Endothelin-1 Pathway Polymorphisms and Outcomes in Pulmonary Arterial Hypertension.

ABSTRACT:

Rationale

Pulmonary arterial hypertension (PAH) is a progressive fatal disease. Variable response and tolerability to PAH therapeutics suggests that genetic differences may influence outcomes. The endothelin pathway is central to pulmonary vascular function, and several polymorphisms and/or mutations in the genes coding for endothelin (ET)-1 and its receptors correlate with the clinical manifestations of other diseases.

Objectives

To examine the interaction of ET-1 pathway polymorphisms and treatment responses of patients with PAH treated with ET receptor antagonists (ERAs).

Methods

A total of 1,198 patients with PAH were prospectively enrolled from 45 U.S. and Canadian pulmonary hypertension centers or retrospectively from global sites participating in the STRIDE (Si

SUBMITTER: Benza RL 

PROVIDER: S-EPMC4731699 | biostudies-literature | 2015 Dec

REPOSITORIES: biostudies-literature