Pulmonary arterial hypertension sensitive to calcium channel blocker, but not advanced pulmonary hypertension treatment: a case report.

ABSTRACT:

Background

Calcium channel blockers (CCB), the first accepted treatment, is effective only in a small number of idiopathic pulmonary arterial hypertension (I-PAH) patients with vasoreactivity [these patients are identified by a positive acute pulmonary vasoreactive test (AVT) response]. While the majority of I-PAH patients is non-vasoreactive and CCB non-responders, modern advanced pulmonary hypertension (PH)-specific therapies, which act on one of the three different mechanistic pathways-endothelin, nitric oxide (NO), and prostacyclin pathways, are effective. Treatment response to advanced PH-specific vasodilators in PAH patients with vasoreactivity is unknown.

Case summary

A 30-year-old woman with I-PAH was referred to our centre with worsening symptoms and deteriorating P

SUBMITTER: Hirakawa K 

PROVIDER: S-EPMC9446687 | biostudies-literature | 2022 Sep

REPOSITORIES: biostudies-literature