Pulmonary Arterial Hypertension in Connective Tissue Diseases Beyond Systemic Sclerosis

Authors

Christopher Lewis, Henry Ford HealthFollow
Ryan Sanderson
Nektarios Vasilottos
Alexander Zheutlin
Scott Visovatti

Recommended Citation

Lewis C, Sanderson R, Vasilottos N, Zheutlin A, and Visovatti S. Pulmonary Arterial Hypertension in Connective Tissue Diseases Beyond Systemic Sclerosis. Heart Fail Clin 2023; 19(1):45-54.

Document Type

Article

Publication Date

1-1-2023

Publication Title

Heart Fail Clin

Abstract

Pulmonary arterial hypertension (PAH) associated with connective tissue disease (CTD) (CTD-PAH) is a devastating condition that may progress rapidly to cause right ventricular dysfunction, resulting in significant morbidity and mortality. The pathobiology, epidemiology, natural history, early diagnosis, and treatment response of PAH associated with scleroderma (SSc-PAH) have been the subjects of intense research efforts over the previous decade. The success of these efforts has resulted in increased awareness and earlier detection of SSc-PAH. Practitioners are less aware of the risk of PAH associated with other CTDs; the aim of this article is to discuss the broader scope of CTD-PAH.

Medical Subject Headings

Humans; Pulmonary Arterial Hypertension; Hypertension, Pulmonary; Connective Tissue Diseases; Scleroderma, Systemic; Early Diagnosis

PubMed ID

36435572

Volume

19

Issue

1

First Page

45

Last Page

54