Resolution of Severe PAH After Late VSD Closure in an 8-Year-Old: Clinical Prudence or Adventurism?

Pulmonary artery hypertension (PAH) is a fatal disease associated with high mortality, especially in countries with limited health resources in terms of lack of access to diagnostic and therapeutic evaluations. Therefore, it is necessary to discover inexpensive and available serum biomarkers for examining patients.

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.