[Tricuspid atresia with hypoplasia of aortic isthmus in absence of transposition of great vessels (author's transl)].
A case of tricuspid atresia with a concordant ventriculoarterial connection associated with hypoplasia of the aortic isthmus and malformation of left atrioventricular valve is presented. Hyoplasia of aortic isthmus in absence of transposition of great vessels was explained by a posterior deviation of infundibular septum that reduced subaortic outflow tract and created an ample bulboventricular orifice. In the left atrioventricular valve an anterolateral papillary muscle that was prolonged in a thick chorda tendineaee fused to ventricular face of the cusp up to its insertion in the atrioventricular ring was observed. A systolic murmur of high tonality compatible with regurgitation of the left atrioventricular valve and a notch that was registered in postmortem angiography were attributed to this anomalous chorda tendinea. In the posterior wall of the heart there were two descending coronary arteries. One descended fro the crux cordis; the other, to its right, adjacent ot the acute margin, followed the insertion of the trabecular septum, delimited the outlet chamber, and was anastomosed to the anterior descending coronary artery.
D001017:Aortic Coarctation / Q000150:complications*; D005260:Female; D006337:Heart Murmurs; D006343:Heart Septal Defects / Q000150:complications; D006801:Humans; D007231:Infant, Newborn; D008943:Mitral Valve / Q000002:abnormalities*; D014261:Tricuspid Valve / Q000002:abnormalities*
Atresia tricúspide con hipoplasia del istmo aórtico en ausencia de transposición de los grades vasos.