Left and right ventricular diverticula, ventricular septal defect and ectopia cordis in a patient with Cantrell's syndrome

Abstract

A patient with Cantrell's syndrome is presented with ectopia cordis, ventricular septal defect and a left ventricular as well as a right ventricular diverticulum. One-stage correction was performed with resection of both diverticula. Closure of the ventricular septal defect was complicated due to exposure problems as a result of the malposition of the heart. Although the presentation of Cantrell's syndrome can be variable, one-stage repair is technically feasible and should be considered as the treatment of choice in this rare syndrome.

Prenatal sonographic diagnosis of ectopia cordis

“…About 90% of fetuses with ectopia cordis have associated cardiovascular malformations, which determine the prognosis. 10,11 In our series, the only survivor, case 3, had no congenital heart disease or associated anomaly, whereas the infant in case 4 had transposition of the great vessels and severe defects that resulted in neonatal death be-fore surgery could be performed. It is difficult to demonstrate intracardiac defects by sonographic or pathologic examination in the first trimester, when cases 1 and 2 were diagnosed.…”

“…Surgical correction of ectopia cordis has successfully been performed using 1-stage and 2-stage procedures. [11][12][13][14][15] The procedures are complex and generally require coverage of the naked heart, placement of the heart into the thoracic cavity, and sternal or thoracic reconstruction. Primary repair in the neonatal period is the best type of management because simple closure of the sternal defect in the first month of life avoids the more complex reconstruction necessary in older children.…”

Ectopia Cordis in a First-Trimester Sonographic Screening Program for Aneuploidy

“…As all of these cases were diagnosed late in pregnancy or postnatally, these findings probably represent a more accurate prevalence of associated defects in this population than cases diagnosed early in pregnancy, many of which may have had undiagnosed structural heart disease because they are terminated or have early intrauterine demise. Similarly, pentalogy of Cantrell, a condition that is frequently associated with ectopia cordis, is by definition associated with intracardiac defects, including an atrial septal defect, a left ventricular diverticulum, tetralogy of Fallot, a double‐outlet right ventricle, and hypoplastic left heart syndrome, 12 , 20 conditions that are also difficult to diagnose in the first trimester.…”

Ectopia Cordis in a First-Trimester Sonographic Screening Program for Aneuploidy

“…1,2 The first diagnosed case was reported in 1981 in a 34-week fetus, 17 and the first-trimester diagnosis with the use of transvaginal sonography was accomplished 1 decade later. Similarly, pentalogy of Cantrell, a condition that is frequently associated with ectopia cordis, is by definition associated with intracardiac defects, including an atrial septal defect, a left ventricular diverticulum, tetralogy of Fallot, a double-outlet right ventricle, and hypoplastic left heart syndrome, 12,20 conditions that are also difficult to diagnose in the first trimester. Humpl et al 19 reviewed a series of 10 patients with ectopia cordis; 7 had intracardiac structural defects, and 6 had associated noncardiac malformations.…”