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.

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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Introduction. Electrocardiography (ECG) is one of the fundamental procedures used as a diagnostic tool in pediatric cardiology. ECG is indispensable in the evaluation of children with arrhythmias, some of which like in long QT syndrome and Wolff- Parkinson-White (WPW) syndrome could be life threatening. Aim. The aim of the study was to analyze diagnostic tests of the cardiovascular system in children with abnormal ECG screening performed at school. Material and methods. Abnormal ECG screening in 31 children, aged 7-13 years old (average 10.6 years) was re-analyzed. In all 31 children referred to pediatric cardiology department ECG and echocardiogram were performed. In case of arrhythmia suspicion, 24-hour Holter ECG monitoring and exercise test were also done. Results. Based on ECG screening performed at school, initially in 22 out of the 31 children significant changes in ECG were found and minor in 9. After verification in the pediatric cardiology department 50% of children with previously diagnosed significant changes in ECG were considered to have a normal ECG. Only in 2 children pathology was confirmed (WPW syndrome and QT prolongation). The most frequent falsely diagnoses in ECG included: hypertrophy of the left ventricle, heart axis deviation, nonspecific repolarization changes, partial right bundle branch block. Conclusions. 1. The interpretation of the ECG in children requires taking into account the age differences and should be carried out by a pediatric cardiologist. 2. Most of the changes found in screening ECG in children were irrelevant without clinical significance.