Complete thoracic ectopia cordis

Abstract

Thoracic ectopia cordis is a rare congenital defect with very few reported survivors after surgical correction. We report a case of complete thoracic ectopia cordis with double outlet right ventricle. The diagnosis was established antenatally and a repair was undertaken soon after birth. The child remained stable and was extubated on the fifth post-operative day. Forty-eight hours later the child succumbed to an unexplained respiratory arrest. Also presented is a review of the different surgical strategies for this unusual condition.

A rare case of cardiac anomaly: prenatally diagnosed ectopia cordis

“…While cases with all of these five findings are defined as definite Cantrell's pentology, cases with four of these five findings are defined as possible Cantrell's pentology (1,10). In our patient, the heart was localized in the thoracoabdominal region and Ectopia cordis is frequently accompanied by congenital heart disorders including ventricular septal defect, atrial septal defect, pulmonary stenosis, fallot tetralogy, right ventricular diverticle, double outlet right ventricle and more rarely with single ventricle, transposition of the great arteries and atrioventricular septal defect (1,7,9,11). In addition, extracardiac disorders including omphalocele, gastrochisis, scoliosis, cleft lip-palate and central nervous system disorders may also be observed in association with ectopia cordis (4,9).…”

“…Thoracoabdominal ectopia cordis may be a part of middle line defects defined as Cantrell's pentology (4,10). In Cantell's pentology, other congenital heart disorders are present in association with abdominal wall, sternum, diaphragm and pericardium disorders (1,9). While cases with all of these five findings are defined as definite Cantrell's pentology, cases with four of these five findings are defined as possible Cantrell's pentology (1,10).…”

Thoracoabdominal ectopia cordis: a case report

“…Ektopia kordisin nedeni tam olarak bilinmemekle birlikte trizomi 18, triploidi ve X'e bağlı geçişli koromozomal anomalilerle birlikteliği bildirilmiştir (7,8 (10). Bizim olgumuzda ise interatriyal septumda, sekundum atriyal septal defekt vardı.…”

“…Bizim olgumuzda ise interatriyal septumda, sekundum atriyal septal defekt vardı. Ayrıca ek anomali olarak başta omfalosel olmak üzere, gasroşizis, skolyoz, yarık damak-dudak, hipoplastik akciğer ve hidrosefali de görülebilir (10,11). Bizim olgumuzda en sık görülen ekstrakardiyak anomali olan omfalosel vardı.…”

“…Ektopia kordisin cerrahi tedavisi, torasik tipte olduğu gibi, güç ve ek kardiyak anomali varsa yüksek mortaliteyle sonuçlanabilir (9,10). Toraks uygun büyüklüğe ulaşana kadar aşamalı cerrahi önerilir.…”