Taliha Oner - MD, Associate Professor in Pediatric Cardiology, University of Health Sciences Dr. Siyami Ersek Thoracic and Cardiovascular Surgery Training and Research Hospital, Department of Pediatric Cardiology Istanbul, Turkey.
Pinar Dervisoglu - Specialist in Pediatric Cardiology, Sakarya University Medical Faculty Department of Pediatric Cardiology, Sakarya, Turkey.
Ahmet Celebi - MD, Professor in Pediatric Cardiology, University of Health Sciences, Dr. Siyami Ersek Thoracic and Cardiovascular Surgery Training and Research Hospital, Department of Pediatric Cardiology Istanbul, Turkey.

Background: We aimed to determine the importance of myocardial tissue Doppler flow rates, pulmonary artery/aorta diameter (PA/Ao), pulmonary venous flow transit time (pPTT), pulmonary artery acceleration time (PAAcTc), and right atrium area index (RAAI) parameters in the prediction of prognosis patients with a diagnosis of pulmonary arterial hypertension (PAH). Materials and Methods: : This retrospective study was done on ۳۰ patients with systemic pulmonary arterial hypertension confirmed via catheterization who had negative vascular reactivity test results (pediatric patients with primary pulmonary hypertension and secondary pulmonary hypertension due to congenital heart disease). PA/Ao diameter, PAAcTc, pPTT, and RAAI were calculated. The patients were grouped as those with PA/Ao <۱, ۵>, SaO۲% , and pro-B-type natriuretic peptide (BNP) , and those receiving combination therapy or monotherapy, and the parameters were compared between the groups. Results: pPTT was shorter in the group with SaO۲ > ۹۰%, which was attributed to the high number of patients with idiopathic PAH in this group and more severe PAH stage. PAAcTc was shorter in patients who showed no clinical improvement on monotherapy and required multidrug therapy. RAAI was significantly increased in patients with pro-BNP > ۱۰۰ pg/ml. A decreased pPTT, accompanied by a lower PAAcTc in the group with PA/Ao >۱.۵, indicated the severity of PAH in this group. Conclusion: When managing PAH patients, the presence of RAAI > ۱۸ cm۲/m۲, PA/Ao > ۱.۵, and PAAcTc < ۸۰ msn indicates a higher disease severity, and suggested that the patients’ treatment regimen should be re-evaluated based on these parameters. Also, the RAAI parameter shows the closest association with pro-BNP levels, which is one of the correct prognosis indicators.

echocardiography, tissue Doppler, Pulmonary arterial hypertension, right atrium area index