BACKGROUND: Right ventricular (RV) failure is a major determinant of symptoms and shortened survival in pulmonary arterial hypertension (PAH). This study assessed the prognostic relevance of increased right heart (RH) dimensions determined by echocardiography and RH reverse remodeling (RHRR) with targeted therapies in idiopathic PAH (IPAH). METHODS: The study prospectively monitored 102 therapy-naïve IPAH patients for the presence of clinical worsening. Baseline evaluation included RH catheterization and echocardiography. RHRR at the 1-year follow-up was defined by a decrease in RV end-diastolic area, right atrial area, and the left ventricular systolic eccentricity index. RESULTS: At the 1-year follow-up, 18 of 102 patients (17.6%) presented with RHRR. A decrease in pulmonary vascular resistance was the only independent determinant of RHRR. The 94 surviving patients were monitored for 995 ± 529 days. RHRR was an independent prognostic factor and significantly improved the power of the prognostic model based on traditional clinical and hemodynamic parameters. The respective event-free survival rates at 1, 3, and 5 years were 94%, 94%, and 94% in patients with RHRR and 75%, 55%, and 24% in those without RHRR (p = 0.0001). Interestingly, RHRR was able to further stratify patients' risk assessment through the Registry to Evaluate Early And Long-term PAH Disease Management risk score. CONCLUSIONS: RHRR after 1 year of treatment is an independent predictor of prognosis in IPAH. The likelihood of RHRR is proportional to decreased pulmonary vascular resistance.
BACKGROUND: Right ventricular (RV) failure is a major determinant of symptoms and shortened survival in pulmonary arterial hypertension (PAH). This study assessed the prognostic relevance of increased right heart (RH) dimensions determined by echocardiography and RH reverse remodeling (RHRR) with targeted therapies in idiopathic PAH (IPAH). METHODS: The study prospectively monitored 102 therapy-naïve IPAH patients for the presence of clinical worsening. Baseline evaluation included RH catheterization and echocardiography. RHRR at the 1-year follow-up was defined by a decrease in RV end-diastolic area, right atrial area, and the left ventricular systolic eccentricity index. RESULTS: At the 1-year follow-up, 18 of 102 patients (17.6%) presented with RHRR. A decrease in pulmonary vascular resistance was the only independent determinant of RHRR. The 94 surviving patients were monitored for 995 ± 529 days. RHRR was an independent prognostic factor and significantly improved the power of the prognostic model based on traditional clinical and hemodynamic parameters. The respective event-free survival rates at 1, 3, and 5 years were 94%, 94%, and 94% in patients with RHRR and 75%, 55%, and 24% in those without RHRR (p = 0.0001). Interestingly, RHRR was able to further stratify patients' risk assessment through the Registry to Evaluate Early And Long-term PAH Disease Management risk score. CONCLUSIONS: RHRR after 1 year of treatment is an independent predictor of prognosis in IPAH. The likelihood of RHRR is proportional to decreased pulmonary vascular resistance.
Authors: Rebecca R Vanderpool; Kendall S Hunter; Michael Insel; Joe G N Garcia; Edward J Bedrick; Ryan J Tedford; Franz P Rischard Journal: Chest Date: 2021-10-09 Impact factor: 10.262
Authors: Pavel Jansa; Samuel Heller; Michal Svoboda; Michal Pad'our; David Ambrož; Vladimír Dytrych; Michal Širanec; Tomáš Kovárník; Marián Felšőci; Martin Hutyra; Aleš Linhart; Jaroslav Lindner; Michael Aschermann Journal: J Clin Med Date: 2020-11-09 Impact factor: 4.241
Authors: Ze Ming Goh; Nithin Balasubramanian; Samer Alabed; Krit Dwivedi; Yousef Shahin; Alexander M K Rothman; Pankaj Garg; Allan Lawrie; David Capener; A A Roger Thompson; Faisal Alandejani; Jim M Wild; Christopher S Johns; Robert A Lewis; Rebecca Gosling; Michael Sharkey; Robin Condliffe; David G Kiely; Andrew J Swift Journal: Heart Date: 2022-08-11 Impact factor: 7.365