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idiopathic pulmonary artery dilatation - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

idiopathic pulmonary artery dilatation

MONDO:0015664

Also known as:idiopathic dilatation of the pulmonary artery

Overview

Idiopathic pulmonary artery dilatation is a rare developmental defect during embryogenesis characterized by the dilatation of the main pulmonary artery, with or without dilatation of the right and left pulmonary artery branches, and not attributed to any other cardiac, pulmonary and/or arterial wall disease. It may present with exertional dyspnea, fatigue, cough, hemoptysis, palpitation and chest pain, but may also be asymptomatic. In serious cases, trachea constriction due to postural changes may lead to attacks of cyanosis with severe dyspnea. Sudden cardiac death has been reported in some cases.

ResearchLimited published research (4 articles)

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

No clinical trials have been registered for idiopathic pulmonary artery dilatation.

4 publications have been identified in PubMed for idiopathic pulmonary artery dilatation. Research spans Case Report / Case Series (100%).

Recent Publications

Ramanathan S (2026). [PMID: 42171567](https://pubmed.ncbi.nlm.nih.gov/42171567/). *JACC Case Rep*. [Case Report / Case Series]
Sun J (2025). [PMID: 41584275](https://pubmed.ncbi.nlm.nih.gov/41584275/). *Front Cardiovasc Med*. [Case Report / Case Series]
Shigeeda W (2025). [PMID: 40108730](https://pubmed.ncbi.nlm.nih.gov/40108730/). *Gen Thorac Cardiovasc Surg Cases*. [Case Report / Case Series]
Komori T (2025). [PMID: 40279701](https://pubmed.ncbi.nlm.nih.gov/40279701/). *Respir Investig*. [Case Report / Case Series]

Data assembled from 3 of 12 sources · Last updated Sep 19, 2026, 3:00 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:1676

GARD

Genetic and Rare Diseases Info Center

GARD:6757

Questions for Your Care Team

→Are there specialists or centers of excellence for this condition in my area?
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idiopathic pulmonary artery dilatation

Overview

Research Landscape

Recent Publications

Medical References

External Resources

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team