Profile of congenital heart disease with pulmonary arterial hypertension

Congenital Heart Disease (CHD) is a significant health issue globally, with Pulmonary Arterial Hypertension (PAH) being a serious and life-limiting complication.Understanding the clinical profile of patients with this comorbidity is crucial for effective management and improved outcomes.This study aims to describe the demographic and clinical characteristics of CHD patients with PAH.This was a retrospective, descriptive study conducted at Siti Khodijah Hospital, Sidoarjo, East Java, Indonesia.Data were collected from the medical records of all patients diagnosed with CHD and PAH from 2023-2024.Patient data included demographics age, gender, type of CHD, clinical symptoms, echocardiography, cardiac catheterization results, and treatment modalities.The data were analyzed to identify the prevalence of specific CHD types and the associated clinical features.A total of 107 pediatrics patients were included in the study.The majority were female (52%).The most common type of CHD identified was ventricular septal defect (31%), atrial septal defect (27%), followed by patent ductus arteriosus (17%), and others (25%).Common clinical symptoms at diagnosis included shortness of breath, cyanosis, fatigue.Diagnostic findings frequently revealed right ventricular hypertrophy, increased pulmonary artery pressure with mild PAH (29%), moderate PAH (38%) and severe PAH (34%).A proportion of patients had received prior surgical intervention, but still developed or had persistent PAH.The findings of this study provide a preliminary profile of CHD patients with PAH at our institution hospital.The data highlight the prevalence of certain CHD types and the associated clinical manifestations.These insights are essential for informing clinical practice, improving diagnostic protocols, and developing targeted treatment strategies for this specific patient population in our hospital.