Congenital pulmonary venous return anomaly

Orpha code: 3090OMIM code:

Definition

A rare developmental defect during embryogenesis where some or all of the pulmonary veins drain into the right atrium or the systemic veins, with or without the presence of pulmonary venous obstruction, leading to various manifestations such as fatigue, exertional dyspnea, pulmonary arterial hypertension, cyanosis and progressive congestive heart failure. The two main subtypes are congenital partial pulmonary venous return anomaly (PAPVC; see this term), where one or a few of the pulmonary veins are anomalous, and congenital total pulmonary venous return anomaly (TAPVC, see this term), where all of the pulmonary veins are anomalous.

Disease data
Classification

Clinical group

Synonyms
Congenital pulmonary venous connection anomaly
Wrodzona wada połączenia żyły płucnej
ORPHA code
3090
OMIM code
-
ICD10 code
-
ICD11 code
-

No additional description.

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