Potts Shunt Improves Right Ventricular Function and Coupling With Pulmonary Circulation in Children With Suprasystemic Pulmonary Arterial Hypertension

Background: Pulmonary arterial hypertension is a progressive disease with poor outcomes in children. Right ventricular (RV) function is the most important determinant of the prognosis. Novel application of Potts shunt between left pulmonary artery and descending aorta has been introduced in an attempt to improve the survival. However, the effect of Potts shunt creation on RV function, RV-PA coupling, and survival has not been studied. Methods and Results: We evaluated the first 12 consecutive pediatric patients (9 male, median age 11.2 years and weight 32.8 kg) who underwent elective Potts shunt placement for suprasystemic pulmonary arterial hypertension between 2013 and 2017 with echocardiographic indices of RV function, RV work, RV-PA coupling, and pulmonary hemodynamics. Of 12 patients, 1 was excluded because of insufficient preshunt data, 2 died, 1 required lung transplant, and 8 survived for a median of 27 months postshunt. In survivors, WHO functional class significantly (P=0.01) improved and the majority (5 of 8) came off pulmonary vasodilators at the most recent follow-up. Postshunt RV systolic function improved (P=0.03), RV afterload decreased (P <0.01), RV work decreased (P=0.02), and RV-proximal PA coupling improved (P<0.01). Conclusions: This proof of concept study shows that Potts shunt allows improvement in functional status and mid-term transplant-free survival in the majority of the recipients by improvement in RV systolic function and RV-PA coupling in children with suprasystemic pulmonary arterial hypertension.