Publications by authors named "Małgorzata Zuk"

15 Publications

  • Page 1 of 1

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

Children (Basel) 2021 Apr 22;8(5). Epub 2021 Apr 22.

Department of Cardiology, The Children's Memorial Health Institute, 04-730 Warsaw, Poland.

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.
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http://dx.doi.org/10.3390/children8050326DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8145230PMC
April 2021

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).

J Clin Med 2021 Apr 12;10(8). Epub 2021 Apr 12.

Pulmonary Circulation Centre, Department of Cardiac and Vascular Diseases, Faculty of Medicine, Medical College Jagiellonian University, John Paul II Hospital in Krakow, u. Pradnicka 80, 31-202 Krakow, Poland.

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 ( = 55; 94.8%) at a mean age of 40.6 ± 6.9 years. Patients (children) were both females ( = 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 ( = 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 ( = 20; 34.5%) and concern ( = 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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http://dx.doi.org/10.3390/jcm10081640DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8069741PMC
April 2021

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

Pediatr Cardiol 2020 Dec 9;41(8):1725-1729. Epub 2020 Aug 9.

Department of Cardiology, The Children's Memorial Health Institute, Al. Dzieci Polskich 20, 04-730, Warsaw, Poland.

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.
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http://dx.doi.org/10.1007/s00246-020-02434-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7695665PMC
December 2020

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

J Clin Med 2020 Jun 3;9(6). Epub 2020 Jun 3.

Department of Cardiac and Vascular Diseases, Jagiellonian University Medical College, John Paul II Hospital in Krakow, 31-202 Krakow, Poland.

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, = 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 ( = 20; 25%), mental retardation ( = 32; 40%), hypothyroidism ( = 19; 23.8%) and Down syndrome ( = 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.
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http://dx.doi.org/10.3390/jcm9061717DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7356296PMC
June 2020

Fatal thromboembolism of major aortopulmonary collateral arteries.

Kardiol Pol 2019 May 24;77(5):574-575. Epub 2019 May 24.

Department of Cardiology,The Children’s Memorial Health Institute, Warsaw, Poland

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http://dx.doi.org/10.33963/KP.14847DOI Listing
May 2019

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

Pediatr Cardiol 2019 Apr 4;40(4):820-826. Epub 2019 Mar 4.

Department of Cardiology, The Children's Memorial Health Institute, Al. Dzieci Polskich 20, 04-730, Warsaw, Poland.

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 SaO and SvO 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.
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http://dx.doi.org/10.1007/s00246-019-02077-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6451723PMC
April 2019

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

Pediatr Cardiol 2017 Apr 27;38(4):754-761. Epub 2017 Feb 27.

Department of Cardiology, The Children's Memorial Health Institute, Al. Dzieci Polskich 20, 04-730, Warsaw, Poland.

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.
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http://dx.doi.org/10.1007/s00246-017-1575-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5388718PMC
April 2017

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

Cardiol Young 2017 Aug 20;27(6):1123-1132. Epub 2016 Dec 20.

9Great Ormond Street Hospital for Sick Children,London,United Kingdom.

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.
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http://dx.doi.org/10.1017/S1047951116002493DOI Listing
August 2017

[The influence of age, place of living, education and number of earlier pregnancies on attendance of pregnant women to screening tests--questionnaire study].

Przegl Lek 2015 ;72(5):257-62

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.
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February 2016

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

Kardiol Pol 2016 ;74(2):159-167

Department of Cardiology, The Children's Memorial Health Institute, Warsaw, Poland.

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 2004–2013.

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.
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http://dx.doi.org/10.5603/KP.a2015.0120DOI Listing
February 2017

Potts shunt in child with suprasystemic pulmonary arterial hypertension.

Kardiol Pol 2015 ;73(3):221

Department of Cardiology, The Children's Memorial Health Institute, Warsaw, Poland.

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http://dx.doi.org/10.5603/KP.2015.0046DOI Listing
February 2017

[Coexistence of congenital heart diseases with other congenital defects].

Med Wieku Rozwoj 2012 Oct-Dec;16(4):284-9

Klinika Kardiologii, Instytut-Pomnik Centrum Zdrowia Dziecka, Al. Dzieci Polskich, Warszawa.

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.
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April 2013

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

Kardiol Pol 2012 ;70(4):370-6

Department of Cardiology, Children's Memorial Health Institute, Warsaw, 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.
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July 2012

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

Endokrynol Pol 2011 ;62(5):416-20

Department of Gynaecological Endocrinology, Jagiellonian University Medical College, Krakow, Poland.

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.
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April 2012

[The significance of cardiac symptoms in patients referred to pediatric cardiology outpatient clinics].

Med Wieku Rozwoj 2005 Apr-Jun;9(2):139-51

Klinika Kardiologii, Instytut Pomnik Centrum Zdrowia Dziecka, Al. Dzieci Polskich 20, 03-719 Warszawa, Poland.

Objective: To determine the significance of cardiac symptoms in patients referred to paediatric cardiology outpatient clinics.

Material And Methods: All patients above l month of age referred to paediatric cardiac outpatient clinics between 01-Apr-2004 and 31-Dec-2004. Data was collected prospectively in Internet data base. Patients were divided into 3 groups: patients referred by paediatricians to regional paediatric cardiology outpatient clinic (group 1, N=3383), patients referred to Academic Paediatric Cardiac outpatient clinics by paediatricians (group 2, N= 7461) and by cardiologists (group 2a, N=793).

Results: Average age of patients was 6.4 +/- 5.8 years. The most common reasons for referral included cardiac murmur, chest pain, syncope, earlier diagnosed congenital heart disease (CHD). The proportion of patients referred by cardiologists and paediatricians because of murmur was 30% vs 56%, arrhythmia 12.6% vs 8% and CHD 44% vs 8%. The percentage of the significant cardiac pathology in all groups (l/ 2/ 2a) was 38/35/76; in patients with chest pain -- 9/18/0; with syncope -- 56/70/80; with murmur -- 19/21/43; with CHD -- 68/69/93 and with arrhythmia -- 57/80/92.

Conclusions: l. There were no significant differences between patients referred by paediatricians from regional and academic outpatient cardiology clinics. 2. Isolated cardiac symptoms such as chest pain, cardiac murmur and syncope were caused by significant cardiac pathology only in about 1/5 cases. 3. Cardiac murmur was the most common reason for referral to cardiac outpatient clinic in younger patients, in older group syncope and arrhythmia were more frequent.
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October 2005