Improvement of Right Ventricular Function in Pulmonary Arterial Hypertension with Disease-Specific Therapy - A Clinical Observational Study.
BACKGROUND
Pulmonary arterial hypertension (PAH) is a serious and progressive disorder that can result in right ventricular (RV) dysfunction and mortality. Consequently, it is important to monitor RV function during management of PAH. The aim of this study was to investigate the change in RV function by echocardiography before and after disease-specific therapy.


METHODS
We recruited 31 PAH patients with functional class (FC) III or IV. All the patients received a comprehensive assessment before disease-specific therapy was administered, including observation of clinical symptoms, 6-min walk distance (6MWD), serum brain natriuretic peptide (BNP) level, and transthoracic echocardiography. The assessment was repeated 12 weeks after therapy.


RESULTS
Twenty-eight patients with a mean age of 40 years completed the study, of whom 82% were women. We found that the etiologies were mainly connective tissue disease-associated and idiopathic PAH. Of the patients in our study, 36% received endothelin receptor antagonist and 64% received phosphodiesterase-5 inhibitor. There was a significant improvement in FC after disease-specific therapy (p < 0.001). The 6MWD increased from 326 to 403 m (p < 0.001), and the serum BNP level decreased from 242 to 130 pg/mL (p = 0.008) after treatment. Echocardiography showed significant reduction in the right atrial and RV areas, pulmonary artery pressure, RV free wall thickness, and inferior vena cava diameter. The myocardial performance index and left ventricular eccentricity index were significantly reduced after therapy.


CONCLUSIONS
For PAH patients in our study, disease-specific therapy for 12 weeks resulted in an improvement in FC, 6MWD, serum BNP levels, and RV function.


BACKGROUND
Disease-specific therapy; Pulmonary arterial hypertension; Right ventricular function.
Department of Cardiology;
Wan-Jing
WJ
Ho
Department of Cardiology;
Chia-Pin
CP
Lin
Department of Cardiology;
Chun-Li
CL
Wang
Department of Cardiology;
Lung-An
LA
Hsu
Department of Rheumatology, Allergy and Immunology, Chang Gung Memorial Hospital, College of Medicine, Chang Gung University, Taoyuan, Taiwan.
Kuang-Hui
KH
Yu
Department of Rheumatology, Allergy and Immunology, Chang Gung Memorial Hospital, College of Medicine, Chang Gung University, Taoyuan, Taiwan.
Shue-Fen
SF
Luo
Department of Rheumatology, Allergy and Immunology, Chang Gung Memorial Hospital, College of Medicine, Chang Gung University, Taoyuan, Taiwan.
Ji-Yih
JY
Chen
Department of Cardiology;
Tsu-Shiu
TS
Hsu