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Revista Cubana de Pediatría

versión impresa ISSN 0034-7531


GONZALEZ MOREJON, Adel Eladio; SERRANO RICARDO, Giselle; DIAZ RAMIREZ, Francisco  y  MARCANO SANZ, Luis. Pulmonary atresia with intact ventricular septum, a thematic review. Rev Cubana Pediatr [online]. 2012, vol.84, n.4, pp.401-405. ISSN 0034-7531.

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.

Palabras clave : pulmonary atresia; pediatric echocardiography; interventional cardiology; cardiovascular surgery.

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