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. 2013;9(1):94-97.

Cardiac anomalies in Cantrell's pentalogy: From ventricular diverticulum to complete thoracic ectopia cordis

Hakim Kaouthar, Ayari Jihen, Jebri Faten, Msaad Hela, Ouarda Fatma, Chaker Lilia, Boussaada Rafik

Cardiac anomalies in Cantrell's pentalogy: From ventricular diverticulum to complete thoracic ectopia cordis

Hakim Kaouthar et al. Cardiol Tunis. 2013.

Abstract

Cantrell's pentalogy is a very rare syndrome associating varying degrees of midline wall defects and congenital cardiac anomalies. It is characterized by a combination of five anomalies that are: a midline supra umbilical abdominal wall defect, a sternal defect, an anterior diaphragmatic defect, a diaphragmatic pericardial defect and a congenital intra cardiac defect. Ectopia cordis, defined as a developmental defect in which the heart is abnormally located partially or totally outside the thorax, is in some cases a part of this syndrome. We report two cases of Cantrell's pentalogy in which cardiac ectopia was complete in one case and limited to left ventricular diverticulum in the other case. Both cases had a common intracardiac defect which is a double outlet right ventricle. The first case underwent surgical repair of the intracardiac lesions with resection of the diverticulum associated to repair of the midline defects with good outcome. The second case that presented with complete extra thoracic ectopia cordis died because of sepsis. We review through this article the main characteristics of Cantrell's pentalogy, we highlight the diversity of anatomic lesions and study the prognosis of this syndrome.

Keywords: Ectopia Cordis; Pentalogy of Cantrell.

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Figures

Figure 1
Figure 1
Profile (A) and face views (B) of the heart on Computed tomodensitometry scan showing a 56 mm long left ventricular diverticulum
Figure 2
Figure 2
Complete extra thoracic ectopia cordis and omphalcele
See this image and copyright information in PMC

References

    1. Cantrell RJ, Haller A, Mark M, Ravitch MD. A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium, and heart. Surg Ginecol Obstet. 1958;107:602–14. - PubMed
    1. Toyama WM. Combined congenital defects of the anterior abdominal wall, sternum, diaphragm, pericardium and heart: a case report and review of the syndrome. Pediatrics. 1972;50:778–86. - PubMed
    1. Fonkalsrud EW. Chest wall abnormalities. In: Bove AE, Geha AS, Hammond GL, Laks H, Naunheim KS, editors. Glenn’s thoracic and cardiovascular surgery. Appleton And Lange; East Norwalk, CT: 1991. pp. 581–591.
    1. Amato JJ, Douglas WI, Desai U, Burke S. Ectopia cordis. Chest Surg Clin North Am. 2000;10:297–316. - PubMed
    1. Apte AV. Thoraco-abdominal ectopia Cordis: A Rare Entity - Case Report and Review of litterature. People’s Journal of Scientific Research. 2008;1:31–33.

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