Agnieszka Tomik

Bland-White-Garland syndrome – a rare and serious cause of failure to thrive

Patient: Male, 0 Final Diagnosis: Bland-White-Garland syndrome Symptoms: Cardiomegaly, feeding problems Medication: — Clinical Procedure: Reimplantation of the left coronary artery to the aorta Specialty: Pediatrics and Neonatology Objective: Rare disease Background: Bland-White-Garland syndrome (BWGS) is a very rare disease characterized by anomalous origin of the left coronary artery from the pulmonary trunk (ALCAPA). WBGS affects 1 in every 300 000 live births. Children typically present with dyspnea, pallor, and failure to thrive. Without surgical repair, most of these children die during the first months of life. Case Report: This case report describes 3-month-old boy admitted to the hospital because of feeding problems. The boy was born at term, with birth weight 3200 g, and was 10 points in Apgar score. He was breast-fed from birth. From the seventh week of age, his mother observed his increasing difficulties with feeding. Physical examination revealed pale skin, diminished heart sounds, tachycardia, cardiomegaly, and hepatomegaly. Results of urine and blood tests and ultrasonography of the central nervous system and abdomen were normal. The chest radiography showed cardiomegaly and electrocardiogram revealed anterolateral myocardial infarction. On echocardiography, an anomalous left coronary artery arising from the pulmonary artery was found. The life-saving treatment of choice was immediate surgical reimplantation of the left coronary artery to the aorta. Conclusions: Children with congenital heart disease are often prone to malnutrition, but in rare cases failure to thrive and breast-feeding problems can be the first symptoms of life-threatening diseases like myocardial infarction secondary to Bland-White-Garland syndrome (BWGS).

Normative values for heart rate variability parameters in school-aged children: simple approach considering differences in average heart rate.

Background: Heart rate variability (HRV) analysis is a clinical tool frequently used to characterize cardiac autonomic status. The aim of this study was to establish normative values for short-term HRV parameters by considering their main determinants in school-aged children. Methods: Five-minute electrocardiograms were taken from 312 non-athlete children (153 boys) at age of 6 to 13 years for computation of conventional time- and frequency-domain HRV parameters. Heart rate (HR), respiratory rate, age, body mass index, and sex were considered as their potential determinants. Multiple regression analysis revealed that HR was the principal predictor of all standard HRV indices. To develop their universal normative limits, standard HRV parameters were corrected for prevailing HR. Results: The HRV correction for HR yielded the parameters which became independent on both sex and HR, and only poorly dependent on age (with small effect size). Normal ranges were calculated for both time- and frequency-domain indices (the latter computed with either fast Fourier transform and autoregressive method). To facilitate recalculation of standard HRV parameters into corrected ones, a calculator was created and attached as a Supplementary Material that can be downloaded and used for both research and clinical purposes. Conclusion: This study provides HRV normative values for school-aged children which have been developed independently of their major determinants. The calculator accessible in the Supplementary Material can considerably simplify determination if HRV parameters accommodate within normal limits.

Transient complete right bundle branch block following right heart catheterization in children – a report of two cases

Corresponding author: Prof. Bożena Werner MD, PhD, Department of Pediatric Cardiology and General Pediatrics, Medical University of Warsaw, 63 A Żwirki i Wigury St, 02-091 Warsaw, Poland, phone: +48 22 317 95 75, e-mail: bozena.werner@wum.edu.pl Accepted: 19.01.2016, accepted: 2.06.2016. Transient complete right bundle branch block following right heart catheterization in children – a report of two cases