| Summary: | Pentalogy of Cantrell is a type of ectopia cordis including: defect in anterior diaphragm, pericardium, anterior abdominal wall in addition to extrusion of part of the heart outside the mediastinum and structural heart disease. We were faced by a case of 2 years old girl presented by a visible large pulsating swelling in the lower part of the chest and upper part of anterior abdominal wall in addition to other manifestations of pulmonary congestion and heart failure. After echocardiography, MSCT was done for completing the diagnosis, the cardiac lesion was a large apical VSD with biventricular dilatation and the apex of the heart forms a long tail like swelling passing through a large anterior pericardial and diaphragmatic defect to lie under the anterior abdominal wall. Under full CPB and cardioplegic arrest; the excess aneuresmal part of cardiac apex was excised and the Apical VSD was closed the aneuresmectomy opening because it was in accessible through the RT atrium. The PT needed high inotric support, prolonged ventilation and ICU stay and discharged on day 15 with maximized medical treatment because the ICU and pre discharge echo shows only minimal improvement of the already severely impaired myocardial function EF ∼ 30% and severe PH.
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