Approach to child with heart disease

1. Approach to child with heart disease Pushpa Raj Sharma Professor of Child Health Institute of Medicine

2. Diseases of heart Blood vessels Endocardium Myocardium Pericardium

3. Congenital Cyanotic: 22% Acyanotic: 68% VSD 25% ASD 6% PDA 6% TOF 5% PS 5% AS 5% Acquired Kawasaki disease Rheumatic Tubercular Collagen Prevalence Ceylon Med J 2001 Sep; 46 (3): 96-8; Indian J Pediatr. 2001 Aug;68 (8):757-7 Nelson’s Textbook of pediatrics; 17 ed.

4. Common acyanotic lesions • Ventricular septal defects • Atrial septal defects • Atrio-ventricular septal defects • Patent ductus arteriosus • Truncus arteriosus • Pulmonary stenosis • Aortic stenosis • Mitral stenosis/incompetence • Coarctation of aorta • Tricuspid regurgitation

5. Common Cyanotic Lesions Decreased flow 1. Tetralogy of Fallot 2. Tricuspid Atresia 3. Severe Pulmonic Stenosis 4. Ebstein’s anamoly Increased Flow 5. Transposition of great vessles 6. VSD with pulmonary atresia

6. Common Lesions producing cyanosis 7. Truncus Arteriosus 8. Hypoplastic left heart 9. Single ventricle 10. TAPVR with infradiaphragmatic obstruction

7. Failure to thrive Exercise intolerence Easy fatigability Chest indrawing Sweating during feeding Bluish spells Fever with rigor Palpitation Convulsion Fast breathing Oedema Hepatomegaly, spleenomegaly Clubbing Cyanosis Focal neurological lesion Other organ defects Chromosomal anomalies Presenting complaints/signs

8. Cyanosis: is it a cardiac cause or lung cause • Hyperoxia test • Neonates with cyanotic congenital heart disease usually do not have significantly raised arterial Pao2 during administration of 100% oxygen.

9. Small VSD Asymptomatic A loud, harsh, or blowing holosystolic murmur. Large VSD dyspnea, feeding difficulties, poor growth, profuse perspiration, recurrent pulmonary infections, and cardiac failure in early infancy. Ventricular Defect 80% Syndromes associated with this condition

10. VSD: ECG is normal but may show right ventricular hypertrophy, if present indicates defect is large and presence of pulmonary hypertension or pulmonry stenosis

11. Ventricular Septal Defect (VSD) Small VSDs, the chest radiograph is usually normal Large VSD: The presence of right ventricular hypertrophy, olegeimic lung fields (pulmonary hypertension or an associated pulmonic stenosis), gross cardiomegaly with prominence of both ventricles, the left atrium.

12. Ventricular Septal defects • 30–50% of small defects close spontaneously, most frequently during the 1st 2 yr of life. • Small muscular VSDs are more likely to close (up to 80%) than membranous VSDs are (up to 35%). • infants with large defects have repeated episodes of respiratory infection and heart failure despite optimal medical management. • Surgical repair prior to development of an irreversible increase in pulmonary vasculalr resistance (usually prior to the patient's second birthday).

13. Most common form of ASD (fossa ovalis) In large defects, a considerable shunt of oxygenated blood flows from the left to the right atrium. Mostly asymptomatic The 2nd heart sound is characteristically widely split and fixed. Atrial Septal Defects: secundum Secundum

14. Situated in the lower portion of the atrial septum and overlies the mitral and tricuspid valves. In most instances, a cleft in the anterior leaflet of the mitral valve is also noted. Combination of a left-to-right shunt across the atrial defect and mitral insufficiency C/F similar to that of an ostium secundum ASD Atrial Septal Defects:primum

15. Enlargement of the right ventricle Enlargement of atrium Large pulmonary artery increased pulmonary vascularity is. Atrial Septal Defect

16. The electrocardiogram in patients with a complete AV septal defect is distinctive. The principal abnormalities are (1) superior orientation of the mean frontal QRS axis with left axis deviation to the left upper or right upper quadrant, (2) counterclockwise inscription of the superiorly oriented QRS vector loop, (3) signs of biventricular hypertrophy or isolated right ventricular hypertrophy, (4) right ventricular conduction delay (RSR′ pattern in leads V3 R and V1 ), (5) normal or tall P waves, and (6) occasional prolongation of the P-R interval

17. Atrial Septal Defects • Secundum ASDs are well tolerated during childhood. • Antibiotic prophylaxis for isolated secundum ASDs is not recommended. • Surgery or transcatheter device closure is advised for all symptomatic patients and also for asymptomatic patients with a Qp:Qs ratio of at least 2:1. • Ostium primum defects are approached surgically

18. Patent Ductus Arteriosus • Small defect no symptoms. • Large defect: • Wide pulse pressure • Enlarged heart • Thrill in L second IS • Continuous murmur • X-ray: prominent pulmonary artery with increased vascular markings.

19. Prominent pulmonary artery. Prominent right ventricle Prominent vascularity in the hilar areas Decreased vascualr marking in the periphery. No treatment Primary Pulmonary Hypertension

20. Mitral insufficiency: Rheumatic High volume load Inflammatory process Enlarged left ventricles Dilatation of the left atrium Pulmonary congestion Symptoms of left sided failure Spontaneous improvement Repeated insult Chronic mitral insufficiency Raised Pulmonary AP Symptoms of right heart failure Enlarged right ventricle and atrium

21. Signs of heart failure Heaving apical impulse Apical systolic thrill Accentuated 2nd sound Holosystolic murmur radiating to axilla ECG: bifid P waves and left ventricular hyertrophy X-ray: prominent left atrium and ventricle (straight left border) Mitral insufficiency: Rheumatic Prophylaxis against recurrence of rheumatic fever

22. Rheumatic valvular disease: Mitral stenosis • Takes 10 years to develop • Symptoms proportionate to severity • Left ventricular failure right ventricular failure • Loud first heart sound with opening snap. • Diastolic murmur • Absent murmur if heart failure. • Surgical intervention if symptomatic

23. Loud 1st sound Diastolic murmur left atrial enlargement prominence of the pulmonary artery enlarged right-sided heart chambers; ECG: prominent notched P wave. Mitral Stenosis

24. Presenting complaint Precordial pain Cough Dyspnoea Abdominal pain Vomiting Fever Other organs involvement Signs: Position: leaning forward. Puffy face Friction rub Absent apical impulse Muffled heart sounds Pulsus paradoxus Distended neck veins Low QRS complex, T inversion Pericardial Effusion

25. A relatively large pericardial effusion must be present to cause an enlarged cardiac shadow with the usual “water bottle” configuration on a chest roentgenogram Pericardial Effusion

26. The test that differentiates The cardiac seize and the vascularity in the chest X-ray

27. Cardiac disease with normal/decreased vasculature • Viral myocarditis • Tetralogy of Fallot • Pulmonary atresia • Tricuspid atresia • Endocardial fibroelastosis • Aberrant left coronary artery • Cystic medial necrosis • Diabetic mother

28. Tetralogy of Fallot Ventricular septal defect Pulmonic stenosis Overriding aorta Right ventricular hypertrophy Cyanotic

29. Cardiac disease with increased vasculature • Atrioventricular septal defects • Congestive cardiac failure • Transposition of great arteries with VSD • Total anomalous pulmonary venous drainage • Truncus arteriosus • Single ventricle without pulmonary stenosis • Hypoplastic left heart syndrome

30. Enlarged heart Plethoric lung fields specially at bases Congestive Cardiac Failure