Takanori Suzuki, Satoru Kawai, Satona Tanaka, Hidetoshi Uchida, Hiromu Ukai, Yoji Nomura, Hiroyuki Katsuragawa, Junichi Kawada, Shuichiro Yoshida
Kariya Toyota General Hospital, Aichi Children’s Health and Medical Center and Fujita Health University. Kyoto University Hospital.
Japan
Journal of the American College of Cardiology Case Reports
JACC Case Rep 2026;
DOI: 10.1016/j.jaccas.2026.109910
Abstract
Background: Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension rarely linked to congenital heart disease (CHD).
Case summary: An infant with a ventricular septal defect developed pulmonary hypertension. At 3 months, cardiac catheterization showed operable hemodynamics with preserved pulmonary vasoreactivity. Pulmonary artery banding was performed. Despite tadalafil, macitentan, and prostacyclin, pulmonary vascular resistance increased without pulmonary edema. Lung perfusion scintigraphy demonstrated perfusion defects, prompting transplantation. At 2 years and 10 months, he underwent bilateral lung transplantation with intracardiac repair. The explanted lungs demonstrated fibrous intimal thickening of pulmonary venules and hemosiderin deposition, indicating PVOD.
Discussion: PVOD may coexist with CHD and mimic CHD-associated pulmonary arterial hypertension, without pulmonary edema during vasodilator therapy.
Take-home messages: PVOD should be considered in children with CHD developing progressive pulmonary hypertension despite favorable hemodynamics and preserved vasoreactivity. The absence of pulmonary edema during vasodilator therapy does not exclude PVOD.
Category
Class I. Pulmonary Veno-occlusive Disease and Pulmonary Capillary Hemangiomatosis
Class I. Pulmonary Hypertension Associated with Congenital Cardiovascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes