1 research outputs found
Children and adolescents with pulmonary arterial hypertension : baseline and follow-up data from the polish registry of pulmonary hypertension (BNP-PL)
We present the results from the pediatric arm of the Polish Registry of Pulmonary
Hypertension. We prospectively enrolled all pulmonary arterial hypertension (PAH) patients,
between the ages of 3 months and 18 years, who had been under the care of each PAH center
in Poland between 1 March 2018 and 30 September 2018. The mean prevalence of PAH was
11.6 per million, and the estimated incidence rate was 2.4 per million/year, but it was geographically
heterogeneous. Among 80 enrolled children (females, n = 40; 50%), 54 (67.5%) had PAH associated
with congenital heart disease (CHD-PAH), 25 (31.25%) had idiopathic PAH (IPAH), and 1 (1.25%)
had portopulmonary PAH. At the time of enrolment, 31% of the patients had significant impairment
of physical capacity (WHO-FC III). The most frequent comorbidities included shortage of growth
(n = 20; 25%), mental retardation (n = 32; 40%), hypothyroidism (n = 19; 23.8%) and Down syndrome
(n = 24; 30%). The majority of children were treated with PAH-specific medications, but only half of
them with double combination therapy, which improved after changing the reimbursement policy.
The underrepresentation of PAH classes other than IPAH and CHD-PAH, and the geographically
heterogeneous distribution of PAH prevalence, indicate the need for building awareness of PAH
among pediatricians, while a frequent coexistence of PAH with other comorbidities calls for a
multidisciplinary approach to the management of PAH children