IMPLANTATION OF PULMONARY ARTERY FLOW RESTRICTORS IN PATIENT WITH COMMON ARTERIAL TRUNK AND FUNCTIONALLY UNIVENTRICULAR HEART

INTRODUCTION: Common arterial trunk (CAT) is a conotruncal defect that is very rare in the setting of a functionally univentricular heart. METHODS: We present a rare case of CAT with a functionally univentricular heart in whom pulmonary artery flow restrictors were implanted as a first-stage palliation in the univentricular pathway. RESULTS: A boy was born at 40 weeks gestation (birth weight 4.1 kg) following a prenatal diagnosis of CAT type I, inlet VSD, and apical muscular VSD. Postnatal echocardiography confirmed the diagnosis of CAT, large inlet VSD 9mm, large apical muscular VSD 6mm, rudimentary ventricular septum, with unusual position, tricuspid truncal valve arising exclusively from the small right ventricle, Double inlet left ventricle, and suspicion for both AV valves having three leaflets. Ventricular function was good with no valve regurgitation or stenosis, however we deemed it impossible to do a biventricular correction and create a normal ventricular septum, and it was decided to follow the univentricular heart pathway. Modified Micro Vascular Plugs (MVP) (Medtronic, Galway, Ireland), as branch pulmonary artery flow restrictors (both 9mm in diameter) were implanted at the age of 12 days. At the age of 1 month, the right pulmonary artery flow restrictor was changed to a larger size (13mm), as there was pulmonary overcirculation in the right upper lobe. The patient has optimal weight gain, oxygen saturation of 85%, and receives aspirin 15mg daily. CONCLUSIONS: Not only is the functionally univentricular heart extremely rare in patients with Common arterial trunk, but the off-label use of modified MVP as pulmonary artery flow restrictors in similar cases, to our knowledge, has been published only once in the literature.