Differential right ventricular afterload during exercise in idiopathic pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension.

Pulmonary vascular resistance (PVR) and pulmonary arterial compliance (PAC)-the two primary components of right ventricular (RV) afterload-are interdependent and exhibit an inverse relationship. However, their variations across pulmonary hypertension subtypes and between rest and exercise remain unknown. We aimed to investigate the PVR-PAC relationship at rest and during exercise in idiopathic pulmonary arterial hypertension (IPAH) and chronic thromboembolic pulmonary hypertension (CTEPH).

Effect of riociguat on exercise following balloon pulmonary angioplasty in patients with inoperable chronic thromboembolic pulmonary hypertension in Japan (THERAPY-HYBRID-BPA): a multicentre, double-blind, randomised, controlled, phase 4 trial.

Background: Balloon pulmonary angioplasty (BPA) and medical therapy are the most common treatment options for inoperable chronic thromboembolic pulmonary hypertension (CTEPH), but their combined use has not been sufficiently studied. This study aimed to evaluate the efficacy during an exercise test and the safety of the continuation of riociguat post-BPA in patients with inoperable CTEPH and normalised haemodynamic variables.

Methods: This multicentre, double-blind, randomised, controlled, phase 4 trial was conducted at four high-volume CTEPH centres in Japan.

Prevalence of occult HFpEF and age-specific efficacy of passive leg raise in pulmonary hypertension.

Background: Occult heart failure with preserved ejection fraction (HFpEF), characterized by elevated pulmonary arterial wedge pressure during exercise (PAWP), has gained attention in the context of pulmonary hypertension (PH). This study aimed to evaluate the prevalence of occult HFpEF and assess the diagnostic efficacy of passive leg raise (PLR) in patients with confirmed or suspected PH.

Methods: A total of 619 patients with normal resting PAWP underwent symptom-limited exercise testing using a supine cycle ergometer in conjunction with right heart catheterization (RHC) for PH diagnosis and treatment assessment.

Prevalence and Determinants of Depression and Anxiety in Patients With Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension.

Background: Depression and anxiety screening has not been adequately examined in patients with pulmonary hypertension (PH). We assessed depression and anxiety prevalence and their determinants in pulmonary arterial hypertension (PAH) and chronic thromboembolic PH (CTEPH).

Methods And Results: This cross-sectional study included 234 patients with PH (age 57 [42-68] years; 75% female; PAH/CTEPH/other: 103/126/5).

Preference for advance care planning in patients with pulmonary hypertension.

Implementing advance care planning (ACP) is recommended in clinical guidelines. However, in pulmonary hypertension, patients' preference toward ACP remains unclear. We aimed to elucidate the preference of patients with pulmonary hypertension for ACP conversations and the association of ACP with important patient factors underlying treatment decision-making.

Efficacy of balloon pulmonary angioplasty in patients with chronic thromboembolic pulmonary disease and exercise pulmonary hypertension.

Background: The efficacy of balloon pulmonary angioplasty (BPA) in patients with chronic thromboembolic disease (CTEPD) without pulmonary hypertension (PH) remains unknown. Exercise PH (Ex-PH) is associated with impaired exercise capacity, even when pulmonary hemodynamics are normal at rest. We hypothesized that patients with Ex-PH could be the candidates for BPA.

Determinants of physical quality of life in patients with chronic thromboembolic pulmonary hypertension after treatment: Insights from invasive exercise stress test.

Background: Impaired quality of life (QoL) is prevalent among patients with chronic thromboembolic pulmonary hypertension (CTEPH) despite improved survival due to medical advances. We clarified the physical QoL of patients with CTEPH with mildly elevated pulmonary hemodynamics and evaluated its determinants using a database of patients with CTEPH evaluated for hemodynamics during exercise.

Methods: The QoL was measured in 144 patients with CTEPH (age, 66 (58-73) years; men/women, 48/96) with mildly elevated mean pulmonary artery pressure (<30 mm Hg) at rest after treatment with balloon pulmonary angioplasty and/or pulmonary endarterectomy using the Short-Form 36 (SF-36) questionnaire.

Pulmonary artery pressure-perfusion relation during exercise in patients with chronic thromboembolic pulmonary hypertension using pulmonary arteriography and right-heart catheterization.

Background: In pulmonary hypertension (PH), pulmonary artery pressure (PAP) does not increase to pulmonary perfusion (PP) < 50%. During exercise, PAP may be increased even at PP > 50% for the early detection of PP disorders. The relationship between PP estimated by pulmonary angiography (PAG) and PAP was evaluated in patients with chronic thromboembolic PH (CTEPH) treated by balloon pulmonary angioplasty with near-normal PH.

Reversible Cardiac Hypertrophy in Pulmonary Arterial Hypertension Treated With High-Dose Epoprostenol.

Although current guidelines recommend the use of prostanoid infusion that includes epoprostenol for high-risk pulmonary arterial hypertension patients, epoprostenol has many adverse effects. We report a case of a heritable pulmonary arterial hypertension patient who had transient biventricular hypertrophy during high-dose administration of epoprostenol. In this case, biventricular hypertrophy with worsening of dyspnea was observed during the uptitration of epoprostenol.

EmPHasis-10 Health-Related Quality of Life and Exercise Capacity in Chronic Thromboembolic Pulmonary Hypertension After Balloon Angioplasty.

Background Whether pulmonary hemodynamic parameters and functional capacity are associated with quality of life in patients with chronic thromboembolic pulmonary hypertension remains unknown. This study aimed to evaluate disease-specific quality of life using the emPHasis-10 questionnaire and assess its determinants in patients with chronic thromboembolic pulmonary hypertension with normalized pulmonary hemodynamics. Methods and Results This cross-sectional study included 187 health status assessments of 143 patients with chronic thromboembolic pulmonary hypertension (median age, 68 [58-75] years; men/women, 51/136; use of home oxygen therapy, 51 patients [27%]) after balloon pulmonary angioplasty with normalized mean pulmonary artery pressure <25 mm Hg at rest.

Successful epoprostenol withdrawal and termination with an aid of the exercise stress test in pulmonary arterial hypertension.

Continuous infusion of intravenous epoprostenol (EPO) improves exercise capacity and survival in pulmonary arterial hypertension (PAH); however, it is associated with side effects. This study aimed to describe our experience on safe EPO withdrawal with the aid of an exercise stress test. This retrospective review included patients with PAH who were successfully withdrawn from EPO.

Novel diagnostic and therapeutic approaches to pulmonary hypertension due to the unilateral absence of a pulmonary artery.

We report the case of a 64-year-old female diagnosed with severe pulmonary hypertension due to the unilateral absence of a pulmonary artery. The four-dimensional computed tomography scan is a useful modality for revealing detailed anatomical findings for differential diagnoses and surgical decision-making. The patient had severe pulmonary hypertension with a mean pulmonary artery pressure (PAP) of 74 mmHg and was treated with triple upfront combination therapy, leading to significant improvement in pulmonary haemodynamics (to 27 mmHg in mean PAP) and functional capacity (WHO functional class, from III to II; 6-min walk distance, from 211 to 276 m).

Factors contributing to exercise capacity in chronic thromboembolic pulmonary hypertension with near-normal hemodynamics.

Background: Despite improved survival for patients with chronic thromboembolic pulmonary hypertension (CTEPH) due to progressive medical and interventional treatment, impaired exercise capacity remains common due to poorly understood mechanisms. We aimed to clarify the exercise capacity of CTEPH patients with near-normal pulmonary hemodynamics and evaluate its determinants among the hemodynamic, peripheral (e.g.

Pulmonary Artery Pressure as a Treatment Target to Improve the Prognosis of Idiopathic Pulmonary Arterial Hypertension - Insight From a Cohort From Two Japanese Pulmonary Hypertension Centers.

The prognosis of pulmonary arterial hypertension (PAH) has been improving since the introduction of epoprostenol (EPO). The 3-year survival of naïve idiopathic PAH (IPAH) and hereditary PAH (HPAH) was 96% in a recent prospective Japanese registry. This increase in survival in Japan may have been due to the reduction of pulmonary artery pressure (PAP) by a rapid and sufficient dosage of EPO.

Exercise intolerance in chronic thromboembolic pulmonary hypertension after pulmonary angioplasty.

Introduction: Exercise pulmonary hypertension is common in patients with chronic thromboembolic pulmonary hypertension (CTEPH) who experience shortness of breath during exercise and reduced exercise capacity despite normalised pulmonary arterial pressure (PAP) at rest; however, the relationship between exercise pulmonary hypertension and exercise capacity remains unclear. Here we aimed to determine whether exercise pulmonary hypertension is related to exercise capacity and ventilatory efficiency in CTEPH patients with normalised resting haemodynamics after pulmonary balloon angioplasty (BPA).

Patients And Methods: In total, 249 patients with CTEPH treated with BPA (mean±sd age 63±14 years; male:female 62:187) with normal mean PAP (mPAP) (<25 mmHg) and pulmonary arterial wedge pressure (≤15 mmHg) at rest underwent cardiopulmonary exercise testing with right heart catheterisation.

Etiology of Exercise-Induced Pulmonary Hypertension Can Be Differentiated by Echocardiography - Insight From Patients With Chronic Pulmonary Thromboembolism With Normal Resting Hemodynamics by Balloon Pulmonary Angioplasty.

Background: Exercise-induced pulmonary hypertension (PH) is often seen in chronic thromboembolic PH (CTEPH) patients with normalized resting hemodynamics, but it is difficult to differentiate precapillary PH as pulmonary vascular dysfunction and post-capillary PH from occult-left ventricular dysfunction (LVD). The aim of this study was to examine whether the exercise-induced elevation of pulmonary arterial wedge pressure (PAWP) can be predicted by the echocardiographic index at rest.

Methods and results: A total of 71 CTEPH patients (67±11 years old, male/female=15/56) treated by pulmonary angioplasty with near-normal pulmonary arterial pressure (PAP) and normal PAWP at rest underwent symptom-limited exercise test using supine cycle ergometer with right heart catheterization.