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

Abstract

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.

Characteristics and outcomes of patients with chronic thromboembolic pulmonary hypertension in the era of modern therapeutic approaches: data from the Polish multicenter registry (BNP-PL)

“…The design of the BNP-PL registry ( https://clinicaltrials.gov/ct2/show/NCT03959748 ), enrollment criteria, and data collection were recently described in detail. 33 – 35 The CTEPH adult arm of the registry enrolls adults with CTEPH from all 16 CTEPH reference centers in Poland accredited by the National Health Fund (NHF) for medical treatment of CTEPH. This ensures that all diagnosed patients in Poland are included in the registry.…”

High CD200 Expression on T CD4+ and T CD8+ Lymphocytes as a Non-Invasive Marker of Idiopathic Pulmonary Hypertension–Preliminary Study

“…Another limitation of this study is the lack of patients’ clinical follow-up, including hemodynamic assessment, echocardiographic exam, 6MWT, and CD200, and CD200R evaluation. The study group was heterogeneous, however, some studies indicate similar pathogenesis of changes in microcirculation in patients with PAH and CTEPH [ 39 , 40 , 41 ]. This study does not define a cause-effect relationship, although demonstrated results can be used in further studies.…”

The Impact of COVID-19 Pandemic on Children with Pulmonary Arterial Hypertension. Parental Anxiety and Attitudes. Follow-Up Data from the Polish Registry of Pulmonary Hypertension (BNP-PL)

“…Therefore, there is a very high risk of a severe or fatal course of COVID-19 [ 9 ]. Children with Pulmonary Arterial Hypertension (PAH) are a unique group of patients treated in tertiary centers with special treatment strategies monitored during frequent hospital and out-patient evaluations [ 10 ].…”

“…In Poland, children with PAH are treated based on the current guidelines and AEPC 2019 updated consensus statement [ 8 , 10 ] adjusted to the National Health Fund criteria for reimbursement of PAH-targeted therapies. According to the National Health Fund requirements, each drug handover must be conducted during hospitalization.…”