ABSTRACT

Pulmonary arterial hypertension is a chronic progressive disease that can be caused by many etiologic factors. If not treated; progressive increase in pulmonary vascular resistance leads to right ventricular failure and premature death. Accumulating PAH patients in experienced centers will ensure appropriate diagnosis, follow-up and treatment options.

The mean age of patients at first admission was 10.7±5 (3-20) years. Only one of the patients (7%) had primary PAH, and two patients (13%) had PAH associated with chronic lung disease. Twelve patients (80%) had PAH secondary to congenital heart disease. Of these patients, 10 had Eisenmenger syndrome and two were operated for congenital heart disease with a left to right shunt. Six patients (60%) had VSD, and two (20%) had atrioventricular septal defect in 10 patients with Eisenmenger syndrome. The most common symptom was easy fatigue (83%). All of the patients had telecardiography, electrocardiogram and echocardiography, and 87% of patients underwent six minute walking test and angiography. All patients undergoing angiography were tested for vasoreactivity and all were negative. When the treatments at the last controls of the patients are evaluated; 53.3% of the patients received monotherapy, 46.7% received combination therapy. The most frequently started monotherapy was bosentan (46.7%). Four patients were died during follow-up.

Fifteen patients (11 girls, 4 boys) who were followed up for PAH in the Department of Pediatric Cardiology between January 2008 and December 2016 were evaluated retrospectively.

This study was conducted to determine the epidemiological characteristics, quality of life, treatment modalities and efficacy of our treatment in patients with pulmonary arterial hypertension (PAH).