Cardiac Dysfunction in Pediatric VSD with PAH

Our study cohort included eight children aged 6–10 years who were hospitalised in the Affiliated Hospital of Southwest Medical University, China on June 2022. Four of the children were diagnosed by echocardiography as VSD without PAH (control group, n = 4) and the other four were diagnosed by echocardiography and right cardiac catheterisation as moderate or severe PAH secondary to VSD (PAH group, n = 4). A diagnosis of PAH by right-heart catheterisation was defined as a mean pulmonary arterial pressure >25 mmHg at rest, a pulmonary capillary wedge pressure <15 mmHg and a pulmonary vascular resistance of >3 Wood units. We excluded patients receiving targeted therapy for PAH and those diagnosed with other intracardiac malformations, such as patent ductus arteriosus, large atrial septal defect, or other related conditions, like congenital lung disease, bronchial asthma and congenital pulmonary vascular malformation.
During the cardiac operation, atrial appendage specimens were collected from all patients before cardiopulmonary bypass and blood samples were collected via the jugular vein before performing the midline sternotomy. The plasma and right atrial appendage specimens were then aliquoted and stored at −80°C until RNA extraction.