CHEST Guidelines-Unique-Predictors-of-Mortality-in-Patients-With-Pu

Unique-Predictors-of-Mortality-in-Patients-With-Pu

Pdf Summary

The study analyzed mortality predictors in patients with Pulmonary Arterial Hypertension (PAH) associated with systemic sclerosis (SSc), using data from the REVEAL Registry involving 500 SSc-APAH and 304 non-SSc-CTD-APAH patients. It aimed to identify why SSc-APAH patients experience higher mortality compared to other connective tissue disease-associated PAH (CTD-APAH) patients.<br /><br />Three-year survival rates were notably lower in SSc-APAH patients (61.4% for previously diagnosed and 51.2% for newly diagnosed) compared to non-SSc-CTD-APAH (80.9% and 76.4%, respectively). Unique mortality predictors for SSc-APAH included being male over 60 years, systolic blood pressure under 110 mm Hg, 6-minute walk distance less than 165 meters, mean right atrial pressure exceeding 20 mm Hg, and pulmonary vascular resistance over 32 Wood units. This contrasts with non-SSc-CTD-APAH, where a 6-minute walk distance over 440 meters was a protective factor.<br /><br />The study reinforces the earlier findings that PAH due to systemic sclerosis is associated with poorer outcomes and higher mortality than other forms of CTD-APAH. Identifying critical risk factors, including low baseline exercise capacity, low blood pressure, and severe disease markers, allows for targeted patient monitoring and potentially more aggressive treatment strategies. The report emphasizes the importance of these predictors for mortality in managing SSc-APAH patients effectively, suggesting that older men with advanced disease markers require closer monitoring and treatment adjustments to improve survival outcomes.<br /><br />This research remains essential for healthcare providers aiming to assess risk and tailor interventions to improve the long-term health of patients with SSc-APAH. The REVEAL registry provides comprehensive data that identifies demographic and clinical features most impactful on disease progression and outcome, underscoring the complexity and critical nature of managing SSc-associated PAH.

Keywords

Pulmonary Arterial Hypertension

Systemic Sclerosis

Mortality Predictors

REVEAL Registry

SSc-APAH

CTD-APAH

Survival Rates

Risk Factors

Disease Markers

Patient Monitoring