The Effects of Pulmonary Vasodilating Agents on Right Ventricular Parameters in Severe Group 3 Pulmonary Hypertension: A Pilot Study

Abstract

Pulmonary arterial hypertension (PAH)-approved vasodilators improve right ventricular (RV) function in patients with PAH. However, whether PAH-approved drugs ameliorate RV morphology and function in lung disease-associated pulmonary hypertension (lung-PH) remains unclear. We aimed to prospectively evaluate the changes in RV volume and ejection fraction (RVEF) in 14 consecutive severe lung-PH patients treated with PAH-approved vasodilators. Severe lung-PH was defined as a mean pulmonary arterial pressure (MPAP) of ≥35 mmHg or an MPAP of ≥25 mmHg with a cardiac index (L/min/m 2 ) of <2. Right heart catheterization and cardiac magnetic resonance (CMR) imaging were performed at baseline and at 3 months after starting sildenafil with or without other PAH-approved drugs. Follow-up was conducted at 3 months in 11 participants; compared with baseline values, MPAP and pulmonary vascular resistance (PVR) decreased by 18% and 37%, respectively. Baseline CMR imaging revealed an elevated RV end-diastolic volume index (RVEDVI; mL/m 2 ) of 117.5 ± 35.9 and a below-average RVEF of 25.2% ± 7.2%; after 3 months, RVEDVI decreased by 23.7% (P = 0.0061) and RVEF increased by 32.9% (P = 0.0165). Among the 11 patients, 3 were thought to be a stable and homogenous subset in terms of background lung disease and medical management administered. These 3 patients exhibited similar ameliorations in PVR and RVEF, compared with the other 8 patients. PAH-approved drug treatment may improve RV dilatation and systolic function among patients with severe lung-PH. This study was approved by University Hospital Medical Information Network Clinical Trials Registry (UMIN-CTR) on September 1, 2013 (UMIN000011541).

Echocardiographic evaluation of the right ventricular dimension and systolic function in dogs with pulmonary hypertension

“…Exclusion criteria for this study were the identification of a congenital heart disease, acquired cardiomyopathies, pericardial disease, severe pleural effusion, atrial fibrillation, and breeds typically prone to arrhythmogenic right ventricular cardiomyopathy (boxers and bulldogs). In addition, all dogs receiving sildenafil or pimobendan were excluded from the study, due to the potential influence of these drugs on TRPG and RV systolic function …”

Pulmonary capillary hemangiomatosis-predominant vasculopathy in a patient with rheumatoid arthritis-associated interstitial lung disease: An autopsy report

“…In the present case, we used sildenafil (phosphodiesterase (PDE)-5 inhibitor), considering that he had a component of PAH. Prior reports, including ours, have indicated a favorable effect of PDE5 inhibitor(s) in severe Group 3 PH patients [ [18] , [19] , [20] ]. Regarding the use of pulmonary vasodilators to PH patients with ILD, current guidelines for PH recommend the use of such drugs only when the degree of PH is disproportionately high (“PAH phenotype”) in patients with ILD [ 3 ].…”

Diagnosis and Management of Combined Post- and Precapillary Pulmonary Hypertension in A Patient with Multiple Comorbidities

“…Another promising single arm prospective study included 14 patients with severe PH associated with lung disease, 6 of which were diagnosed with CPFE. Repeated cardiac magnetic resonance imaging revealed improvement of right ventricle dilatation and dysfunction after 3 months of treatment with sildenafil, implying potential benefit in this group of patients [15].…”