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).

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.

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.

[Clinical and therapeutic considerations in children with infective endocarditis]. / Kliniczne i terapeutyczne asperkty zapalenia wsierdzia u dzieci.

PURPOSE: the aim of this study was to analyse the clinical course, complications and treatment in children with infective endocarditis (IE). MATERIAL AND METHODS: study group concerned 39 children with IE, aged 2.5 months - 15 years. The diagnosis was based on clinical symptoms, laboratory results and echocardiographic evaluation. RESULTS: among 39 children, in 36 cardiac anomalies (in 35 congenital and in 1 acquired heart lesions) were recognized prior to development of IE. In 3 children with normal heart, endocarditis was part of septicaemia. Positive blood cultures were present in 69.2% of patients, mostly Streptococci and Staphylococci. In one patient Aspergillus fumigatus was the etiologic factor of endocarditis. In 17 children cardiac failure was diagnosed and in 7 acute severe regurgitation of aortic valve in 3, mitral valve in 2, both aortic and mitral insufficiency in 2. One child with perforation of the tricuspid valve leaflet presented signs of renal failure. In 15 children the following complications were diagnosed: cerebral and/or systemic emboli, mycotic aneurysm of the sinus of Valsalva, abscess of aortic annulus, rupture of valve leaflets with severe insufficiency and recurrence of hemodynamically significant VSD. All children were given intravenous antibiotics. 13 patients were operated, 6 had cardiosurgery as an emergency. Four children died including 1 after cardiosurgery. CONCLUSIONS: 1. Acute heart failure in children with infective endocarditis is most often caused by severe aortic and / or mitral valve regurgitation. 2. Urgent cardiosurgery is the treatment of choice in children with acute heart failure or big and mobile vegetations. 3. Echocardiography plays a major role in the diagnosis of infective endocarditis and its complications.