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Congenital pulmonary vein (PV) stenosis or atresia is a rare progressive life-threatening great vessels anomaly characterized by narrowing and obstruction of one or more normally positioned PV at their junction with the left atrium, that usually presents during early infancy with dyspnea, tachypnea, and repeated pulmonary infections, and eventually, when all PV of one lung are affected, results in pulmonary hypertension (PH) and consecutive pulmonary arterial hypertension (PAH). It may manifest as an isolated lesion or associated with other cardiac defects such as congenital pulmonary venous return anomaly and septal defects.
Biomarker and diagnostic research for congenital pulmonary veins atresia or stenosis has been reported in the published literature.
Estimated prevalence: Unknown (Unknown prevalence).
2 clinical trials registered, 1 recruiting. Interventions under study include drug therapy and other interventions. Pipeline includes 1 PHASE1, 1 NA. Research is primarily sponsored by academic and government institutions.
299 publications have been identified in PubMed for congenital pulmonary veins atresia or stenosis. Kisho has analyzed 84 by research type. Research spans Review / Meta-Analysis (31%), Epidemiology / Natural History (18%), and Clinical Trial Publication (17%).
Research Type | Count | % of Total |
|---|---|---|
Research summaries | 26 |
Data assembled from 4 of 12 sources · Last updated Sep 20, 2026, 8:31 PM UTC
European rare disease database
Genetic and Rare Diseases Info Center
31%
Disease patterns and progression | 15 | 18% |
Clinical study results | 14 | 17% |
Patient case studies | 11 | 13% |
Laboratory research | 9 | 11% |
Testing and diagnosis research | 6 | 7% |
Other research | 3 | 4% |
Cicala N (2026). [PMID: 41796665](https://pubmed.ncbi.nlm.nih.gov/41796665/). *Ann Vasc Surg*. [Review / Meta-Analysis]
Jiang C (2026). [PMID: 41167585](https://pubmed.ncbi.nlm.nih.gov/41167585/). *J Pediatr Surg*. [Clinical Trial Publication]
Dreifuss NH (2026). [PMID: 41457173](https://pubmed.ncbi.nlm.nih.gov/41457173/). *Cardiovasc Intervent Radiol*. [Review / Meta-Analysis]
Fargen KM (2026). [PMID: 40789758](https://pubmed.ncbi.nlm.nih.gov/40789758/). *J Neurointerv Surg*. [Epidemiology / Natural History]
Palaparthi S (2026). [PMID: 42091310](https://pubmed.ncbi.nlm.nih.gov/42091310/). *Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu*. [Review / Meta-Analysis]
Zhang D (2025). [PMID: 41438765](https://pubmed.ncbi.nlm.nih.gov/41438765/). *Front Immunol*. [Basic Science / Preclinical]
Zhang L (2025). [PMID: 40955047](https://pubmed.ncbi.nlm.nih.gov/40955047/). *Ren Fail*. [Epidemiology / Natural History]
Robin B (2025). [PMID: 40449923](https://pubmed.ncbi.nlm.nih.gov/40449923/). *Neoreviews*. [Review / Meta-Analysis]
Taiji R (2025). [PMID: 40742880](https://pubmed.ncbi.nlm.nih.gov/40742880/). *Radiographics*. [Review / Meta-Analysis]
Zeng C (2025). [PMID: 40028855](https://pubmed.ncbi.nlm.nih.gov/40028855/). *J Am Heart Assoc*. [Basic Science / Preclinical]
AI-curated news mentioning congenital pulmonary veins atresia or stenosis
Updated May 7, 2026
A case report highlights the use of optical coherence tomography to assess rapidly progressive pulmonary vein stenosis in a patient with fibrosing mediastinitis. This study contributes to the understanding of diagnostic techniques in rare pulmonary conditions.
A recent study explores surgical and catheter-based interventions for pediatric pulmonary vein stenosis, highlighting advancements in treatment approaches. This research contributes to the understanding of effective management strategies for this rare condition.
A recent publication discusses the ethical dilemmas surrounding the management of pediatric pulmonary vein stenosis, highlighting the challenges of treating patients as a limited resource. The study emphasizes the need for a balanced approach in addressing the complexities of this rare condition.