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Background Cardiac pacing can be challenging after a Fontan operation, and limited data exist regarding strategies to plan these epimyocardial systems while minimizing the number of surgical procedures. Methods A retrospective review of all our 47 patients (mean age 18 years, standard deviation 9 years) with a Fontan palliation who received an epimyocardial cardiac implantable electronic device (CIED) between 2002 and 2020 with regard to the stability of the epimyocardial lead parameters and the incidence of system revisions. Results Over the last 18 years, 84 implantations or revisions of the epimyocardial CIED in 47 Fontan patients were performed. Mean age at time of the first implantation was 9.4 (range 0.28-29.3) years. Follow-up period ranges from 0.11 to 18.2 (mean 7.7, standard deviation 4.2) years. A total of 123 pacing leads were implanted of which 99 are still active. From 2010 triple lead cardiac resynchronization devices were used in 17 patients to better cope with lead problems. The initial pacing threshold of the leads inactivated during this study period proved significantly higher (mean 1.66 V) than in the "all leads" group (mean 1.27 V, p = .0005) or the group of the still active leads (mean 1.17 V, p = .00004). Conclusions When implanted with a low pacing threshold, the bipolar epimyocardial electrodes show stable and good long-term results in young patients with a Fontan circulation. Resynchronization pacing systems and the prospective implantation of reserve leads may help to reduce the rate of resternotomies and provide a flexible concept to deal with lead failure.

Temporary intermittent complete heart block (CHB) occurred the day after interventional closure of an ASD with a 30 mm Gore Septal Occluder (GSO™) in a 2 years and 11-month-old female. CHB disappeared without further treatment and stable sinus rhythm recovered within 3 days. Only short episodes of 2nd degree AV-block (Wenckebach periodicity) at rare intervals were documented in Holter-monitors the following 2 months. Eleven months after device implantation the patient suffered from long lasting episodes of CHB. Surgical removal of the device resulted in incomplete recovery of AV-conduction. Histopathological work-up of the explanted GSO showed complete endothelialization of the device and regular scar formation. One year after surgery, the child had sinus rhythm during daytime but needed VVI-pacing while sleeping. Young age, inferior localization of the defect, and use of a large device have been individual risk factors for CHB in this patient. Clinical course and histologic findings indicate that mechanical compression was the only cause for CHB. The cumulative number of reports of CHB after use of different ASD-devices supports the recommendation to postpone the intervention in asymptomatic patients to preschool-age. Early removal of a pushing device may increase the chance of complete recovery from CHB.

Background Modern fluoroscopic angiography systems permit rendering of three-dimensional volumetric data sets using rotational angiography (3D-RA). Along with magnetic resonance imaging (MRI) or multi-detector-row computed tomography (MDCT) data sets, they can be fused with live fluoroscopy images for roadmapping during therapeutic procedures, but the value of multimodality fused data sets has not been clarified. Methods In a pediatric cardiac catheterization laboratory, we analyzed 78 interventional cardiovascular procedures in which 3D models of 3D-RA, MRI, or MDCT were used for 3D-guidance. Accuracy of 2D-3D registration as well as overall procedural benefit was independently rated by two pediatric interventionalists. Fluoroscopy time, radiation dose, and contrast dye consumption were evaluated and, grouping a subgroup analysis, the parameters were compared between patients who underwent stenting of aortic coarctation with and without 3D-roadmapping. Results 3D-guiding was used in 78 cases, 75 of these cases were with accurate 2D-3D registration. 3D-roadmapping was rated superior to conventional biplane imaging in 74 cases. 3D-guidance was used in 64 cases to define the ideal C-arm angulation and in 60 cases for accurate device positioning. Median dose-area product in the total investigation was 706.3 μGym2 (104.8–7249.7 μGym2), 3.3 ml/kg (0.9–13.7 ml/kg) of contrast dye was used, and total fluoroscopy time was 14.5 min (2.9–68.1 min). Fluoroscopy time for 3D-guided stenting of aortic coarctation is significantly lower (8.35 versus 10.2 min; P = 0.04). Conclusion 3D-image fusion with live fluoroscopy can be applied successfully in catheter-based interventions of congenital heart disease. 3D-guidance facilitates catheter manipulations and interventions, allows preselection of ideal projection angles, reduces fluoroscopic time and the number of control angiographies. © 2013 Wiley Periodicals, Inc.

Nephropathy is known to occur in patients with long-standing cyanotic congenital heart disease (CCHD). In order to assess the incidence, nature and degree of the problem among such patients, discriminating urine analyses were performed in 26 patients with CCHD, with a mean age of 22 (10-42) y. Ten patients showed reduced glomerular function, six of whom also had advanced glomerulopathy. Glomerular filtration rates were below normal in half of the patients and occurred with glomerular-type proteinuria in five, with tubular-type proteinuria in one and without pathological proteinuria in four. An elevated haematocrit and duration of cyanosis were identified as the main risks factors for the development of glomerulopathy. The risk of developing glomerular lesions rose sharply during the second decade of life. Nephropathy in CCHD is common and the dominant feature is glomerular damage, which is related to the duration of cyanosis and the extent to which the haematocrit is elevated.

Summary 1. Renal postischaemic reperfusion injury constitutes a significant problem after kidney transplantation. The polysaccharide fucoidin improves postischaemic function in lamb hearts, presumably by blocking selectin-mediated leucocyte adhesion. 2. In the present study, eight pairs of ischaemic pig kidneys were reperfused in an ex vivo model with autologous blood with or without fucoidin (100 mg/L). 3. Fucoidin resulted in a significant decrease in renal blood flow (45 ± 5 vs 178 ± 22 mL/min per 100 g; P

The impact of an atrial septal defect in the elderly with reduced diastolic elasticity of the left ventricle is unclear. We studied the hemodynamic changes during balloon occlusion of atrial septal defects in patients over 60 years of age. In 18 patients (61-78 years old; median, 70), the left atrial pressure and the mitral valve inflow was measured during complete balloon occlusion of the defect and after deflation of the balloon. In seven patients, the left atrial pressure and the E/A ratio of the mitral valve inflow increased markedly (P = 0.02). Mean atrial pressures reached values of 27 mm Hg and the v-wave peak values of 55 mm Hg. Two patients received a transcatheter device closure and developed congestive heart failure. In the elderly, an atrial septal defect can have a decompressive impact on the left ventricle. Therefore, caution appears to be warranted if atrial septal closure is planned.

To avoid x-ray exposure prior to interventional closure of atrial septal defects (ASDs), we recently developed a technique for diagnostic catheterization and balloon sizing of the defect by echocardiographic guidance without fluoroscopy. We report on our first experiences with this technique. Fourteen patients with atrial septal perforations (mean age, 23 years; range, 1-66 years) underwent diagnostic catheterization and balloon sizing prior to possible interventional defect closure. Mean size of the defects was 16 mm (7-29 mm). Mean left-to-right shunt was Qp/Qs = 2.0 (range, 1.0-4.0). Without fluoroscopy, the procedures were performed in two children by transthoracic echocardiography (TTE) and in 12 patients by both TTE and transesophageal echocardiography (TEE). Mean procedure time was 59 minutes (range, 35-90 minutes). We conclude that oxymetry, pressure recordings, and the estimation of the balloon-stretched size of atrial septal perforations can be performed safely by echocardiographic guidance without fluoroscopy. The x-ray exposure for patient selection prior to a transcatheter closure of an ASD can be avoided with this technique.

Aims: To assess plasma B-type natriuretic peptide (BNP) levels in patients with univentricular hearts late after volume unloading by total cavopulmonary connection (TCPC). Methods: Plasma BNP was measured by sandwich immunoassay in 67 patients after a modified Fontan procedure. BNP levels were compared with age and sex-specific normal values, clinical and echocardiographic data, and results of exercise testing. Results: BNP had a wide range of 5–290 pg/ml, but was normal in 81% of patients, and median BNP was only 13pg/ml. There was no difference between males and females, between patients with left or right ventricular morphology, and no correlation was found between BNP and age, post-operative follow-up period, maximum exercise capability, peak oxygen uptake, or blood oxygen saturation. New York Heart Association class I patients had lower BNP than class II patients (p=0.04). Plasma BNP concentration was positively correlated to the number of specific sequelae (r=0.59, p

Catecholamine treatment for hypotension is common practice in neonatal intensive care units. In the presence of left ventricular hypertrophy, the positive inotropic action of catecholamines with mainly β-receptor specificity can lead to excessive hypercontractility and paradoxical hypotension. This reports presents two cases of hypercontractile heart failure during β-agonist treatment in very low birthweight (VLBW) infants. Both patients (27 wk, 5 d; and 26 wk, 6 d of gestation) underwent surgical arterial duct ligation and coarctation repair. After operation they developed arterial hypotension that failed to respond to epinephrine (adrenaline) or dobutamine treatment. Echocardiography demonstrated a complete systolic obliteration of the left ventricular cavity. Epinephrine and dobutamine were stopped, and norepinephrine (noradrenaline) was successfully used to control the hypotension. Conclusion: Treatment of hypotension with β-agonists in VLBW infants with left ventricular hypertrophy can lead to hypotension with and left ventricular obliteration with paradoxical hypertrophy can lead to hypercontractility and left ventricular obliteration with paradoxical hypotension. Careful echocardiographic monitoring is indicated in such cases.