Pulmonary arterial endothelial and smooth muscle cell homeostasis is regulated through the bone morphogenetic protein (BMP) and transforming growth factor beta (TGF-β) receptor pathways. Pathway imbalance results in pulmonary hypertension (PH). Each pathway has ligands and modulators influencing this balance. How these pathways differ in pediatric PH patients is unknown.
Pulmonary hypertension (PH) is a rare, complex disease affecting both children and adults. Efforts to provide health education are imperative as health literacy impacts patient and caregiver capacity to acquire, process, and understand health information and make informed health decisions.
The authors of this manuscript titled “Elective thoracic surgical resections for pulmonary arteriovenous malformations—a 16 year single-center experience,” presents excellent surgical results for elective operations performed in 24 patients with pulmonary parenchymal arteriovenous malformations.
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.
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.
