Neonatal Repair of Hemitruncus-Excellent short and long term outcomes

M. Nathan, D. Rimmer, G. Piercey, P. del Nido*, J. E. Mayer, E. Bacha, F. Pigula. Childrens' Hospital, Boston, MA,

Anomalous origin of one of the branch pulmonary arteries from the aorta with two normal semilunar valves (hemitruncus) is a rare entity. There have been several small case series reported. We report here our experience with hemitruncus from 1982- 2005.
Retrospective case review of all cases of conotruncal anomalies at Children’s Hospital Boston, revealed 13 patients with hemitruncus. Diagnosis was established in most cases by echocardiogram, there was one missed diagnosis with diagnosis established intraoperatively. 8 patients were operated in the neonatal period, 1 at 6 weeks , 2 a t 2 months, 1 at 5 months and 1 at 2 years. 12 of the 13 had anomalous right pulmonary artery from aorta and 1 had left pulmonary artery from aorta. Associated anomalies included muscular VSD in one, left pulmonary vein membrane in one, coarctation and arch hypoplasia in one
There was one operative death in this series in an infant who died post repair of associated TEF from infected right atrial thrombus from a central venous lines. One patient required reoperation for supravalvar aortic stenosis and RPA stenosis 1 year postop. Freedom from death was 0.92 at 10 years and freedom from reoperation 0.83 at 10 years
Hemitruncus anamolies are a rare form of conotruncal abnormaliy that can and should ideally operated in the neonatal period with excellent longterm results.
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