Pediatric Heart Disease

1. Pediatric Heart Disease

2. Infants may have one of the following: • Cyanosis (cyanotic CHD or R to L shunt) • Pulmonary and tricuspid atresia • CHF (L to R shunt) or shock • Shock from outflow obstruction • Diagnostic evaluation • CXR • EKG

3. Cyanotic Heart Defects • Tetralogy of Fallot • Transposition of the great vessels • Truncus arteriosus • Total anomalous pulmonary venous return • Tricuspid valve Abnormalities • Severe pulmonic stenosis

4. Tetralogy of Fallot • Consists of VSD, obstructed right vent. outflow tract, dextroposed overriding aorta, RVH. • TeT spells-worsening obstruction of flow in the pulmonary artery leading to greater R to L shunt. Causes cyanosis and dyspnea.

5. Transposition of Great Vessels • Appears in 1st week of life. • Aorta comes from R vent. and pulmonary art. Comes from L vent. • Must have a VSD or ASD for survival • If suspected, start Prostaglandin E1

6. Truncus Arteriosus • Large arterial trunk from the ventricular portion of the heart. • Supplies blood to systemic and pulmonary circulation. • Present with increased pulm. blood flow, dyspnea and CHF

7. Coarctation of the Aorta • Narrowing of the aortic lumen. • Present with CHF and feeding difficulty. • Decreased pulse amplitude in the lower ext. • Hypertension in the upper ext. • Older kids present with exercise intolerance and rib notching.

8. Ductal Dependent heart defects • Depend on a patent ductus arteriosus. • Systemic blood flow depends on a R to L shunt from the pulmonary artery through the DA to aorta. • Pulmonic blood flow depends on a L to R shunt from the aorta through the DA to the pulmonary art.

9. Defects dependent on R to L shunt via the PDA include: • Critical Aortic Stenosis • Hypoplastic left heart • Severe coarctation • Usual Clinical Presentation • Circulatory collapse at the end of first week of life

10. Treatment • Prostaglandin infusion keeps the DA open until surgery. • Start at 0.1 mcg/kg/min and titrate

11. CHF • Usually w/in the first 6 months when PVR has decreased allowing L to R shunt (VSD or PDA) • Triad of CHF in infancy • Tachypnea • Tachycardia • Hepatosplenomegaly

12. Treatment • Supportive • Lasik • Digoxin except in IHSS or TOF • Inotropes • Pressors • Vasodilators • Afterload reducing drugs • Head up • NPO • Treat infections

13. Atrial septal defects • RA and RV enlargement • Pulmonary over-circulation • High pressure • Low volume • ASD low morbidity and mortality • Repair may be surgical or trans cath

14. Most common CHD May occur in any septal location Hemodynamic significance depends on the size of the defect. Spontaneous closure in the first 6 mo. In 30-40%. Surgical repair required if: Infant has failure to thrive Pulmonary HTN R to L shunt Vent. Septal Defect

15. Aortic Stenosis • May have a mono, bi or tricuspid valve. • If severe, avoid prosthetic valve until they are old enough to receive an adult sized one.

16. In critical AS, LV unable to pump adequate flow past the aortic valve. • If no PDA, not compatible with life

17. Kawasaki’s Disease • Acute self limited multisystem vasculitis • Clinical presentation • Fever 5 or more days with 4 of 5 other clinical features • Bilat. conjunctival inection w/o exudates • Mucous membranes changes of upper resp. tract Erythema and edema of hands/feet (early) and desquamation (subacute phase) • Exantham polymorphous, truncal • Acute cervical lymphadenopathy

18. Lab findings include high WBC, left shift, hemolytic anemia, high platelets, high CRP/ESR, pyuria, bilirubinuria • On CXR infiltrates, cardiomegaly, long PR or QT, dysrhythmias • Cardiac echocardiography show coronary aneurysms 80-90% of time

19. Treatment • Hospitalization for IVIG • ASA • Cardiac eval. • Corticosteroids may decrease coronary aneurysms

20. Myocarditis • Causes • Idiopathic • Inflammatory • Acute rheumatic fever • Collagen vascular disease • Lyme disease • Toxins • HIV • Viruses • Adenovirus • Coxsackie A and B

21. Often misdiagnosed • Classic presentation • CHF or fulminant cardiogenic shock • Syncope from dysrhythmias May present with cough, wheeze/tachypnea, congestion, or fever. Consider in a child with wheezing and no history of asthma or in a febrile child whose wheezing is not responding to treatment.

22. Diagnostic eval • CXR-cardiomegaly • EKG changes • Elevated troponin • Definitive diagnosis-biopsy or MRI

23. Treatment • Bedrest • Oxygen • Inotropes • May need transplant 35% mortality

24. SVT • Most common dysrhythmia • Present with tachycardia, poor feeding, tachypnea, pallor, lethargy, chest pain. • HR usually >230 BPM • Unstable sync. Cardiovert 0.5 j/kg. Lidocaine 1 mg/kg should be given prior d/t risk of V fib. • Adenosine 0.1 mg/kg the 0.3 mg/kg

25. A fib Usually d/t rheumatic heart disease or dilated cardiomyopathy. Unstable-cardiovert 0.5 J/kg Medical therapy (dig) is usually not effective Anticoagulation is not necessary

26. V Tach • Usually seen with congenital heart disease, but also with myocarditis, cardiomyopathy, or prolonged QT. • Unstable sync. Cardiovert 1-2 J/kg • Start Lidocaine or Procainamide if pt not stable for transfer

27. V Fib • Initial energy for defib is 2 J/kg. Can be doubled. • A trial of amiodarone 5mg/kg infusion followed by defib. • If successful, Amiodarone infusion 5-15 micrograms/kg/min. • After 3 defib attempts and a second 2 mg/kg bolus infusion usually indicates futility.