Congenital pulmonary veins atresia or stenosis

Summary

Congenital pulmonary veins atresia or stenosis (MONDO:0017864) is a disease and 4 clinical trials. Top therapeutic interventions include ferumoxytol and losartan. A subtype of congenital anomaly of the great arteries — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Prevalence: Unknown (Worldwide)
• Clinical trials: 4

Clinical features

No curated clinical features (Orphanet) for this disease.

Identifiers

Disease identifiers

Field	Value
Canonical name	congenital pulmonary veins atresia or stenosis
Mondo ID	MONDO:0017864
Orphanet	3188
ICD-11	469101490
SNOMED CT	234062003
UMLS	C5680865
MedGen	1826176
GARD	0004598
Is cancer (heuristic)	no

Also known as: pulmonary vein stenosis · pulmonary veins stenosis

Disease family

This is a subtype of congenital anomaly of the great arteries. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease › human disease › disease by body system or component › cardiovascular disorder › congenital anomaly of cardiovascular system › congenital heart malformation › congenital anomaly of the great arteries › congenital pulmonary veins atresia or stenosis

Related subtypes (14): aortic arch interruption, aortic arch defects, idiopathic pulmonary artery dilatation, scimitar syndrome, fixed subaortic stenosis, congenital pulmonary valve stenosis, aorto-ventricular tunnel, aneurysm or dilatation of ascending aorta, premature closure of the arterial duct, absence of the pulmonary artery, congenital patent ductus arteriosus aneurysm, pulmonary artery hypoplasia, pulmonary branch stenosis, primary pulmonary vein stenosis

Subtypes (1): congenital pulmonary vein atresia

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

No druggable-target or therapeutic data for this disease’s cohort.

Clinical trials & evidence

Clinical trials

Clinical trials: 4.

Phase distribution (across all retrieved trials)

Phase	Trials
Not specified	2
PHASE1/PHASE2	1
PHASE1	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT02769130	PHASE1/PHASE2	SUSPENDED	Pilot Trial: the Safety and Feasibility of Losartan for Pulmonary Vein Stenosis
NCT06440408	PHASE1	RECRUITING	Calculating Wall Shear Stress in Infant Pulmonary Veins
NCT04696289	Not specified	ACTIVE_NOT_RECRUITING	Predicting Pediatric Pulmonary Vein Stenosis Outcomes Using Data Acquired During a Cardiac Catheterization
NCT03456193	Not specified	WITHDRAWN	Allogeneic Left Atrial and Pulmonary Vein Transplant for Pulmonary Vein Stenosis

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
FERUMOXYTOL	4	1
LOSARTAN	4	1