Reduced RVSWI Is Associated With Increased Mortality in Connective Tissue Disease Associated Pulmonary Arterial...

Area of Science:

• Cardiology
• Pulmonology
• Rheumatology

Background:

• Pulmonary arterial hypertension (PAH) carries a poor prognosis, particularly when linked to connective tissue disease (CTD).
• Right ventricular (RV) dysfunction is noted in scleroderma-related PAH, but its presence in the general CTD-associated PAH population is unclear.

Purpose of the Study:

• To investigate the association between RV function measurements and survival in a large cohort of CTD-associated PAH patients.
• To determine if intrinsic RV dysfunction contributes to reduced survival in CTD-associated PAH.

Main Methods:

• Compared clinical characteristics of 845 PAH patients with and without CTD.
• Utilized Kaplan-Meier survival analysis over 4 years.
• Examined the association between right ventricular stroke work index (RVSWI) and mortality in CTD-associated PAH.

Main Results:

• Significant differences in 6-minute walk distance, Borg dyspnea index, RVSWI, and pulmonary artery pulsatility index were observed between CTD-PAH and non-CTD-PAH groups.
• Lower RVSWI in CTD-PAH patients was associated with decreased survival, approaching statistical significance after adjustments.
• Pulmonary vascular resistance was similar between groups despite RV dysfunction differences.

Conclusions:

• RV dysfunction, indicated by reduced RVSWI, is linked to decreased survival in CTD-associated PAH.
• Findings suggest intrinsic RV dysfunction negatively impacts long-term survival in this patient group.
• This highlights the importance of assessing RV function beyond pulmonary vascular resistance in CTD-PAH management.