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Extensive screening in a newborn with prenatal suspicion of VACTERL syndrome identified an anomalous origin of the left pulmonary artery from the descending aorta with an arterial duct and left aortic arch, and normal intra-cardiac anatomy. Other anatomical anomalies suggested heterotaxy syndrome. At one-month-old, re-implantation of the 3.5 mm left pulmonary artery was performed by direct tension-low anastomosis. Post-operative course was complicated by severe left pulmonary atelectasis, and the patient died 20 days later.

Treatment of the aneurysms comprising the aortic arch is challenging. Surgical reconstruction usually requires aortic cross-clamping, cardiac arrest, and even deep hypothermia for a bloodless field. In this report, we present our surgical technique providing normothermic ascending aorta, aortic arch, and proximal descending aorta replacement with selective cannulation and perfusion of the whole body.

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Abnormal origin of the left pulmonary artery from the descending aorta and heterotaxy syndrome: an undescribed phenotypic association

A surgical technique for ascending aorta, aortic arch and descending aorta replacement without cross-clamp, circulatory arrest or hypothermia

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2 results

Abnormal origin of the left pulmonary artery from the descending aorta and heterotaxy syndrome: an undescribed phenotypic association

A surgical technique for ascending aorta, aortic arch and descending aorta replacement without cross-clamp, circulatory arrest or hypothermia