%0 Journal Article
%T Atresia pulmonar con septum interventricular intacto Pulmonary atresia with intact ventricular septum, a thematic review
%A Adel Eladio Gonz¨¢lez Morej¨®n
%A Giselle Serrano Ricardo
%A Francisco D¨ªaz Ram¨ªrez
%A Luis Marcano Sanz
%J Revista Cubana de Pediatr£¿-a
%D 2012
%I Editorial Ciencias M¨¦dicas
%X La atresia pulmonar con septum interventricular intacto es una cardiopat¨ªa cong¨¦nita cian¨®tica aparentemente sencilla caracterizada por incorporar diversas variantes morfol¨®gicas y lesiones asociadas que conllevan a un diagn¨®stico y tratamiento m¨¢s complejo del que inicialmente podr¨ªa esperarse. La elevada mortalidad antes de los 6 meses de edad obliga a instaurar una conducta agresiva y precoz. Con esta revisi¨®n nos hemos propuesto efectuar la evaluaci¨®n integral de la enfermedad desde sus elementos conceptuales hasta las variantes terap¨¦uticas a adoptar, para lo cual se describen los aspectos m¨¢s importantes en cuanto a morfolog¨ªa, fisiopatolog¨ªa, diagn¨®stico y tratamiento. Se discute, asimismo, el papel de la ecocardiograf¨ªa, de la cirug¨ªa y de las t¨¦cnicas de cardiolog¨ªa intervencionista. La estrategia fundamental del tratamiento es separar las circulaciones sist¨¦mica y pulmonar sin provocar disminuci¨®n del gasto card¨ªaco ni aumentos en la presi¨®n venosa central. Se pretende, adem¨¢s, rescatar al ventr¨ªculo derecho para el circuito pulmonar siempre que la circulaci¨®n coronaria no sea dependiente de aquel. Se resalta, mediante algoritmo, la importancia que reviste la integraci¨®n de modalidades quir¨²rgicas, intervencionistas e h¨ªbridas en el acometimiento terap¨¦utico de esta cardiopat¨ªa. Pulmonary atresia with intact ventricular septum is an apparently simple cyanotic congenital cardiopathy, characterized by several morphological variants and associated lesions leading to a complex diagnosis and treatment. The high mortality rate recorded in under 6 months-old infants demands an early aggressive management to face this situation. This review was intended to make a comprehensive assessment of the disease from its basic conceptual elements to the therapeutic variants to be adopted. To this end, the most important elements of morphology, physiopathology, diagnosis and treatment were addressed, in addition to discussing the roles of echocardiography, surgery and interventional cardiology techniques. The fundamental strategy of treatment is to separate the systemic circulation from the pulmonary circulation, causing neither reduction of the cardiac output nor increase of the central venous pressure. It is also aimed to return the right ventricle to the pulmonary circuit, provided that the coronary circulation does not depend on it. The algorithm presented in this paper underlined the importance of the integration of the surgical, interventional and hybrid modalities for the therapeutic management of this cardiopathy.
%K atresia pulmonar
%K ecocardiograf¨ªa pedi¨¢trica
%K cardiolog¨ªa intervencionista
%K cirug¨ªa cardiovascular
%K pulmonary atresia
%K pediatric echocardiography
%K interventional cardiology
%K cardiovascular surgery
%U http://scielo.sld.cu/scielo.php?script=sci_arttext&pid=S0034-75312012000400009