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.
NCT03959748) is the first multicenter and prospective registry of adult and pediatric patients with PAH and CTEPH created in any of the Central-Eastern European countries. In the present report, we describe the design of the registry. Objectives of the BNP-PL registry The BNP-PL registry is an initiative of the Working Group on Pulmonary Circulation of the Polish Cardiac Society in cooperation with Polish PH reference centers 6-9 to assess prospectively the epidemiology, clinical course, and disease management of patients with PAH and CTEPH. The specific objectives of the study are shown in Supplementary material, Table S1. Introduction Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are rare types of pulmonary hypertension (PH). Consequently, patient registries are key instruments that provide data for clinical research and improve patient care and healthcare planning. 1 Currently, most of our knowledge on PAH and CTEPH epidemiology, management, and treatment outcomes come from registries originating in the Western populations .2-5 However, a global view on the epidemiology of PH reveals important geographical differences. 1 The Database of Pulmonary Hypertension in the Polish population (Baza Nadciśnienia Płucnego [BNP-PL]) (ClinicalTrials.gov identifier,
BackgroundDespite advancements in pharmacological and device-based treatment, heart failure (HF) continues to impose an enormous burden for health care system worldwide. Decompensation of HF is one of the main causes of hospitalization, therefore the identification of patients with the highest risk of such complication is still of great clinical importance. The prognostic significance and utility of global longitudinal strain (GLS) has been previously studied in patients with the broad spectrum of cardiovascular diseases in various endpoints, however its role in assessing the risk of hospitalization due to HF exacerbation of optimally treated outpatients has not been fully explored. Therefore, the aim of the study was to verify whether the GLS of the left ventricle (LV) derived by 2D speckle tracking echocardiography has, independently of other well-known clinical parameters, an additional impact on the risk of HF decompensation in stable patients with LV systolic dysfunction of ischemic origin.MethodsIn 193 clinically stable HF outpatients with LV ejection fraction (LVEF) ≤ 50%, GLS, additionally to other clinical parameters, was analyzed. During 34 (14–71) months of follow-up, 58 patients were hospitalized due to HF decompensation (EVENT).ResultsEVENT was significantly associated with age, QRS width, NYHA functional class, left atrium diameter, LV systolic and diastolic volume, LVEF, hemoglobin, brain natriuretic peptide, diuretic treatment, absence of beta-blockers, impaired renal function and history of diabetes in univariate Cox analyzes. GLS with pre-specified cut-off value of -9.4% was also significantly associated with the EVENT (HR 15.16; 95% CI 1.81–126.91). After adjusting for above-mentioned parameters GLS was still a significant predictor of hospitalization due to HF decompensation.ConclusionsGLS measurement can provide incremental information on the risk of HF decompensation in stable outpatients with LV systolic dysfunction of ischemic origin.
The COVID-19 pandemic has impacted healthcare systems worldwide. Little is known about the impact of the pandemic on medical and psycho-social aspects of children with rare diseases such as pulmonary arterial hypertension and their parents. The study is based on children registered in The Database of Pulmonary Hypertension in the Polish Population and a parent-reported survey deployed during the first 6 months of the pandemic. The questionnaire consisted of six question panels: demographic data, fear of COVID-19, General Anxiety Disorder-7 (GAD-7), social impact of pandemic, patients’ medical status, and alarming symptoms (appearance or exacerbation). Out of 80 children registered, we collected 58 responses (72.5% response rate). Responders (parents) were mostly female (n = 55; 94.8%) at a mean age of 40.6 ± 6.9 years. Patients (children) were both females (n = 32; 55%) and males with a mean age of 10.0 ± 5.1 years. Eleven (19%) children had symptoms of potential disease exacerbation. Eight parents (72.7%) decided for watchful waiting while others contacted their GPs or cardiologists (n = 6; 54.5%). Three children had to be hospitalized (27.3%). Most planned hospitalizations (27/48; 56.2%) and out-patient visits (20/35; 57.1%) were cancelled, delayed, or substituted by telehealth services. Among the participating parents, the study shows very high levels of anxiety (n = 20; 34.5%) and concern (n = 55; 94.8%) and the need for detailed information (52; 89.6%) regarding COVID-19 and medical service preparedness during the pandemic. The COVID-19 pandemic has influenced child healthcare and caused high levels of anxiety among parents.
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