Pulmonary Veno-Occlusive Disease Presenting as Congenital Heart Disease-Associated Pulmonary Hypertension
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.
How strong is the evidence?
Evidence Hub reads this as a case report, done in people. Its evidence level is 5 of 5 (Very low · case / narrative / animal / in vitro / in silico). Case reports, narrative reviews, and animal, cell or computer-model studies give the weakest evidence for human health.
Read the paper
Related studies
- Protective effects of tadalafil and N-acetyl cysteine therapy on cisplatin-induced testicular toxicity
- The Use of Generative AI for Scientific Literature Searches for Systematic Reviews: ChatGPT and Microsoft Bing AI Performance Evaluation
- Sildenafil as a Candidate Drug for Alzheimer’s Disease: Real-World Patient Data Observation and Mechanistic Observations from Patient-Induced Pluripotent Stem Cell-Derived Neurons
- Ozone-modified properties of pumpkin seed oil as anti-H. pylori, anticancer, anti-diabetic and anti-obesity agent
- Effectiveness of Low-Intensity Extracorporeal Shock Wave Therapy in Erectile Dysfunction: An Analysis of Sexual Function and Penile Hardness at Erection: An Umbrella Review
- Molecular Docking of Silymarin as a Ligand for Aromatase Enzyme and HIV-1 Reverse Transcriptase Using Molegro Virtual Docker
Go deeper in Evidence Hub
Evidence Hub adds a study data sheet (design, sample size, evidence level, key numbers), an AI summary and a chat grounded in the paper's text.