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

Objectives: 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.

Methods: 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.

Results: 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.

Conclusions: 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.