Lea Behrend, Muneaki Matsubara, Takuya Osawa, Thibault Schaeffer, Jonas Palm, Carolin Niedermaier, Paul Philipp Heinisch, Nicole Piber, Alfred Hager, Peter Ewert, Jürgen Hörer, Masamichi Ono
University hospital of Technische Universität München. University Hospital of Munich and Ludwig-Maximilians-Universität, Munich.
Germany
European Journal of Cardiothoracic Surgery
Eur J Cardiothorac Surg 2025;
DOI: 10.1093/ejcts/ezaf058
Abstract
Objectives: We aimed to evaluate outcomes after Kawashima procedure with special regard to the development of pulmonary arterio-venous malformations.
Methods: All patients who underwent Kawashima procedure between 1992 and 2022 were reviewed.
Results: Twenty-one patients underwent Kawashima procedure at a median age of 14.5 (interquartile range, 8.4-40.4) months. There were no hospital deaths and 2 late deaths. Survival after Kawashima procedure at 5, 10, and 15 years was 90.5, 82.9, and 69.1%, respectively. It was 100% at 10 years for children 9 months old or younger at Kawashima procedure, compared with 77.0% for older children (p = 0.281). Hepatic vein incorporation was achieved in 16 patients (76.2%) at a median age of 3.3 (2.7-13.8) years and at a median interval of 2.6 (1.9-8.6) years. Survival after hepatic vein incorporation at 5, 10, and 15 years was 92.3, 83.1, and 55.4%, respectively. Pulmonary arterio-venous malformations developed in 4 patients after Kawashima procedure, which improved after hepatic vein incorporation in 3 patients. Of 4 patients who developed pulmonary arterio-venous malformations after hepatic vein incorporation, 2 patients died, and 2 patients survived. All of them had bilateral superior vena cava and hepatic venous flow was excluded in one lung as the cause of pulmonary arterio-venous malformations.
Conclusions: Kawashima procedure could be performed with low operative risk on a patient aged less than 9 months. Despite the current early Kawashima and subsequent hepatic vein incorporation strategy, the incidence of pulmonary arterio-venous malformations did not decrease. Therefore, leaving antegrade pulmonary blood flow at Kawashima procedure is recommended.
Category
Pulmonary Arteriovenous Malformations
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
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