This study aimed to explore the potential causal link between genetic predisposition to various connective tissue diseases (CTDs), namely systemic lupus erythematosus (SLE), Sjögren's syndrome (SS), polymyositis (PM), dermatomyositis (DM), systemic sclerosis (SSc), mixed connective tissue disease (MCTD), and rheumatoid arthritis (RA), and the incidence of pulmonary arterial hypertension (PAH) utilizing Mendelian randomization (MR). 

The 7th International Leh Symposium on “Molecular Mechanisms and Therapeutics of Hypoxic Lung” from August 4 to 9, 2024, organized at the Mahabodhi International Meditation Centre in Saboo, Leh, Ladakh, India, ensured the participation of a galaxy of national and international researchers, who explored the complex clinico-physiological processes associated with high-altitude adaptive and maladaptive mechanisms.

The hemodynamic definitions of pulmonary hypertension consider resistive loading (pulmonary vascular resistance [PVR]), but there are increasing evidence that pulsatile loading (pulmonary artery compliance [PAC]) has functional and prognostic importance.

Pulmonary arterial hypertension (PAH) is a chronic progressive exacerbation of cardiopulmonary vascular disease. The patients' exercise endurance decreased progressively and the survival rate was low. Current basic therapy and targeted drug therapy can improve the quality of life (QoL) of PAH patients, but the long-term efficacy and prognosis are not good.

Elevated circulating hepcidin levels have been reported in patients with pulmonary artery hypertension (PAH). Hepcidin has been shown to promote proliferation of human pulmonary artery smooth muscle cells (PASMCs) in vitro, suggesting a potential role in PAH pathogenesis.

Balloon pulmonary angioplasty (BPA) is now a widely accepted treatment for inoperable chronic thromboembolic pulmonary hypertension (CTEPH), but it still faces the problem of high complications. Herein, we report a rare case of severe vagal response during the BPA of a total occlusion lesion in a patient with CTEPH. 

Patients with borderline pulmonary hypertension (PH) often experience shortness of breath or exacerbation of PH during exercise, known as exercise-induced PH. However, the pathogenesis of exercise-induced post-capillary PH (post-EIPH) and its treatment strategies remain unclear.

The right ventricular stroke work index (RVSWI) reflects the active work of the right ventricle (RV), but its clinical usefulness is not yet fully known in pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH).

Pulmonary veno-occlusive disease (PVOD) is a lethal variant of pulmonary hypertension. The degree of pulmonary arterial involvement varies. Here, we compare two PVOD patients who were transplanted at 8 years of age, whereof one is a homozygous EIF2AK4 mutation carrier.