İlker Ertuğrul

Regression of Symptomatic Multiple Cardiac Rhabdomyomas Associated with Tuberous Sclerosis Complex in a Newborn Receiving Everolimus

UNLABELLED Cardiac rhabdomyoma is the most common primary cardiac tumor, is considered to be a hamartoma of developing cardiac myocytes. Cardiac rhabdomyoma is associated with tuberous sclerosis complex (TSC) in 50-86% of cases. Mutations in TSC-1/TSC-2 genes result in increased mammalian target of rapamycin (mTOR) pathway activation responsible for the hamartomatous lesions of tuberous sclerosis complex. Therapy with mTOR inhibitors is currently under investigation as a treatment option for tumors associated with TSC. In this report we present a case with multiple symptomatic rhabdomyomas associated with tuberous sclerosis complex, deemed to be ineligible for surgical removal, treated with everolimus (mTOR inhibitor). CONCLUSION As we observed in our patient, in cases with inoperable symptomatic rhabdomyomas associated with TSC, everolimus, an mTOR inhibitor, may be the treatment of choice, which should be confirmed with additional studies.

Midterm Results of Implantable Cardioverter Defibrillators in Children and Young Adults from a Single Center in Turkey

Despite concerns about complications with the implantable cardioverter defibrillator (ICD), it is effective for the prevention of sudden cardiac death (SCD). We aimed to analyze our midterm experience with ICD in children and young adults.

Closure of the patent ductus arteriosus with the Amplatzer Duct Occluder II: a clinical experience.

BACKGROUND The aim of our study was to share our clinical experience on cases with patent ductus arteriosus treated with the Amplatzer Duct Occluder II. METHODS Between 2008 and 2012, 26 of 31 patients with patent ductus arteriosus underwent successful transcatheter closure of patent ductus arteriosus using the Amplatzer Duct Occluder II. Mean age was 3.3 years and mean weight was 15.7 kilograms. The presence of a residual shunt, left pulmonary artery or aortic obstruction was explored by administering contrast material during the procedure. The patients were discharged 24 hours after the procedure. RESULTS The procedure was successful in 26 of 31 patients and failed in five patients. According to the Krichenko classification, 26 patients had type A, one patient had type B and 4 patients had type C ductus. The mean narrowest ductus diameter was 3.2 mm and the mean ductus length was 6.7 mm. Complete angiographic occlusion occurred immediately after the procedure in 22 out of 26 patients in whom the ductus was closed successfully with the Amplatzer Duct Occluder II. Complete occlusion was achieved in the remaining patients with residual shunt one month after the procedure. The procedure was preceded by closure with an Amplatzer Duct Occluder I in two patients and an Amplatzer Vascular Plug I in one patient. CONCLUSION Amplatzer Duct Occluder II is highly effective in transcatheter closure of patent ductus arteriosus. We think that an alternative closure device and alternative techniques can be attempted in patients with type C ductus. The success rate could increase with accumulating experience.

Increased P-wave dispersion a risk for atrial fibrillation in adolescents with anorexia nervosa

ABSTRACT Studies have shown that a prolonged P-wave dispersion is a risk factor for the development of atrial fibrillation. The aim of this study was to evaluate P-wave dispersion in adolescents with anorexia nervosa at diagnosis. We evaluated electrocardiographic findings, particularly the P-wave dispersion, at initial assessment in 47 adolescents with anorexia nervosa. Comparison of P-wave dispersion between adolescents with anorexia nervosa and controls showed a statistically significant higher P-wave dispersion in patients with anorexia nervosa (72 ± 16.3 msec) when compared to the control group (43.8 ± 9.5 msec). Percent of body weight lost, lower body mass index, and higher weight loss rate in the patients with anorexia nervosa had no effect on P-wave dispersion. Due to the fact that anorexia nervosa has a high mortality rate we believe that cardiac pathologies such as atrial fibrillation must also be considered in the medical evaluation.

Transjugular approach for radiofrequency ablation of permanent junctional reciprocal tachycardia in a newborn with bilateral femoral vein thrombosis.

Although radiofrequency ablation is the first line therapy in some children with supraventricular tachycardia, its application in small children is still limited. Herein, we presented a premature newborn diagnosed as multidrug-resistant permanent junctional reciprocal tachycardia, and treated by radiofrequency ablation via the jugular vein approach because of bilateral femoral vein thrombosis. We think that when there is limited vascular access, the transjugular route for radiofrequency ablation might be considered as an alternative treatment in newborns with multidrug-resistant supraventricular tachycardia.

A new approach to an old hypothesis; phototherapy does not affect ductal patency via PGE2 and PGI2

Abstract Objective: Numerous investigations have demonstrated that phototherapy (PT) directly or indirectly causes ductal patency by photorelaxation effect. In this observational study, we aimed to assess the effect of PT on the incidence of patent ductus arteriosus (PDA) together with prostaglandins (PGE2) and (PGI2) levels in preterm infants. Methods: Preterm infants whose gestational age < 34 weeks and who required PT in the first 3 d of life were enrolled in this prospective study. The clinical signs of PDA, the data of detailed echocardiographic study were recorded and plasma PGE2 and PGI2 levels were measured before and after PT. The outcome measures were the status of ductus arteriosus and alterations of PGE2 and PGI2 levels under the effect of PT. Results: A total of 44 preterm infants were enrolled in the study, of these 21 (47.7%) were in Group 1 (Non-PDA Group) and 23 (52.3%) were in Group 2 (PDA Group). After PT, ductal reopening occurred in three infants (14.3%) in Group 1, while ductus closed in four infants in Group 2 (17.3%). PT does not seem to effect ductal patency for both groups (p = 0.250 and p = 0.125, respectively). PGE2 levels were not different before and after PT for both groups (p = 0.087, p = 0.408, respectively). However, PGI2 levels were significantly decreased after PT in both groups (p = 0.006, and p = 0.003, respectively). Conclusion: There was no effect of PT on ductal patency. We can conclude that PGs were eliminated simultaneously with ductal closure and photorelaxation effect did not influence PG levels.

Electrical storm in an adolescent with arrhythmogenic right ventricle cardiomyopathy treated with cardiac transplantation

Arrhythmogenic right ventricle dysplasia/cardiomyopathy (ARVD/C) is an inherited cardiomyopathy characterized by fibro fatty replacement of the right and less frequently left ventricle (1, 2). Ventricular arrhythmias requiring implantable cardioverter defibrillator (ICD) are common in patients with ARVD/C and electrical storm (≥3 life-threatening ventricular arrhythmia within 24-hour period) resulting in ICD discharges is a major cause of morbidity and mortality (3). Radiofrequency ablation with three-dimensional (3-D) mapping and navigation systems has been recently advocated as a preferred treatment for recurrent ventricular arrhythmias (4). We had experience of a case of a15-year-old boy who presented with chest pain and decreased exercise capacity. He had undergone surgical closure for atrial septal defect and complicated with ventricular tachycardia/fibrillation postoperatively at 11 years-old. No history of congenital heart defect in family and syncope were found. Premature ventricular contractions were determined occasionally in 24-hour Holter monitoring. Echocardiography revealed a dilatation of the right ventricle and the right ventricle outflow tract. The dilated infundibulum and increased trabeculation in the right and left ventricle were shown in the cardiac angiography and magnetic resonance imaging. Non-sustained monomorphic ventricular tachycardia (VT) with the rate of 260 beats/minute was induced by programmed stimulation with a single extra-stimulus from the right ventricle. He was diagnosed as ARVD/C and ICD was implanted for primary prevention. Two years later, the episodes of ventricular tachycardia/fibrillation were repeated 35 times within one month. Amiodarone and sotalol administration was initiated and the ablation treatment was planned. A single 4 mm openirrigation ablation catheter (Medtronic, MN, USA) was advanced to right ventricle via femoral vein by using the EnSite NavX 3D mapping and navigation system (St Jude Medical, MN, USA). The area with <0.5 mV during voltage mapping was considered as scar tissue. Radiofrequency ablation was applied to around the scar at the temperature 45°C with 30-35 Watt energy. Total procedure time was 280 minutes. Two months later, the electrical storm repeated again and the patient was arrested in a short time. He was immediately connected to the pump after cardiac resuscitation and underwent cardiac transplantation from an adult cadaver one day later. He has been on follow-up with no symptom for three years. Stec et al. (4) reported a pregnant woman with an electrical storm due to ARVD/C of successful endocardial catheter ablation, by using 3-D mapping and navigation system. Although ventricular tachycardia frequency is reduced after catheter ablation, ventricular arrhythmia recurrence is still common in ARVD/C (2). It appears that ICD is currently an indispensable treatment option in ARVD/C. Philips et al. (2) claimed that VT-free period after epicardial ablation was longer than those after endocardial ablation. They speculated that it was because of epicardial distribution of ARVD/C. In our case, recurrence of VT may be associated with endocardial ablation. Nevertheless, catheter ablation of ventricular tachycardia in ARVD/C can be considered as a beneficial method in terms of reducing the side effects of antiarrhythmic drugs and prolonging the life of ICD battery (2). The management of an electrical storm should be individualized for each patient and the treatment may indicate extracorporal membrane oxygenation and cardiac transplantation.

The Impact of Rapid Left Ventricular Pacing during Pediatric Aortic Valvuloplasty on Postprocedural Aortic Insufficiency

OBJECTIVE This study aims to determine efficacy and safety of rapid left ventricular pacing (RLVP) during balloon aortic valvuloplasty and effect on development of postprocedural aortic insufficiency (AI) in children. DESIGN This is a retrospective comparison of 56 children (mean age 18.3 month; 1 day-15 years of age) who underwent valvuloplasty by using RLVP with standard method (without pacing) during same time period (74 children; mean age 12.6 month; 1 day-18 years of age). RESULTS The systolic valvular gradient decreased from a mean 67 ± 20.4 mm Hg (26-120 mm Hg) before the procedure to a mean 27.6 ± 17.8 mm Hg (0-120 mm Hg) after the procedure. Procedure failed in two of the patients (3.6%) who received the RLVP; only two patients developed severe AI. Among those patients who underwent the standard method procedure failed in 6 patients (8.3%), whereas severe AI was seen in 15 (20.2%). RLVP decreased the incidence of postprocedure severe AI significantly (P < .01) but it had no effect on the gradient reduction of the procedure (P > .05). RLVP did not change the procedure duration, but decreased the duration of fluoroscopy significantly (P < .01). CONCLUSION RLVP can be used effectively and safely for pacing during balloon aortic valvuloplasty procedures in all age groups; it decreases procedural failure rate, eases the procedure and prevents the development of AI through the stabilization of the balloon.

A rare association: inferior vena cava anomalies and congenital heart diseases

The numerous variations of abnormalities of the inferior vena cava (IVC) result in anomalies such as isolated left IVC, double IVC and more than 60 types of malformation. These anomalies are rare and recognized incidentally during surgical or radiological procedures. They may lead to clinical complications during abdominal surgery, and predispose to venous thrombosis. Although they have no definite relationship to other congenital cardiac lesions, identification of these anomalies are important for pre-operative planning and post-operative follow-up. This report presents two cases of congenital IVC anomalies accompanied by congenital heart diseases.

A rare complication of radiofrequency ablation: skin burn.

Radiofrequency ablation is the first-line treatment for arrhythmias with high success and low complication rates. Skin burns have been reported rarely after electrophysiological procedures, especially procedures in which higher-power energy is used and multiple ablations are performed. Here, we report a case of skin burn that developed after radiofrequency ablation for ventricular tachycardia originating from the right ventricular outflow tract.