Double outlet right ventricle

Caring for someone with this diagnosis? Eleplan keeps diagnoses, medications, documents and every specialist in one plan.

Start free with Eleplan

Double outlet right ventricle with atrioventricular septal defect, pulmonary stenosis, heterotaxy

ORPHA:423712Clinical subtype

Also called DORV with atrioventricular septal defect, pulmonary stenosis, heterotaxy

What it is

Orphanet has not published a description for this disease yet. The identifiers, classification and cross-references below are still current.

Key facts

Inheritance
Multigenic/multifactorial
Classified as
Clinical subtype

Recorded for the broader condition

Prevalence
1-5 / 10 000 (at birth, Germany)Double outlet right ventricle
Age of onset
Infancy, NeonatalDouble outlet right ventricle

Orphanet records these for the broader condition rather than for this specific form. They are a starting point, not a figure for this subtype — subtypes often differ, and some are defined by being rarer than the condition they sit under.

ICD-10 codes

Q24.8filed under a broader ICD-10 category — shared with 10 other rare diseases

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Cross-references

MONDO 0018499UMLS C5680071

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

Powered by Eleplan

A rare diagnosis is just the start. Eleplan keeps the whole care plan in one place.

Diagnoses, medications, documents, appointments, and the whole care team — organized and always in sync, so you are not retelling the same story to every new specialist. With Ellie, your AI care assistant, on top of it. Free to start.