Stephanie Talbot

Most common cause of death in yr life w/ ventricular septal defects being most common Associated chromosomal abnormalities Exact cause unknown, associated maternal risk factors: alcohol consumption, exposure environmental toxins & infection, Phenylketonuria, diabetes mellitus & family hx congenital heart defects Consequence defects = CHF & hypoxia

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EO1.1: Discuss the classification of congenital heart dx based on the hemodynamic classification system

EO1.1: Discuss the classification of congenital heart dx based on the hemodynamic classification system
Increased pulmonary Flow Obstructed blood flow

Decreased pulmonary Flow

EO1.1: Discuss the classification of congenital heart dx based on the hemodynamic classification system

Mixed blood flow 1. Transposition of the great arteries 2.Truncus arteriosus 3.Total anomalous pulmonary venous return 4.Hypoplastic left heart syndrome

EO-1.2:Describe embryonic heart development,

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EO-1.2:Describe embryonic heart development

EO-1.2:Describe embryonic heart development

Blood & nutrients enters via umbilical vein liver, rest goes to inferior vena cava via Ductus arteriosus RA from RA, Blood shunts through the foramen ovale into the LA (due to higher pressures in RA) LV and pumped through the aorta to the head & upper extremities Blood from head & upper extremities entering RA RV pulmonary artery, where major portion shunted through the Ductus arteriosus into the descending aorta (sm. Amts blood flows to lings)back to placenta

 Before birth pressures > R. heart due to the collapsed pulmonary system, result=low systemic vascular pressure

expansion of lungs (on that first breath) inspired O2 dilating pulmonary vessels ( blood begins flow to lungs) decrease in pressure on R. heart & pulmonary arteries. At same time, clamping cord causes increase in blood volume &systemic pressure rises increase pressure L. heart change of pressures causes flow through fetal shunts to reverse. Closure due to increased O2 in blood & decreased prostaglandins & acidosis Foramen ovale closes soon after birth, the ductus arteriosus by day 4

EO-1.3:Describe common diagnostic procedures used to confirm congenital heart anomalies & associated nsg responsibilities
Basis s/s:

tachypnea & tachycardia @ rest dyspnea, retractions HTN activity intolerance- during feeding weight gain due to NSG: fluid retention hepatomegaly

Chest x-ray

EO-1.3:Describe common diagnostic procedures used to confirm congenital heart anomalies & associated nsg responsibilities
EchocardiogramECG-

EO-1.3:Describe common diagnostic procedures used to confirm congenital heart anomalies & associated nsg responsibilities

Cardiac catheterization -O2sat in chambers & great vessels, pressure changes, C.O or stroke volume, anatomic abnormalities NSG:

Pre height & weight, allergies, mark pulses, SaO2, NPO (morning dose meds held) Post O2sat and cardiac monitoring: pulses, temp & colour, VS, bleeding @ site, I/O, accucheck (give infants dextrose containing IV)

EO-1.4:Describe problems w/ Increased Pulmonary Blood Flow Cardiac Defects Characterized by: Intracardiac communication between septum Abnormal connection between great vessels Blood shunts from high pressure of L. side heart low pressure of R. side increase in volume on R. side & thus increases pulmonary blood flow
Sign & Symptoms: 1. Poor feeding, anorexia 2. Growth failure, poor weight gain 3. Tachypnea, tachycardia, dyspnea, orthopnea, grunting, nasal flaring, retractions 4. CHF, murmur 5. Activity intoleranceexcessive sweating when feeding

EO-1.4:Describe problems w/ Increased Pulmonary Blood Flow Cardiac Defects

Atrial Septal Defect Abnormal opening between atria increased flow oxygenated blood right side of atrium r. atrial enlargement s/s: asymptomatic, @ risk dysrhythmias, obstructive pulmonary dx, emboli formation later life Tx: surgical patch closure, closure w/ deviceamplatzer septal occluder, low dose aspirin 6mths

EO-1.4:Describe problems w/ Increased Pulmonary Blood Flow Cardiac Defects

blood flows aorta pulmonary artery (L-R Patent Ductus Arteriosus shunt)increased vol & pressure R. ventricle hypertrophy & pulmonary congestion increased work L. heart. s/s: asymptomatic, widened pulse pressure, @ risk endocarditis & obstructive pulmonary vascular dx
Tx: administer indomethicin (prostaglandin inhibitor) surgical division or ligation via thoracotomy coils to occlude PDA in cath lab

EO-1.4:Describe problems w/ Increased Pulmonary Blood Flow Cardiac Defects

Atrial Septal Defect
increased flow oxygenated blood right side of atrium r. atrial enlargement s/s: asymptomatic, @ risk atrial dysrhythmias, obstructive pulmonary dx, emboli formation later life Tx: surgical patch closure, open repair w/ bypass before school aged closure w/ deviceamplatzer septal occluder, low dose aspirin 6mths

EO-1.4:Describe problems w/ Increased Pulmonary Blood Flow Cardiac Defects

Ventricular Septal Defect
blood flows from L. to R. ventricle (L-R shunt)increased volume to R. ventricle (hypertrophy) & lungs which increases pulmonary vascular resistance Assoc. other defects s/s: @ risk bacterial endocarditis & pulmonary vascular dx Tx: Pulmonary artery banding Suture, knitted Dacron patch sewn over opening Device closure in cath lab

EO-1.4:Describe problems w/ Increased Pulmonary Blood Flow Cardiac Defects

NSG Interventions: 1. Prepare child & family for diagnostic studies 2. Administer meds HF 3. Ensure adequate nutrition
Prevent crying to decrease O2 demands, feed semiupright, soft nipple decrease fatigue, gavage feeding may be necessary, burp frequently, monitor growth

4. Monitor VS 5. Provide rest

Age approp play, cluster care

6. Position semi upright 7. Prevent infection 8. Meet age developmental needs 9. Prophylaxis antibiotics for endocarditis for unrepaired ASD, VSD, PDA.

Characterized by:

 Narrowing of aorta increased blood flow to head & upper extremities & Coarctation of aorta decreased blood flow to lower parts body s/s: 1. Increased BP & bounding pulses of arms > legs 2. Lower body cooler than upper 3. In children epistaxis, dizziness, headaches, fainting Tx: Surgical repair by 2yrs- removal narrowed portion w/ anastamosis & graft.

Post op HTN tx IV sodium nitroprusside, esmolol or milrinone followed by angio-tension converting enzyme inhibitors or blockers

Aortic Stenosis

Narrowed aortic valve resistance blood flow in L. ventricle, decreased C.O 7 L. Ventricular hypertrophy s/s: 1. Decreased C.O 2. Faint pulses 3. Hypotension 4. Tachycardia 5. Poor feeding 6. Aortic murmur Tx: 1. Balloon angiography in cath lab. If critical stage 1 Norwood procedure 2. Aortic valvotomy Complications: aortic insufficiency or valve regurgitation, tearing valve leaflets, loss pulse in catheterized limb

Pulmonic Stenosis

Narrowed pulmonic valve R. ventricular hypertrophy & decreased pulmonary blood flow if R. ventricular failure increased R. atrial pressure can cause re-opening of foramen ovale s/s: asymptomatic, cyanosis or CHF, systolic thrill, murmur, cardiomegaly. @ risk bacterial endocarditis Tx:In infants: transventricular (closed) valvotomy (Brock procedure) In children: pulmonary valvotomy w/ bypass Also balloon angioplasty to dilate valve

EO 1.5: NSG Interventions for problems with Obstructive Blood Flow Defects
1. Monitor VS- esp. BP for hypotension 2. Monitor for heart failure 3. Monitor for cyanosis & hypoxemia 4. Prepare child & family for diagnostic procedures 5. Ensure adequate nutrition

6. Administer meds 7. Administer prophylactic antibiotics for bacterial endocarditis 8. Meet age approp. Developmental needs 9. Teach care to parents

Characterized by: an obstruction and an anatomical defect (atrial & ventricular septal most common) Pressure on the R. heart exceeds L desaturated blood from R. heart shunting to the L. heart & systemic system hypoxia & cyanosis

Signs & Symptoms:

1. Low SaO2, Cyanosis 2. Polycythemia, Clubbing digits 3. Poor feeding, anorexia, fatigue, activity intolerance, growth failure, weak cry 4. Squatting increases systemic vascular resistance 7 improves pulmonary blood flow 5. Hypercyanotic (tet spells0 6. Tachycardia, tacypnea, dyspnea 7. Heart murmur

Tetrology of Fallot

4 defects: VSD, overriding aorta, pulmonary stenosis & R. ventricular hypertrophy Blood shunts either R-L or L-R depending on pressures pulmonary stenosis decreased blood flow to lungs R. shifted aorta over VSD causing blood from both R/L ventricles to flow into aorta Mixed blood then flows to body

Signs & Symptoms

Therapeutic Management

Anoxic spells during crying or after feedings @ risk for emboli, seizures, loss consciousness or sudden death following anoxic spell

1. Surgery: Pallative- BlalockTaussig shunt, provides blood flow to pulmonary artery fro L/R subclavian via tube graft Closure VSD & resection of infundibular stenosis w/ placement pericardial patch to enlarge R. ventricular outflow tract

NSG Interventions:

6. Admin. Meds 1. Observe for 7. Admin antibiotics squatting prophylaxis 2. Cluster care 8. For hypercyanotic spells, place knee3. Admin O2 chest position, 4. Prevent crying & admin O2, admin anticipate needs morphine 9. Meet age approp. 5. Prepare child & developmental family for needs diagnostic tests 10.Teach care to parents

Characterized by

1. Cyanosis & hypoxemia Fully saturated 2. Tachycardia, dyspnea, systemic blood tachypnea mixes w/ 3. Cardiac murmur, HF desaturated 4. Poor feeding, growth pulmonary blood failure, activity overall deintolerance, weak cry, saturation lethargy Pulmonary system 5. Polycythemia becomes congested 6. Clubbing of digits & C.O decreases 7. Risk for bacterial endocarditis, emboli, stoke

Signs & Symptoms

 Pulmonary artery leaves L. ventricle & Transposition of Great aorta leaves R. ventricle Arteries No communication between systemic & pulmonary circulation Septal & ductal defects allow mixing of blood so that some desaturated blood makes it to the lungs Most common defect is patent foramen ovale s/s: cyanosis, those w/ septal defects less cyanosis and CHF & cardiomegaly Tx: Goal=intracardiac mixing

Admin prostaglandins to ductus arteriosus open temporaril71 y Arterial & coronary artery switch switch procedure 1wk life Potential complication= narrowing @ anastomosis site

EO 1.7: NSG Interventions Problems with Mixed Blood Flow Defects

1. Prepare child & family for diagnostic procedures 2. Assess VS assess poor cardiac fxn 3. Monitor infants receiving prostaglandin E for apnea, hypotension, hypothermia 4. Cluster care 5. Admin O2 6. Prevent crying & anticipate needs 7. Ensure adequate nutrition 8. Admin antibiotics prophylactically 9. Meet age approp. Developmental needs 10.Teach care to parents

EO 1.9/10: NSG DX & Goals for children w/ alterations cardiac fxn r/t congenital heart anomalies
NSG DX Activity intolerance r/t fatigue Ineffective bx pattern decreased cardiac output Delayed growth & development r/t inadequate oxygenation Imbalanced nutrition: Less than body requirements Risk for deficient fluid volume Goals/outcomes

1. Conserve energy 2. Adequate ventilation 3. Adequate tissue perfusion/oxygenat ion 4. Normal growth & development milestones 5. Adequate nutrition 6. Maintain fluid balance

EO 1.11: Describe Jones Criteria used dx Rheumatic Fever & associate NSG responsibilities
Inflammatory disorder occurs 2-6wks after Pharyngitis inflammatory hemorrhagic bullous lesions called Aschoff bodies that cause swelling, fragmentation, & alterations connective tissue group A beta hemolytic streptococci causes infection Self limiting Affects joints, skin, brain & serous surfaces Cardiac valve damage most common complication

EO 1.11: Describe Jones Criteria used dx Rheumatic Fever

Major Manifestations

1. Carditis 2.Polyarthritis
3. Erythema marginatum 4. subQ nodules 5. Chorea - also called St. Vitus dance or Sydenham chorea

1. 2. 3. 4.

Clinical findings Arthralgia Fever Lab findings: elevated values for acute-phase reactants Erythrocyte sedimentation rate & c-reactive protein level Supporting Evidence of Antecedent +ive throat culture or rapid streptococcal antigen test Elevated/rising streptococcal antibody titre

Minor Manifestations

EO 1.11: Describe Therapeutic Management used dx Rheumatic Fever

Goal/Management:

1. Eradication group A hemolytic streptococci (primary prevention) 2. Prevention permanent cardiac damage
Penicillin (oral or IM) 10dys Bed rest Prednisone may be indicated for some w/ heart failure Tx CHF. Surgery for valve repair @ risk atrial fibrillation & emboli

3. Palliation symptoms

4. Prevention recurrences- children susceptible recurrence for rest life

Salicylates to control inflammation joints, reduce fever & discomfort

Seconday prevention monthly IM, 2 daily oral doses penicillin V, or 1 daily dos sufadiazine-sufisoxazole benzathine penicillin G for 5-10yrs+

EO 1.11: Describe NSG Interventions used dx Rheumatic Fever

1. Carditis
Admin penicillin Prophylaxis taken till age 20 or for 5yrs after attack, whichever first Bed rest
Admin aspirin Change position q2 Decrease stimulation, safe environment-no forks, assist w/ mobility, sm. Frequent meals

2. Arthritis

3. Chorea

4. Nodules & rash: nothing

5. Alleviate anxiety 6. Prevent recurrent infection 7. Minimize boredom 8. Provide client teaching Stress to parents & teachers that the sudden & involuntary movements of chorea are transitory & will disappear.

EO 1.16: Describe Etiology/Pathophysiology Kawasaki Disease

Etiology=unknown Widespread inflammation sm. Then the medium blood vessels Effected vessels enlarge for weeks Myocarditis in acute phase Affected vessels try heal by producing more cells in vessels (myointimal proliferation)vessel never same, thicker & subject scarring & calcification

Almost all morbidity & mortality result cardiac complications Coronary thrombosis sluggish blood flow in dilated vessel Stenosis & scarring impeded blood flow which predisposes pt to myocardial ischemia

EO 1.16: Describe Diagnostic Evaluation of Kawasaki Disease

No specific test Based s/s Lab findings:
Anemia Leukocytosis w/ shift left (immature leukocytes) Thrombocytosis w/ hypercoagulability Elevated ESR ^ C-reactive protein Transient elevation liver enzymes Decreased albumin Microscopic urinalysis reveals pyuria w/ mononuclear cells Cerebral spinal fluid analysisaseptic meningitis

EO 1.16: Describe Clinical Manifestations of Kawasaki Disease

4.Edematous hands Acute Phase & feet w/ 1. Abrupt onset fever erythematous unresponsive to palms & soles antibiotic & 5.Temporary arthritis antipyretics 6.Myocarditis w/ 2. Bulbar conjunctivae ECG changesof eyes reddened decreased LV fxn, pericardial effusion, 3. Inflammation & mitral pharynx & oral regurgitation mucosa, red, 7.Tachycardia w/ cracked lips, gallop loop STRAWBERRY TONGUE

EO 1.16: Describe Clinical Manifestations of Kawasaki Disease

Subacute Phase- lasts till outward s/s disappear

Convalescent

All s/s have resolved, but labs not normal: ESR & C-reactive protein elevated Thrombocytosis may still be present Arthritis cont Coronary complications may remain

EO 1.16: Describe Complications of Kawasaki Disease