• Erika B Rosenzweig, Ashish Ankola, Usha Krishnan, William Middlesworth, Emile Bacha, and Matthew Bacchetta.
• Division of Pediatric Cardiology, Department of Pediatrics and Medicine, Columbia University Medical Center-New York Presbyterian Hospital, New York, NY. Electronic address: esb14@cumc.columbia.edu.
• J. Thorac. Cardiovasc. Surg. 2021 Apr 1; 161 (4): 1438-1446.e2.

ObjectivesDespite advances in treatment of idiopathic pulmonary arterial hypertension (IPAH), there remains no medical cure, and patients can experience disease progression leading to right heart failure, progressive exercise intolerance, and death. The reversed Potts shunt (left pulmonary artery to descending aorta) was reintroduced for treatment of end-stage IPAH to permit decompression of the suprasystemic right ventricle by right to left shunting, with preservation of upper body oxygenation. The shunt has the potential to delay the need for lung transplantation and offer a treatment for those who are transplant ineligible. To optimize shunt design and avoid the potential complications of bidirectional shunting, we developed a novel approach using a unidirectional-valved shunt (UVS) in patients with IPAH with suprasystemic pulmonary arterial pressure and poor right ventricular function.MethodsA single-center retrospective review was performed of UVS cases done at Columbia University Medical Center-New York Presbyterian between November 1, 2016, and May 1, 2019.ResultsFive patients (4 female; ages 12-22 years) underwent UVS. All had suprasystemic pulmonary arterial pressure, poor right ventricular function, and World Health Organization functional class IV symptoms at baseline. All patients are alive and transplant-free at latest follow-up (range 3-33 months; median 6 ± 11 months).ConclusionsThe UVS may offer an alternative solution to lung transplantation in adolescents and young adults with IPAH. Longer-term follow-up is needed to determine the ultimate impact of unidirectional unloading of the right ventricle in these patients and to determine whether the UVS will enable a broader approach to the treatment of patients with IPAH.Copyright © 2019 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.

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