Learning and research

Cellular communication network 2 (CCN2) is a secreted matricellular protein associated with pulmonary arterial hypertension (PAH) but has not been studied relative to PAH severity, outcomes, or right ventricle (RV) structure and function in a large human cohort and preclinical animal model.

Pulmonary arterial hypertension (PAH) is a disease that can eventually progress to right ventricular failure. Heart rate variability (HRV), including standard deviation of R-to-R intervals (SDNN), has been associated with increased mortality across different populations. The purpose of this study was to examine the association between HRV and disease severity in PAH.

Congenital heart disease (CHD) occurs at increased prevalence at high altitude, but there may be a paradoxical later onset of the development of Eisenmenger syndrome. We hypothesized that congenital heart disease patients at high altitude are protected from an early onset of irreversible pulmonary hypertension.

159 meeting abstracts from PVRI 2025 Rio.

Pulmonary arterial hypertension (PAH) is characterized by progressive narrowing and obliteration of distal, pre-capillary pulmonary vessels. Yet, noninvasive biomarkers that reflect this disease-defining process are lacking. A systematic review of PAH studies that measured circulating progenitor cells (CPCs) or circulating endothelial cells (CECs) in PAH by flow cytometry was performed to understand how future studies, leveraging state-of-the-art single-cell analyses, can advance the field.

To study the contribution of QRS prolongation to transplant-free survival, we conducted an observational study of 68 patients with sarcoidosis-related pulmonary hypertension. Every 10-ms increase in QRS interval was associated with an adjusted HR of 1.23 (95% CI: 1.06, 1.44) for the composite outcome of lung transplantation and all-cause mortality.

Pulmonary embolism (PE) is a leading cause of mortality in lung transplant recipients, with early cases associated with particularly poor outcomes. Identified risk factors include elevated BMI, renal dysfunction, ABO mismatch, donor malignancy, and specific immunosuppressive agents. Tailored risk assessments and targeted interventions are essential to mitigating PE-related mortality.

Benchmarks of clinical management are essential for improving the quality of care. However, the lack of established quality metrics for pulmonary arterial hypertension (PAH) contributes to practice heterogeneity. We assessed our center's diagnostic practices, therapeutic practices, and risk-adjusted survival patterns over time for the purpose of establishing quality benchmarks. 

In this Community Call, we discussed:

• Defining echocardiographic degrees of right heart size and function in pulmonary vascular disease from the PVDOMICS study
• Skeletal muscle SIRT3 deficiency contributes to pulmonary vascular remodeling in pulmonary hypertension due to heart failure with preserved ejection fraction