ObjectiveCardiac pacing is sometimes required for patients with congenital heart disease for various reasons. Because of complex anatomy, repetitive previous heart surgery and patient size, epicardial leads are of special importance in these patients. Using epicardial leads has been discussed controversly regarding pacing performance and lead survival. The aim of this study was to review the long-term data on pacing performance as well as lead survival of epicardial leads implanted in our center.MethodsRetrospective chart review and review of the literature.Results82 consecutive pediatric patients or adult patients with congenital heart disease with 158 epicardial leads (Medtronic 4968, bipolar, steroid - eluting) were included. We found 1.2% pacemaker-related early postoperative complications. The incidence of lead dysfunction was 7.5% (12/158) for primary (i.e. directly related to the lead itself) lead dysfunction and 3.2% (5/158) of lead abandonment for reasons not directly related to the lead itself. Primary lead dysfunction occured after a median of 3.83 years. Lead survival at 2, 5 and 10 years was 98.7%, 93% and 92.4%. There were no infections reported. Stable median measurements for impedance (RA/RV/LV of 577/483/610 Ohm), sensing threshold (RA/RV/LV of 2.0/11.0/10.0 mV) and pacing threshold (RA/RV/LV of 0.75 V at 0.4 ms/1.0 V at 0.49 ms/1.0 V at 0.45 ms) indicated a good mid- to longterm performance. The only risk factor for primary lead dysfunction was young age at implantation.ConclusionThe use of epicardial leads in pediatric and adult patients with congenital heart disease shows good longterm outcomes in terms of pacing performance and lead survival. The authors encourage using epicardial leads in patients with congenital heart disease based on the patient‘s individual characteristics.

A secundum atrial septal defect (ASD 2) was closed percutaneously via the transfemoral approach in a 3-year-old boy with interrupted inferior vena cava and azygos continuation. The procedure was guided by transesophageal echocardiography and fluoroscopy using conscious sedation. Successful transhepatic and jugular accesses in similar patients are described. This is the first pediatric report describing a transfemoral closure of a secundum atrial septum defect via azygos continuation.

Postero-septal accessory pathways (AP) are a rare cause of intraventricular dyssynchrony and severe LV dysfunction in children. Beside the common treatment with radiofrequency ablation of septal substrates we present the case of a successful pharmacologic resynchronization in a 13/12 years old male toddler with Wolff-Parkinson-White syndrome (WPW) and severe LV dysfunction (left ventricular biplane EF of 31 %) due to intraventricular dyssynchrony with septal to posterior wall motion delay (SPWMD) of 350 ms. Interventricular mechanical delay (IVMD) was 65 ms. Using propafenone, pharmacologic cardiac resynchronization could be achieved. Pharmacologic resynchronization should be considered as safe and effective alternative to catheter ablation in very young children.

A 14-year-old boy with a heart murmur was referred to the authors' department because structural heart disease could not be ruled out by standard echocardiographic views. The best apical four-chamber view was obtained with the patient turned to a right lateral decubitus position and the transducer shifted almost to the posterior axillary line. A biplane chest x-ray also showed a counterclockwise heart axis deviation. Magnetic resonance imaging confirmed the suspected congenital absence of the pericardium.

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An acute rupture of the ascending aorta occurred in a 12-year-old boy after balloon angioplasty and was successfully treated by emergency covered stent implantation during resuscitation and preparation for support with extracorporeal membrane oxygenation. Neuroprotection with sedation, core temperature cooling and mild hyperventilation were undertaken. The boy recovered quickly. There were no neurological sequalae with a good interventional result at discharge and at follow-up after 9 months.

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