Portopulmonary hypertension syndrome

Also known as: Portopulmonary syndrome

Pulmonary arterial hypertension in portal hypertension.

Medical specialty: Pulmonology
Subspecialty: Pulmonology / Pulmonary vascular disease
Sex & age distribution: Male: affected; Female: affected, female predominance; Children: rare
Gene(s) involved: BMPR2

Etiology & mechanism

Vasoconstriction and remodeling from portal circulation mediators.

Gene(s) involved (1)

BMPR2

Symptoms & signs (5)

chest paindyspneaedemafatiguesyncope

SyndromeScope — educational reference. Content is model-generated and not clinically verified; always confirm against OMIM, Orphanet and GeneReviews before clinical use.