Revista Española de Cardiología (English Edition) Revista Española de Cardiología (English Edition)
Rev Esp Cardiol. 2011;64:615-8 - Vol. 64 Num.07 DOI: 10.1016/j.rec.2010.07.003

Cardiac Malformations in Patients With Pentalogy of Cantrell and Ectopia Cordis

Jesús de Rubens Figueroa a,, Erika Flor Sosa Cruz a, Luisa Díaz García b, Daniel Carrasco Daza c

a Departamento de Cardiología, Instituto Nacional de Pediatría, México DF, Mexico
b Departamento de Investigación, Instituto Nacional de Pediatría, México DF, Mexico
c Departamento de Patología, Instituto Nacional de Pediatría, México DF, Mexico

Keywords

Congenital heart disease. Ectopia cordis. Pentalogy of Cantrell.

Abstract

We studied 21 patients who presented with a diagnosis of pentalogy of Cantrell. Their mean age was 40 days. All patients presented with congenital heart disease. Six presented with ectopia cordis. Every patient was subjected to echocardiography. Double outlet right ventricle, an atrial septal defect and dextrocardia were seen in 5 patients (24%). Four patients survived. Seventeen died, 12 from sepsis or septic shock. Autopsies were performed on 10 patients. All of the ectopia cordis patients died. Pentalogy of Cantrell is uncommon, and its association with ectopia cordis indicates poor prognosis.

1885-5857/© 2011 Sociedad Española de Cardiología. Published by Elsevier España, S.L.U. All rights reserved

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