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A newborn baby was transferred to our hospital because of a pulsating mass of the abdominal wall just above the umbilical insertion. The patient was comfortable, not cyanotic and breathing spontaneously, but with mild tachypnoea. Physical examination was unremarkable except for a pulsating purple mass in the midline just above the umbilicus (middle panel). The chest x ray showed a moderate dextroposition of the heart without any pulmonary pathology. The ECG was normal. Echocardiography revealed a normal intracardiac anatomy except for a long and narrow appendiform diverticulum at the apex of the left ventricle of almost 3 cm, contracting synchronously with the left ventricle. The left superior caval vein persisted and entered into the right atrium via the coronary sinus.
Multisliced computed tomography showed a bifid lower part of the sternum, a small omphalocoele, a ventral diaphragmatic hernia, the absence of the apical part of the pericardial membrane, and the abnormal left ventricle with its diverticulum. The heart was noted to be in a dextroverse position. No other abnormalities were encountered.
The diagnosis of pentalogy of Cantrell consists of a combination of an abdominal wall defect, a diaphragmatic defect in the sternal part, a sternal defect, absence of the inferior pericardial membrane, and a heart defect, most frequently tetralogy of Fallot or a left ventricular diverticulum. The diagnostic results above confirmed our suspicion of this syndrome.
Surgical resection of the left ventricular diverticulum was indicated because of the risk of incarceration of the intestinal part in the diaphragmatic hernia. A preoperative cardiac catheterisation was performed revealing normal intracardiac pressures and saturations. Angiography of the left ventricle showed the ventricle with the diverticulum and visualised a normal aorta with two normal coronary arteries (right upper panel). The right and left coronary artery systems were normally dispersed over the myocardium and no main branch descended into the wall of the diverticulum.
Based on this information a resection of the left ventricular diverticulum (right lower panel) was performed and the apex of the left ventricle was closed, using two pledgets to prevent myocardial rupture. The abdominal wall was closed successfully after repair of the diaphragm in the same session at one week of age. At 3 weeks of age the patient was discharged in good general condition.
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