Differences in right ventricular function and response to targeted therapy between patients with IPAH and PAH-CHD

Tengteng Zhu; Panyun Wu; Zhen Tan; Quan Jin; Yusi Chen; Lian Li; Zewei Chen; Yirui Tang; Jiang Li; Zhenfei Fang

doi:10.3389/fphar.2023.1124746

Differences in right ventricular function and response to targeted therapy between patients with IPAH and PAH-CHD

Front Pharmacol. 2023 Feb 13:14:1124746. doi: 10.3389/fphar.2023.1124746. eCollection 2023.

Authors

Tengteng Zhu¹, Panyun Wu¹, Zhen Tan¹, Quan Jin¹, Yusi Chen¹, Lian Li¹, Zewei Chen¹, Yirui Tang¹, Jiang Li¹, Zhenfei Fang¹

Affiliation

¹ Department of Cardiovascular Medicine, The Second Xiangya Hospital, Central South University, Changsha, Hunan, China.

Abstract

Background and aims: Pulmonary arterial hypertension (PAH) is a chronic pulmonary vascular disorder characterized by elevated pulmonary vascular resistance (PVR) and pulmonary arterial pressure (PAP). Right heart failure is a life-threatening complication of PAH and predicts a poor prognosis. PAH associated with congenital heart disease (PAH-CHD) and idiopathic PAH (IPAH) are two prevalent PAH subtypes in China. In this section, we set out to explore baseline right ventricular (RV) function and its response to targeted agents between IPAH and PAH-CHD. Methods and results: Consecutive patients diagnosed with IPAH or PAH-CHD by right heart catheterization (RHC) in the Second Xiangya Hospital from November 2011 to June 2020 were included. All patients received PAH-targeted therapy and the RV function was assessed by echocardiography at baseline and during follow-up. A total of 303 patients (age, 36.23 ± 13.10 years; women, 213 (70.3%); mean PAP [mPAP], 63.54 ± 16.12 mmHg; PVR, 14.74 ± 7.61 WU) with IPAH (n = 121) or PAH-CHD (n = 182) were included in this study. Compared with PAH-CHD, patients with IPAH had worse baseline RV function. As of the latest follow-up, forty-nine patients with IPAH and six patients with PAH-CHD died. Kaplan-Meier analyses showed better survival in PAH-CHD versus IPAH. After PAH-targeted therapy, patients with IPAH had less improvement in 6 MWD, World Health Organization functional class, and RV functional parameters compared with patients with PAH-CHD. Conclusion: Compared with patients with PAH-CHD, patients with IPAH had worse baseline RV function, unfavourable prognosis, and inadequate response to targeted treatment.

Keywords: congenital heart disease; pulmonary arterial hypertension; pulmonary hypertension; right ventricle; right ventricular function; targeted therapy; treatment.