Isolated pseudohypoplasia of the right ventricle.

We present a case of a 12-year-old girl with severe cyanosis due to abnormal moderator band producing a hidden trabecular component of the right ventricle, mimicking isolated hypoplasia of the right ventricle. A marked hypoplasia was confirmed by echocardiography and catheterization. At operation an anomalously thickened moderator band obstructing the apical infundibulum was found. Repair consisted of a section of the moderator band and closure of the atrial septal defect. Postoperatively the right ventricle showed normal dimension and function.

[Ectopia cordis and cardiac anomalies]. / Anomalías cardíacas en la ectopia cordis.

Ectopia cordis is a rare disease that occurs in 5.5 to 7.9 per million live births. Only 267 cases had been reported as of 2001, most (95%) associated with other cardiac anomalies. We studied the cardiac malformations associated in 6 patients with ectopia cordis. Depending on where the defect was located, the cases of ectopia were classified into four groups: cervical, thoracic, thoraco-abdominal, and abdominal. All 6 patients died before the third day of life, 4 during delivery. Three of the patients were included in the thoracic group, whereas the other 3 belonged to the thoraco-abdominal group. All the patients had associated ventricular septal defects, 3 double-outlet right ventricle (50%) and the rest (50%) tetralogy of Fallot-pulmonary atresia. Two patients with double-outlet right ventricle presented mitral-valve pathology, a parachute valve and an atresic mitral valve. None of these cardiac anomalies have been reported to date.

Anomalías cardíacas en la ectopia cordis / Ectopia cordis and cardiac anomalies

La ectopia cordis es una enfermedad rara, con una incidencia de 5,5-7,9 casos por millón de nacimientos. Hasta el año 2001 se han descrito 267 casos, la mayoría de ellos (95 por ciento) con otra cardiopatía asociada. Estudiamos las cardiopatías asociadas a 6 casos de ectopia cordis.En función de la zona afectada, los pacientes con ectopia cordis se clasifican en 4 grupos: cervical, torácica, toracoabdominal y abdominal. Los 6 pacientes descritos fallecieron antes de los 3 días de vida, cuatro de ellos en el momento del nacimiento. Tres pertenecían al grupo de ectopia torácica y otros tres al de toracoabdominal. Todos tenían comunicación interventricular, asociada a doble salida del ventrículo derecho en 3 (50 por ciento) y a tetralogía de Fallot-atresia pulmonar en otros 3 (50 por ciento). En 2 corazones con doble salida existía afección de la válvula mitral, estenosis por válvula en paracaídas y válvula mitral atrésica. Este tipo de anomalías no ha sido descrito anteriormente (AU)