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Penthalogy of Cantrell

Marecos, Edgardo; Ramos, Miguel H.;
Gomez Rinesi, JuanF

Cardiocentro SRL- Corrientes. Argentina

Fetal echocardiography has a privilege place among routine diagnostic procedures due to its availability and efficiency to discover cardiac pathologies during gestation.

Objectives: This presentation demonstrates one of the anomalies that can be diagnosed during gestation, the penthalogy of Cantrell, which consists of thoracoabdominal ectopis cordis and the following five elements; notch in the lower portion of the sternum, defect in the supraumbilical abdominal wall or laparoquisis, anterior deficiency of the diaphragm, intracardiac defects, and pericardiodiaphragmatic defects.

Reported case: Consists of a patient without previous control, that came to the physician's office with a 34 week pregnancy in which fetal anasarca was detected. Fetal echocardiography was requested. The study was done using an ATL Ultramark 8 (2,5 and 3 Mhz transducers). The fetus was detected to have ascitis, subcutaneous edema, and severe tricuspid insufficiency, also, ectopia cordis with both vessels coming out from the right ventricle, severe interauricular defect, and a considerable enlargement of right cavities.

The hourglass-shaped heart showed ventricles protruding from the thoracic wall and the diaphragm inside the onphalocele sac.

Caesarean section was performed 2 weeks after diagnostic study. The patient died 4 hrs after birth; authorization for autopsy was requested to confirm imaging findings.

Conclusion: The penthalogy of Cantrell is a complex syndrome that consists of malformations compromising thorax, abdomen, diaphragm and/or pericardium and cardiac anomalies. Its diagnosis can be done during pregnancy. The complications that usually appear after birth need to be managed by a team integrated by pediatricians, cardiovascular surgeons and cardiologists.

 

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2nd Virtual Congress of Cardiology

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