Idiopathic pulmonary arterial hypertension and congenital heart defects: similarities and differences

Objective: to study the clinical and pathogenetic features of idiopathic pulmonary arterial hypertension (IPAH) and pulmonary arterial hypertension (PAH) in congenital heart defects, based on a 10-year follow-up of patients in the Rostov region. Materials and methods: the analysis included patients over the age of 18 who are registered in the Rostov region with various etiologies of PAH, including with IPAH and PAH-CHD. Two groups of patients were identified: 1st – patients with IPAH (n=32) and 2nd – with PAH in congenital septal heart defects (defects of the atrial or interventricular septa) (n=30). All patients underwent electrocardiography, echocardiography, and condition assessment using the clinical condition assessment scale, as well as the effectiveness of a screening questionnaire for early diagnosis of PAH. Results: it was found that patients with IPAH are older, seek medical help more often, have more pronounced clinical symptoms compared to patients in the 2nd group, and require more aggressive treatment. Patients with PAH in congenital heart defects are less likely to seek help, and have a longer period from the onset of the first symptoms to the detection of pulmonary hypertension. Patients in group 1 required increased PAH-specific therapy in 53% of cases, and patients in group 2 – in 19% (p<0.05). Conclusions: clinical and pathogenetic features of idiopathic PAH and against PAH in congenital heart defects have been identified, which makes it possible to optimize the early diagnosis and treatment of such patients