INDEX SR NO 1. 2. 3. 4. 5.

CONTENT OBJECTIVES TERMINOLOGY INTRODUCTION TYPES KAWASAKI DISEASE PAGE NO 2 3 5 6 7

6. 7. 8. 9. 10. 11. 12. 13.

MYOCARDITIS CARDIOMYPPATHY RHEUMATIC FEVER RHEUMATIC HEART DISEASE NURSING CARE PLAN CONCLUSION BIBLIOGRAPHY ABSTRACTS

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OBJECTIVES
Central objective All the student will have depth understanding about acquired heart diseases, rheumatic fever and rheumatic heart diseases Specialized objective 1) 2) 3) 4) 5) 6) 7) 8) 9) 10) To introduce with the acquired heart diseases To enlist the terminology related to acquired heart diseases To classify acquired heart diseases To learn about Kawasaki disease To get knowledge about myocarditis To explain cardiomyopathy To describe rheumatic fever To discuss rheumatic heart diseases To describe nursing management of acquired heart disease To formulate care plan with congenital heart disease

TERMINOLOGY
Acquired: Anything that is not present at birth but develops some time later. In medicine, the word "acquired" implies "new" or "added." Autoimmune: Pertaining to autoimmunity, a misdirected immune response that occurs when the immune system goes awry and attacks the body itself. Blood pressure: The blood pressure is the pressure of the blood within the arteries. It is produced primarily by the contraction of the heart muscle . The first ( systolic pressure) is measured after the heart contracts and is highest. The second ( diastolic pressure) is measured before the heart contracts and lowest. Cancer: An abnormal growth of cells which tend to proliferate in an uncontrolled way and, in some cases, to metastasize (spread). Carbon dioxide: A gas which is the byproduct of cellular metabolism and which collects in the tissues, is cleared from the tissues by the blood within the veins, is carried by the hemoglobin in the red blood cells, and removed from the body via the lungs in the exhaled air. Abbreviated CO2. Cardiac output: The amount of blood that is pumped by the heart per unit time, measured in liters per minute (l/min). Cardiomyopathy: Disease of the heart muscle (the myocardium ). From the Greek roots: "cardio-", heart + "mys", muscle + "pathos", disease = disease (of the) heart muscle. Cardiovascular: The circulatory system comprising the heart and blood vessels which carries nutrients and oxygen to the tissues of the body and removes carbon dioxide and other wastes from them. Chest pain : There are many causes of chest pain. One is angina which results from inadequate oxygen supply to the heart muscle. Angina can be caused by coronary artery disease or spasm of the coronary arteries. Chest pain can also be due to a heart attack (coronary occlusion) and other important diseases such as, for example, dissection of the aorta and a pulmonary embolism Cholesterol: The most common type of steroid in the body, cholesterol has gotten something of a bad name. However, cholesterol is a critically important molecule. Clinical trials: Trials to evaluate the effectiveness and safety of medications or medical devices by monitoring their effects on large groups of people. Congestive heart failure : Inability of the heart to keep up with the demands on it and, specifically, failure of the heart to pump blood with normal efficiency. When this occurs, the heart is unable to provide adequate blood flow to other organs such as the brain, liver and kidneys. Heart failure may be due to failure of the right or left or both ventricles. Defibrillator: A device used to correct a dangerously abnormal heart rhythm, usually ventricular fibrillation, or to restart the heart by depolarizing its electrical conduction system and delivering brief measured electrical shocks to the chest wall or the heart muscle itself. Dilated cardiomyopathy: A disorder in which the chambers of the heart are dilated (enlarged) because the heart muscle is weakened and cannot pump effectively. There are many causes, the
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most common being myocardial ischemia (not enough oxygen supplied to the heart muscle) due to coronary artery disease. Dysfunction: Difficult function or abnormal function. Electrocardiogram : A recording of the electrical activity of the heart. An electrocardiogram is a simple, non-invasive procedure. Electrodes are placed on the skin of the chest and connected in a specific order to a machine that, when turned on, measures electrical activity all overaround the heart. Output is usually in the form of a long scroll of paper displaying a printed graph of activity. Extrinsic:not an essential or inherent part of a something such as a structure. Fibrillation: In matters of the heart (cardiology), fibrillation is incoordinate twitching of the heart muscle fibers. Heart attack: The death of heart muscle due to the loss of blood supply. The loss of blood supply is usually caused by a complete blockage of a coronary artery, one of the arteries that supplies blood to the heart muscle. Death of the heart muscle, in turn, causes chest pain and electrical instability of the heart muscle tissue. Heart disease: Any disorder that affects the heart.

ACQUIRED HEART DISORDER

Figure 1: SHOWING NORMAL HEART Paediatric cardiologist treats various types of heart disease in children. There are two types of heart disease in the paediatric population. There is heart disease that is genetic that newborns are born with and then there is heart disease that can be acquired through the environment in which a child is raised. The field of pediatric cardiology has noticed more and more children are suffering from acquired heart disease. This kind of heart disease is preventable if precautions are taken and a pediatric cardiologist informs the parent. This type of heart disease occurs in childhood 1-4 per 1000 children. Some children develop a heart problem after an illness. This is called an acquired heart defect. The four main types of acquired heart defect are Kawasaki disease, myocarditis, cardiomyopathy and rheumatic heart disease. Some children acquire a heart problem after an illness in childhood. This is called an acquired heart defect. This is different to a congenital (at birth) heart defect, which occurs when a child is born with a heart problem.

TYPES OF ACQUIRED HEART DISEASE

Kawasaki disease an illness that occurs mainly in young children and may leave the heart muscle or coronary arteries damaged.
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Myocarditis the heart muscle becomes inflamed and may be damaged after a viral infection. Cardiomyopathy a disease of the heart muscle, caused by a genetic disorder or after an infection. It leads to poor heart function. Rheumatic heart disease caused by rheumatic fever, this disease leads to heart muscle and valve damage.

KAWASAKI DISEASE

A child showing characteristic lip changes, and red eyes seen in Kawasaki disease Definition of Kawasaki Disease Kawasaki disease is an acute, distinct, self-limited, idiopathic exanthematous febrile disease of young children which is notable for vasculitis of coronary blood vessels with potential dilation and subsequent aneurysms, thrombosis, rupture, or myocardial ischemia.

Epidemiology 1) Worldwide, affects all races but most prevalent in Japan 2) Predominant age is 1-5 years the peak is 18-24 months. In Japan, it is 6-11 months 3) 80% are <5 years old. 50% are <2 years old.

Classification Kawasaki disease is considered to be a necrotizing vasculitis (also called necrotizing angeititis), which may be identified histologically by the occurrence of necrosis (tissue death), fibrosis, and proliferation of cells associated with inflammation in the inner layer of the vascular wall. Kawasaki disease may be further classified as a medium-sizedvessel vasculitis, affecting medium and small sized blood vessels, such as the smaller cutaneous vasculature (veins and arteries in the skin) that range from 50 to 100m in diameter. KD is also considered to be a primary childhood vasculitis, a disorder associated with vasculitis that mainly affects children under the age of 18.

Causes: Like all autoimmune diseases, the cause of Kawasaki disease is presumably the interaction of genetic and environmental factors, possibly including an infection. The specific cause is unknown, but current theories center primarily on immunological causes for the disease. Evidence increasingly points to an infectious etiology, but debate continues on whether the cause is a conventional antigenic substance or a superantigen.

Path physiology 1) Stage I (1st two weeks) neutrophilic infiltrates involving pericardium, myocardium, endocardium, and vascular endothelium of the coronary arteries; changes in the cardiac conduction system. This is the febrile stage where patients present with the most symptoms 2) Stage II (2-4 weeks after onset) vasculitis persists and necrosis may develop resulting in aneurysm dilatation in coronary arteries. Possible thrombosis in the aneurysm with subsequent obstruction of coronary blood flow 3) Stage III (4-8 weeks after onset) coronary and myocardial inflammation starts to subside. 4) Stage IV (>8 weeks after onset) scar formation and calcification of coronary arteries with stenosis, recanalization of the
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coronary lumen, myocardial fibrosis PHASES 1) Acute (lasting up to two weeks) most of the following signs and symptoms are manifested 2) Subacute (day 10-21) resolution of fever, rash, and LAD. Children will have persistence of irritability, poor appetite, develop coronary artery disease, and the skin on their fingers and toes will start to peel and may continue to next phase. 3) Chronic or convalescent phase (within 2 months of onset) resolution of symptoms and lab abnormalities

CLINICAL MANIFESTATION:

(A) Bilateral, non-exudative conjunctivitis with perilimbal sparing - "conjunctival injection". (B) Strawberry tongue and bright red, swollen lips with vertical cracking and bleeding. (C) Erythematous rash involving perineum.

(D) Erythema of the palms, which is often accompanied by painful, brawny edema of the dorsa of the hands. (E) Erythema of the soles, and swelling dorsa of the feet. (F) Desquamation of the fingers. (G) Erythema and induration at the site of a previous vaccination with Bacillus Calmette-Gurin (BCG). (H) Perianal erythematous desquamation.

Kawasaki disease often begins with a high and persistent fever that is not very responsive to normal treatment with paracetamol or ibuprofen. High-grade fever (greater than 39 C or 102 F; often as high as 40 C or 104 F), The duration of fever is on average one to two weeks; in the absence of treatment, it may extend for three to four weeks. However, when appropriate therapy is started the fever is gone after two days.

Affected children develop red eyes because of non-suppurative conjunctivitis, iritis and bilateral anterior uveitis. Inflammation of the mucous membranes in the mouth, along with erythema (redness), edema (swelling) with fissures (cracks in the lip surface), desquamation (peeling) and exsudation of the lips are also evident.

The oropharynx mucosa has enanthema and the tongue maintains an unusual red appearance termed "strawberry tongue" (marked erythema with prominent gustative papillae) Keratic precipitates (detectable by a slit lamp but usually too small to be seen by the unaided eye), and swollen lymph nodes may also be present and can be the first manifestation of the disease. Rashes occur early in the disease, and the cutaneous rash observed in patients with KD is non-specific, polymorphic, non-itchy and normally observed up to the fifth day of fever. Cutaneous exanthema may comprise macular-papular erythematous and fissure lesions, the most common type, in addition to urticariform type rash, purpuric, multiform-like erythema and peeling of The skin in the genital area, hands, and feet (especially around the nails and on the palms and soles) may occur in later phases. Some of these symptoms may come and go during the course of the illness. It is a syndrome affecting multiple organ systems, and in the acute stage of KD, systemic inflammatory changes are evident in many organs.
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Myocarditis, pericarditis, valvulitis, aseptic meningitis, pneumonitis, lymphadenitis, and hepatitis may be present and are manifested by the presence of inflammatory cells in the affected tissues.

LESS COMMON MANIFESTATIONS System GIT Manifestations Diarrhea, abdominal pain, vomiting, liver dysfunction, pancreatitis, Hydrops gallbladder, cholangitis, intussusception, intestinal pseudo-obstruction, ascites, splenic infarction. Polyarthritis and arthralgia. Myocarditis, pericarditis, valvular heart disease. Urethritis, prostatitis, cystitis, priapism, Interstitial nephritis, orchitis, nephrotic syndrome. Aseptic meningitis, and sensorineural deafness. Influenza-like illness, plural effusion, Atelectasis. Erythema and induration at BCG vaccine site, Beau's lines, and finger gangrene.

MSS CVS GU CNS RS Skin

Diagnosis

CRITERIA FOR DIAGNOSIS OF KAWASAKI DISEASE Fever of 5 days' duration associated with at least 4 of the following 5 changes Bilateral nonsuppurative conjunctivitis One of more changes of the mucous membranes of the upper respiratory tract, including pharyngeal injection, dry fissured lips, injected lips, and "strawberry" tongue One or more changes of the extremities, including peripheral erythema, peripheral edema, periungual desquamation, and generalized desquamation Polymorphous rash, primarily truncal Cervical lymphadenopathy >1.5 cm in diameter Disease cannot be explained by some other known disease process A diagnosis of Kawasaki disease can be made if fever and only 3 changes are present in conjunction with coronary artery disease documented by two-dimensional echocardiography or coronary angiography. Source: Nelson's essentials of pediatrics, Review
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Kawasaki disease can only be diagnosed clinically (i.e. by medical signs and symptoms). There exists no specific laboratory test for this condition. Many other serious illnesses can cause similar symptoms, and must be considered in the differential diagnosis, including scarlet fever, toxic shock syndrome, juvenile idiopathic arthritis, and childhood mercury poisoning

Investigations A physical examination will demonstrate many of the features listed above. Blood tests Complete blood count (CBC) may reveal normocytic anemia and eventually thrombocytosis Erythrocyte sedimentation rate (ESR) will be elevated C-reactive protein (CRP) will be elevated Liver function tests may show evidence of hepatic inflammation and low serum albumin

Other optional tests

Electrocardiogram may show evidence of ventricular dysfunction or, occasionally, arrhythmia due to myocarditis Echocardiogram may show subtle coronary artery changes or, later, true aneurysms. Ultrasound or computerized tomography may show hydrops (enlargement) of the gallbladder Urinalysis may show white blood cells and protein in the urine (pyuria and proteinuria) without evidence of bacterial growth Lumbar puncture may show evidence of aseptic meningitis Angiography was historically used to detect coronary artery aneurysms and remains the gold standard for their detection, but is rarely used today unless coronary artery aneurysms have already been detected by echocardiography.

Treatment The treatment for Kawasaki disease is intravenous gammaglobulin (immunoglobulin), made from donated blood transfusions. Large doses of intravenous gammaglobulin will usually stop the fever and other symptoms of Kawasaki disease. Treatment should be administered within 10 days of the onset of fever to minimize heart problems.
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Children may also be prescribed aspirin for some weeks following the onset of Kawasaki disease, to prevent problems with coronary arteries. Medical Treatment

1) Aspirin 80-100mg/kg/day during febrile phase then 5mg/kg/day since aspirin therapy is associated with Reyes syndrome, flu and Varicella vaccine should be given! 2) IV immunoglobulin 2g/kg at the time of diagnosis lowers risk of coronary artery aneurysms and shorten duration of acute phase 3) Steroids are shown to increase incidence of aneurysm and should be avoided

Prognosis

With early treatment, rapid recovery from the acute symptoms can be expected and the risk of coronary artery aneurysms greatly reduced. Untreated, the acute symptoms of Kawasaki disease are self-limited (i.e. the patient will recover eventually), but the risk of coronary artery involvement is much greater. Overall, about 2% of patients die from complications of coronary vasculitis. Patients who have had Kawasaki disease should have an echocardiogram initially every few weeks, and then every one or two years to screen for progression of cardiac involvement. Overall, life-threatening complications resulting from therapy for Kawasaki disease are exceedingly rare, especially compared with the risk of non-treatment. There is also evidence that Kawasaki disease produces altered lipid metabolism that persists beyond clinical resolution of the disease. Complications The primary complications of Kawasaki disease involve the development and rupture of coronary artery aneurysms. Giant aneurysms may occur and may be resistant to Kawasaki disease therapy. Some recommend coronary artery bypass grafting using arterial grafts that can grow with the child. Transplant has been performed in some children who had large aneurysms in vessels not amenable to bypass. Dehydration may result from fever and anorexia. Hyponatremia secondary to SIADH has been reported in a very young infant. Joint inflammation in the acute phase may limit mobility. Pancreatitis, hydrops (vasculitis) of the gallbladder, hepatitis, meningitis, and orchitis may complicate care. Endovascular ultrasonography has shown that some resolved aneurysms are associated with marked intimal thickening.
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MYOCARDITIS

Histopathological image of myocarditis at autopsy in a patient with acute onset of congestive heart failure.

In medicine (cardiology), myocarditis is inflammation of heart muscle (myocardium). It resembles a heart attack but coronary arteries are not blocked. The definition of myocarditis varies, but the central feature is an infection of the heart, with an inflammatory infiltrate, and damage to the heart muscle, without the blockage of coronary arteries that define a heart attack (myocardial infarction) or other common non-infectious causes

Causes Infections Bacterial infections:

Viral infections:

Fungal infections:

Coxsackie Cytomegalovirus Hepatitis C Herpes HIV Parvovirus

Chlamydia Mycoplasma Streptococcus Treponema

Aspergillus Candida Coccidioides Cryptococcus Histoplasma Schistosomiasis

Toxins

Immunologic Drugs (ethanol, Allergic (acetazolamide, amitriptyline)

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anthracyclines and some other forms of chemotherapy, and antipsychotics, e.g. clozapine, also some designer drugs such as mephedrone Physical agents

Rejection after a heart transplant Autoantigens (scleroderma, systemic lupus erythematosis, sarcoidosis, systemic vasculitis such as Churg-Strauss syndrome, Wegener's granulomatosis) Toxins (arsenic, toxic shock syndrome toxin, carbon monoxide, snake venom) Heavy metals (copper, iron)

electric shock, hyperpyrexia, and radiation

PATHOPHYSIOLOGY Damage to myocardium occurs through the following mechanisms:

Direct cytotoxic effect of the causative agent Secondary immune response, which can be triggered by the causative agent Cytokine expression in the myocardium (eg, tumor necrosis factor-alpha, nitric oxide synthase) Aberrant induction of apoptosis

Myocardial damage has 2 main phases, as follows:

Acute phase (first 2 wk): Myocyte destruction is a direct consequence of the offending agent, which causes cell-mediated cytotoxicity and cytokine release, contributing to myocardial damage and dysfunction. Detection of the causal agent is uncommon during this stage. Chronic phase (>2 wk): Continuing myocyte destruction is autoimmune in nature, with associated abnormal expression of human leukocyte antigen (HLA) in myocytes (and in the case of viral myocarditis, persistence of viral genome in myocardium).

CLINICAL MANIFESTATION: The signs and symptoms associated with myocarditis are varied, and relate either to the actual inflammation of the myocardium, or the weakness of the heart muscle that is secondary to the inflammation.
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Signs and symptoms of myocarditis include: Chest pain (often described as "stabbing" in character)

Congestive heart failure (leading to edema, breathlessness and hepatic congestion)

Palpitations (due to arrhythmias) Sudden death (in young adults, myocarditis causes up to 20% of all cases of sudden death) Fever (especially when infectious, e.g. in rheumatic fever) Symptoms in infants and toddlers tend to be more non-specific with generalized malaise, poor appetite, abdominal pain, chronic cough.

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Later stages of the illness will present with respiratory symptoms with increased work of breathing and is often mistaken for asthma.

Since myocarditis is often due to a viral illness, many patients give a history of symptoms consistent with a recent viral infection, including fever, rash, diarrhea, joint pains, and easy fatigueability. Myocarditis is often associated with pericarditis, and many patients present with signs and symptoms that suggest concurrent myocarditis and pericarditis. Diagnosis Endomyocardial biopsy specimen. Extensive eosinophilic infiltrate involving the endocardium and myocardium (hematoxylin and eosin stain). Myocardial inflammation can be suspected on the basis of electrocardiographic results (ECG), elevated C-reactive protein (CRP) and/or Erythrocyte sedimentation rate (ESR) and increased IgM (serology) against viruses known to affect the myocardium. Markers of myocardial damage (troponin or creatine kinase cardiac isoenzymes) are elevated. The electrocardiogram (ECG) findings are diffuse T wave inversions; saddle-shaped ST-segment elevations may be present (The gold standard is still biopsy of the myocardium, generally done in the setting of angiography). Histopathological features are: myocardial interstitium with abundant edema and inflammatory infiltrate, rich in lymphocytes and macrophages. Focal destruction of myocytes explains the myocardial pump failure. Cardiac magnetic resonance imaging (cMRI or CMR) has been shown to be very useful in diagnosing myocarditis by visualizing markers for inflammation of the myocardium.

Treatment There is no cure for myocarditis. Treatment focuses on the underlying cause, as well as supporting the heart to function and maintain adequate circulation. Treatment can include: Medication to control blood pressure and body fluids (diuretics) Intravenous immunoglobulin or purified antibodies, to reduce inflammation Bed rest and avoiding strenuous physical activity.

Immunisations against the viral diseases that can trigger myocarditis, including rubella and influenza etc. supportive therapy for symptoms of acute heart failure with use of diuretics, nitroglycerin/nitroprusside, and angiotensin-converting enzyme (ACE) inhibitors
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Inotropic drugs (eg, dobutamine, milrinone) may be necessary for severe decompensation, although they are highly arrhythmogenic. Long-term treatment follows the same medical regimen, including ACE inhibitors, beta blockers, and aldosterone receptor antagonists. However, in some instances, some of these drugs cannot be implemented initially because of hemodynamic instability.

Withdrawal of the offending agent is called for, if applicable (eg, cardiotoxic drugs, alcohol). Treat underlying infectious or systemic inflammatory etiology. Nonsteroidal anti-inflammatory agents should be avoided in the acute phase, as their use may impede myocardial healing and actually exacerbate the inflammatory process and increase the risk of mortality. Anticoagulation may be advisable as a preventive measure, as in other causes of heart failure, although no definitive evidence is available. Antiarrhythmics can be used cautiously, although most antiarrhythmic drugs have negative inotropic effects that may aggravate heart failure.

Supraventricular arrhythmias should be converted electrically. High-grade ventricular ectopy and ventricular tachyarrhythmia should be treated cautiously with beta blockers and antiarrhythmics. Patients are usually very sensitive to digoxin and should use it with caution and in low doses. (Digoxin increases expression of proinflammatory cytokines and mortality rate in animal models.) Complete heart block is an indication for temporary transvenous pacing. Implantable defibrillators rarely are indicated in lymphocytic myocarditis unless extensive scarring has occurred. In the case of frequent nonsustained or polymorphic ventricular ectopy or tachyarrhythmia, temporary or wearable defibrillator support (eg, LifeVest) may be considered.

Medication Summary

In general, treatment of both acute and chronic myocarditis is aimed at reducing congestion and improving cardiac hemodynamics in heart failure, as well as providing supportive therapy with the hopes of prolonging survival

Angiotensin converting enzyme inhibitors Capotril: oral suspension:1mg/mL tablet:12.5mg,25mg,50mg,100mg Hypertension (Off-label) Calcium channel blockers: Amlodipine : Tablet:2.5mg, 5mg, 10mg Hypertension

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Neonates: 0.05-0.1 mg/kg/dose q8-24hr, titrate dose up to 0.5 mg/kg/dose q6-24hr

Initial: 0.1 mg/kg PO qDay/BID May gradually increase to 0.6 mg/kg/day or 5 mg/day

Infants: 0.15-0.3 mg/kg/dose; titrate dose upward to maximum 6 mg/kg/day in 1-4 divided doses; 2.5<6 Years Old 6 mg/kg/day usually required Children: 0.3-0.5 mg/kg/dose; titrate to maximum 6 mg/kg/day divided BID/QID Older children: 6.25-12.5 mg/dose q12-24hr; titrate to no more than 6 mg/kg/day divided BID/QID Adolescents: 12.5-25 mg/dose q8-12hr; may increase by 25 mg/dose q1-2Weeks to maximum 450 mg/day Loop diuretics:Frusemide injectable solution:10mg/mL oral solution:10mg/mL,40mg/5mL

Safety and efficacy not established Potential toxic dose: 4 mg/kg

6-17 years old: 2.5-5 mg PO qDay

Cardiac glycosides Digoxin (Rx) - Lanoxin Capsule:0.05mg,0.1mg,0.2mg

tablets: 20mg,40mg,80mg

Edema Initial: 1-2 mg/kg IV/IM/PO x1 Neonates (<28 do)

Elixir: 0.05mg/mL injectable solution: 0.1mg/mL,0.25mg/mL tablet:0.125mg,0.25mg

0.5-1 mg/kg IV/IM q8-24hr; no more than 2 mg/kg/dc OR 0.5-1 mg/kg PO q8-24hr; no more than 6 mg/kg/d, Continuous Infusion: 0.05 mg/kg/hr IV, titrate to effect Extended release: 20 mg PO qDay; maintain dose for 1-2 weeks if tolerated; may increase to 40 mg PO qDay if necessary; 80 mg PO qDay Left Ventricular Dysfunction Following Myocardial
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Beta-adrenergic blockers; carvedilol (Rx) capsule, extended release

10mg,20mg,40mg,80mg

Infarction Immediate release: 6.25 mg PO BID, initially, THEN increase as tolerated after 3-10 days to 12.5 mg PO BID, THEN increase to 25 mg PO BID (target dose) 10-20 mg PO qDay; increase q3-10Days based on tolerance to target dose of 80 mg PO qDay Additional Information Less effective than thiazide diuretics in black and geriatric patients Shown to decrease mortality in hypertension and post-myocardial infarction Take with food May break capsule & sprinkle on spoonful of applesauce, eat immediately

tablet

3.125mg,6.25mg,12.5mg,25mg

Congestive Heart Failure (CHF) Immediate release: 3.125 mg PO BID for 2 weeks, THEN increase q2Weeks as tolerated to 6.25 mg, 12.5 mg & 25 mg PO BID Extended release: 20 mg PO qDay; maintain dose for 1-2 weeks if tolerated; may increase to 40 mg PO qDay if necessary; 80 mg PO qDay Hypertension (HTN) Immediate release: 6.25 mg PO BID initially THEN increase after 7-14 days as tolerated first to 12.5 mg PO BID, THEN to 25 mg PO BID

Further Inpatient Care

Further outpatient care consists of largely supportive care, with slow rehabilitation and implementation of evidence-based medical therapy. Repeat assessment with echocardiography may be helpful to determine the persistency of cardiac dysfunction.

Further Outpatient Care

Ongoing chronic inflammation may cause dilated cardiomyopathy and subsequent heart failure. Patients with a history of myocarditis should be monitored at intervals of 1-3 months initially, with gradual return of physical activity.

Deterrence/Prevention Vaccination should reduce the incidence of myocarditis caused by measles, rubella, mumps, poliomyelitis, and influenza. Development of vaccines for other cardiotropic viruses may prevent viral myocarditis in the future.
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Complications

Cardiogenic shock may occur in fulminant cases of myocarditis. Severe heart block requiring permanent pacemaker placement occurred in 1% of patients in the Myocarditis Treatment trial.

Prognosis: Patients who have survived fulminant myocarditis have a good prognosis Patient Education Patients should be advised of the current understanding of the natural history of myocarditis and the strengths and limitations of different diagnostic testing and therapeutic options. Overall, neurohormonal agents are given in a similar manner as in patients presenting with new-onset heart failure. Serial assessment is needed to determine the potential resolution of acute myocarditis, and during the early recovery period, strenuous exercise and digoxin should be avoided. Data regarding the risks of relapse with drug withdrawal following recovery is not available, so it is generally not recommended in practice.

CARDIOMYOPATHY
A primary disorder of the heart muscle that causes abnormal myocardial performance and is not the result of disease or dysfunction of other cardiac structures myocardial infarction, systemic hypertension, valvular stenosis or regurgitation

Opened left ventricle of heart shows a thickened, dilated left ventricle with subendocardial fibrosis manifested as increased whiteness of endocardium.

Cardiomyopathy (cardio=heart +myo=muscle + athy=disease/abnormality) is diseased heart muscle that cannot function (contract) adequately.

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Cardiomyopathy results in the failure of the heart muscle to meet the needs of the body for oxygen rich blood and removal of carbon dioxide and other waste products

Cardiomyopathy Cardiomyopathy is a weakening of the heart muscle or a change in heart muscle structure. It is often associated with inadequate heart pumping or other heart function problems. The official definition of cardiomyopathy of the American Heart Association in 2006 is as follows:

"Cardiomyopathies are a heterogeneous group of diseases of the myocardium associated with mechanical and/or electrical dysfunction that usually (but not invariably) exhibit inappropriate ventricular hypertrophy or dilatation and are due to a variety of causes that frequently are genetic. Cardiomyopathies either are confined to the heart or are part of generalized systemic disorders, which may lead to cardiovascular death or progressive heart failure-related disability." The definition divides heart disease into 1) primary cardiomyopathies, those that usually affect the heart alone (primary) 2) secondary cardiomyopathies, those that are a result of an underlying condition affecting many areas of the body. The primary cardiomyopathies are further divided into inherited (genetic) diseases, those that are acquired, and those that are a combination of both. Common types of cardiomyopathy include: Dilated cardiomyopathy is a condition in which the heart becomes weakened and enlarged. It cannot pump blood efficiently. Many different medical problems can cause this type of cardiomyopathy. 2. Hypertrophic cardiomyopathy (HCM) is a condition in which the heart muscle becomes thick. This thickening makes it harder for blood to leave the heart. This type of cardiomyopathy is usually passed down through families. 3. Restrictive cardiomyopathy refers to a group of disorders in which the heart chambers are unable to properly fill with blood because of increased stiffness in the heart.
1.

In most cases of cardiomyopathy in children, the cause is unknown. However, possible factors include: Heart valve problems
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 Viral infections that trigger myocarditis A family history of cardiomyopathy Genetic disorders including Noonan syndrome. ETIOLOGIES 1) Viral myocarditis most common cause 2) Cardiovascular conditions - i.e. Kawasaki disease, congenital heart defects, hypertension, cardiac transplantation or surgery, persistent arrhythmias problems, abnormal coronaries 3) Infectious or inflammatory diseases 4) Immunologic diseases (i.e. HIV) 5) Obesity or dietary deficiencies 6) Toxin reactions - i.e. drug, alcohol, radiation exposure 7) Connective tissue and autoimmune diseases 8) Endocrine diseases 9) Pregnancy related complications 10) Genetic Mutations

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PATHOPHYSIOLOGY As cardiac output decreases the body develops compensatory mechanisms so supply can equal demand.

This may include both decreasing demand, e.g. lessening exercise,and increasing supply, e.g. releasing intrinsic catecholamines to increase stroke volume and heart rate.

When these mechanisms allow supply to equal demand, the patient is in a compensated state. Ideally, a patient is maintained in this state on an outpatient regiment as long as tolerated.

If the cardiac function worsens, the patient will eventually develop decompensate shock (demand > supply) and will require admission to the ICU. In cardiogenic shock, the cardiac output is low and systemic vascular resistance (SVR) is high. Myocardialischemia may also develop secondary to decreased coronary perfusion pressure, increased myocardial demand, or a hypertrophic myocardium. Symptoms Symptoms of cardiomyopathy vary. Some people, including children, have no symptoms in the earlier stages. Symptoms, where they occur, may include:

Swelling of the hands and feet Swelling of the abdomen with fluid Breathlessness Fatigue Irregular heart rhythm Dizziness, light-headedness and fainting during physical activity.

DIAGNOSIS

The diagnosis of cardiomyopathy begins with history.

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Usually, the patient's symptoms consist of complaining of fatigue, chest discomfort , weakness, and shortness of breath.

Other information may be gathered from past medical history including history of high blood pressure, high cholesterol, and diabetes. Noting the presence of other underlying illnesses such as sarcoidosis, amyloidosis, thyroid disorders, and rheumatoid arthritis Social history including smoking, alcohol, and drug abuse can also be helpful in making the diagnosis. Family history is often important, especially if there is concern about sudden cardiac death at an early age. Physical examination often will include monitoring vital signs including heart rate, blood pressure, respiratory rate, and oxygen saturation. The significant information from lung examination listening for fluid and from listening to the heart sounds. Murmurs may provide information about leaking heart valves. Examination of the jugular veins in the neck and swelling or fluid in the feet and ankles can be clues to the diagnosis of heart failure. Blood tests may be helpful in screening for anemia, abnormalities in electrolytes, and kidney and liver function. An electrocardiogram (EKG) is a screening test to look for electrical abnormalities in the heart. Evidence of previous heart attack or ventricular hypertrophy (heart muscle thickening) can be noted on the EKG. An ultrasound of the heart (echocardiogram) can help evaluate wall motion function of the heart, the integrity of the heart valves, and the ventricle ejection fraction. It may also provide visualization of the sac (pericardium) that surrounds the heart. Chest X-ray may reveal an enlarged or abnormal heart shape or excess fluid accumulation within the lungs.

Treatment

Medications angiotensin-converting enzyme (ACE) inhibitors, diuretics and beta blockers to improve the hearts pumping capability Devices a pacemaker to coordinate the contractions between the left and right ventricle, or an implantable cardioverter defibrillator (ICD) to monitor the heart rhythm and deliver electrical shocks to control abnormal, rapid heartbeats Surgery and transplant a heart transplant, which may be an option for patients with advanced symptoms or who have not been successfully treated by other measures.
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Patients presenting in decompensate shock need immediate admission to the ICU and treatment to provide end organ perfusion. Patients presenting with carcinogenic shock can present with the following: SIGNS AND SYMPTOMS Depressed mental status *Changes in mixed venous saturation and Tachycardia lactate can predict patient Hypotension (Hypertension due to decompensation prior to an arrest increased systemic vascular resistance, situation, and therefore should be but monitored closely. poor perfusion) Upon admission to the ICU, treatment Arrhythmias measurements are implemented to Abdominal discomfort / emesis increase cardiac output and decrease Poor urine output total body demand. Prolonged capillary refill time

Increasing Cardiac Output

Initiation of inotropic support: milrinone, dopamine,dobutamine, and if required epinephrine. Dopamine improves systolic function, but increases SVR. Dobutamine improves systolic function and decreases SVR. Milrinone improves diastolic dysfunction and also decrease SVR. Should the patient continue hypotensive, epinephrine is started. It is a very effective inotrope, but increases SVR and myocardial oxygen demand. Anti-arrhythmia treatment should be initiated if required. Mechanical Support: If all other medical treatments are optimized and cannot provide adequate cardiac output, patients should be considered either for extracorporeal membranous oxygenation (ECMO) or a ventricular assist device (VAD) placement. ECMO provides both pulmonary and cardiac support, can be initiated in acute situations, and can act as a bridge to heart transplant. Given the tenuous positions of the cannulas, patients often need to remain deeply sedated and perhaps even paralyzed. They also require systemic anticoagulation and are therefore at risk of developing intra-cranial hemorrhages. VADs can be used for either right or left ventricular support, but do not provide pulmonary support. It should therefore not be considered in patients whose lungs cannot provide adequate oxygenation or ventilation. Cardiomyopathy patients rarely have pulmonary disease that cannot be reversed upon improvement of cardiac output. VADs are placed in the operating room since a thorocotomy is required for cannula insertion, and also act as a bridge to heart transplant.
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Since the cannulas are in firmer position, patients are extubated and can often have many activities of daily living. Patients also require systemic anticoagulation for a VAD and are therefore also at risk of developing intra-cranial hemorrhages. Patients have remained on VADs for several years awaiting a transplant as compared to only 1 - 2 months on ECMO. Decreasing Demand

Intubation should be considered in any patient that remains in cardiogenic shock despite initiation of inotropic support. The work of breathing consumes a tremendous amount of total cardiac output and in infants can account for up to 40%. Intubation medications should be carefully selected since patients are often at risk of arresting upon initiation of positive pressure ventilation. The drugs of choice usually are Etomidate or Ketamine for sedation and a fast acting paralytic. Diuretics should be started or continued. Intravascular volume status, e.g. CVP(central venous pressure), should be monitored closely as urine output improves. Patients with dilated cardiomyopathy in particular should not be preload diminished. Enteral feeds should only be started slowly as cardiac output improves with inotropes. The mixed venous saturations and lactate should be monitored closely as feeds are begun.Fevers should be aggressively controlled due to the tremendous increase in demand. Active infections / sepsis should be treated with appropriate antibiotics / antifungals. Sedation and paralysis is often considered in patients with severe cardiogenic shock to decrease the overall demand created by movement.

RHEUMATIC FEVER

Background Rheumatic fever causes chronic progressive damage to the heart and its valves. Until 1960, it was a leading cause of death in children and a common cause of structural heart disease. The disease has been known for many centuries.
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Epidemiology Mortality/Morbidity

Morbidity from acute rheumatic fever (ARF) is directly proportional to the rate of streptococcal infections. Infections that are not treated adequately are most likely to cause the major sequelae noted in the list of Jones criteria in Physical. Morbidity also is related to the care that the patient receives. Cardiac involvement is the major cause of long-term morbidity. ARF causes inflammation of valvular endocardium. One or more valves (most commonly the mitral valve) may be permanently deformed. Those valves are then dysfunctional and may lead to problems including left ventricular dilation and congestive heart failure, sometimes decades later. Vegetations may develop on damaged valves and become infected leading to endocarditis. Myocarditis is present but is not the direct cause of heart failure. It estimated that worldwide, approximately 60% of all patients with ARF will develop rheumatic heart disease. Further, they estimate a world burden of 2.4 million children aged 5-14 years affected or a total population of 15-20 million living with rheumatic heart disease. Patients without carditis during the initial episode have a relatively low risk of developing carditis during recurrences, although scattered case reports of carditis occurring only during a recurrence exist. Migratory polyarthritis occurs early in the disease course and is a common complaint for patients with rheumatic fever. Joint involvement ranges from arthralgia without objective findings to overt arthritis with warmth, swelling, redness, and exquisite tenderness. The larger joints such as the knees, ankles, elbows, and wrists are involved most frequently. An inverse relationship between severity of joint involvement and risk of carditis appears to exist. In approximately 75% of cases, the acute attack lasts only 6 weeks. Ninety percent of cases resolve in 12 weeks or less. Fewer than 5% of patients have symptoms that persist for 6 months or more. Literature began to appear in 1998 suggesting that acute rheumatic fever might be another disorder associated with PANDAS pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections. As of 2009, this is considered an unproven hypothesis.

Age:Although individuals of any age group may be affected, most cases are reported in persons aged 515 years. Causes

Acute rheumatic fever (ARF) has been linked definitively with a preceding streptococcal infection, usually of the upper respiratory tract. Evidence is very strong that the M protein in certain streptococci subtypes is responsible for antigenicity.
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The perfect causes for Rheumatic Fever in children are still to be understood I e.the bodys own immune system and therefore it is considered as autoimmune disease. In this autoimmune disease, the body develops antibodies against its own cells and therefore the cells consider cells as foreign bodies and reacts very badly. It can affect any part of the body but generally the vital organ that is affected the most is heart.

Pathogenesis

Rheumatic fever develops in children and adolescents following pharyngitis with GABHS (ie, Streptococcus pyogenes). The organisms attach to the epithelial cells of the upper respiratory tract and produce a battery of enzymes, which allows them to damage and invade human tissues. After an incubation period of 2-4 days, the invading organisms elicit an acute inflammatory response, with 3-5 days of sore throat, fever, malaise, headache, and elevated leukocyte count. In a small percent of patients, infection leads to rheumatic fever several weeks after the sore throat has resolved. Only infections of the pharynx initiate or reactivate rheumatic fever. Direct contact with oral (PO) or respiratory secretions transmits the organism, and crowding enhances transmission. Patients remain infected for weeks after symptomatic resolution of pharyngitis and may serve as a reservoir for infecting others. GABHS organisms are gram-positive cocci, which frequently colonize the skin and oropharynx. These organisms may cause suppurative diseases (eg, pharyngitis, impetigo, cellulitis, myositis, pneumonia, puerperal sepsis). GABHS organisms also may be associated with nonsuppurative diseases (eg, rheumatic fever, acute poststreptococcal glomerulonephritis). Group A streptococci (GAS) elaborate the cytolytic toxins, streptolysins S and O. Of these 2 toxins, streptolysin O induces persistently high antibody titers that provide a useful marker of GAS infection and its nonsuppurative complications. Acute RHD often produces a pancarditis, characterized by endocarditis, myocarditis, and pericarditis. Endocarditis is manifested as mitral and aortic valve insufficiency. Severe scarring of the valves develops during a period of months to years after an episode of acute rheumatic fever, and recurrent episodes may cause progressive damage to the valves. The mitral valve is affected most commonly and severely (65-70% of patients); the aortic valve is affected second most commonly (25%). The tricuspid valve is deformed in only 10% of patients, almost always in association with mitral and aortic lesions, and the pulmonary valve is rarely affected. Severe valve insufficiency during the acute phase may result in congestive heart failure (CHF) and even death (1% of patients).
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 Whether myocardial dysfunction during acute rheumatic fever is primarily related to myocarditis or is secondary to CHF from severe valve insufficiency is not known. When pericarditis is present, it rarely affects cardiac function or results in constrictive pericarditis. Chronic manifestations occur in adults with previous RHD from residual and progressive valve deformity. RHD is responsible for 99% of mitral valve stenosis in adults, and it may be associated with atrial fibrillation from chronic mitral valve disease and atrial enlargement. Risk factors Overcrowding, poor hygiene and lack of access to medical services are all important. In hot countries, skin infection is a more important source of streptococci than pharyngitis. Young age is a risk factor with most cases occurring between age 5 and 15 years. History

Acute rheumatic fever (ARF) is associated with 2 distinct patterns of presentation. o The first pattern of presentation is sudden onset. It typically begins as polyarthritis 2-6 weeks after streptococcal pharyngitis and is usually characterized by fever and toxicity. o If the initial abnormality is mild carditis, ARF may be insidious or subclinical. Age at onset influences the order of complications. Younger children tend to develop carditis first, whereas older patients tend to develop arthritis first.

Physical Diagnosis of acute rheumatic fever (ARF) requires a high index of suspicion. Guidelines of diagnosis used by the American Heart Association include major and minor criteria (ie, modified Jones criteria). In addition to evidence of a previous streptococcal infection, the diagnosis requires 2 major Jones criteria or 1 major plus 2 minor Jones criteria. Major criteria Carditis: This occurs in as many as 40% of patients and may include cardiomegaly, new murmur, congestive heart failure, and pericarditis, with or without a rub and valvular disease. Migratory polyarthritis: This condition occurs in 75% of patients and is polyarticular, fleeting, and involves the large joints. Note that one group of authors has suggested that atypical cases may only involve small joints.[10] Subcutaneous nodules (ie, Aschoff bodies): These nodules occur in 10% of patients and are edematous, fragmented collagen fibers. They are firm, painless nodules on the extensor surfaces of the wrists, elbows, and knees.

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Erythema marginatum: This condition occurs in about 5% of patients. The rash is serpiginous and long lasting. Chorea (also known as Sydenham chorea and "St Vitus dance"): This characteristic movement disorder occurs in 5-10% of cases. Sydenham chorea consists of rapid, purposeless movements of the face and upper extremities. Onset may be delayed for several months and may cease when the patient is asleep. Note that a murmur in a patient with Sydenham's predicts carditis with a high degree of probability.[20]

Minor criteria Clinical findings include arthralgia, fever, and previous history of ARF. Laboratory findings include elevated acute-phase reactants (eg, erythrocyte sedimentation rate, C reactive protein), a prolonged PR interval, and supporting evidence of antecedent group A streptococcal infections (ie, positive throat culture or rapid streptococcal screen and an elevated or rising streptococcal antibody titer). Laboratory Studies

No specific confirmatory laboratory tests exist for acute rheumatic fever. However, several laboratory findings indicate continuing rheumatic inflammation. Some are part of the Jones minor criteria. Streptococcal antibody tests disclose preceding streptococcal infection. The CDC has stated that a rapid antigen test in the appropriate clinical setting is sufficient to make the diagnosis of active GABHS infection and begin treatment. Isolate group A streptococci via throat culture. A significant percentage will result in a culture positive for group A beta-hemolytic Streptococcus (GABHS). However, a culture positive for GABHS does not definitively prove active infection. Some patients are carriers. Acute-phase reactants (eg, erythrocyte sedimentation rate [ESR], C-reactive protein [CRP] in serum and leukocytosis) may show an increase in serum complement, mucoproteins, alpha-2, and gamma globulins. Anemia is usually caused by suppression of erythropoiesis. PR-interval prolongation is present in approximately 25% of all cases and is neither specific for nor diagnostic of acute rheumatic fever. Synovial fluid analysis may demonstrate an elevated white blood cell count with no crystals or organisms.

Imaging Studies

Echocardiography may be helpful in establishing carditis.

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Chest radiography should be performed to determine presence of cardiomegaly and congestive heart failure.

Prehospital Care: Although no specific prehospital interventions exist for those with acute rheumatic fever, the patient's presentation may warrant establishment of intravenous access and placement of a cardiac monitor

The first step in treating rheumatic fever is to eradicate the bacteria which initially caused the immunologic response. This is usually accomplished with the use of penicillin. Initially the patient is given therapeutic dose of penicillin 400000 units of procaine penicillin intramuscularly twice daily for 10days. This is followed by prophylactic penicillin using benzathine penicillin 1.2 mega units every 21 days or 0.6 mega units every 15 days. For penicillin-allergic patients, there are other options such as erythromycin (E-Mycin, Eryc, Ery-Tab, PCE, Pediazole, Ilosone) or azithromycin (Zithromax, Zmax). Bed rest and diet. Bed rest at the hospital, it is mandatory for all patients, at least for three weeks, during which carditis usually occurs. Duration must be greater for those with medium and severe carditis (2-6 months, depending on the evolution) Limiting physical activity depends on the carditis severity. Resumption of normal activity is done after 3 months (mild carditis), 6 months (avreage severity carditis) and much later or never in case of severe carditis. SUPRESSIVE THERAPY: Aspirin or steroids are given as suppressive therapysince untreated rheumatic fever subsides in 12 weeks in 80 percent of the patientseither of two suppressive agents are given. Steroids are more potent suppressive agent Pericardial rub tends to disappear after starting the steroids and new friction rub may not appear. In selecting the suppressive agents following guidelines are adopted If the patient has carditis with congestive cardiac failure use steroids. It is mandatory.Carditis without congestive cardiac failure one may use either steroids or aspirin If the patient does not have carditis it is preferable to use aspirin

The dose is 60 mg/kg /day eighing more then 20 kg and 40 mg weighing less then 20 kg.this is continued for three weeks and then reduced to 50 mg/ kg for 1 week and then 40 mg/k for another week.Following the reduction in dose is 5 mg/kg till it is fnished The dose of aspirin is 90 to 120 mg / kg /day in 4 divided doses.It is necessary to reemphasise that surgical replacement of the mitral or aortic valve or both is indicatedif the patient is deteriorating inspite of aggressive anticongestive measures
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MANAGEMENT OF CHOREA:

The most difficult and unpredictable symptom to treat is the chorea (involuntary movements). It often responds to antipsychotic medications such as haloperidol (Haldol) but may continue for a protracted period. For patients who develop Sydenham's chorea, it can be the most difficult of the symptoms, since it involves involuntary movements and can interfere with daily activities. These individuals must remain on chronic long-term antibiotics to prevent recurrence of the strep infection, which has been known to cause recurrence of the chorea. The patient and parent should re assured and told about the self limiting course of the disease. The patient should be provided complete physical and mental rest.Phenobarbitone 0 mg thice daily may be prescribed. Other drugs like chlorprozine,valium,diphenhydramine or or phenargan can also be used to provide sedation. Carditis is treated by high-dose steroids but other cardiac medications may be needed to control the inflammation of the heart. This is a serious condition and is most often initially managed in an acutecare setting such as a hospital.

Further Inpatient Care

Most patients with acute rheumatic fever (ARF) will be managed as inpatients by a multidisciplinary team of pediatrics, internal medicine, cardiology, infectious disease, and rheumatology specialists.

Further Outpatient Care Several regimens exist to prevent recurrences"secondary prevention." o Duration of prophylaxis is determined by the number of previous attacks, time since last attack, the risk of exposure to streptococcal infections, patient age, andvery importantlypresence or absence of cardiac involvement. Penicillin is still the drug of choice and may be given daily by mouth or monthly by intramuscular injection. Macrolides are acceptable in penicillin-allergic patients. o Those who have had carditis should be treated well into adulthood and may require lifelong prophylaxis.Those without carditis may be treated until they reach their 20s and after at least 5 years have elapsed since the past episode. Duration may increase if patients in this group are at risk for exposure to streptococcal infection. Complications : Carditis, Mitral steno sis, Mitral regurgitation, Aortic steno sis,Congestive heart failure (CHF) Prognosis

Sequelae are limited to the heart and are dependent upon the severity of the carditis during the acute attack.
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RHEUMATIC HEART DISEASE

Rheumatic heart disease is permanent damage to the heart valves caused by rheumatic fever. Rheumatic fever can develop if someone with a type of strep throat caused by group A betahemolytic streptococcus bacteria (GAS) does not receive proper antibiotic treatment. The American Heart Association says that GAS is the most common type of bacterial infection of the throat. According to the American Heart Association, most cases of rheumatic fever occur in children ages 5 to 15, so it is important for parents to be aware of the risk of rheumatic heart disease. Mortality/Morbidity Rheumatic heart disease is the major cause of morbidity from rheumatic fever and the major cause of mitral insufficiency and stenosis in world. Variables that correlate with severity of valve disease include the number of previous attacks of rheumatic fever, the length of time between the onset of disease and start of therapy, and sex. (The disease is more severe in females than in males.) Insufficiency from acute rheumatic valve disease resolves in 60-80% of patients who adhere to antibiotic prophylaxis. Race Race (when controlled for socioeconomic variables) has not been documented to influence disease incidence. Age:Rheumatic fever is principally a disease of childhood, with a median age of 10 years, although it also occurs in adults (20% of cases).
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Causes Rheumatic fever is thought to result from an inflammatory autoimmune response. Rheumatic fever only develops in children and adolescents following group A beta-hemolytic streptococcal pharyngitis, and only streptococcal infections of the pharynx initiate or reactivate rheumatic fever. PATHOPHYSIOLOGY

Rheumatic fever develops in some children and adolescents following pharyngitis with group A betahemolytic Streptococcus (ie, Streptococcus pyogenes) as described in rheumatic fever. History A diagnosis of rheumatic heart disease is made after confirming antecedent rheumatic fever. The modified Jones criteria (revised in 1992) provide guidelines for the diagnosis of rheumatic fever. The Jones criteria require the presence of 2 major or 1 major and 2 minor criterion for the diagnosis of rheumatic fever. The major diagnostic criteria include carditis, polyarthritis, chorea, subcutaneous nodules, and erythema marginatum. The minor diagnostic criteria include fever, arthralgia, prolonged PR interval on ECG, elevated acute phase reactants (increased erythrocyte sedimentation rate [ESR]), presence of C-reactive protein, and leukocytosis. Additional evidence of previous group A streptococcal pharyngitis is required to diagnose rheumatic fever. One of the following must be present:

Positive throat culture or rapid streptococcal antigen test result Elevated or rising streptococcal antibody titer History of previous rheumatic fever or rheumatic heart disease

These criteria are not absolute; the diagnosis of rheumatic fever can be made in a patient with chorea alone if the patient has had documented group A streptococcal pharyngitis. After a diagnosis of rheumatic fever is made, symptoms consistent with heart failure, such as difficulty breathing, exercise intolerance, and a rapid heart rate out of proportion to fever, may be indications of carditis and rheumatic heart disease.
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Physical: Physical findings in a patient with rheumatic heart disease include cardiac and no cardiac manifestations of acute rheumatic fever. Some patients develop cardiac manifestations of chronic rheumatic heart disease. Cardiac manifestations of acute rheumatic fever Pancarditis is the most serious and second most common complication of rheumatic fever (50%). In advanced cases, patients may complain of dyspnea, mild-to-moderate chest discomfort, pleuritic chest pain, edema, cough, or orthopnea. For a graph illustrating the time course for the carditis relative to the other clinical manifestations of rheumatic fever Upon physical examination, carditis is most commonly detected by a new murmur and tachycardia out of proportion to fever. New or changing murmurs are considered necessary for a diagnosis of rheumatic valvulitis. Other cardiac manifestations include congestive heart failure and pericarditis. New or changing murmurs Apical pansystolic murmur is a high-pitched, blowing-quality murmur of mitral regurgitation that radiates to the left axilla. The murmur is unaffected by respiration or position. Intensity varies but is grade 2/6 or greater. The mitral insufficiency is related to dysfunction of the valve, chordae, and papillary muscles. Apical diastolic murmur (also known as a Carey-Coombs murmur) is heard with active carditis and accompanies severe mitral insufficiency. The mechanism for this murmur is relative mitral stenosis, as the large volume of regurgitate flow traverses the mitral valve during ventricular filling. It is heard best with the bell of the stethoscope, while the patient is in the left lateral position and the breath held in expiration. Basal diastolic murmur is an early diastolic murmur of aortic regurgitation and is high-pitched, blowing, decrescendo, and heard best along the right upper and mid-left sternal border after deep expiration while the patient is leaning forward. Congestive heart failure Heart failure may develop secondary to severe valve insufficiency or myocarditis.The physical findings associated with heart failure include tachypnea, orthopnea, jugular venous distention, rales, hepatomegaly, a gallop rhythm, edema, and swelling of the peripheral extremities.
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Pericarditis A pericardial friction rub indicates that pericarditis is present.Increased cardiac dullness to percussion and muffled heart sounds are consistent with pericardial effusion. A paradoxical pulse (and accentuated fall in systolic blood pressure with inspiration) with decreased systemic pressure and perfusion and evidence of diastolic indentation of the right ventricle on echocardiogram reflect impending pericardial tamponade. In this clinical emergency, the pericardial effusion should be evacuated by pericardiocentesis. Noncardiac manifestations Common noncardiac (and diagnostic) manifestations of acute rheumatic fever include polyarthritis, chorea, erythema marginatum, and subcutaneous nodules. Other clinical, noncardiac manifestations include abdominal pain, arthralgias, epistaxis, fever, and rheumatic pneumonia. Polyarthritis is the most common symptom and is frequently the earliest manifestation of acute rheumatic fever (70-75%). Characteristically, the arthritis begins in the large joints of the lower extremities (knees and ankles) and migrates to other large joints in the lower or upper extremities (elbows and wrists). Affected joints are painful, swollen, warm, erythematous, and limited in their range of motion. The pain is out of proportion to clinical findings. The arthritis reaches maximum severity in 12-24 hours, persists for 2-6 days (rarely more than 3 wk) at each site, and rapidly responds to aspirin. Aspirin improves symptoms in affected joints and prevents further migration of the arthritis. Polyarthritis is more common and more severe in teenagers and young adults than in younger children. Sydenham chorea occurs in 10-30% of patients with rheumatic fever. Patients present with difficulty writing, involuntary grimacing, purposeless (choreiform) movements of the arms and legs, speech impairment, generalized weakness, and emotional lability. Physical findings include hyperextended joints, hypotonia, diminished deep tendon reflexes, tongue fasciculations ("bag of worms"), and a "milk sign" or relapsing grip demonstrated by alternate increases and decreases in tension when the patient grips the examiner's hand. In the absence of a family history of Huntington chorea, the diagnosis of acute rheumatic fever is almost certain. A long latency period (1-6 mo)

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between streptococcal pharyngitis and the onset of chorea is observed; a history of an antecedent sore throat is frequently not obtained. Daily handwriting samples can be used as an indicator of progression or resolution of disease. Complete resolution of the symptoms typically occurs with improvement in 1-2 weeks and full recovery in 2-3 months. However, cases have been reported in which symptoms wax and wane for several years. Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS) may be associated with chorea. Children have been identified in whom group A streptococcal infection appears to have triggered a relapsing-remitting symptom complex characterized by obsessive-compulsive disorder (somatic obsessions and checking, cleaning, and repeating compulsions), and neurologic abnormalities, such as cognitive defects and motoric hyperactivity. The symptoms are prepubertal in onset and may include emotional lability, separation anxiety, and oppositional behaviors. Erythema marginatum, also known as erythema annulare, is a characteristic rash that occurs in 513% of patients with acute rheumatic fever. It begins as 1-3 cm in diameter, pink-to-red nonpruritic macules or papules located on the trunk and proximal limbs but never on the face. The lesions spread outward to form a serpiginous ring with erythematous raised margins and central clearing. The rash may fade and reappear within hours and is exacerbated by heat. Thus, if the lesions are not well visualized, they can be accentuated by the application of warm towels, a hot bath, or the use of tangential lighting. The rash occurs early in the course of the disease and remains long past the resolution of other symptoms. When present, the nodules appear over the extensor surfaces of the elbows, knees, ankles, knuckles, and on the scalp and spinous processes of the lumbar and thoracic vertebrae where they are attached to the tendon sheath. They are firm, nontender, and free from attachments to the overlying skin and range in size from a few mm to 1-2 cm. They vary in number from one to dozens (mean 3-4). Histologically, they contain areas resembling the Aschoff bodies seen in the heart. Cardiac manifestations of chronic rheumatic heart disease Valve deformities, thromboembolism, cardiac hemolytic anemia, and atrial arrhythmias are the most common cardiac manifestations of chronic rheumatic heart disease. Mitral stenosis occurs in 25% of patients with chronic rheumatic heart disease and in association with mitral insufficiency in another 40%. Progressive fibrosis (ie, thickening and calcification of the valve)
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takes place over time, resulting in enlargement of the left atrium and formation of mural thrombi in that chamber. Aortic stenosis from chronic rheumatic heart disease is typically associated with aortic insufficiency. The valve commissures and cusps become adherent and fused, and the valve orifice becomes small with a round or triangular shape. Thromboembolism occurs as a complication of mitral stenosis. It is more likely to occur when the left atrium is dilated, cardiac output is decreased, and the patient is in atrial fibrillation. The frequency of this complication has decreased with the use of anticoagulation and the development of surgical repair for the valve abnormality. Cardiac hemolytic anemia is related to disruption of the RBCs by a deformed valve. Increased destruction and replacement of platelets also may occur. Atrial arrhythmias are typically related to a chronically enlarged left atrium (from a mitral valve abnormality). Successful cardioversion of atrial fibrillation to sinus rhythm is more likely to be successful if the left atrium is not markedly enlarged, the mitral stenosis is mild, and the patient has been in atrial fibrillation for less than 6 months. Patients should be anticoagulated before cardioversion to decrease the risk of systemic embolization. LABORATORY STUDIES Throat culture Throat culture findings for group A beta hemolytic Streptococcus are usually negative by the time symptoms of rheumatic fever or rheumatic heart disease appear. Rapid antigen detection test This test allows rapid detection of group A streptococcal antigen and allows the diagnosis of streptococcal pharyngitis and the initiation of antibiotic therapy while the patient is still in the physician's office. Because the rapid antigen detection test has a specificity of greater than 95% but a sensitivity of only 60-90%, a throat culture should be obtained in conjunction with this test. Antistreptococcal antibodies The clinical features of rheumatic fever begin at the time antistreptococcal antibody levels are at their peak. The elevated level of antistreptococcal antibodies is useful, particularly in patients that present with chorea as the only diagnostic criterion. Sensitivity for recent infections can be improved by testing for several antibodies. Antibody titers should be checked at 2-week intervals in order to detect a rising titer.
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Acute phase reactants The C-reactive protein and erythrocyte sedimentation rate are elevated in rheumatic fever due to the inflammatory nature of the disease. Both tests have a high sensitivity but low specificity for rheumatic fever. They may be used to monitor the resolution of inflammation, detect relapse when weaning aspirin, or identify the recurrence of disease. Heart reactive antibodies Tropomyosin is elevated in acute rheumatic fever. Rapid detection test for D8/17 This immunofluorescence technique for identifying the B cell marker D8/17 is positive in 90% of patients with rheumatic fever. It may be useful for identifying patients who are at risk for developing rheumatic fever. Imaging Studies Chest roentgenography Cardiomegaly, pulmonary congestion, and other findings consistent with heart failure may be seen on chest radiography. When the patient has fever and respiratory distress, chest radiography helps differentiate heart failure from rheumatic pneumonia. Doppler-echocardiogram In acute rheumatic heart disease, Doppler-echocardiography identifies and quantities valve insufficiency and ventricular dysfunction. With mild carditis, Doppler evidence of mitral regurgitation may be present during the acute phase of disease but resolves in weeks to months. In contrast, patients with moderate-to-severe carditis have persistent mitral and/or aortic regurgitation. The most important echocardiographic features of mitral regurgitation from acute rheumatic valvulitis are annular dilatation, elongation of the chordae to the anterior leaflet, and a posterolaterally directed mitral regurgitation jet. In chronic rheumatic heart disease, echocardiography may be used to track the progression of valve stenosis and may help determine the time for surgical intervention. The leaflets of affected valves become diffusely thickened, with fusion of the commissures and chordae tendineae. Increased echodensity of the mitral valve may signify calcification.

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 Parasternal long-axis view demonstrating the typical diastolic aortic insufficiency jet observed with rheumatic heart disease (red jet extending from the aorta into the left ventricle). (LV=left ventricle; LA=left atrium; Ao=aorta; RV=right ventricle). Heart catheterization In acute rheumatic heart disease, this procedure is not indicated. With chronic disease, heart catheterization has been performed to evaluate mitral and aortic valve disease and to balloon stenotic mitral valves. Postcatheterization precautions include hemorrhage, pain, nausea and vomiting, and arterial or venous obstruction from thrombosis or spasm.Complications may include mitral insufficiency after balloon dilation of the mitral valve, tachyarrhythmias, bradyarrhythmias, and vascular occlusion Other Tests On ECG, sinus tachycardia most frequently accompanies acute rheumatic heart disease. Alternatively, some children develop sinus bradycardia from increased vagal tone. No correlation between bradycardia and the severity of the carditis is noted. Histologic Findings Pathologic examination of the insufficient valves may reveal verrucous lesions at the line of closure. Aschoff bodies (perivascular foci of eosinophilic collagen surrounded by lymphocytes, plasma cells, and macrophages) are found in the pericardium, perivascular regions of the myocardium, and endocardium. The Aschoff bodies assume a granulomatous appearance with a central fibrinoid focus and eventually are replaced by nodules of scar tissue. Anitschkow cells are plump macrophages within Aschoff bodies. In the pericardium, fibrinous and serofibrinous exudates may produce an appearance of "bread and butter" pericarditis. Medical Care Medical therapy in rheumatic heart disease includes attempts to prevent rheumatic fever (and thus rheumatic heart disease). In patients who develop rheumatic heart disease, therapy is directed toward eliminating the group A streptococcal pharyngitis (if still present), suppressing inflammation from the autoimmune response, and providing supportive treatment for congestive heart failure. Prevention of rheumatic fever in patients with group a beta
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hemolytic streptococci (GABHS) pharyngitis For patients with GABHS pharyngitis, a meta-analysis supports a protective effect against rheumatic fever when penicillin is used following the diagnosis. Oral (PO) penicillin V remains the drug of choice for treatment of GABHS pharyngitis, but ampicillin and amoxicillin are equally effective. When PO penicillin is not feasible or dependable, a single dose of intramuscular benzathine penicillin G or benzathine/procaine penicillin combination is therapeutic. For patients who are allergic to penicillin, administer erythromycin or a first-generation cephalosporin. Other options include clarithromycin for 10 days, azithromycin for 5 days, or a narrow-spectrum (first-generation) cephalosporin for 10 days. As many as 15% of patients who are allergic to penicillin are also allergic to cephalosporins. Do not use tetracyclines or sulfonamides to treat GABHS pharyngitis. For recurrent group A streptococci (GAS) pharyngitis, a second 10-day course of the same antibiotic may be repeated. Alternate drugs include narrow-spectrum cephalosporins, amoxicillin-clavulanate, dicloxacillin, erythromycin, or other macrolides. Control measures for patients with GABHS pharyngitis are as follows:

Hospitalized patients: Place hospitalized patients with GABHS pharyngitis of pneumonia on droplet precautions, as well as standard precautions, until 24 hours after initiation of appropriate antibiotics. Exposed persons: People in contact with patients having documented cases of streptococcal infection first should undergo appropriate laboratory testing if they have clinical evidence of GABHS infection and should undergo antibiotic therapy if infected. School and childcare centers: Children with GABHS infection should not attend school or childcare centers for the first 24 hours after initiating antimicrobial therapy.

Treatment for patients following rheumatic heart disease (RHD) Preventive and prophylactic therapy is indicated after rheumatic fever and acute rheumatic heart disease to prevent further damage to valves. Primary prophylaxis (initial course of antibiotics administered to eradicate the streptococcal infection) also serves as the first course of secondary prophylaxis (prevention of recurrent rheumatic fever and rheumatic heart disease). An injection of 0.6-1.2 million units of benzathine penicillin G intramuscularly every 4 weeks is the recommended regimen for secondary prophylaxis for most US patients. Administer the same dosage every 3 weeks in areas where rheumatic fever is endemic, in patients with residual carditis, and in high-risk patients.

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 Although PO penicillin prophylaxis is also effective, data from the World Health Organization indicate that the recurrence risk of GABHS pharyngitis is lower when penicillin is administered parentally. The duration of antibiotic prophylaxis is controversial. Continue antibiotic prophylaxis indefinitely for patients at high risk (eg, health care workers, teachers, daycare workers) for recurrent GABHS infection. Patients with rheumatic fever and carditis but no valve disease should receive prophylactic antibiotics for 10 years or well into adulthood, whichever is longer. Finally, patients with rheumatic fever with carditis and valve disease should receive antibiotics for at least 10 years or until age 40 years. Patients with rheumatic heart disease and valve damage require a single dose of antibiotics 1 hour before surgical and dental procedures to help prevent bacterial endocarditis. Patients who had rheumatic fever without valve damage do not need endocarditis prophylaxis. Do not use penicillin, ampicillin, or amoxicillin for endocarditis prophylaxis in patients already receiving penicillin for secondary rheumatic fever prophylaxis (relative resistance of PO streptococci to penicillin and aminopenicillins). Alternate drugs recommended by the American Heart Association for these patients include PO clindamycin (20 mg/kg in children, 600 mg in adults) and PO azithromycin or clarithromycin (15 mg/kg in children, 500 mg in adults). The guidelines for endocarditis prophylaxis in patients with valve damage from rheumatic heart disease have changed. Antibiotic prophylaxis is no longer recommended.[7] ANTIBIOTICS

PENICILLIN oral solution:125mg,250mg tablet:250mg,500mg, Pneumococcal Infections < 12 years old

ANTIINFLAMMATORY AGENTS Aspirin

Steroids Dosing Forms & Strengths

Tablet:81mg,300mg,325mg,500mg, oral solution:5mg/5mL 650mg tablet: Analgesic/Antipyretic <12 years old: 10-15 mg/kg/dose PO q4hr, up to 60-80 mg/kg/day

Systemic

25-50 mg/kg/day 12 years or older: As in adults q6-8hr PO No more than 3 Juvenile Rheumatoid Arthritis
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1mg 2.5mg 5mg 10mg 20mg 50mg

Anti-

<25 kg: 60-100 mg/kg/day PO inflammatory/Immunosuppressive divided q6-8hr (maintain serum >12 years old 0.5-2 mg/kg/day PO qD or divided salicylate 150-300 mcg/mL) BID; not to exceed 80 mg/day 125-500 mg q6-8hr 25 kg or heavier: 2.4-3.6 g/day PO Acute Asthma Kawasaki Disease Primary Prevention of <12 years old: 2 mg/kg/day PO qD or Rheumatic Fever Febrile phase: 80-100 mg/kg/day divided BID for 3-10 days; not to PO divided q6hr exceed 60 mg/day Children: 250 mg BID-TID PO x 10 days After defervesces: 3-6 mg/kg PO >12 years old: 40-60 mg PO qD for qDay divided BID for 3-10 days Adolescents: 500 mg BIDTID PO x 10 days Other Information Nephrotic Syndrome Recurrent Rheumatic Toxic dose: 200 mg/kg Fever Prophylaxis Children: 250 mg BID PO Angiotensin-converting enzyme (ACE) inhibitors Enalapril (Vasotec) injectable solution:1.25mg/mL oral solution:1mg/mL tablet:2.5mg,5mg,10mg,20mg Hypertension (1 Month Old - 16 Years Old) Neonates: 0.05-0.1 mg/kg/dose q8-24hr, titrate dose up to 0.5 mg/kg/dose q6-24hr captopril oral suspension:1mg/mL tablet:12.5mg,25mg,50mg,100mg Hypertension (Off-label) 2 mg/kg PO qD; not to exceed 80 mg/day

g/day

Initial 0.1 mg/kg/day PO qDay or divided q12hr; Infants: 0.15-0.3 mg/kg/dose; titrate dose upward to no more than 5 mg/day, increase PRN q2Weeks maximum 6 mg/kg/day in 1-4 divided doses; 2.5-6 to no more than 0.5 mg/kg/day (40 mg/day) mg/kg/day usually required 0.01-0.02 mg/kg/day divided q12hr IV infusion Hypertensive Crisis 0.05-0.1 mg/kg direct IV injection Older children: 6.25-12.5 mg/dose q12-24hr; titrate to no more than 6 mg/kg/day divided BID/QID
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Children: 0.3-0.5 mg/kg/dose; titrate to maximum 6 mg/kg/day divided BID/QID

Adolescents: 12.5-25 mg/dose q8-12hr; may increase by 25 mg/dose q1-2Weeks to maximum 450 mg/day

Diet The diet should be nutritious and without restrictions except in the patient with congestive heart failure. In these patients, fluid and sodium intake should be restricted. Potassium supplementation may be necessary if steroids or diuretics are used. Further Outpatient Care Patients usually show significant improvement after initiation of anti-inflammatory therapy. However, they should not be allowed to resume full activities until all clinical symptoms have abated and laboratory values have returned to normal levels. The importance of prophylaxis against recurrent streptococcal pharyngitis and rheumatic fever should be emphasized with each patient. Each recurrent episode of rheumatic carditis produces further valve damage and increases the likelihood that valve replacement will be required. Patients should remain on antibiotic prophylaxis at least until their early twenties. Many physicians believe that lifelong prophylaxis is appropriate. Complications Potential complications include heart failure from valve insufficiency (acute rheumatic carditis) or stenosis (chronic rheumatic carditis). Associated cardiac complications include atrial arrhythmias, pulmonary edema, recurrent pulmonary emboli, infective endocarditis, intracardiac thrombus formation, and systemic emboli. Prognosis Manifestations of acute rheumatic fever resolve over a period of 12 weeks in 80% Patient Education Emphasize the importance of prophylaxis against recurrent streptococcal pharyngitis and rheumatic fever with each patient. For excellent patient education resources, visit eMedicine's Heart Center. Also, see eMedicine's patient education article Mitral Valve Prolapse.

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Nursing Consideration of client with acquired heart disease: The objectives of nursing care for child with A.h.d. are to: 1. 2. 3. 4. Encourage compliance with drug regimens. Facilitate recovery from the illness. Provide emotional support. Prevent the disease.

A. Encourage compliance with drug regimens: Since compliance is a major concern in long term during therapy every effort is made to encourage adherence to the therapeutic plan. When compliance is poor monthly injections may be substituted for daily oral administration of antibiotics. B. Facilitate recovery from the illness: The major role of the nurse is to assist the child and parents to understand the need for the amount of bed rest ordered by the physician. The aim of the bed rest is to minimize the effect of the inflammatory process on all body systems, particularly the heart. If the metabolic needs oxygen consumption and expected cardiac response can be very low. The damage to heart muscle and valve tissue may be restricted to what has already occurred. During the febrile phase, fluids are encouraged to prevent dehydration but overdehydration is avoided because hypervolemia places increased demands on the heart As soon as a child is willing to eat a liquid or soft diet is given in small frequent amounts to minimize the exertion of chewing. The diet is gradually increased to meet the childs nutritional requirements overfeeding is to be avoided. Pain from Carditis or polyarthritis is decreased by administration of anti-inflammatory agents (aspirin). Alternating warm and cold snacks to the affected joints may reduce swelling and inflammation and provide direct and immediate reduction of pain. During chorea the only treatment is prevention of injury during the period when involuntary jerking and possibly violent movements occur.

Restraints to prevent the child from falling from a chair, padding on the sides of the beds or chair to prevent injury.
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Assist once during walking. Feeding the child to prevent injury. Bed rails to prevent the child from falling out of bed.

Behavioral changes and learning difficulties are discussed with school, classmates, peer group and parents. C. Provide emotional support: The parents and child need to know that all the manifestations of rheumatic fever are temporary expected for the cardiac involvement let the child verbalize his feelings. Find ways for bed activities, i.e. quiet play and school work while in hospital and home. Allow for parent of normal routine at home. Classmates and sibling can show their concern by card, letters etc. D. Prevent the disease: The nurse should be alert to any child who has abrupt onset of signs and symptoms of a streptococcal sore throat. After streptococcal pharyngitis has been diagnosed, antimicrobial treatment is given to eradicate the infection. A single injection of benzathine penicillin is the most reliable from the therapy, oral penicillin or erythromycin may be given daily for 10 days, the nurse help the parents to be cooperative. The main nurses role in prevention is to help educate the public as well as the individual patient and family. Massive screening program with throat cultures and support of patient cooperation in therapy can eradicate the disease. N.B.: from the previous discussion about A.R.F. try to develop the appropriate nursing care plan.

Plan of nursing care for a child undergoing heart surgery: Pre-operative care: a-Pre-operative assessment: It can be done though observation. It will facilitate care planning. Postoperatively, it will include:

Baseline vital signs (apical pulse and blood pressure) in all examinations should be obtained for evaluation at rest and with activity-report any change.

Admission history and physical examination.

Height and weight measurement for fluid replacement. Pre-operative studies, i.e. E.C.G. Lab Tests should be prepared. Sleep/ awake patterns.
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Additional observation (normal activities, rest be noted & reported)- physical and emotional stress should be reported. Elimination pattern to avoid constipation post-operatively (should be planned in advance). b. Pre-operative preparation: It should be given throughout the pre-operative period. Instructing child and parents about tests will be done; operation. when appropriate. Take child and parents to the operating suite and ICU to be familiar with the physical settings (lights, gown, .etc). Show them the equipment used gradually. Place child temporarily into oxygen tent to make him accustomed to it and to oxygen mask through play (sometimes fear of suffocation). Introduce them to post-operative procedure, semi fowlers position, how to turn from side to side. Practice deep inspiration and expiration. Through play, nurse demonstrates deep act of coughing, breathing to the childlet him practice it (tell him that they may cause some discomfort in practice post-operatively). Demonstrate postural drainage and percussion. Prepare them (if old enough) for nasogastric tube catheter for bladder-by telling them about it. During preparation, the nurse must be aware of the level of childs anxiety (so discussion should be ended if she felt the child is very anxious). c. Physical preparation for surgery: Shaving the child skin the evening before the operation. Cleansing enema may be ordered. Sedation is given for rest (or Dr. order). Vital signs are checked.

Fluid intake: to estimate the childs fluid consumption and which fluids are preferred.

Postoperative Care: Immediate Admission to ICU: Oxygen immediately. Check each catheter in his chest and attached to the suction machine. Check vital signs. If cardioscopy will be in use, attach it to the patient, then EKG, heart rate and rhythm will observed constantly. I.V. fluids must be checked and regulated carefully.
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Then: Tell patient that operation is over when he re-awakes. Narcotic may be given as Dr. order. Vital signs are taken each hour or more often if necessary (temperature rectally as cool atmosphere in oxygen tent may affect temperature). Temperature: if there is fever, reduce it by tepid water sponges, ice bags or ice water mattress. Respiration: - Check depth and rate of respiration and chest expansion on both sides is equal. - Do suction if respiration becomes noisy and rapid. Pulse: it should be observed for: regular rhythm and power. Blood pressure: Should be taken and notifying doctor for any change. Observe: skin color, moisture (dry moist). (Warm cold). Monitor fluids: if cut down is used for I.V.; not be too rapid, it must be in proper position (no filtration). Oral fluids are restricted in immediate post-operative period to decrease load on heart, therefore mouth care will also decrease childs thirsty. Input and output of all fluids must be accurately calculated and recorded. Urine must be analyzed for specific gravity to assess kidney function. Fluids requirement is based on childs weight and body surface area. If no voiding within 8-12 hours after operation, catheter may be used. Care for chest tubes (under-water seal). Observe the drainage, bleeding, be sure that they are patent (free from blood clots) and well tighten to patient. Important: be sure that the bottles. Below bed level and observe the amount, color and consistence. If cyanosis, sudden sharp chest pain around the catheter and dyspnea and tachycardia occur. Clamp the chest tube and call Dr. immediately. Encourage child for deep breathing and coughing exercise and support his chest especially over the incision area and praise him. Change position every hour. Support the body with pillows if he cant maintain his position. Encourage him to move himself. Observe: color of legs and its coldness, bleeding or infection at side of operation. To provide rest, good plan of N.C. to decrease disturbance. Encourage patient to be out of bed (according to his condition and severity of operation) on wheel chair after chest tubes have been removed. Child may regress in his behavior (cry- more physical contact- more demanding) understand his behavior as a nurse once his condition improves help him to gain control of his situation. Before discharge: plan of care must be done with the health team and parents.

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ABSTRACTS 1.Prevalence of rheumatic heart disease in children and young adults in Nicaragua. Abstract Rheumatic heart disease (RHD) results in morbidity and mortality that is disproportionate among individuals in developing countries compared to those living in economically developed countries. The global burden of disease is uncertain because most previous studies to determine the prevalence of RHD in children relied on clinical screening criteria that lacked the sensitivity to detect most cases. The present study was performed to determine the prevalence of RHD in children and young adults in Len, Nicaragua, an area previously thought to have a high prevalence of RHD. This was an observational study of 3,150 children aged 5 to 15 years and 489 adults aged 20 to 35 years randomly selected from urban and rural areas of Len. Cardiopulmonary examinations and Doppler echocardiographic studies were performed on all subjects. Doppler echocardiographic diagnosis of RHD was based on predefined consensus criteria that were developed by a working group of the World Health Organization and the National Institutes of Health. The overall prevalence of RHD in children was 48 in 1,000 (95% confidence interval 35 in 1,000 to 60 in 1,000). The prevalence in urban children was 34 in 1,000, and in rural children it was 80 in 1,000. Using more stringent Doppler echocardiographic criteria designed to diagnose definite RHD in adults, the prevalence was 22 in 1,000 (95% confidence interval 8 in 1,000 to 37 in 1,000). In conclusion, the prevalence of RHD among children and adults in this economically disadvantaged population far exceeds previously predicted rates. The findings underscore the potential health and economic burden of acute rheumatic fever and RHD and support the need for more effective measures of prevention, which may include safe, effective, and affordable vaccines to prevent the streptococcal infections that trigger the disease.

2.Multiple Brain Abscesses: An Initial Presentation In A Child With An Undiagnosed Sinus Venosus Atrial Septal Defect Rukhsana Abdullaha, Mary Baldaufa, Shabana Azama, Mohamud Hassaneina and Sarita Dhuper
, a,

Abstract

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Cerebral abscess is a serious condition and in children the commonest cause is cyanotic congenital heart disease. We report a 12-year old male who presented with multiple brain abscesses. After an extensive evaluation including a transesphageal echocardiogram (TEE), we found a previously undiagnosed sinus venous atrial septal defect (ASD). He was treated medically with antibiotics. This is an unusual initial presentation of asymptomatic acynotic congenital heart disease in children. We recommended a complete cardiac evaluation including TEE with saline contrast study in the work up of an idiopathic cerebral abscess. Article Outline 1. Inroduction Cerebral abscess is an uncommon condition in children associated with high mortality and morbidity. Early recognition and treatment is the cornerstone to favorable outcome. Brain abscess can occur in any age group in children but the peak incidence is between 4 and 7 years of age. [1] Cerebral abscess results from invasion of infectious organism into the parenchyma of the brain, by direct invasion or through hematogenous spread. The most common predisposing conditions in children are cyanotic congenital heart disease and dental, sinus and ear infections. Other uncommon conditions are meningitis, penetrating head injury, and an immunosuppressive state. [1] and [2] The pathogenesis is undetermined in 1015% of cases. [3] Clinical presentation of brain abscess includes headache, fever and vomiting in 6070% of cases. Seizures, altered mental status and focal neurological signs occur in 2550% of cases. [1] and [4] CT scan and MRI are the essential diagnostic tools for accurate diagnosis. [5] Surgical drainage and antimicrobial therapy are the treatments of choice for most of the brain abscess. However, with the advent of CT and MRI, medical management has been used successfully in carefully selected cases. [3] Acyanotic congenital heart disease is an unusual cause of brain abscess in children. Patent foramen ovale has been reported in few cases as an association for cryptogenic brain abscess, in the adult population. [6] and [7] We are reporting a case of 12-year old boy who presented with multiple brain abscesses secondary to a previously undiagnosed sinus venosus ASD and responded well to medical therapy alone. 3.Acute rheumatic fever and the evolution of rheumatic heart disease: A prospective 12 year follow-up report Hasan A. Majeed,aSudhair Batnagerb, Abdul Mohsen Yousofb, Faisal Khuffasha and Abdul Razzak Yusufb,

Abstract Sixty four children who presented with the initial attack of acute rheumatic fever and maintained continuous regular secondary prophylaxis, were followed up prospectively for 12.3 years (an observation
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period of 775 patient-years). The prevalence rate of rheumatic heart disease in the 29 children who had carditis in the initial attack and in the 35 children who had no carditis initially was 49 vs 0%, respectively. The overall prevalence rate of rheumatic heart disease was 20%. Mitral incompetence developed in 11 patients (17%), aortic incompetence in 2 (3%) and mitral stenosis in 2 (3%). None of the patients developed aortic stenosis. Two recurrences developed with a recurrence rate of 0.003 per patient per year. One patient needed cardiac surgery and there was no mortality. These data strongly suggest that continuous regular secondary prophylaxis can prevent or significantly reduce the development of mitral and aortic valve stenosis, the prevalence rate of rheumatic heart disease and mortality. 4.Incidence of rheumatic fever and prevalence of rheumatic heart disease in India .M. Vijaykumara, Jagat Narulab, K. Srinath Reddy Abstract Rheumatic heart disease (RHD) has been documented in Indian medical literature since the early nineteenth century, contrary to the popular belief that rheumatic fever (RF) and its sequelae were exclusively prevalent in temperate limates until the early twentieth century. Rheumatic heart disease has, in the past 50 years, emerged as a major contributor to cardiovascular morbidity in India. Despite the paucity of clear information regarding secular trends, the few available community surveys indicate that there are at present more than 1 million patients with RHD. Even a conservative estimate of the incidence of RF suggests that at least 50 000 new episodes occur every year. The younger age of onset (juvenile RHD) seen in India is a special feature of both public health and clinical importance. These patterns of RF and RHD, which may be similar to those in other developing countries, underscore the importance of effective public health strategies for prevention and control.
,c

and Edward L. Kapland

CONCLUSION: Acquired heart diseases and rheumatic heart diseases are important disease conditions in pediatric care. mortality and morbidity rates are very high and if untreated then prognosis is also very poor. So it is very much important to understand all these disease conditions in detail with definition, causes, path physiology, clinical signs and management. In depth understanding of disease process improves the knowledge and skill and it also helps us to deliver qualitative care when we came across with the client in an actual situation. as a pediatric nursing
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specialty this presentation gives us knowledge about all these disease condition and enhances our efficacies towards qualitative care in dealing with the clients with same disease conditions Thank you

BIBLIOGRAPHY: Asssuma Beevi.textbook Of Peditric Nursingfirst edition,elesvier publications,2010.pp:261-271 Bauchner Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier; 2007:435-450 Datta parul , Pediatric H. Failure to thrive. In: Kliegman RM, Behrman RE, Jenson HB, Nursing , Second edition , 2006, J.P brothers Ltd., New Delhi.pp:312-315. Dorowthy Marlow R, Pediatric Nursing, Sixth edition, 2002, W. B.Saunders Pvt.Ltd., Philadelphia Page Number:1014-1049. Ghai o.p.Textbook of preventive edition,,2007,pp:375,386,380. and social medicinethird edition,jaypee brothers.third

Gupta m.c.mahajan BK,Textbook of preventive and social medicinethird edition,jaypee brothers,New delhi,pp:348-360. Gupta Piyush.*2004+,Essentials Of Paediatric Nursing,first edition,2009,vora medical publication ,Mumbai.pp:303;312. Forfor and arneils,Texbook Of Paediatricseventh edition, 2009.elsevierchurchil publication, living stone, New delhi.page no: Meherban singh , pediatrics:Clinical Methods, Third edition,sagar publications,2006.pp:284-301. Nelson,Texbook Of Paediatric, eight edition,elsveier India pvt.ltd,New delhi.2009,page no:1036-1060. Wongs, Essentials Of Pediatric Nursing, Seventh edition, Jaypee brothers, Mosby Publication, Missouri. Page Number:980 1012. References ^ a b c Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; & Mitchell, Richard N. (2007). Robbins Basic Pathology (8th ed.). Saunders Elsevier. pp. 403-406 ISBN 978-1-4160-2973-1 2. ^ "rheumatic fever" at Dorland's Medical Dictionary 1.
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3. 4.

^ Jones TD (1944). "The diagnosis of rheumatic fever.". JAMA 126: 4814. ^ Ferrieri P; Jones Criteria Working, Group (2002). "Proceedings of the Jones Criteria workshop". Circulation 106 (19): 25213. doi:10.1161/01.CIR.0000037745.65929.FA. PMID 12417554. http://circ.ahajournals.org/cgi/content/full/106/19/2521?ck=nck. 5. ^ Steven J Parrillo, DO, FACOEP, FACEP. "eMedicine Rheumatic Fever". http://www.emedicine.com/emerg/topic509.htm. Retrieved 2007-07-14. 6. ^ "Guidelines for the diagnosis of rheumatic fever. Jones Criteria, 1992 update. Special Writing Group of the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease of the Council on Cardiovascular Disease in the Young of the American Heart Association". JAMA 268 (15): 206973. 1992. doi:10.1001/jama.268.15.2069. PMID 1404745. 7. ^ Saxena, Anita (2000). "Diagnosis of rheumatic fever: Current status of Jones criteria and role of echocardiography". Indian Journal of Pediatrics 67 (4): 2836. doi:10.1007/BF02758174. PMID 11129913. 8. ^ http://www.utmb.edu/Pedi_Ed/CoreConceptsOfPediatrics/Cardiology/page_40.htm 9. ^ Abbas and Lechtman. Basic Immunology: Functions and Disorders of the Immune System. Elsevier Inc. 2004. 10. ^ Fa KC, da Silva DD, Oshiro SE, et al. (May 2006). "Mimicry in recognition of cardiac myosin peptides by heart-intralesional T cell clones from rheumatic heart disease". J. Immunol. 176 (9): 566270. PMID 16622036. http://www.jimmunol.org/cgi/pmidlookup?view=long&pmid=16622036. 11. ^ a b Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease. St. Louis, Mo: Elsevier Saunders. ISBN 0-7216-0187-1. http://www.robbinspathology.com/. 12. ^ "Rheumatic Heart Disease/Rheumatic Fever". American Heart Association. http://www.americanheart.org/presenter.jhtml?identifier=4709. Retrieved 2008-02-17. 13. ^ "WHO Disease and injury country estimates". World Health Organization. 2009. http://www.who.int/healthinfo/global_burden_disease/estimates_country/en/index.html. Retrieved Nov. 11, 2009. 14. ^ NLM/NIH: Medline Plus Medical Encyclopedia: Rheumatic fever 15. ^ Porth, Carol (2007). Essentials of pathophysiology: concepts of altered health states. Hagerstown, MD: Lippincott Williams & Wilkins. ISBN 0-7817-7087-4.

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