NCM 102

Pediatric Nursing
Prepared by: Adahlia T. Basco RN, MAN

Growth and Development

Growth:
Is physical change and increase in size. It can be measured quantitatively. Indicators of growth includes height, weight, bone size, and dentition. Growth rates vary during different stages of growth and development. The growth rate is rapid during the prenatal, neonatal, infancy and adolescent stages and slows during childhood. Physical growth is minimal during adulthood

Development:
Is an increase in the complexity of function and skill progression. It is the capacity and skill of a person to adapt to the environment. Development is the behavioral aspect of growth

DEVELOPMENTAL MILESTONE Is a standard of reference by which to compare a childs behavior at specific ages

Sigmund Freud
Age 0-2 years old -4 years old 4-5 years old puberty Name Oral Anal Phallic Latency Pleasure Source Mouth: sucking, biting, swallowing Anus: defecating or retaining feces Genitals Sexual urges sublimated into sports and hobbies. Same-sex friends also help avoid sexual feelings Conflict Weaning away from mothers breast Toilet training Oedipus (boys), Electra (girls)

puberty onwards

Genital

Physical sexual changes reawaken repressed needs. Direct sexual feelings towards others lead to sexual gratification.

Social Rules

Erick Erikson
STAGE 1. Infancy AGE Birth-18 mos CENTRAL TASK Trust vs Mistrust (+) RESOLUTION Learn to trust others (-) RESOLUTION Mistrust, withdrawal, estrangement Compulsive, self-restraint or compliance. Willfulness & defiance. Lack of selfconfidence. Pessimism, fear of wrongdoing. Over-control & over-restriction.

2. Early childhood

1 to 3 y/o

Autonomy vs Self control w/o Shame & doubt loss of self esteem Ability of cooperate & express oneself Initiative vs guilt Learns to become assertive Ability to evaluate ones own behavior

3. Late childhood

3 to 5 y/o

Erick Erikson`s
4.School Age 6 to 12 y/o Industry vs Inferiority Learns to create, develop & manipulate. Develop sense of competence & perseverance. Loss of . School Age hope, sense of being mediocre. Withdraw al from school & peers.

5. Adolescence

1220 y/o

Identity vs role Coherent sense confusion of self. Plans to actualize ones abilities

Feelings 5. of Adolescence confusion, indecisive ness, & possible anti-social behavior.

Erick Erikson`s
6. Young Adulthood 18-25 y/o Intimacy vs isolation Intimate relationship with another person. Commitment to work and relationships. Impersonal relationships. Avoidance of relationship, career or lifestyle commitments. Self-indulgence, self-concern, lack of interests & commitments. Sense of loss, contempt for others.

7. Adulthood

25-65 y/o

Generativity vs Creativity, stagnation productivity, concern for others. Integrity vs despair Acceptance of worth & uniqueness of ones own life. Acceptance of death.

8. Maturity

65 y/o to death

INFANCY
Birth to 1 year Erickson: Trust vs Mistrust Freud: Oral Solitary play Consistent primary caregiver

SMILES: After two months of sleepless nights and round-the-clock soothing, you've seen plenty of your baby's tears. Maybe you've spotted a fleeting smile, but then again, it could have been gas. Now it's time for the real reward. By around 2 months of age, your baby will smile in response to you! The sound of your voice or the sight of your face is often all it takes to trigger baby's irresistible grin.

LAUGHS: If the frequent sound of baby's crying has you on edge, take heart. By 4 months, you can look forward to another sound, possibly the sweetest you'll ever hear - your baby's laughter. The best part is how easily a baby laughs. Silly faces, tickling, and peek-a-boo are usually more than enough to set off lots of squeals and giggles.

SLEEP: Like no other baby milestone, a full night of sleep becomes the Holy Grail for new parents. While it is unrealistic and unhealthy to expect a newborn to sleep all night, parents can rest assured that relief will come soon. By 4-6 months, most babies are capable of sleeping through the night.

SITS UP: How different the world looks when you're not stuck on your belly! Around 5 or 6 months, most babies can sit up with support either by resting on their hands in front of them or by leaning on pillows or furniture. Babies can usually sit alone steadily by 7-9 months.

CRAWLS: If you have an 8-month-old, you may want to put your gym membership on hold. You're about to get plenty of exercise chasing your suddenly mobile baby around the house. By 9 months, most babies crawl using both hands and feet, though some babies never crawl, preferring to creep or wriggle instead. Crawling is not an essential baby milestone, and infants who choose to scoot or creep still tend to reach other milestones on schedule.

Waving "bye-bye" is not just a cute trick - it is an actual expression of language. By 9 months most babies begin to make the link between sounds, gestures, and meaning. They understand that waving is connected to the phrase "bye-bye."

Just when spoon-feeding begins to lose its luster, babies are ready to feed themselves. Between 9-12 months, babies develop better control over their hands and fingers, making it easier to grab small objects - like finger foods! Unfortunately, babies this age love to explore taste and texture, so food is not the only thing they'll try to pop into their mouths. Environmental safety should, therefore, become a big parental concern at this age.

By 12 months, most babies begin to stand briefly without support. They also take small steps while holding onto furniture or other objects, an activity called "cruising." In the weeks or months before they walk independently, babies may spend hours cruising to practice for the real thing.

You might call it the crown jewel of baby milestones. No other moment is met with more anticipation (or camera clicks) than baby's first step on his or her own. But not all babies walk by their first birthday. The normal range is anywhere from 9 to 17 months, with most babies taking at least a few steps by about 13 months.

Says a Word "Mama! Dada!" There's nothing like hearing your baby call your name, and it usually happens right around the oneyear mark. By this time, most babies can say at least one real word and actively try to imitate others. It won't be long before you finally get to hear what's on your little one's mind.

INFANCY
Physiological: BW x 2 in 6 months BW x 3 in 1 year Posterior fontanel closes in 2-3 months Anterior fontanel remains open until 18 mos Height increase by 50% in 1 year HC > chest circumference until 1 year HC is measured until 2 y/o Tooth eruption begins in 4 6 mos

Weaning is the transition from the bottle or breastfeeding to a cup, typically begun at 8 9 mos.

TODDLER
1 3 years Erickson: Autonomy vs Shame & Doubt Independence Freud: Anal child gratifies by controlling body excretion Toilet training Parallel play

TODDLER
Physiological: BW x 4 in 3 years Anterior fontanel closes Sphincter control begins around age 2 All deciduous teeth by 2 years Pot belly appearance HC = CC Bow legs are normal around 2 3 years Appetite decreases

PRESCHOOL
3 6 years Erickson: Initiative vs Guilt Freud: Genital or Phallic Associative Play Sense of self esteem Loves school

PRESCHOOL
Physiological: Growth of extremities Weight increases by 4-6 lbs/year Handedness established Body systems mature Day time toilet training complete All deciduous teeth complete, see dentist

SCHOOL AGE
6 12 years Erickson: Industry vs Inferiority Freud: Latency Competitive play Privacy is important Group play Same sex friends

SCHOOL AGE

Physiological: Increased physical strength & capabilities Slower physical growth Permanent teeth starts to replace deciduous teeth Boys and girls remain close in size

ADOLESCENCE
13 18 years Erickson: Identity vs Role Confusion Freud: Genital Independence from family Peer group Intense relationship

ADOLESCENCE Physiological: Rapid growth rate: 4/ year in boys, 3/year in girls & slows at age 16 Primary & secondary sex characteristics develops Puberty 2 years early in girls than boys Boys genital development Girls breast development

CONGENITAL HEART DEFECTS

FOUR CLASSIFICATIONS OF CHD

1. Increased Pulmonary Blood Flow (L-R) 2. Obstruction to Blood Flow 3. Mixed Blood Flow (O2 and UnO2) 4. Decreased Pulmonary Blood Flow

INCREASED PULMONARY BLOOD FLOW Blood flow from L side of the heart (increased pressure) to the R side of the heart (decreased pressure) through some abnormal opening or connection between the 2 systems of the great arteries.

VENTRICULAR SEPTAL DEFECT, or holes in the walls that separate the left and right sides of the heart

Most common CHD Acyanotic Mild cases can close in 1st year of life 30% of all cases of CHD

Management
Surgery: Palliative pulmonary artery banding Complete repair Knitted Dacron Patch is sutured over the opening via cardiopulmonary bypass Medications: Digoxin Diuretics

I.2. Atrial Septal Defect

Acyanotic type Most common in girls 3 types: 1. Ostrium Primum (ASD1) opening is in the lower end of the septum 2. Ostrium Secundum (ASD2) opening is near the center of the septum 3. Sinus Venosus Defect - opening near junction of the superior vena cava and right atrium

Clinical Manifestations

Harsh systolic murmur Dyspnea w/ feeding and frequent respiratory infections Decrease CO may result w/: Tachycardia, cool skin Delayed capillary refill AV node involvement may result in arrhythmias

Management:

Surgery - done bet 2 and 3 years of age, Dacron or Silastic patch is sutured into place for occlusion Non Surgical cardiac catheterization

Atrioventricular Canal Defect

Also called as endocardial cushion defect Incomplete fusion of endocardial cushions Associated with Downs Syndrome

Management:
Palliative Pulmonary artery banding Surgery Patch closure of the septal defects Reconstruction of the AV valve or valve replacement

. Patent Ductus Arteriosus

Failure of ductus arteriosus to close within the first week of life Wide pulse pressure and bounding pulses runoff blood from aorta to pulmonary artery
Machinery like murmur

A PATENT DUCTUS ARTERIOSUS is a blood vessel between the aorta and main pulmonary artery that all babies require in fetal life but which usually closes within the first couple of days of life.

Some babies are born with other connections between the two main arteries leaving the heart, i.e., aortopulmonary window or truncus arteriosus. These babies are also at risk for having too much blood flow to the lungs.

Holes between the two upper chambers of the heart (atrial septal defects) rarely cause problems with congestive heart failure no matter how large.

In older children where the structure of the heart is normal, it is usually due to a weakening of the heart muscle, or cardiomyopathy, or Kawasaki Disease, which all can lead to congestive heart failure.

CARDIOMYOPATHY can also be seen in babies and can be due to a number of problems such as rhythm disturbances or infections.

Management
Indomethacin(indocin) to close the patent ductous If medical management fails, use of coils to occlude PDA in catheterization(palliative:PA Banding) Surgical division or ligation of the patent vessel via a left thoracotomy

Management common to all LR/ Acyanotic Heart Defects

Small frequent feeding High calorie feeding, gavage feeding Cluster care to allow for rest periods Fluid restrictions

II. DEFECTS WITH OBSTRUCTION TO BLOOD FLOW

An obstruction defect is a type of defect where one of the valves or ventricles is narrowed to such a degree that it partially or completely blocks the flow of blood. There are several types of obstruction defects, depending upon where the malformation occurs.

Types of Obstruction Defects

Pulmonary stenosis

Aortic stenosis Coarctation of the aorta

Symptoms of Obstruction Defects

Cyanosis (a bluish color to the skin due to lack of blood oxygen) Chest pain Unusual fatigue Dizziness More serious symptoms include congestive heart failure or high blood pressure.

II.1 Pulmonary Stenosis Narrowing of the pulmonary valve or pulmonary artery just distal to the valve

Pulmonary Stenosis

In children, these problems can include: a valve that has leaflets that are partially fused together. a valve that has thick leaflets that do not open all the way. the area above or below the pulmonary valve is narrowed.

different types of pulmonary stenosis:

-valvar pulmonary stenosis -supravalvar pulmonary stenosis -subvalvar (infundibular) -pulmonary stenosis -branch peripheral pulmonic stenosis

Causes of pulmonary stenosis

improper development of the pulmonary valve in the first 8 weeks of fetal growth a number of factors, though most of the time this heart defect occurs sporadically (by chance) with no clear reason evident for its development.

Symptoms of pulmonary stenosis

heavy or rapid breathing shortness of breath fatigue rapid heart rate swelling in the feet, ankles, face, eyelids, and/or abdomen fewer wet diapers or trips to the bathroom

Diagnosis: chest x-ray electrocardiogram (ECG or EKG) echocardiogram (echo) cardiac catheterization

Treatment
Specific treatment for pulmonary stenosis will be determined by your child's physician based on: your child's age, overall health, and medical history extent of the condition your child's tolerance for specific medications, procedures, or therapies expectations for the course of the condition your opinion or preference

balloon angioplasty (to dilate narrow valve) or valvuloplasty Valvotomy (Brock procedure for infant) Valvectomy (with or without transannular patch) pulmonary valve replacement

Post-procedure care for your child:

cath lab interventional procedure surgical repair Ventilator intravenous (IV) catheters arterial line nasogastric (NG) tube urinary catheter chest tube heart monitor

Long-term outlook after pulmonary stenosis repair:

recommends antibiotics be given to prevent bacterial endocarditis after discharge from the hospital. repeat interventional cath lab procedures may be necessary during infancy and childhood to stretch the valve open. Replacement of the pulmonary valve. Regular follow-up care.

Aortic Stenosis
Narrowing or stricture of the aortic valve Clinical Manifestations: Infants
Decrease CO w/ faint pulses Hypotension, tachycardia Poor feeding

Children
Sign of exercise intolerance Chest pain, dizziness when standing for a long period of time Murmur

AORTIC STENOSIS

TYPES:

VALVULAR STENOSIS
SUBVALVULAR STENOSIS SUPRAVALVULAR STENOSIS

Aortic Stenosis

Aortic Valve
found between the left ventricle and the aorta. It has three leaflets that function like a one-way door, allowing blood to flow forward into the aorta, but not backward into the left ventricle.

Aortic Valve

In children, these problems can include a valve that:

bicuspid aortic valve leaflets that are partially fused together. has thick leaflets that do not open all the way. becomes damaged by rheumatic fever or bacterial endocarditis. area above or below the valve is narrowed (supravalvar or subvalvar).

Bicuspid Aortic Valve

Causes
improper development of the aortic genetic link, either occurring due to a defect in a gene, a chromosome abnormality, or environmental exposure, causing heart problems to occur more often in certain families. Acquired aortic stenosis may occur after a strep infection that progresses to rheumatic heart fever.

DEGENERATION AND CALCIFICATION

BICUSPID AORTIC VALVE

BICUSPID AORTIC VALVE

AORTIC VALVE BEGINS TO DEGENERATE

CALCIUM ACCUMULATES IN THE VALVE

CALCIUM ACCUMULATES IN THE VALVE

DEPOSIT INFRINGE ON THE VALVULAR OPENING

NARROWING OF AORTIC VALVE

CAUSES: UNKNOWN(CONGENITAL) DEGENERATION OR CALCIFICATION BICUSPID AORTIC VALVE RHD

NARROWING OR STRICTURE OF AORTIC VALVE

RESISTANCE TO BLOOD FLOW

DECREASED CARDIAC OUTPUT LEFT VENTRICULAR HYPERTROPHY PULMONARY VASCULAR CONAGESTION and POSSIBLE PULMONARY EDEMA

SIGNS AND SYMPTOMS

A characteristic murmur is present Infant severe defects demonstrates signs of: -decreased cardiac output - faint pulses -Hypotension -tachycardia -poor feeding(too sleepy to feed)

Children show signs of : -exercise in tolerance -chest pain -dizziness when standing long period of time

DIAGNOSIS:
Ultrasound Scan oxygen saturation monitor chest x-ray EKG (electrocardiogram) Echocardiography Cardiac catheterization

Management
Beta blocker or calcium channel blocker to reduce hypertrophy before correction

Non Surgical treatment for valvular aortic stenosis: Balloon angioplasty during cardiac catheterization to dilate narrowed valve Surgical treatment for valvular aortic atenosis: Aortic valvotomy under inflow occlusion (palliative);valve replacement may be required a second procedure Surgical treatment for Subvalvular Aortic Stenosis: may involve incising a membrane if one exists or cutting the fibromuscular ring; patch may be required

SURGICAL TREATMENT FOR SUBVALVULAR STENOSIS

In cases where the narrowing in the aorta is above the aortic valve (supravalvular stenosis), the obstructing portion is removed and the remaining parts of the vessel are stitched together

SURGICAL TREATMENT FOR SUPRAVALVULAR STENOSIS

VALVOTOMY aortic valve replacement aortic homograft pulmonary homograft

(Ross procedure)

Post Operative Care

Ventilator intravenous (IV) catheters arterial line nasogastric (NG) tube urinary catheter chest tube heart monitor

Coarctation of the Aorta

Narrowing of the descending aorta , usually just below the aortic arch causing an increase in pressure proximal to the defect and decrease in pressure distal to the defect Usually have other congenital lesions (Bicuspid Aortic Valve and VSD)

COARCTATION OF THE AORTA

Types
Preductal coarctation Ductal coarctation Postductal coarctation

Clinical Manifestations

Increased circulation above the narrowing resulting in bounding radial pulses, Headaches, dizziness, epistaxis, fainting BP on upper extremities circulation in lower extremities resulting in leg cramps Diminished or absent pedal pulses Cool lower extremities

ASSESSMENT
If the coarctation is slight: absence of palpable femoral pulses Children who have an obstruction proximal to the left subclavian artery: absent brachial pulses leg pain obvious nodules on the ribs

DIAGNOSIS:
History Physical examination Lab Studies Imaging Studies

Lab Studies:
Laboratory studies in neonatal patients who present in shock include the following: Septic workup includes blood, urine, and cerebral spinal fluid (CSF) cultures. Electrolyte levels, BUN, creatinine, and glucose concentrations should be tested. Measure arterial blood gases and serum lactate levels.

MEDICAL CARE
Early presentation Treatment in patients with congestive heart failure (CHF) includes the use of diuretics and inotropic drugs. Prostaglandin E1 (0.05-0.15 mcg/kg/min) is infused intravenously to open the ductus arteriosus. Ventilatory assistance is provided to patients with markedly increased work of breathing.

Infusion of inotropic drugs (dopamine, dobutamine, epinephrine) is useful when ventricular dysfunction is present, especially with hypotension.
A Foley catheter is inserted to monitor renal perfusion and urine output.

Arterial blood gases are tested to monitor acidosis.

-An umbilical artery catheter may be placed in neonates to assess the response to prostaglandin infusion with regard to improving lower-body blood flow. -Patients stabilized by the above interventions are better candidates for surgical or catheter intervention. -In the presence of associated defects, the significance of coarctation on the clinical course of the patient should be assessed with echoDoppler and/or catheterization and angiographic studies.

SURGICAL CARE

**balloon angioplasty

**Stents **Resection and end-to-end anastomosis of aorta **patch aortoplasty **left subclavian flap aortoplasty **tubular bypass grafts

Balloon Angioplasty

NURSING MANAGEMENT
Diet Persistent hypertension has been shown to increase the incidence of coronary artery disease (CAD); therefore, periodically examine patients who have undergone CoA repair for hypertension and recommend a healthy low-fat diet. Measure cholesterol levels and intervene pharmacologically in older patients as indicated, with a total cholesterol goal of less than 200 g/dL. Patients with persistent hypertension may require varying degrees of salt restriction. Emphasize dietary counseling and avoidance of obesity and smoking.

Activity

Patients with CoA and hypertension who are awaiting surgical repair should limit heavy isometric exercises to a degree commensurate with the degree of hypertension. Generally, the duration of hypertension after CoA repair is related in part to the duration of hypertension prior to diagnosis and repair of coarctation. Patients who undergo repair of coarctation in infancy usually remain normotensive in the absence of significant residual arch obstruction and require no specific activity restrictions or limitations. With growth, coarctation may recur, and some patients may be normotensive at rest but have significant upper extremity hypertension provoked by exercise. Such patients who desire to participate in competitive athletics should undergo exercise stress testing prior to clearance.

Patients who undergo repair later in life and who have had a significant period of preoperative hypertension are at particular risk for sustained postoperative hypertension, which may be permanent. Restrict heavy isometric exercise and other activities in these patients, commensurate with the degree of hypertension and BP control. Use exercise testing to assess BP response to exercise as a means of delineating reasonable exercise limitations.

Defect with Mixed Blood Flow

Transposition of Great Arteries
TOTAL ANOMALOUS PULMONARY VENOUS CONNECTION (TAPVC) TOTAL ANOMALOUS PULMONARY VENOUS DRAINAGE (TAPVD)

TOTAL ANOMALOUS PULMONARY VENOUS RETURN (TAPVR)

Transposition Of Great Arteries

Aorta arises from the R ventricle instead of the left and pulmonary artery arises from the L instead of the right No communication between systemic and pulmonary circulation; no oxygenation happens

Clinical Manifestation
Cyanosis after 1st cry Cardiomegaly and polycythemia- in response to hypoxemia which may cause thromboembolism leading to CVA CHF Acceptable saO2 should be 75%

Management: Palliative: Rashkind procedureor Balloon atrial-septostomy (BAS) via cardiac catheterization to increase blood mixing and to maintain cardiac output over long period Surgical correction:
Complete: Intra-atrial baffle repair to direct venous blood to mitral valve and pulmonary valve to tricuspid valve using prosthesis (mustard procedure)

Complete: Arterial switch procedure with reimplantation of coronary arteries (done during the first week of life)
Complications:
Coronary insufficiency Arrythmia Ventricular dysfunction Heart failure

Total Anomalous Pulmonary Venous Connection

Very rare defect Failure of pulmonary vein to join left atrium, instead, connected to right atrium or superior vena cava Absent spleen is often associated with this disorder

Total Anomalous Pulmonary Venous Connection

Signs and symptoms

RV hypertrophy,hypoplastic LV Mild to moderate cyanosis CHF

management
Complete: open heart with CP bypass ASD patch closure Anastomosis of pulmonary vein to LA TAPVC or TAPVR ligation
COMPLICATIONS

reobstruction Bleeding Dysrythmia heartblock

Truncus Arteriousus
Rare defect One major artery or trunk arises from the left and right ventricles in place of a separate aorta and pulmonary artery, usually accompanied by VSD

Signs and Sypmtoms CHF Cyanosis and hypoxemia Murmur Poor growth Activity intolerance

COMPLICATIONS BRAIN ABCESS BACTRIAL ENDOCARDITIS

Management
Modified Rastelli procedure:
VSD patch closure with excision of PA from aorta and attaching them to RV via homograft
COMPLICATIONS

CHF Bleeding Pulmonary artery hypertension Arrythmia Residual VSD

Hypoplastic Left Heart Syndrome

Underdeveloped left side of the heart which lacks adequate strength to pump blood into the systemic circulation, causing R ventricle to hypertrophy Increased pressure on the R side of the heart, UnO2 blood is shunted to the left side through the foramen ovale

SIGNS AND SYMPTOMS

Cyanosis until PDA closes leading to progressive deterioration and decrease in cardiac output and possibly cardiovascular collapsed.

Management
Inotropes IV. Prostaglandin E1 IV to

keep PDA open Norwood Procedure: PA anastomosis to create new aorta and creation of large VSD Bidirectional Glenn Shunt: pulmonary to systemic artery anastomosis to increase blood flow to the lung

 Modified Fontan Procedure Connects RA and PA An opening in the RA baffle is done to decrease pressure Separates oxygenated from unoxygenated blood but does not restore normal anatomy or hemodynamics

 Heart Transplant- best option

Heart transplant problem Shortage of newborn organ donor Risk of rejection Chronic immunosupression and infection

 DEFECTS WITH DECREASED PULMONARY BLOOD FLOW

 a heart defect present at birth (congenital) in which one of the valves (tricuspid valve) between two of the heart's chambers isn't formed. Instead, there's solid tissue between the chambers. If your baby is born with tricuspid atresia, blood cannot flow through the heart and into the lungs to pick up oxygen as it normally would. The result is the lungs can't supply the rest of your baby's body with the oxygen it needs. Babies with tricuspid atresia tire easily, often short of breath and have blue tinged skin

Tricuspid Atresia

SIGNS AND SYMPTOMS

Blue tinge to the skin and lips (cyanosis) Difficulty breathing (dyspnea) Tiring easily, especially during feedings Slow growth Most babies who have tricuspid atresia begin to show these signs and symptoms within the first two months of life. Some babies with tricuspid atresia may also develop signs and symptoms of congestive heart failure, including: Fatigue and weakness

 Persistent cough or wheezing with white or pink blood-tinged phlegm Swelling (edema) in the legs, ankles and feet Swelling of the abdomen (ascites) Sudden weight gain from fluid retention Decreased alertness Irregular or rapid heartbeat

Difference between normal or triscuspid valve with defect

causes
Heredity Factors Down syndrome, may increase your baby's risk of Tricuspid Atresia

The exact cause of tricuspid atresia is unknown, but several factors may increase the risk of a baby being born with this condition:

Risk
A mother who had German measles (rubella) or another viral illness during early pregnancy A parent who has a congenital heart defect Excessive alcohol consumption during pregnancy A mother who has diabetes Use of some types of medications during pregnancy, such as the acne drug isotretinoin (Accutane) and lithium (Eskalith), which is used to treat bipolar disorder Babies who have Down syndrome, a genetic condition that results from an extra 21st chromosome, also often have a congenital heart defect.

Possible complications later in life

Formation of blood clots that may lead to a clot blocking an artery in the lungs (pulmonary embolism) or to a stroke Easily tiring when participating in sports or other exercise Heart rhythm abnormalities (arrhythmias) Abnormal loss of protein from the digestive tract (protein-losing enteropathy) Infection of the heart valves (endocarditis) For this reason, your child will need lifelong care from a heart specialist (cardiologist) to monitor for complications and treat them as necessary.

 Heart rhythm abnormalities (arrhythmias) Abnormal loss of protein from the digestive tract (protein-losing enteropathy) Infection of the heart valves (endocarditis) For this reason, your child will need lifelong care from a heart specialist (cardiologist) to monitor for complications and treat them as necessary.

Diagnostic Procedure
Before birth Ultrasound technology It's possible for a baby to be diagnosed with tricuspid atresia before he or she is born. Doctors can identify the condition on a routine ultrasound exam during pregnancy.

Tetralogy of Fallot
is a congenital heart defect which is classically understood to involve four anatomical abnormalities It is the most common cyanotic heart defect, representing 55-70%, and the most common cause of blue baby syndrome. It was described in 1672 by Niels Stensen, in 1673 by Edward Sandifort, and in 1888 by the French physician tienne-Louis Arthur Fallot , for whom it is named.

"Tetralogy" - refers to four heart problems -four cardiac abnormalities characteristic of TOF

1. 2. 3. 4.

A ventricular septal defect(VSD) Pulmonary stenosis Overriding aorta Right ventricular hypertrophy

 It is the most common cyanotic heart defect, representing 55-70%, and the most common cause of blue baby syndrome. It was described in 1672 by Niels Stensen, in 1673 by Edward Sandifort, and in 1888 by the French physician tienne-Louis Arthur Fallot , for whom it is named.

Epidemiology

TOF occurs in approximately 3 to 6 per 10,000 births and represents 5-7% of congenital heart defects. Its cause is thought to be due to environmental or genetic factors or a combination. It is associated with chromosome 22 deletions and diGeorge syndrome It occurs slightly more often in males than in females. Embryology studies show that it is a result of anterior malalignment of the septum , resulting in the clinical combination of a VSD, pulmonary stenosis, and an overriding aorta. Right ventricular hypertrophy results from this combination, which causes resistance to blood flow from the right ventricle.

Pathophysiology
Decreased pulmonary blood flow
VSD and overriding aorta Mixing of oxygenated and unoxygenated blood Blood shunt from right to left ventricle and vice versa Reduces blood flow to the lungs Diluted and O2 poor blood reaches the body Low O2 saturation Tet or pink spells
Hypertrophy of the right ventricle

Pressure builds up in the right side of the heart

A Healthy Heart Cross-Section

Tetralogy of Fallot

A baby experiencing tet spell

Tet spells Infrequently, babies with tetralogy of Fallot will suddenly develop the ff. deep blue skin, nails and lips after crying, feeding or upon awakening. these episodes are called "Tet spells" and result from a rapid drop in the amount of oxygen in the blood. Toddlers or older children may instinctively squat when they are short of breath. Squatting increases blood flow to the lungs.

Signs and symptoms

A bluish coloration of the skin caused by blood low in oxygen (cyanosis) Shortness of breath and rapid breathing Loss of consciousness (fainting) Clubbing of fingers and toes (an abnormal, rounded shape of the nail bed) Lack of appetite Poor weight gain Tiring easily during play Irritability

Diagnostic tests
Echocardiography

Chest x-ray

 The classic coeur en sabot or boot-shaped cardiac silhouette is caused by the elevation of the apex due to right ventricular hypertrophy, combined with a concavity in the area of the main pulmonary artery.

Cardiac Catherization

Complete blood count

Electrocardiogram

Treatment
Specific treatment for tetralogy of Fallot will be determined by your child's physician based on: your child's age, overall health, and medical history extent of the condition your child's tolerance for specific medications, procedures, or therapies expectations for the course of the condition your opinion or preference

Medical Management
Emergency management of tet spells (acute hypoxia) Beta blockers Propanolol acute episode Morphine, Phenylephrine O2 therapy not effective Squatting in the knee chest position

Surgical Management
Palliative surgery Will redirect a large portion of the partially oxygenated blood leaving the heart for the body into the lungs, increasing flow through the pulmonary circuit, and greatly relieving symptoms in patients. The first Blalock-Thomas-Taussig shunt surgery was performed on 15-month old Eileen Saxon on November 29, 1944 with dramatic results.

 The Pott shunt Waterson procedure - are other shunt procedures which were developed for the same purpose. 2. Total Surgical Repair - first total surgical repair was performed in 1954 - This first total repair was performed by C. Walton Lillehei at the University of Minnesota in 1954 on a 10-month boy

Total Surgical Repair

The surgery generally involves making incisions into the heart muscle, relieving the right ventricular outflow tract stenosis by careful resection of muscle, and repairing the VSD using a Gore-Tex patch or a homograft. Additional reparative or reconstructive work may be done on patients as required by their particular anatomy.

 The patient must undergo pre-operative work-out in order to attain the very best surgical outcome for the patient NPO post midnight the night before the operation. Nursing Management POST-OPERATIVE CARE patient will continue to be monitored closely.(Neurologic and vital signs) the patient must be free of infection continually assess the patients comfort level

ACQUIRED PEDIATRIC HEART DISEASES

Congestive Heart Failure occurs when a cardiac output is inadequate to meet the demands of the body and results in the accumulation of excessive blood volume in the pulmonary and/ or systemic venous system.

CHF usually results from:

Congenital hearts defects Post cardiac surgery Rheumatic Fever Severe anemia Hypocalcaemia Myocarditis

 Results when the myocardium of the heart cannot pump and circulate enough blood to supply O2 and nutrients to body cells. Blood pools in the heart (excessive preload) or in the pulmonary or venous systems. Apt for children under 1 year of age.

 Congenital heart disease (primary cause of first 3 years of life) Acquired heart disease- rheumatic heart disease (caused by damage to the heart from group A strep infections), endocarditis, myocarditis Noncardiovascular causes- acidosis, pulmonary disease, various metabolic disease cardiac malformations, such as tetralogy of Fallot abnormalities of the heart valves underdevelopment of one or both ventricles coarctation of the aorta, which is a narrowing of the vessel bringing blood to the heart

Non cardiac causes:

1. Volume overload 2. HPN 3. Anemia 4. Sepsis 5. Renal Failure

SIGNS AND SYMPTOMS

Tachycardia Venous congestion Right-sided Hepatomegaly Ascites Edema Increase venous pressure Left-sided Tachypnea Dyspnea Orthopnea Cyanosis Rales/ crackles Pulmonary edema Low cardiac output Fatigue or low energy, Pallor Difficulty of feeding Sweating/ diaphoresis Poor growth Dizziness Altered consciousness Generalized edema Abrupt gain weight

General Signs & Symptoms:

Tachycardia 1st sign, attempts to beat faster, to move blood forward Apical heart beat displaced laterally and downwards Lower extremity edema late sign in children

LEFT SIDE CHF

Mitral valve stenosis Aortic stenosis Ischemic Heart Disease

Overworking of the left side of the heart

Pulmonary Congestion

Failure of the left side to contract properly

Dyspnea, wheezing, Rales/crackles, Productive cough, Salivation, cyanosis

 RIGHT SIDE CHF

Tricuspid valve stenosis Pulmonary stenosis, COPD, Pulmonary embolism, Left sided heart failure

Overworking of the right side of the heart

Venous Congestion

Failure of the right side to contract properly

Jugular vein distention Weight gain, dependent pitting edema, ascites, Hepato-splenomegaly Esophageal varices, jaundice

Rheumatic Heart Disease

A systemic inflammatory disease that affects connective tissue in the heart, joints, CNS, and subcutaneous tissue. Caused by a delayed immune response to untreated group A beta hemolytic streptococcal infection. Causes cardiac damage in 50% of all cases.

Minor Manifestations
Prolonged PR interval History of rheumatic fever Fever Elevated ESR Leukocytosis Arthralgia severe joint pain

Major Manifestations (Jones Criteria)

Carditis Erythema marginatum truncal erythematous, non-pruritic rush Subcutaneous nodules over bony prominences Chorea sudden involuntary movements Polyarthritis inflammation of more than one joint

Pathophysiology:
Congenital Heart Disorder Acquired Heart Diseases Noncardiovascular causes cardiac malformations abnormalities of the heart valves underdevelopment of one or both ventricles coarctation of the aorta ventricular septal defects patent ductus arteriosus complications of open heart surgery
ENLARGEMENT OF VENTRICLES

BLOOD CANNOT BE PUSHED FORWARD EFFECTIVELY

BLOOD CANNOT BE PUSHED FORWARD EFFECTIVELY

RIGHT VENTRICLE Back up of blood into systemic circulation Venous pressure Cardiac Output to lungs Edema of extremities & other body organs including brain, distended neck veins, flushed face, headache, shortness of breath, dyspne LEFT VENTRICLE Back up of blood to pulmonary veins High pressure in pulmonary capillaries, cardiac output to system Pulmonary congestion, rales, cough, pallor, weakness, fatigue DECREASE RENAL BLOOD FLOE

Stimulation of reninangiotensin system and aldosterone secretion

Stimulation of thirsts center and sodium retention

Tachycardia, edema

Clinical Manifestations:
Dyspnea and tachypnea Tachycardia Orthopnea Peripheral edema Feeding difficulties, anorexia Easy fatigability Restlessness Pallor or grayish tint to the skin Weight gain

Diaphoresis Growth Failure or failure to thrive, meaning that the child's growth and weight gain are slower than expected Non productive, irritative cough Neck vein distention Hepatomegaly pain and tenderness of the abdomen coolness of extremities to the touch

Pharmacological Treatment:

digoxin - a medication that helps strengthen the heart muscle, enabling it to pump more efficiently. - Side effects: loss of appetite, nausea or vomiting, headaches, irregular heartbeat or skipped heart diuretics - helps the kidneys remove excess fluid from the body. - Side effect: fatigue, decrease blood pressure, kidney complications, excessive loss of potassium

potassium-sparing diuretics - helps the body retain potassium, an important mineral that is often lost when taking diuretics. -Side Effect: On their own this group of drugs may raise potassium levels beyond the normal range, termed hyperkalemia, which risks potentially fatal arrhythmias. potassium supplements - replaces the potassium lost when taking diuretics.

ACE (angiotensin-converting enzyme) inhibitors - dilates the blood vessels, making it easier for the heart to pump blood forward into the body. -Dizziness, headache, diarrhea, constipation, loss of appetite, nausea, loss of taste, flushing, fatigue, cough or increased urination may occur beta blockers - decrease the heart rate and blood pressure, and improve heart function by blocking the stress hormone adrenalin. - You may experience dizziness, lightheadedness, drowsiness, and blurred vision as your body adjusts to the medication.

SURGICAL MANAGEMENT:

ARTIFICIAL PACEMAKER -used for a small battery-operated device that helps the heart beat in a regular rhythm. Some are permanent (internal) and some are temporary (external). An artificial pacemaker can replace a defective natural pacemaker or blocked pathway.

CARDIAC ABLATION

- In cardiac ablation, a form of energy renders a small section of damaged tissue inactive. This puts an end to arrhythmias that originated at the problematic site.

Left ventricular assist devices, or LVADs

-used to mechanically pump blood through the hearts of individuals with heart failure as they await transplantation, can reverse reduced heart muscle performance.
Extracorporeal membrane oxygenation (ECMO) -is an extracorporeal technique of providing both cardiac and respiratory support oxygen to patients whose heart and lungs are so severely diseased that they can no longer serve their function.

Heart transplantation or cardiac transplantation -is a surgical transplant procedure performed on patients with end-stage heart failure or severe coronary artery disease -is an open-heart surgery in which a severely diseased or damaged heart is replaced with a healthy heart from a recently deceased person

NURSING MANAGEMENT:

Improved myocardial efficiency

Administer Digoxin as prescribed by the physician Carefully calculate dosage; it is given in very small amount in children and infant Count apical pulse in 1 full minute before administering Observe for vomiting and report to the physician Observe for the development of premature ventricular contraction when digoxin is initially started and report to the physician Be aware of signs of digitalis intoxication altered emotional status digitalis blues decreased appetite Bradycardia Arrythmias gastrointestinal symptoms

 Reduce energy requirements Avoid necessary activities such as frequent complete baths and clothing changes Prevent excessive crying Use pacifier Hold baby Eliminate sources of distress (hunger, wet diapers, etc.) Remove accumulated sodium and fluid Administer diuretics as prescribed by the physician.

 Hypokalemia may cause weakened myocardial contractions and may precipitate digoxin toxicity. Oral potassium supplements may be indicated when a child is on diuretics for an extended period of time. Restrict sodium intake The child may be placed on a low-sodium diet. Be aware of the prescribed diet and the amount of sodium in foods and fluids offered to the child. Question the child about his likes and dislikes so that the diet can be made as appealing as possible. Interpret the diet and its purpose to the child and his parents. Infants may require low-sodium formulas.

Be aware of the sidE effects of the prescribed medication. Weigh the child at least daily to observe response. Maintain an accurate record of intake and output. Record urine specific gravity. Encourage foods such as bananas and orange juice that have a high potassium content to prevent potassium depletion associated with many diuretics.

 Relieve the respiratory distress associated with pulmonary engorgement Improve tissue oxygenation Administer oxygen therapy Maintain the infant in a neutral thermal environment. Provide adequate nutrition to meet the caloric requirements of the child Provide foods that the child enjoys in small amount, because he may have a poor appetite due to liver enlargement. Infant feeding Feed frequently in small amounts. Feed slowly in sitting position, allowing frequent rest periods. Supplement oral feedings with gavage feeding if the infant is unable to take an adequate amount of formula by mouth.

Kawasaki Disease
Mucocutaneous lymph node syndrome Acute febrile illness of unknown cause which may result in obstruction, stenosis or aneurysm formation of the arteries. Common in children of Asian decent. Vasculitis is the most life threatening complication

KAWASAKI's disease-also known as motorcycle syndrome, lymph node syndrome, mucocutaneous node disease, infantile polyarteritis and Kawasaki syndrome, is a poorly understood self-limited vasculitis that affects many organs, including the skin and mucous membranes, lymph nodes, blood vessel walls, and the heart.

Clinical Manifestations
Fever lasting longer than 5 days & does no subsides w/ antipyretics Bilateral conjunctival infection Strawberry tongue Rash Lymphadenopathy Peeling of skin soles, palms Edema, irritability, joint pain

Other CAUSES:
complications of open heart surgery chronic anemia, which results in a low red blood cell count poor nutrition drug toxicity

Endocarditis
Inflammation and infection of the endocardium or valves of the heart. Common complication of congenital heart diseases (TOF, VSD, COA) Caused by streptococcal infections that invade the body at the time of oral surgery, urinary infection, or skin infection (impetigo)

Management
Prophylactic antibiotic for children w/ CHD Antibiotic and therapy for underlying infection Long time follow up care to prevent recurrence

Cardiac Surgery
Surgery on the heart and or the blood vessels

Closed Heart Surgery

Cardiac catheterization

Open Heart surgery

Operation under hypothermia

2. Temperature and notify the physician if a fever occurs.

 Maintain aseptic technique Monitor for sign of sepsis Fever chills Diaporesis lethargy Altered level of consciousness

Monitors lines, tubes, or catheters that are in place and remove promptly as prescribed when no longer needed, to prevent infection. Assess for signs of discomfort: Irritability Change in heart rate, respiratory rate, BP Inability to sleep

 Administer pain medication as prescribed noting effectiveness Administered antibiotic and antipyretic as prescribed. Encouraged rest period. Facilitate parent child contact as soon as possible.

 RESPIRATORY DISORDER

TRACT