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pulmonary artery coming from the aorta - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

pulmonary artery coming from the aorta

MONDO:0020391

Also known as:abnormal origin of right or left pulmonary artery from the aortahemitruncus arteriosus

Overview

Pulmonary artery coming from the aorta (PACA) is a cardiac malformation characterized by anomalous origin of one branch of the pulmonary arteries from the ascending aorta and a normal origin of the other pulmonary artery from the right ventricular outflow tract, and presenting with respiratory distress, congestive heart failure and failure to thrive within the first days/months of life.

Clinical Presentation

Built from Human Phenotype Ontology (HPO) phenotype annotations and GeneReviews clinical descriptions. Features represent findings documented across known cases — not an exhaustive list of possible symptoms.

Features include very common findings: Anomalous origin of right pulmonary artery from ascending aorta and Abnormal cardiovascular system morphology; and common findings: Cyanosis, Failure to thrive, Atrial septal defect, and Congestive heart failure and others. 26 total HPO annotations.

Organ System	Phenotype Count	Example Features
Heart and blood vessels	9	Atrial septal defect, Congestive heart failure, Left ventricular outflow tract obstruction

Diagnosis

Biomarker and diagnostic research for pulmonary artery coming from the aorta has been reported in the published literature.

Prognosis

Phenotype severity distribution: 2 very common features, 9 common features.

Estimated prevalence: Unknown (Unknown prevalence).

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 pulmonary artery coming from the aorta.

106 publications have been identified in PubMed for pulmonary artery coming from the aorta. Kisho has analyzed 74 by research type. Research spans Case Report / Case Series (39%), Review / Meta-Analysis (28%), and Epidemiology / Natural History (11%).

Research Type	Count	% of Total
Patient case studies	29	39%

Data assembled from 4 of 12 sources · Last updated Sep 19, 2026, 8:50 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:99050

GARD

Genetic and Rare Diseases Info Center

GARD:4586

Questions for Your Care Team

→Are there specialists or centers of excellence for this condition in my area?
→Is there a patient registry I should consider joining?

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Kisho

pulmonary artery coming from the aorta

Overview

Clinical Presentation

Diagnosis

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Recent Publications

pulmonary artery coming from the aorta

Overview

Clinical Presentation

Diagnosis

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Recent Publications