• • • • TYPE Conoventricular VSD Perimembranous VSD Inlet VSD Muscular VSD VSD SMALL VSD COMPLICATION • Aortic regurgitation • IE • Eisenmenger’s syndrome • Pulmonary hypertension A L to R shunt at the ventricular level has 3 hemodynamic consequences: 1. increase volume RV→increase pulmonary flow→pulmonary hypertension→increase RV pressure→R to L shunt→ increase LV volume load→ LV dilatation and then hypertrophy→ increase pulmonary venous pressure→raises pulmonary capillary pressure→increase pulmonary interstitial fluid→pulmonary edema 2. excessive pulmonary blood flow 3. reduced systemic cardiac output • • • Asymptomatic. Loud pansystolic murmur at LLSE Quiet pulmonary second sound (P2). LARGE VSD • • Heart failure with breathlessness and FTT after 1 week old Recurrent chest infections. Tachypnoea, tachycardia and enlarged liver from heart failure Active precordium Soft pansystolic murmur or no murmur Apical mid-diastolic murmur (from increased flow across the mitral valve after the blood has circulated through the lungs) Loud pulmonary second sound (P2) • • • • • • Normal CXR • • • • Cardiomegaly Enlarged pulmonary arteries Increased pulmonary vascular markings Pulmonary oedema • Normal ECG • Biventricular hypertrophy by 2 months of age. • Close spontaneousely • • - Diuretics, captopril, calories Surgery at 3–6 months old Cardiac cathetherization Pulmonary artery banding Atrial Septal Defect (ASD) Accounts for 10% of all congenital defects. Females > males (2:1) Left to right shunt occurs between two low-pressure heart chambers, majority of cases are asymptomatic during childhood. usually suspected because of incidental heart murmur. Types: Ostium secundum defect (most common) Ostium primum defect – part of AVSD spectrum. SMALL ASD Asymptomatic Normal CXR Normal ECG Partial right bundle branch block, RV hypertrophy pattern. Do not need specific treatment May close spontaneously during early childhood + right heart dilatation, require elective closure. Secundum defects can be closed by percutaneous interventional technique. Defects other than secundum and very large secundum defects need to closed surgically. Closure of ASD is contraindicated if there is significant pulmonary hypertension. LARGE ASD Parasternal heave, wide and fixed splitting of the S2, ejection systolic murmur. Mild cardiomegaly, increased pulmonary vascular marking Patent Ductus Arteriosus (PDA) Diagnosis Risk factors for delayed closure of the PDA: • • • Hypoxia, Acidosis, Immaturity • • • Echocardiographic visualization of a PDA with Doppler flow imaging that demonstrates left-to-right or bidirectional shunting. ↑ pulmonary pressure 2nd to vasoconstriction Systemic hypotension Local release of prostaglandins Clinical Features Small PDA Large PDA • Asymptomatic • Heart failure symptoms • Normal peripheral • Retardation of physical growth pulses • Bounding peripheral arterial pulses • Wide pulse pressure • Enlarged heart • Continuous murmur, thrill at 2nd left intercostal space • Functional closure of the ductus normally occurs soon after birth, within the 1st wk of life. If the ductus remains patent when pulmonary vascular resistance falls, aortic blood then is shunted left to right into the pulmonary artery. • Female predominance 2 : 1. • Associated with maternal rubella infection during early pregnancy. • Premature infants - the smooth muscle in the wall of the preterm ductus is less responsive to high PO2 and less likely to constrict after birth Investigations Chest Radiograph • Prominent pulmonary artery • ↑ pulmonary vascular markings • Cardiomegaly ECG • Right ventricular hypertrophy Echocardiography • Evaluates heart structure & function • Assess blood flow pattern • Estimate PDA size Cardiac Catheterization • Haemodynamic measurements. • Visualization of duct injection of contrast medium Complications • Infective endarteritis • Pulmonary or systemic emboli • Paradoxical emboli. • Aneurysmal dilation of the pulmonary artery or the ductus • Pulmonary hypertension in large PDA Management – aim for closure of the PDA Conservative Operative • COX inhibitors – Inhibit prostaglandin • After 14 days of life, proceeds to operative production management • Indomethacin, ibuprofen • Indications - symptomatic PDA fails to close with pharmacologic interventions or who has • Analgesics – acetaminophen contraindications to COX inhibitors • If heart failure symptoms present • Surgical ligation • Fluid restriction • Transvenous occlusions with coil device • Diuretics * PDA closure is more likely when medication is administered before 14-21 days of age. General contraindications to both indomethacin and ibuprofen: • Thrombocytopenia • Active hemorrhage (including severe IVH) • NEC or isolated intestinal perforation • High plasma creatinine • Oliguria