Potts shunt in children with pulmonary arterial hypertension: institutional experience.

Abstract

Since 2004, different techniques for the Potts shunt have been used in patients with incurable pulmonary arterial hypertension. However, the experience with these interventions in a single institution is limited. We present our series of Potts shunts in children with pulmonary arterial hypertension. Eight patients with suprasystemic pulmonary arterial hypertension (pulmonary artery-to-aorta mean pressure ratio of 1.39 ± 0.30) had a Potts shunt at the median age of 13.5 months. Six patients were followed up for 2-32 (median 17) months after the operation. In all cases, the Potts shunt caused a decrease in arterial blood oxygen saturation at the lower extremities and was the genesis of the arterial blood oxygen saturation gradient between the upper and lower extremities (10.5 ± 1.8% at discharge). Postoperative echocardiography revealed flattening of the ventricular septum as well as a decrease in the right ventricle-to-left ventricle end-diastolic diameter ratio from 1.36 ± 0.14 preoperatively to 0.99 ± 0.22 at discharge (P = 0.010). The 2 patients with the highest preoperative pulmonary artery-to-aorta mean pressure ratio (1.79-1.86) died during the early postoperative period from repeated pulmonary hypertension crises and uncontrolled hypoxaemia. There were no deaths during the follow-up period. The paediatric functional class of the patients improved from IIIb-IV to I-II, syncopal episodes no longer persisted and the weight deficit decreased from 21.8 ± 7.5% preoperatively to 11.5 ± 5.9% at the latest follow-up examination (P = 0.004). The arterial blood oxygen saturation gradient between the upper and lower extremities and the right ventricle-to-left ventricle end-diastolic diameter ratio were the same as they were while the patients were in the hospital. Our limited experience demonstrates that the Potts shunt lowers suprasystemic pulmonary hypertension to the systemic level, limits manifestations of right-sided heart failure and improves the overall functional status of the patients.