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Revista Cubana de Pediatría

Print version ISSN 0034-7531


GONZALEZ MOREJON, Adel Eladio et al. Echocardiographic morphology of the pulmonary atresia with intact ventricular septum. A 20-year study. Rev Cubana Pediatr [online]. 2013, vol.85, n.1, pp.6-16. ISSN 0034-7531.

Introduction: pulmonary atresia with intact ventricular system is a cardiovascular malformation accounting for 1% of congenital cardiopathies seen in the extrauterine life; due to its unfavorable outcomes, this disease is a true challenge for the contemporary medicine. Objectives: the research study comprised the application of the classification guidelines, the characterization of the atresia outlet track, the morphological study of the right ventricle, the assessment of the tricuspid ring and the detection of anomalies in the coronary circulation. Methods: forty three patients diagnosed with this disease and referred to "William Soler" pediatric cardiocenter from January 1992 to November 2011 were studied. Each case was performed bidimensional echocardiography and color-coding Doppler. Results and conclusions: the study confirmed the predominance of the valvular morphological variant of the disease and the presence of moderate and severe levels of right ventricular hypoplexia associated with limited volumetric capacitance of this chamber, with tricuspid valvular hypodevelopment and with anomalous sinusoids-dependent coronary circulation. The permeable oral foramen was the interatrial septal defect that was most associated to this disease and several structural anomalies of the tricuspid system was evidenced in conjunction or not with the prevailing annular hypodevelopment.

Keywords : pulmonary atresia; echocardiography; tricuspid valve.

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