Rationale: Right ventricular (RV) function is an important determinant of prognosis in pulmonary
hypertension. However, noninvasive assessment of the RV function is often limited by …

Objective Pulmonary arterial hypertension related to scleroderma (PAH‐Scl) is associated
with high morbidity and mortality as well as poorer response to therapy and worse outcomes …

Background— Right ventricular failure from increased pulmonary vascular loading is a major
cause of morbidity and mortality, yet its modulation by disease remains poorly understood. …

The lung is a common site of complications of systemic connective tissue disease (CTD),
and lung involvement can present in several ways. Interstitial lung disease (ILD) and …

Objective Pulmonary hypertension (PH) is an important cause of mortality in systemic sclerosis
(SSc), where it can be isolated (pulmonary arterial hypertension [PAH]) or associated with …

… Correspondence and requests for reprints should be addressed to Stephen C. Mathai,
MDMHS, Johns Hopkins University School of Medicine, Division of Pulmonary and Critical Care …

Background: Right ventricular (RV) adaptation to acute and chronic pulmonary hypertensive
syndromes is a significant determinant of short- and long-term outcomes. Although …

Rationale: Pulmonary arterial hypertension (PAH) related to systemic sclerosis (SSc) has a
poorer prognosis compared with other forms of PAH for reasons that remain unexplained. …

Background— Systemic sclerosis–associated pulmonary artery hypertension (SScPAH) has
a worse prognosis compared with idiopathic pulmonary arterial hypertension (IPAH), with a …

Objective Pulmonary arterial hypertension (PAH) affects up to 15% of patients with connective
tissue diseases (CTDs). Previous recommendations developed as part of larger efforts in …