Do tertiary paediatric hospitals deal with the same spectrum of paediatric pulmonary hypertension as multicentre registries?

Abstract

To the Editor: Although national [1–5] and international paediatric registries for pulmonary hypertension (PH) [6, 7] have been published recently, inclusion criteria and, therefore, the distribution of aetiologies and severity of cases have not always been comparable [8]. In order to determine possible differences between registries, we have compared the aetiologies of confirmed PH of prevalent versus incident cases of PH in our own large database (in a tertiary paediatric hospital), and compared these to recently reported large multinational registries. According to pre-specified protocols, any children with clinical symptoms or at risk of PH at our centre were referred for echocardiography. If tricuspid regurgitation velocity was >2.8 m·s−1, right heart catheterisation was performed and the diagnosis of PH was confirmed if mean pulmonary artery pressure was ≥25 mmHg. The Dana Point classification was used [1, 9]. A standardised diagnostic work-up was used in PH cases: thoracic computed tomography scan, hepatic echography, lung function tests, genetic screening, screening for systemic diseases, and HIV serology were used when appropriate. At the beginning of our study, our database included 86 prevalent PH patients. Over the next 3 yrs, 126 incident PH patients were referred. Median age at inclusion was 2.4 yrs (range 0.5–15 …