Navigating Extreme Ventilation-perfusion Mismatches: Hybrid ECMO for ARDS with Right Ventricular Dysfunction in a Patient with Unilateral Agenesis of the Right Pulmonary Artery: A Case Report

BackgroundUnilateral agenesis of the pulmonary artery (UAPA) is a rare congenital anomaly that poses significant challenges in the management of acute respiratory failure due to complex ventilation-perfusion mismatch.The use of extracorporeal membrane oxygenation (ECMO) in such patients has rarely been described. Case ReportWe report a young female with right pulmonary artery agenesis, severe acute respiratory distress syndrome (ARDS), refractory hypoxemia, hypercapnia, shock, and right ventricular (RV) dysfunction, successfully managed with primary hybrid veno-arterialvenous (V-AV) ECMO. ConclusionThis case highlights the role of tailored ECMO configurations in managing complex cardiopulmonary interactions and represents one of the first reported cases of UAPA with ARDS supported using ECMO.

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Journal
Indian Journal of ECMO
Published
2026-02-04
DOI
https://doi.org/10.5005/jaypee-journals-11011-0086.32
Primary Topic
Congenital Heart Disease Studies
Type
article
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article

Navigating Extreme Ventilation-perfusion Mismatches: Hybrid ECMO for ARDS with Right Ventricular Dysfunction in a Patient with Unilateral Agenesis of the Right Pulmonary Artery: A Case Report

Kaladhar Sheshala
Indian Journal of ECMO
Congenital Heart Disease Studies
article

Navigating Extreme Ventilation-perfusion Mismatches: Hybrid ECMO for ARDS with Right Ventricular Dysfunction in a Patient with Unilateral Agenesis of the Right Pulmonary Artery: A Case Report

Kaladhar Sheshala
article en

Abstract

BackgroundUnilateral agenesis of the pulmonary artery (UAPA) is a rare congenital anomaly that poses significant challenges in the management of acute respiratory failure due to complex ventilation-perfusion mismatch.The use of extracorporeal membrane oxygenation (ECMO) in such patients has rarely been described. Case ReportWe report a young female with right pulmonary artery agenesis, severe acute respiratory distress syndrome (ARDS), refractory hypoxemia, hypercapnia, shock, and right ventricular (RV) dysfunction, successfully managed with primary hybrid veno-arterialvenous (V-AV) ECMO. ConclusionThis case highlights the role of tailored ECMO configurations in managing complex cardiopulmonary interactions and represents one of the first reported cases of UAPA with ARDS supported using ECMO.

Indian Journal of ECMOVol. 4(S1)
Yashoda Hospital (IN)
Openalex Percentile: Top 63%
Congenital Heart Disease Studies
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Navigating Extreme Ventilation-perfusion Mismatches: Hybrid ECMO for ARDS with Right Ventricular Dysfunction in a Patient with Unilateral Agenesis of the Right Pulmonary Artery: A Case Report — Kaladhar Sheshala · Indian Journal of ECMO (2026) | TGRS Research Map | TGRS