pulmonary venoocclusive disease 2
A rare form of pulmonary arterial hypertension (PAH) characterized by a capillary infiltration of the pulmonary interstitium, bronchioles and pleura leading to elevated pulmonary arterial resistance and right heart failure. PCH is potentially fatal.
MONDO:0018554
pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis
excluded_subClassOf
MONDO:0009937
pulmonary venoocclusive disease
is_a
PVOD2
related
familial pulmonary capillary hemangiomatosis
related
pulmonary capillary hemangiomatosis
broad
pulmonary venoocclusive disease 2, autosomal recessive
related
SPBC36B7.09