Utility of serial 12-lead electrocardiograms in children with Marfan syndrome.

OBJECTIVES: The goal of this study was to assess the utility of serial electrocardiograms in routine follow-up of paediatric Marfan patients. METHODS: Children ⩽18 years who met the revised Ghent criteria for Marfan syndrome and received a 12-lead electrocardiogram and echocardiogram within a 3-month period were included. Controls were matched by age, body surface area, gender, race, and ethnicity, and consisted of patients assessed in clinic with a normal cardiac evaluation. Demographic, clinical, echocardiographic, and electrocardiographic data were collected. RESULTS: A total of 45 Marfan patients (10.8 [2.4-17.1] years) and 37 controls (12.8 [1.3-17.1] years) were included. Left atrial enlargement and left ventricular hypertrophy were more frequently present on 12-lead electrocardiogram of Marfan patients compared with controls (12 (27%) versus 0 (0%), p<0.001; and 8 (18%) versus 0 (0%), p=0.008, respectively); however, only two patients with left atrial enlargement on 12-lead electrocardiogram were confirmed to have left atrial enlargement by echocardiogram, and one patient had mild left ventricular hypertrophy by echocardiogram, not appreciated on 12-lead electrocardiogram. QTc interval was longer in Marfan patients compared with controls (427±16 versus 417±22 ms, p=0.03), with four Marfan patients demonstrating borderline prolonged QTc intervals for gender. CONCLUSIONS: While Marfan patients exhibited a higher frequency of left atrial enlargement and left ventricular hypertrophy on 12-lead electrocardiograms compared with controls, these findings were not supported by echocardiography. Serial 12-lead electrocardiograms in routine follow-up of asymptomatic paediatric Marfan patients may be more appropriate for a subgroup of Marfan patients only, specifically those with prolonged QTc interval at their baseline visit.

The 2017 Seventh World Congress of Pediatric Cardiology & Cardiac Surgery: week in review: imaging.

The Imaging Program at the 7th World Congress highlighted the versatility and diagnostic power of the current and upcoming imaging tools in Pediatric Cardiology and Cardiac Surgery. Several experts presented interesting as well as practical data on the use of 2D and 3D Echocardiography, magnetic resonance imaging and computed tomography in the fetus, child, and adult with congenital heart disease. Bridging sessions coupled use of these imaging modalities and screening practices in patients with acquired heart disease. Hot topics included nomenclature of ventricular septal defects, the challenging diagnosis of double outlet right ventricle, cardiac tumors, and imaging of aortapathies. Several talks concentrated on the quantitative assessment of ventricular function and reviewed numerous exciting new modalities that currently serve as research tools. In summary, Imaging Sessions truly represented how far we have advanced the field of Imaging in Pediatric Cardiology and Cardiovascular Surgery.