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A rare vascular anomaly characterized by congenital absence of the right or left pulmonary artery, usually ending within 2 cm of its expected origin from the pulmonary trunk. Patients often also have other cardiovascular abnormalities and respective symptoms and are then typically diagnosed in infancy or childhood, while isolated cases generally present with a mild clinical course and may go undiagnosed until adulthood. Presenting clinical features in isolated cases include hemoptysis, exertional dyspnea, and recurrent respiratory infections. The condition is typically accompanied by marked changes of lung tissue and may, if unrecognized, result in massive hemoptysis and pulmonary hypertension.
Features include common findings: Abnormal heart morphology, High blood pressure in lung arteries (pulmonary arterial hypertension), Recurrent respiratory infections, and Abnormal heart rhythm on EKG (abnormal ekg) and others; and sometimes findings: Cyanosis, Growth delay, Atrial septal defect, and Congestive heart failure and others.
Organ System | Phenotype Count | Example Features |
|---|---|---|
Heart and blood vessels | 14 |
Biomarker and diagnostic research for absence of the pulmonary artery has been reported in the published literature.
Phenotype severity distribution: 8 common features.
Estimated prevalence: Unknown (Unknown prevalence).
No clinical trials have been registered for absence of the pulmonary artery.
141 publications have been identified in PubMed for absence of the pulmonary artery. Research spans Case Report / Case Series (39%), Review / Meta-Analysis (28%), and Basic Science / Preclinical (13%).
Research Type | Count | % of Total |
|---|---|---|
Patient case studies | 55 | 39% |
Data assembled from 4 of 12 sources · Last updated Sep 20, 2026, 11:36 AM UTC
European rare disease database
Genetic and Rare Diseases Info Center
Common questions about absence of the pulmonary artery
Lungs and breathing | 8 | High blood pressure in lung arteries (pulmonary arterial hypertension), Recurrent respiratory infections, Recurrent pneumonia |
Blood and immune system | 1 | Recurrent respiratory infections |
Lab test results | 1 | Abnormal heart rhythm on EKG (abnormal ekg) |
Brain and nerves | 1 | Exercise intolerance |
Growth and development | 1 | Growth delay |
Research summaries
39 |
28% |
Laboratory research | 18 | 13% |
Clinical study results | 10 | 7% |
Disease patterns and progression | 10 | 7% |
Testing and diagnosis research | 6 | 4% |
Other research | 2 | 1% |
New treatment approaches | 1 | 1% |
Cueto-Robledo G (2026). [PMID: 41575407](https://pubmed.ncbi.nlm.nih.gov/41575407/). *Mayo Clin Proc*. [Case Report / Case Series]
Palaparthi S (2026). [PMID: 42091310](https://pubmed.ncbi.nlm.nih.gov/42091310/). *Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu*. [Review / Meta-Analysis]
Wang Z (2026). [PMID: 40423720](https://pubmed.ncbi.nlm.nih.gov/40423720/). *Pediatr Cardiol*. [Epidemiology / Natural History]
Calé R (2026). [PMID: 41790757](https://pubmed.ncbi.nlm.nih.gov/41790757/). *PLoS One*. [Basic Science / Preclinical]
Cealera E (2026). [PMID: 42123103](https://pubmed.ncbi.nlm.nih.gov/42123103/). *J Clin Med*. [Review / Meta-Analysis]
Jalali A (2026). [PMID: 41511738](https://pubmed.ncbi.nlm.nih.gov/41511738/). *Egypt Heart J*. [Case Report / Case Series]
Cozzi D (2026). [PMID: 41488156](https://pubmed.ncbi.nlm.nih.gov/41488156/). *Radiol Case Rep*. [Case Report / Case Series]
Wolf S (2026). [PMID: 41643939](https://pubmed.ncbi.nlm.nih.gov/41643939/). *J Thromb Haemost*. [Epidemiology / Natural History]
Yasrab M (2026). [PMID: 40413118](https://pubmed.ncbi.nlm.nih.gov/40413118/). *Curr Probl Diagn Radiol*. [Review / Meta-Analysis]
Brandão HV (2026). [PMID: 41711784](https://pubmed.ncbi.nlm.nih.gov/41711784/). *Einstein (Sao Paulo)*. [Case Report / Case Series]
AI-curated news mentioning absence of the pulmonary artery
Updated Sep 8, 2026
A new case report details congenital unilateral absence of pulmonary artery, contributing to the understanding of this rare condition. The findings may inform future research and clinical approaches.
A case report highlights transient cardiac dysfunction linked to new-onset mitral chordal rupture and congenital absence of the right coronary artery. This study contributes to the understanding of rare cardiac conditions.