Current practice of care for adolescent and adult patients after Fontan surgery in Poland.

BACKGROUND: The growing number of adult patients after the Fontan operation requires regular surveillance tests in specialized centers. AIMS: Our study aimed to evaluate the current practice of care for Fontan patients in Poland using a multicenter survey. METHODS: Eight centers were included in the study including 5 adult congenital heart disease (ACHD) and 3 pediatric centers for adolescents. To compare the centers and facilitate interpretation of results, the Fontan Surveillance Score (FSS) was developed. The higher score is consistent with better care, with a maximum of 19 points. RESULTS: We included in the study 398 Fontan patients (243 adults and 155 adolescents [aged 14-18 years]). The median FSS was 13 points with variability between centers (interquartile range 7-14 points). Centers providing continuous care from the pediatric period until 18 years of age achieved a higher FSS compared to ACHD centers (median: 14 points vs. 12 points; P <0.001). Most of the patients, both in the ACHD (82.3%) and pediatric centers (89%), were seen annually and had a physical examination, electrocardiogram, and echocardiogram performed at each visit. However, we observed unsatisfactory utilization of tests identifying early stages of Fontan circulation failure (cardiopulmonary exercise tests, cardiac magnetic resonance, liver biochemistry and imaging, detection of protein-losing enteropathy). CONCLUSIONS: Our results showed that there is no unified surveillance approach for Fontan patients in Poland. The practice of care for adults differs from that of adolescents.

Effect of standardized vs. local preoperative enteral feeding practice on the incidence of NEC in infants with duct dependent lesions: Protocol for a randomized control trial.

Background: Infants with duct dependent heart lesions often require invasive procedures during the neonatal or early infancy period. These patients remain a challenge for pediatric cardiologists, neonatologists, and intensive care unit personnel. A relevant portion of these infant suffer from respiratory, cardiac failure and may develop NEC, which leads to inadequate growth and nutrition, causing delayed or complicated cardiac surgery. Methods: This randomized control trial will recruit term infants diagnosed with a duct dependant lesion within the first 72 h of life. After obtaining written parental consent patients will be randomized to either the physician led enteral feeding or protocol-based feeding group. The intervention will continue up to 28 days of life or day of cardiosurgical treatment, whichever comes first. The primary outcomes include NEC and death related to NEC. Secondary outcomes include among others, number of interrupted feedings, growth velocity, daily protein and caloric intake, days to reach full enteral feeding and on mechanical ventilation. Discussion: Our study will be the first randomized control trial to evaluate if standard (as in healthy newborns) initiation and advancement of enteral feeding is safe, improves short term outcomes and does not increase the risk of NEC. If the studied feeding regime proves to be intact, swift implementation and advancement of enteral nutrition may become a recommendation. Trial registration: The study protocol has been approved by the local ethical board. It is registered at ClinicalTrials.gov NCT05117164.

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 with Bronchopulmonary Dysplasia-Associated Pulmonary Hypertension Treated with Pulmonary Vasodilators-The Pediatric Cardiologist Point of View.

Pulmonary hypertension in children with bronchopulmonary dysplasia (BPD-PH) significantly worsens the prognosis. Pulmonary vasodilators are often used in BPD-PH but the short-term outcome of treatment is not well described. The aim of this study was to evaluate BPD-PH children diagnosed beyond 36 weeks postmenstrual age treated with pulmonary vasodilators (sildenafil, bosentan, or both) and to assess the short and long-term effect of oral pulmonary vasodilators treatment. Twenty patients were included in the study. Cardiology evaluation (WHO-FC, NTproBNP, oxygen saturation, pulmonary to systemic pressure ratio PAP/SAP) was performed at diagnosis and after treatment initiation. In the majority of patients improvement in all evaluated factors was observed. No side effects of vasodilators were observed. PH resolved in 10 patients after a mean of 21.4 months of treatment. Six patients died. The number of poor prognostic factors commonly used to assess patients with pulmonary arterial hypertension (PAH) decreased significantly during BPD-PH treatment. The influence of BPD-PH perinatal risk factors on prognosis was considered but was not confirmed. In conclusion, the treatment of BPD-PH with pulmonary vasodilators was well tolerated and led to a clinical improvement with the possibility of discontinuation without recurrence of PH. Prognostic factors used in pediatric PAH risk stratification also seem to be useful in assessing treatment efficacy and prognosis in patients with BPD-PH.

Which Functional Classification Scale is Optimal for Children with Pulmonary Hypertension (PAH)?

Functional status assessed by the WHO-FC scale derived from adults is a known prognostic factor for pulmonary hypertension. Data on the usefulness of the Panama-FC scale in assessing children with pulmonary hypertension are limited. The study was performed to compare functional status results (WHO-FC and Panama-FC) and to assess the usefulness of these scales in various clinical situations. The reliability of the Panama-FC questionnaire method for facilitating patient evaluation was also examined. 26 functional status assessments (7 in disease progression/after treatment intensification) in both scales were analyzed in 19 patients with PAH confirmed in RHC. WHO-FC, Panama-FC scales, and questionnaire-based on Panama-FC were conducted independently by three different physicians. Results of assessments were compared with each other and with 6MWD, NTproBNP level, and echo parameters (TAPSE, RV/LV ratio). The Panama-FC scale results obtained using the medical interview method and questionnaire did not differ. Both WHO-FC and Panama-FC classes well-reflected disease advancement confirmed by non-invasive parameters (NTproBNP, 6MWD, TAPSE, RV/LV ratio). Differences between grading the class in both scales were observed: 5pts were classified to II (Panama-FC) vs I (WHO-FC), 2pts were in lower risk group in WHO-FC (II) vs Panama (IIIa). Worsening or improvement after treatment intensification in functional status in both scales was connected with the significant change of NTproBNP level. The 6-min walking distance did not change. TAPSE, RV/LV ratio changed significantly in 3pts with IPAH, accordingly to change in WHO-FC and Panama-FC. WHO-FC and Panama-FC well reflect the disease advancement. The questionnaire method simplified the use of the Panama-FC scale. The Panama-FC scale appears to be better for assessing functional status during long-term follow-up, while the WHO-FC scale was more useful in short-term treatment monitoring.

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.

Usefulness of Red Cell Width Distribution (RDW) in the Assessment of Children with Pulmonary Arterial Hypertension (PAH).

Red cell width distribution (RDW) is known to be a prognostic marker in adults with pulmonary hypertension. The value of this test in the pulmonary arterial hypertension (PAH) pediatric population was not yet established. The aim of the study was to evaluate the prognostic value of RDW in children with PAH and utility of this parameter in the management. Data were collected retrospectively in 61 patients with PAH confirmed by right heart catheterization. RDW was measured at diagnosis, 3 and 12 months after initial therapy, during and after deterioration if occurred. Results were compared with NTproBNP, WHO-FC and oxygen blood saturation. Mean RDW at baseline was 15.3 ± 2.4% (12.1-24.4, median 14.7%) and was elevated in 29 patients (47%). There were no significant difference in clinical status, NTproBNP and hemodynamic parameters among patient with normal and elevated RDW at diagnosis. Poor negative correlation with SaO2 and SvO2 was shown. After 3 and 12 months of treatment no significant change of RDW level was found despite of statistically significant improvement of WHO-FC and decrease of NTproBNP level (NS). Episodes of clinical deterioration weren't connected with change of RDW level (16 vs. 15.6% NS). Kaplan-Meier analysis did not show differences in prognosis between patients with normal and elevated RDW. Elevation of RDW was not associated with any measured parameters. Prognostic value of RDW in the pediatric PAH population was not confirmed. Usefulness of RDW in management in PAH pediatric population is limited and required further studies.

Halitosis in children with adenoid hypertrophy.

This paper attempts to identify the sources and evaluate the prevalence of halitosis in children with adenoid hypertrophy. The study included pediatric patients admitted for adenoidectomy due to obstructive symptoms. Patients with possibly other causes of halitosis, were excluded from the study. Halitosis was detected in 30 out of 136 children (22.1%). The effect of adenoid hypertrophy on halitosis was confirmed by the significant reduction in the organoleptic score rated according to the Rosenberg scale (on average by 2°) and the levels of volatile sulfur compounds (on average 84 ppb) post-adenoidectomy, as compared to pre-surgery results. In total, we reported a statistically significant decrease in the abovementioned values in 90% of patients with halitosis. It was found that in patients with halitosis, as compared to patients with no oral malodor, anaerobic bacteria and Staphylococcus aureus were more frequently observed in the adenoid tissue. Streptococcus oralis strains were more commonly reported in the material taken during adenoidectomy in children without oral malodor.

Six-Minute Walk Test in Evaluation of Children with Pulmonary Arterial Hypertension.

Six-minute walk test (6MWT) is a submaximal exercise test applied for evaluation of adults with pulmonary arterial hypertension (PAH). It was widely used as an endpoint in the clinical trials. The aim of the study was to assess the usefulness of 6MWT in management of children with PAH and to establish correlations with other clinical features. 164 6MWT were performed in 15 children between 5 and 18 years with PAH confirmed by right heart catheterization (102 in patients with shunt, 62 without shunt). Distance in 6MWT (6MWD)-% of predicted for age and gender, desaturation at the maximum effort, peak heart rate (HR)-% of maximal HR, were compared to the level of NTproBNP, WHO-FC, echocardiography parameters, and events of PAH treatment intensification. 6MWD had low negative correlation with peak HR (τ -0.1 p = 0,03), negative correlation with NTproBNP (τ -0.17 p = 0.002), and no dependence on echocardiography parameters. The presence of shunt was associated with lower 6MWD, lower blood saturation at rest, and higher desaturation after effort. Patients in III/IV WHO-FC achieved higher rest HR and maximal HR in comparison to patients in I/II WHO-FC (63.1 vs. 55.2% p < 0.01) and lower 6MWD (64.3 vs. 77.5% p < 0.01). In 14 out of 20 6MWT performed after treatment intensification, increase of distance was observed. The results of 6MWT were consistent with clinical status (WHO-FC, NTproBNP) but not with echocardiography parameters. 6MWT may be the source of additional information in management of children with PAH.

Treatment initiation in paediatric pulmonary hypertension: insights from a multinational registry.

Different treatment options for pulmonary hypertension have emerged in recent years, and evidence-based management strategies have improved quality of life and survival in adults. In children with pulmonary vascular disease, therapeutic algorithms are not so clearly defined; this study determined current treatment initiation in children with pulmonary hypertension in participating centres of a registry. Through the multinational Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension registry, patient demographics, diagnosis, and treatment as judged and executed by the local physician were collected. Inclusion criteria were >3 months and <18 years of age and diagnostic cardiac catheterisation consistent with pulmonary hypertension (mean pulmonary arterial pressure ⩾25 mmHg, pulmonary vascular resistance index ⩾3 Wood units×m2, and mean pulmonary capillary wedge pressure ⩽12 mmHg). At diagnostic catheterisation, 217/244 patients (88.9%) were treatment naïve for pulmonary hypertension-targeted therapy. Targeted therapy was initiated after catheterisation in 170 (78.3%) treatment-naïve patients. A total of 19 patients received supportive therapy, 28 patients were not started on therapy, and 26 patients (10.7%) were on targeted treatment before catheterisation. Among treatment-naïve subjects, treatment was initiated with one targeted drug (n=112, 51.6%), dual therapy (n=39, 18%) or triple-therapy (n=5, 2.3%), and calcium channel blockers with one targeted medication in one patient (0.5%). Phosphodiesterase inhibitors type 5 were used frequently; some patients with pulmonary hypertension related to lung disease received targeted therapy. There is a diverse therapeutic approach for children with pulmonary hypertension with a need of better-defined treatment algorithms based on paediatric consensus for different aetiologies including the best possible diagnostic workup.

Prognosis in children with pulmonary arterial hypertension: 10-year single-centre experience.

BACKGROUND: Pulmonary arterial hypertension (PAH) is a rare progressive disease of the pulmonary arterioles with an unfavourable prognosis. AIM: To evaluate survival and prognostic factors in patients with PAH diagnosed and treated at a single centre in the years 2004­2013. METHODS: The study included 55 children (33 girls; 66%, 22 boys; 33%), with an average age 6.2 ± 6.0 years, with idiopathic PAH ­ n = 23 (42%), PAH associated with systemic-to-pulmonary shunts ­ n = 17 (31%), and PAH after corrective cardiac surgery ­ n = 15 (27%). Forty-seven of them (87%) were treated with advanced therapy. RESULTS: During the follow-up with an average time of 5.6 ± 4.7 years 15 (27.3%) children died. The one-, three-, five-, and ten-year survival was, respectively, 83.1%, 77.1%, 70.7%, and 65.2%. The analysis of the survival curves revealed a better prognosis in patients with baseline N-terminal pro-B-type natriuretic peptide (NT-proBNP) level < 605 pg/mL (p = 0.024) and a higher probability of survival of three and five years in children at baseline I/II World Health Organisation functional class (WHO-FC). The higher risk of death was associated with a higher pressure in the right atrium (HR 1.23, p < 0.01) and higher pulmonary resistance (HR 1.1, p < 0.01), whereas no history of syncope had a better prognosis (HR 0.31, p = 0.03). CONCLUSIONS: Survival in the study group was comparable to the currently published register data. Mortality risk factors were connected with the severity of the disease at diagnosis.

[The influence of age, place of living, education and number of earlier pregnancies on attendance of pregnant women to screening tests--questionnaire study]. / Wplyw wieku, miejsca zamieszkania, wyksztalcenia i Iiczby wczesniejszych ciaz na zglaszalnosc ciezarnych do badan przesiewowych- badanie ankietowe.

UNLABELLED: The aim of this study was to assess attendance at the screening programme in pregnancy and the influence of age, number of past pregnancies, level of education and place of residence on the attendance. MATERIAL AND METHODS: Our study was performed on the basis of an anonymous questionnaire handed out 543 women aged 16-45, on the third day of their puerperal, in one of the five obstetric wards in southern Poland. The questionnaire contained questions about participation in recommended for pregnant women screening tests such as: fasting blood glucose level measurement, oral glucose tolerance test, blood type test, measurement of hepatitis B surface antigen and antibodies to VDRL, Rubella, Toxoplasma gondii, hepatitis C virus at least once during pregnancy. RESULTS: The highest attendance rate was related with blood type test, whereas the lowest was related with measurement of antibodies to hepatitis C virus (95.6% vs 22.7%, p < 0.001). A very low percentage of pregnant patients measured Rubella antibodies (29.1%). A larger proportion of the respondents checked antibodies against Toxoplasma gondii (41.6%). The attendance at fasting blood glucose level was 66.9 % and at oral glucose tolerance test was 63.7%. The attendance according as age, place of living, number of past pregnancies and level of education was described in detail. CONCLUSION: Despite current recommendations of Polish Gynecological Society and the ordinance of polish Minister of Health the percentage of women participating in screening tests during pregnancy is still insufficient. Age, place of residence and education remain strong factors influencing attendance at the screening programme in pregnancy.

Bilateral facial palsy in rapidly progressive course of Wegener's granulomatosis: a case report.

Introduction. Wegener's granulomatosis belongs to a group of systemic vasculitis diseases, which is characterized by necrotizing vasculitis and presence of granulomas. In a lot of cases, the first symptoms of the disease are observed in the head and neck region, but the bilateral facial nerve palsy occurs very rarely. Objective. The objective of our report was to describe the unusual course of Wegener's granulomatosis with the bilateral facial nerve paralysis, which subsided after application of steroids and immunosuppressive therapy in combination with surgical treatment. Results and Conclusions. Hearing loss may precede other symptoms in Wegener's granulomatosis. Ear pain and otorrhea may suggest the diagnosis of bacterial purulent otitis media and delay the proper diagnosis. In the presented case, considering the clinical course, it was necessary to apply both pharmacological and surgical treatments.

Early screening for critical congenital heart defects in asymptomatic newborns in Mazovia province: experience of the POLKARD pulse oximetry programme 2006-2008 in Poland.

BACKGROUND: Early diagnosis of critical congenital heart defects (CCHD) may be missed both during prenatal echocardiography and the short stay in the neonatal nursery, leading to circulatory collapse or death of the newborn before readmission to hospital. AIM: To assess the usefulness of pulse oximetry as a screening test in early diagnosis of CCHD in newborns. METHODS: A prospective screening pulse oximetry test was conducted in 51 neonatal units in the Mazovia province of Poland as part of the POLKARD 2006-2008 programme between 16 January, 2007 and 31 January, 2008. Newborns with no circulatory symptoms or coexisting diseases, and no prenatal diagnosis, were enrolled. The test was performed between the 2(nd) and 24(th) hours of life in stable newborns. A double arterial oxygen saturation (SpO(2)) reading < 95% on a lower extremity led to cardiovascular evaluation and echocardiography. RESULTS: From a population of 52,993 newborns (14.2% of births in Poland), a group of 51,698 asymptomatic infants was isolated. CCHD was diagnosed solely by pulse oximetry in 15 newborns, which constituted 18.3% of all CCHD; 14 (0.026%) false positives were obtained and there were four false negative results. The sensitivity of the test was 78.9% and specificity 99.9%. The positive predictive value was 51.7% and negative 99.9%. CONCLUSIONS: Pulse oximetry fulfilling the screening test criteria, performed on a large population of newborns in Poland, proved useful in supporting prenatal diagnostics and postnatal physical examination in the early detection of initially asymptomatic CCHD. Good sensitivity and specificity results of the pulse oximetry test have allowed it to be recommended for use in neonatal units nationwide.

[Coexistence of congenital heart diseases with other congenital defects]. / Wspólistnienie Wrodzonych Wad Ukladukrazenia Z Innymi Wadami Rozwojowymi.

AIM: Evaluating the prevalence and type of non-heart malformations in patients hospitalised in the Department of Pediatric Cardiology of the Children's Memorial Health Institute due to congenital pathology of the circulatory system; comparing the frequency of other congenital malformation in the group researched in relation to the population overall and evaluating their relevance to survival. MATERIAL AND METHODS: Research included 266 infants with heart defects. The patients' data were analysed in retrospect on the basis of medical records, taking into account the kind of heart defect, defects of other systems, and mortality in the first year of life. The frequency of non-heart congenital defects were compared with data from the Polish Registry of Congenital Malformations. RESULTS: In 71 children among 266 patients congenital defects of other systems were diagnosed. The group researched presented a significantly greater occurrence of such defects than the population overall (26.7% vs 1.81%). The most frequent diagnoses were: 1. chromosomal abnormalities (N=26; 9.77%), 2. malformations of the digestive system (N=7; 3.01%), 3. syndromes of multiple congenital defects (N=6 ; 2.26%). The defects usually coexisted with the atrio-ventricular septal defect (18 out of 22 patients - 81.8%) and rarely in neonates with duct-dependent defects (19 out of 122 patients - 15.6%). 32 children died, of whom 10 had congenital non-heart malformation in addition to congenital heart defects. No significant difference in the frequency of death was found in children who presented only with heart pathology in comparison to those with other defects (11.2% vs 14%). CONCLUSIONS: In patients with congenital heart defects other congenital malformations occurred more often than in all the population, especially chromosomal abnormalities, malformations of the digestive system and congenital malformation syndromes. Congenital malformations were not connected with higher mortality of children with heart defects.

Age, place of living and education influences the pregnancy universal thyroid function screening program attendance - questionnaire study.

BACKGROUND: The aim of this study was to assess attendance at the universal screening programme for thyroid function in pregnancy and attempt to evaluate the influence of age, number of past pregnancies, level of education, and place of residence on the attendance. The study was performed by means of a questionnaire. MATERIAL AND METHODS: Our study was performed on the basis of an anonymous questionnaire handed out to 543 women aged 16-45 years, on the third day of their puerperal stay in one of five obstetric wards in southern Poland. The questionnaire contained questions about participation in plasma level measurements of TSH, fT4, total T4, thyroid antibodies or thyroid ultrasound scanning at least once in pregnancy. RESULTS: The rate of attendance at any examination of thyroid function among pregnant women was 26.7%. The highest attendance rate (32.7%) was found among women living in provincial capitals or with higher education (41.3%), whereas the lowest was among women who had completed only primary school (11%) and those living in county towns (15%). The number of previous pregnancies did not influence the thyroid screening attendance. Women over 21 years of age participated in this screening programme more frequently (27.1-30%). CONCLUSION: Less than one third of pregnant women participated in the thyroid function screening. Place of living, education level, and age were the main factors influencing the attendance rate.