[Show abstract][Hide abstract] ABSTRACT: Percutaneous replacement of the tricuspid valve with a bovine jugular venous valve (melody valve) was successfully undertaken in a 9-year-old boy. The patient had a previous history of bacterial endocarditis of the native tricuspid valve in infancy. Initially, a pericardial patch valve was created, followed by surgical replacement of the valve using a biological tissue valve at 4 years of age. Progressive stenosis and regurgitation of the biological valve, with severe venous congestion and resulting hepatic dysfunction prompted percutaneous valve replacement.
Annals of Pediatric Cardiology 09/2015; 8(3):230-232. DOI:10.4103/0974-2069.164687
[Show abstract][Hide abstract] ABSTRACT: Background Limited data exist on the vitamin D status in Fontan patients. We determined the prevalence and potential risk factors of vitamin D deficiency in this patient subset.
Methods and results Data were collected from 27 Fontan patients (55.6% male, mean age 8.1±5.3 years). Protein-losing enteropathy was diagnosed in six patients (22.2%). Vitamin D deficiency was defined as a serum 25-hydroxyvitamin D level of <20 ng/ml. The neutrophil-to-lymphocyte ratio, a marker of systemic inflammation, was calculated. Associations between laboratory measurements and patient characteristics were explored. Mean serum 25-hydroxyvitamin D level was 14.1±10.4 ng/ml. Vitamin D deficiency was found in 19/27 patients (70.3%). Only skin type was associated with vitamin D deficiency (p=0.04). Hyperparathyroidism was present in 5/21 (23.8%) patients, and was more prevalent in patients with protein-losing enteropathy (p<0.001). Parathyroid hormone levels correlated with parameters of systemic inflammation (neutrophil-to-lymphocyte ratio: r=0.484, p=0.026; relative lymphocyte count: r=−0.635, p=0.002). Vitamin D supplementation significantly increased serum 25-hydroxyvitamin D levels (p<0.0001), and was accompanied by a reduction in parathyroid hormone concentrations (p=0.032).
Conclusions A high prevalence of vitamin D deficiency was found among Fontan patients, independent of age, time after Fontan procedure, ventricular morphology, and presence of protein-losing enteropathy. A potentially important link between parathyroid hormone levels and systemic inflammation is suggested.
Cardiology in the Young 08/2015; -1. DOI:10.1017/S1047951115001444 · 0.84 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Objective
To assess feasibility, safety and effectiveness of right ventricular outflow tract (RVOT) stenting in symptomatic young infants.Methods
Multicentre evaluation of 35 patients intended to undergo RVOT stenting in 11 pediatric cardiac centres from 2009 to August 2011.ResultsMedian age and weight at the time of first stent implantation were 8 weeks and 3.3 kg, with 40% of patients <3 kg. A total of 19 patients had suffered from hypoxemic spells, 8 patients were ventilated, 6 on inotropic support and 5 on prostaglandin infusion. Severe concomitant malformations were present in 11 patients, and acute infections in 2. Stenting of the RVOT was successfully performed in 33 patients, improving oxygen saturation from a median of 77 to 90% 2 days after intervention. Besides the 2 patients in whom RVOT stenting was not successful for technical reasons, there were no procedural complications. In 17 of 33 patients, 1–3 reinterventions were performed during follow-up, less than half of those were reinterventions in the RVOT. A total of 27 patients have undergone successful surgical repair 4–162 (median 19.5) weeks after initial RVOT stent implantation, 2 patients are still waiting. There were no perioperative deaths.Conclusions
Stenting of the RVOT provides a safe and effective management strategy for initial palliation in symptomatic young infants, including those patients not suitable or at higher risk for surgical therapy.
[Show abstract][Hide abstract] ABSTRACT: Haemostasis is achieved by coordinated interactions of cellular and plasmic components. Under physiological contitions, pro-haemostatic and anti-haemostatic mechanisms are maintained in a well-balanced equilibrium. Platelets induce initial sealing of the vascular lesion by forming a haemostatic plug, mediate subsequent plasmic haemostasis and contribute to wound healing. The distinction of primary and secondary haemostasis follows the physiological sequence of haemostatic events. Primary haemostasis is controlled by platelet functions (activation, adhesion and aggregation), succeded by activation of the coagulation system with generation of thrombin and subsequent conversion of fibrinogen into fibrin. A network of insoluble fibrin stabilizes the haemostatic plug and prevents its detachment by the flowing blood. In analogy to coagulation, initiation of fibrinolysis is triggered by specific activators and proteases. Direct interactions of platelets, coagulation and fibrinolysis warrant a phase-oriented regulation of haemostasis kinetics.
[Show abstract][Hide abstract] ABSTRACT: The term shock dates back to the Middle Ages. In its meaning of blow, punch or impact, the word found entry into medical terminology by its usage as a military expression. Doctors in antiquity already described clinical pictures, traumata, and blood-losses inducing shock-like states. From about 1500 on, even gunshot wounds were followed by such states. In 1737, H.F. Le Dran published his book on wounds by fire-arms. In its English translation of 1743, the term "shock" as a consequence of such traumata was used for the first time. Up to modern times, the importance of traumata and blood-losses triggering shock states was examinded in more detail. The effects of haemorrhages and haemostasis were also noticed as early as in ancient times. The practice of blood-letting and the inspection of this blood enabled the increasing understanding of normal and pathological haemostasis. In 1772, W. Hewson published his work on blood-loss and haemostasis. Owing to his publication in 1905, P. Morawitz became the founder of the "classical coagulation theory". It was only after the 1960s that the close and complex interactions of shock and haemostasis were examinded and more clearly understood.
[Show abstract][Hide abstract] ABSTRACT: We describe transcatheter therapy for early onset occlusion or stenoses of extracardiac conduits in three children who had undergone Fontan completion. Successful stent implantation was associated with complete resolution of symptoms.
Cardiology in the Young 11/2012; 23(05):1-3. DOI:10.1017/S1047951112001886 · 0.84 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: A vein of Galen malformation - a rare cause of cardiac insufficiency in neonates - is sometimes associated with coarctation of the aorta, two diseases requiring urgent therapy in the neonatal period. We report on a term neonate in whom we first palliated the coarctation by stent implantation, providing time to treat the vein of Galen malformation by endovascular embolisation. Following this, the coarctation was surgically repaired and the stent was explanted.
Cardiology in the Young 12/2011; 22(5):596-9. DOI:10.1017/S104795111100196X · 0.84 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: The aim of this study was to assess the efficacy of radiofrequency catheter ablation (RFCA) in the treatment of hypertrophic obstructive cardiomyopathy in children.
Hypertrophic obstructive cardiomyopathy is an uncommon cause of left ventricular outflow tract obstruction in children. In symptomatic patients, open heart surgical myectomy has hitherto been the only therapeutic option.
In 32 children, at a median age of 11.1 (range 2.9 to 17.5) years and weight of 31 (15 to 68) kg, ablation of the hypertrophied septum was performed using a cool-tip ablation catheter via a femoral arterial approach. The median number of lesions was 27 (10 to 63) and fluoroscopic time was 24 (12 to 60) min.
The majority of patients demonstrated an immediate decrease in the catheter pullback gradient (mean 78.5 ± 26.2 mm Hg pre-RFCA versus mean 36.1 ± 16.5 mm Hg post-RFCA, p < 0.01) and a further reduction in the Doppler echocardiographic gradient (mean 96.9 ± 27.0 mm Hg pre-RFCA versus 32.7 ± 27.1 mm Hg post-RFCA, p < 0.01) at follow-up. One patient died due to a paradoxical increase in left ventricular outflow tract obstruction, and another had persistent atrioventricular block that required permanent pacing. Six patients required further procedures (surgery, pacing, or further RFCA) during a median follow-up of 48 (3 to 144) months.
The preliminary results of RFCA for septal reduction in children with hypertrophic cardiomyopathy are promising and merit further evaluation.
Journal of the American College of Cardiology 12/2011; 58(24):2501-10. DOI:10.1016/j.jacc.2011.09.020 · 16.50 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Cryoablation is safe for the ablation of substrates in proximity to the AV node, because the initial lesion is reversible. We report our results of cryoablation in a transregional center for ablation in children and adolescents.
Data on 39 children and adolescents (4 - 18 years of age) who had been treated with cryo energy were analyzed retrospectively. The diagnosis was AV nodal reentry tachycardia (AVNRT; n = 30), para-Hisian accessory pathway (AP; n = 6) and congenital junctional ectopic tachycardia (JET; n = 4). In addition to non-inducibility, the targeted endpoint for AP-ablation was a missing or decremental concentric retrograde conduction, for ablation of AVNRT the endpoint was no slow pathway, no AH jumps and no echo-beats. The median follow-up was 3 years (270 - 1919 days).
The targeted endpoint was reached in 35/39 patients (90 %), in four patients (10 %) RF energy had to be applied. A recurrence occurred in 7/35 (20 %) successfully treated patients. Two patients had a pre-excitation again after AP ablation, but no symptoms. Thus, 28/35 patients (80 %) remained asymptomatic after cryoablation, and 26/35 (74 %) are definitively cured, regarding all follow-up data. The subgroup of AVNRT patients does not differ from the entire group. There was no AV block in the cryoablation group.
Cryoablation is very safe and effective for the definitive treatment of arrhythmias in children and adolescents. The price for the high safety is a reduced efficacy and a higher recurrence rate.
[Show abstract][Hide abstract] ABSTRACT: Current recommendations discourage elective radiofrequency ablation in patients <5 years old and/or weighing <15 kg, primarily because of the greater complication rate. To describe the current use, complications, and immediate outcomes of cryoablation in this patient population, a multicenter retrospective review of all patients <5 years old and/or weighing <15 kg who were treated with cryoablation for arrhythmia was performed. Eleven centers contributed data for 68 procedures on 61 patients. Of those, 34% were elective and 24% (n = 16) were both cryoablation and radiofrequency ablation. The median age and weight at ablation was 3.5 years (range 8 days to 9.9 years) and 15.2 kg (range 2.3 to 23), respectively. Congenital heart disease was present in 23% of the patients. The immediate success rate of cryoablation alone was 74%. No major complications occurred with cryoablation only; however, 2 of the 16 patients who underwent cryoablation and radiofrequency ablation had major complications. Of the 50 patients receiving cryoablation, 8 (16%) had variable degrees of transient atrioventricular block. The recurrence rate was 20% after cryoablation and 30% after cryoablation plus radiofrequency ablation. In conclusion, cryoablation appears to have a high safety profile in these patients. Compared to older and larger patients, the efficacy of cryoablation in this small, young population was lower and the recurrence rates were higher. Cryoablation's effect on the coronary arteries has not been fully elucidated and requires additional research.
The American journal of cardiology 05/2011; 108(4):565-71. DOI:10.1016/j.amjcard.2011.03.089 · 3.28 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: A variety of devices are available for transcatheter closure of unwanted shunts. We describe our experience with the use of the Amplatzer vascular plug IV in a consecutive series of infants.
A total of eight consecutive infants - all born preterm at gestational ages ranging from 24 to 35 weeks - undergoing transcatheter closure of unwanted shunts - persistently patent arterial duct in five patients, an aorta to right atrium fistula in one, multiple aortopulmonary collateral vessels in one, and an azygos vein to left atrium connection in one - are described. Their age, from birth, ranged between 3 and 11 months, and weight between 2.6 and 11.3 kilograms. All devices were delivered using percutaneous arterial or venous vascular access via a large lumen (0.038 inch) 4-French delivery catheter.
All lesions could be successfully occluded using one or more devices. Device diameters ranged between 4 and 8 millimetres, and exceeded the minimum diameter of the target vessel by 1 to 2 millimetres. Successful occlusion was confirmed either directly at angiography or on follow-up echocardiography. Of the infants who were mechanically ventilated prior to the procedure, three could be successfully weaned following closure of the shunt. There were no procedure-related complications.
The new vascular plug IV is cheap and efficacious in closing a variety of shunts in young infants, and warrants further extended clinical application.
Cardiology in the Young 04/2011; 21(2):178-81. DOI:10.1017/S104795111000171X · 0.84 Impact Factor