A patient presents with LVH. and pulmonary complications. ECG, shows left axis deviation. Most likely diagnosis is –

Congenital absence of tricuspid valve is called tricuspid atresia. Haemodynamics : The inflow portion of R.V. is hypoplastic The systemic venous blood coming to R.A. exits by way of a patent foramen ovale or an ASD. A VSD provides communication between L.V. and outflow portion of R.V. The L.V. thus maintains both the systemic and pulmonary circulation. The saturation of blood in the pulmonary artery and aorta is identical —> Because unoxygenated blood from right atrium mixes with oxygenated blood into left atrium. The pulmonary blood flow is dependent on size of VSD —› Smaller the VSD, less the pulmoanry blood flow. Clinical features : Child is cyanotic from birth Anoxic spells and squatting may be present. Features suggestive of tricuspid atresia : L.V. type of apical impulse Prominent large 'a' waves Enlarged liver with presystolic pulsations (a waves) ECG characterised by Left axis deviation and Left ventricular hypertrophy.