Anomalous origin of left coronary artery in young athletes with syncope.
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Biomedical subjects
Publications and source records attributed to Matthias Gass.
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INTRODUCTION: Recurrent but infrequent syncopes represent a diagnostic challenge, since they frequently remain unexplained despite extensive investigations. This applies specifically for patients who carry an increased risk of potentially lifethreatening arrhythmias, either due to congenital cardiac disease or primary electrical disorders. Implantable loop recorders permit long-term electrocardiographic monitoring. Experience with these devices is still limited in children. PATIENTS AND METHODS: Between January 1999 and August 2005, 12 patients underwent implantation of a loop recorder in our tertiary referral centre. The mean age was 10.9 years, with a range from 2 to 17 years. Of the patients, 6 had structural disease, 3 had primary electrical abnormalities, and 3 had no cardiovascular disease. RESULTS: Resyncope occured in 9 of the 12 patients. Arrhythmic origin of the syncope was diagnosed in 4 of these patients. The events recorded were ventricular fibrillation in 2, intermittent asystole in 1, and pacemaker-syndrome in the other patient. Malignant arrhythmia was ruled out in the remaining 5 patients. There were no complications related to implantation of the loop recorder, and the mean duration until explantation was 8.3 months. CONCLUSIONS: Based on our experience, we suggest that implantation of a loop recorder represents an additional tool for a selected group of children. Due to its invasive nature, it should be restricted to patients at high risk, or those in which there is substantial clinical suspicion of the likelihood of serious arrhythmias when conventional testing has been inconclusive. In this cohort, implantation of the loop recorder either helps to establish the correct diagnosis, or to exclude an arrhythmic event, thus avoiding unnecessary escalation of therapy and providing reassurance for the family.
UNLABELLED: We report our single centre experience with a new fractally coated myocardial unipolar lead (ELC35UP; Biotronik) in 96 pediatric patients (59% male, 41% female). Congenital heart disease (CHD) was associated in 89%. The age at implantation ranged between 2 days and 19 years, median for children with CHD 7.8 years, without CHD 4.7 years. Twenty percent of the children were younger than one year at implantation. Mean follow-up was 30 months (1-57 months). We compared our findings with a steroid eluting epicardial lead (CapSure EPI 4968; Medtronic) in 46 children with comparable age and sex-distribution. We found a lead survival of 87% after 57 months in the ELC35UP group (steroid lead: 87% after 129 months). Pacing energy thresholds were equal after 12 months (median 3.0 microjoules), but the sensing characteristics of the fractally coated lead was significantly superior to the steroid eluting lead with median R waves of 7.0 mV (steroid lead: 3.5 mV) after 12 months. Children with myocardial scar tissue requiring pacemaker therapy after surgery of CHD showed no differences in sensing and pacing thresholds in comparison to children with congenital rhythm disorders. The fractally coated screw-in lead offers technical advantages concerning the subxiphoidal implantation procedure. CONCLUSION: Fractally coated ventricular screw-in leads represent a feasible alternative to the common steroid eluting leads- especially in children requiring pacemaker therapy after surgery for CHD.
We report the observation and analysis of a new adverse event during the insulin tolerance test (ITT) and propose additional safety procedures. An 8-year-old girl with growth hormone insufficiency had a cardiac arrest due to ventricular flutter when she was tested for growth hormone deficiency by the ITT. Severe hypokalaemia (K+ 2.6 mmol/l) was observed after resuscitation. Ergometry ECG revealed catecholaminergic polymorphic ventricular tachycardia, a hereditary arrhythmogenic disease. Consecutive measurements of serum potassium during ITT in 29 short children (21 boys) with growth failure revealed a mean decrease of serum potassium by 1.1 +/- 0.4 mmol/l with the nadir at 30 min after the insulin bolus. Hypokalaemia (serum potassium < 3.5 mmol/l) occurred in all but one child; severe hypokalaemia (serum potassium < 2.9 mmol/l) was measured in every third child. This observation indicates that acute hypokalaemia which is induced by insulin and catecholamine excess occurs frequently in ITT. The case shows that the combination of acute hypokalaemia and the adrenergic counterregulation in ITT is a strong trigger of cardiac arrhythmias, which can become life-threatening if the child has an arrhythmogenic disease. Therefore, we recommend ECG monitoring during ITT to enhance the detection of cardiac arrhythmias. In addition, in the case of a comatose child during ITT the determination of the glucose and potassium level as well as adequate treatment are necessary.
Bradyarrhythmias developing after Fontan-type operations impair the function of the univentricular heart causing fatigue, headaches, ascites, and protein-losing enteropathy (PLE). Transvenous inaccessibility, requiring epicardial implantation, accounts for the reluctance to implant a pacemaker (PM). Between 1997 and 2000, 24 patients (mean age 9.5 years, range 6 months to 19 years) with Fontan-type operations received DDD pacing systems with atrial steroid-eluting stitch-on electrodes (mean capture threshold 1.9 V/0.5 ms, range 0.4-3.5 V) and ventricular screw-in electrodes (mean capture threshold 1.7 V/0.5 ms, range 0.1-3 V). The systems were implanted at the time of conversion from atrio- to cavopulmonary connections in 5 patients, at the time of a total cavopulmonary Fontan operation in 6, and 1-50 months thereafter (mean = 18) in 13 patients. A right ventricular anatomy was present in 13 (54%) of 24 of PM recipients, versus 35% of the overall population. After a mean follow-up of 3.5 years, the PM were functioning in DDD mode in 23 of the 24 patients. Length of hospital stay in the ten patients who underwent repeat sternotomy was 5 days, without procedure related complications. In three children a repeat sternotomy was avoided by implanting the atrial electrodes during the Fontan operation. All patients improved clinically, including resolution of PLE in four patients. Bradyarrhythmias may lead to significant morbidity after Fontan-type operations. Electrophysiological evaluation is advised at follow-up. The indication for implantation of a DDD pacemaker system should be liberal. Placing atrial electrodes during the Fontan operation, especially in the presence of a right ventricular anatomy, avoids repeat sternotomy.