Prognosis in children with pulmonary arterial hypertension: 10-year single-centre experience

Abstract

A b s t r a c tBackground: Pulmonary arterial hypertension (PAH) is a rare progressive disease of the pulmonary arterioles with an unfavourable prognosis. Aim:To evaluate survival and prognostic factors in patients with PAH diagnosed and treated at a single centre in the years 2004-2013. Methods:The study included 55 children (33 girls; 66%, 22 boys; 33%), with an average age 6.2 ± 6.0 years, with idiopathic PAH -n = 23 (42%), PAH associated with systemic-to-pulmonary shunts -n = 17 (31%), and PAH after corrective cardiac surgery -n = 15 (27%). Forty-seven of them (87%) were treated with advanced therapy.Results: During the follow-up with an average time of 5.6 ± 4.7 years 15 (27.3%) children died. The one-, three-, five-, and ten-year survival was, respectively, 83.1%, 77.1%, 70.7%, and 65.2%. The analysis of the survival curves revealed a better prognosis in patients with baseline N-terminal pro-B-type natriuretic peptide (NT-proBNP) level < 605 pg/mL (p = 0.024) and a higher probability of survival of three and five years in children at baseline I/II World Health Organisation functional class (WHO-FC). The higher risk of death was associated with a higher pressure in the right atrium (HR 1.23, p < 0.01) and higher pulmonary resistance (HR 1.1, p < 0.01), whereas no history of syncope had a better prognosis (HR 0.31, p = 0.03).Conclusions: Survival in the study group was comparable to the currently published register data. Mortality risk factors were connected with the severity of the disease at diagnosis.

Children and Adolescents with Pulmonary Arterial Hypertension: Baseline and Follow-Up Data from the Polish Registry of Pulmonary Hypertension (BNP-PL)

“…In the group of IPAH, 16% of patients had a positive acute vasoreactivity test at diagnosis, but at the time of enrolment, only half of them were still considered vasoreactive. Other pediatric registries reported positive results of acute vasoreactivity testing ranging from 6% to 34%, but the comparison of different studies is difficult due to the various protocols of the test [4,6,7,12,26,27]. Still, it is important to check the acute pulmonary vasculature response to vasodilators, since as many as half of the patients with a positive test can be successfully treated with calcium channel blockers in the long-term.…”

“…Prior to the availability of the targeted PAH therapies, the estimated median survival of children with idiopathic pulmonary arterial hypertension (IPAH) was between 10 months and 4.1 years [ 2 , 3 ]. However, it improved significantly with the advent of targeted treatment [ 4 , 5 , 6 , 7 ].…”

Hubungan rasio netrofil limfosit dengan hipertensi arteri pulmonal pada anak dengan penyakit jantung bawaan asianotik

“…4 Faktor risiko yang menyebabkan penyakit semakin berat antara lain: sinkop, aktivitas fungsional menurut World Health Organization-Functional Class (WHO-FC) III/IV, peningkatan kadar NT-proBNP, tingginya resistensi vaskular paru, dan tingginya tekanan atrium kanan. 5 Biomarker yang diteliti dan digunakan pada pasien hipertensi arteri pulmonal semakin luas. 6 Salah satunya adalah rasio neutrofil limfosit.…”

Effect of first-month specific therapy determines long-term clinical outcome in patients with pulmonary arterial hypertension (RCD code: II-1A.4.o)

“…According to analysis in this group of patients, WHO-FC significantly correlated with 6MWD, NT-proBNP, and haemodynamic parameters [17]. Based on this data, NT-proBNP is accepted as an marker in IPAH [18].…”