ABSTRACT
Congenital long QT syndrome (LQTS) is a recognized but rare cause of sudden death in young adults. Death is due to polymorphic tachycardia degenerating into ventricular fibrillation. Implantable cardiovertor defibrillators are being increasingly employed in the management of congenital LQTS patients with recurrent syncopal episodes. Mutations within genes encoding sarcomeric contractile proteins such as troponin cause hypertophic and dilated cardiomyopathy. Hypertrophic cardiomyopathy is the commonest cause of sudden cardiac death in young athletes. Asymmetrical septal hypertrophy is the commonest pattern of hypertrophy, occurring in 60% of cases. Pericardial constriction may be complicated by any form of pericarditis but characteristically occurs in TB, connective tissue diseases, radiotherapy, post-cardiac surgery, bacterial pericarditis, neoplastic infiltration, and chronic renal failure. The prognosis of heart failure has been improved significantly by drugs inhibiting the renin-angiotensm-aldosterone system and those negating the effects of circulating catecholamines.
