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Tissue doppler evaluation of systolic and diastolic cardiac functions in long-term survivors of Hodgkin lymphoma†

Monitoring for late adverse events is crucial in long‐term management of childhood cancer survivors. A case–control study to evaluate long‐term cardiovascular status of childhood Hodgkin lymphoma (HL) using tissue Doppler imaging (TDI) was performed.

Successful Implantation of an Intracardiac Defibrillator in an Infant With Long QT Syndrome and Isolated Noncompaction of the Ventricular Myocardium

Congenital long QT syndrome (LQTS) is an inherited disorder characterized by QT prolongation and polymorphic ventricular tachycardia known as torsade de pointes. The underlying cellular mechanism is prolonged ventricular repolarization caused by mutations in genes encoding cardiac ion channels or membrane adaptors. The disease can be diagnosed at any age and, very rarely, it can be diagnosed prenatally or in the neonatal period. Isolated noncompaction of the ventricular myocardium (INCVM) is defined as the presence of prominent ventricular trabeculations and deep intertrabecular recesses within the endomyocardium. This report describes a newborn baby presenting with polymorphic ventricular tachycardia whose diagnosis was LQTS and INCVM. Ventricular tachycardia did not respond to medical treatment, and a transient epicardial pacemaker was inserted surgically on his 30th day of life for atrioventricular block and bradycardia. The transient epicardial pacemaker was upgraded to an epicardial intracardiac defibrillator on his 40th day. The concomitant occurrence of INCVM, LQTS, and atrioventricular block needs to be evaluated further.

Long-term inhaled iloprost use in children with pulmonary arterial hypertension.

BACKGROUND We performed a retrospective analysis of patients with pulmonary arterial hypertension receiving inhaled iloprost in a single centre to evaluate long-term tolerability, safety, and efficacy of chronic inhaled iloprost therapy in children. METHODS A total of 20 patients with either idiopathic or associated pulmonary arterial hypertension were treated with iloprost between April, 2003 and January, 2010. The median age and weight of the patients were 3.8 years--ranging from 4 months to 19 years--and 12.3 kilograms--ranging from 4 to 73 kilograms-- respectively. Pulmonary arterial hypertension was idiopathic or hereditary in eight patients (40%) and associated with congenital cardiac disease in 12 patients (60%). RESULTS Of the 20 patients, 15 had combined therapy--12 patients with two and three patients with three different classes of drugs. In all, six patients died during follow-up. The median follow-up time was 18 months, ranging from 6 to 74 months. The 6-minute walking test was performed in 7 out of 20 patients at baseline and on follow-up. The median 6-minute walking test increased from 420 to 490 metres after iloprost therapy (p = 0.028). After initiation of iloprost therapy, one patient complained of headache and another had a rash around his mouth, none necessitating discontinuation of therapy. Overall compliance with inhaled iloprost was good. CONCLUSION Pulmonary hypertension is associated with significant morbidity and mortality. Careful assessment of each patient and timely combination of specific vasodilator therapy is needed to improve clinical outcomes. This study suggests that inhaled iloprost, with or without concomitant endotelin receptor antagonist and/or phosphodiesterase inhibitor, is safe and efficacious for treatment of pulmonary arterial hypertension in children.

Transcatheter closure of coronary artery fistula with an Amplatzer Duct Occluder II in a symptomatic infant.

induced platelet aggregation (8), others documented increased aggregation of platelets in the presence of THC (9). Increased myocardial oxygen demand, decreased blood supply, marked vasoconstriction of the coronary arteries and platelet activation all contribute to the development of acute event. In our case, probably, he has had early onset coronary heart disease in whom the cigarette smoking was the single risk factor and cannabis smoking triggered the plaque rupture and induced thrombosis.

Lamotrigine Related Myocarditis

Myocarditis can develop secondary to several medications. Here, we report a case of myocarditis related to the use of lamotrigine. A 15-year-old boy was admitted to another hospital because of a chest pain that was sustained for 30 minutes. He was transferred to our hospital after detection of cardiac enzyme elevation. He was evaluated in our center, where electrocardiography revealed non-specific ST elevation at inferior derivations, and the level of troponin T was found to be 0.47 ng/ml (0-0.1), while creatinin kinase MB was found to be 38 ng/ml (0-4.97). Systolic cardiac functions were normal via echocardiography. Cardiac magnetic resonance imaging showed minimal pericardial effusion and a minimal decrease in left ventricular function. He was hospitalized with the diagnosis of myocarditis. Viral and bacterial agents that can cause myocarditis were excluded via serological tests. He had been on a lamotrigine treatment due to epilepsy, and after cessation of lamotrigine, his cardiac enzyme levels returned to normal. Therefore, we diagnosed him with drug related myocarditis due to lamotrigine. If an etiology cannot be found during the evaluation of a myocarditis case, drug hypersensitivity should be considered. Changing the responsible drug for hypersensitivity may be beneficial for these patients.

Subcutaneous defibrillator implantation in pediatric patients.

Objective: Although sudden cardiac death is rare in children, an intracardiac defibrillator system is indicated in children with various types of cardiomyopathy, primary electrical diseases, and after surgical repair of congenital heart defects. The use of transvenous defibrillator lead systems is limited in pediatric patients because of a small body size and/or limited vascular access. Subcutaneous array leads combined with an abdominally placed generator can enable implantation. Method: This is a retrospective study of 13 patients who underwent subcutaneous defibrillator implantation between September 2010 and March 2015. The subcutaneous system was preferred because patients were not amenable to transvenous lead placement. Results: The median patient age was 4.1 years, and the median patient weight was 12.1 kg. Diagnoses of patients were long-QT syndrome in 6, aborted cardiac arrest with left ventricular non-compaction in 3, hypertrophic cardiomyopathy with sustained ventricular tachycardia in 3, and arrythmogenic right ventricular cardiomyopathy in 1. Revision of the subcutaneous lead was required in 5 patients 2–26 months after the implantation. Appropriate shocks were observed in three patients. Inappropriate shock and lead fractures were observed in one patient during the follow-up period. The failure of therapy was observed in one patient. There were no perioperative complications and no early or late deaths. Conclusion: Subcutaneous defibrillator systems are safe and effective in pediatric patients when the transvenous method is risky and contraindicated. Because the high growth rate in this population leads to lead failures, a close follow-up of this population is essential. (Anatol J Cardiol 2016; 16: 630-4)

Clinical and electrophysiological evaluation of pediatric Wolff-Parkinson-White patients

Objective: Wolff-Parkinson-White (WPW) syndrome presents with paroxysmal supraventricular tachycardia and is characterized by electrocardiographic (ECG) findings of a short PR interval and a delta wave. The objective of this study was to evaluate the electrophysiological properties of children with WPW syndrome and to develop an algorithm for the management of these patients with limited access to electrophysiological study. Methods: A retrospective review of all pediatric patients who underwent electrophysiological evaluation for WPW syndrome was performed. Results: One hundred nine patients underwent electrophysiological evaluation at a single tertiary center between 1997 and 2011. The median age of the patients was 11 years (0.1-18). Of the 109 patients, 82 presented with tachycardia (median age 11 (0.1-18) years), and 14 presented with syncope (median age 12 (6-16) years); 13 were asymptomatic (median age 10 (2-13) years). Induced AF degenerated to ventricular fibrillation (VF) in 2 patients. Of the 2 patients with VF, 1 was asymptomatic and the other had syncope; the accessory pathway effective refractory period was ≤180 ms in both. An intracardiac electrophysiological study was performed in 92 patients, and ablation was not attempted for risk of atrioventricular block in 8 (8.6%). The success and recurrence rate of ablation were 90.5% and 23.8% respectively. Conclusion: The induction of VF in 2 of 109 patients in our study suggests that the prognosis of WPW in children is not as benign as once thought. All patients with a WPW pattern on the ECG should be assessed electrophysiologically and risk-stratified. Ablation of patients with risk factors can prevent sudden death in this population.

Behcet's disease with superior vena cava syndrome related to pacemaker leads: a case report

A 14-year-old male patient presented with swelling on his face and distension in the neck and chest vessels. Transvenous permanent pacemaker had been implanted because of sick sinus syndrome 3 years ago. There was oedema in the head-neck, he had jugular venous distension and varicose enlargement in the superficial veins of …

Endovascular stents for treatment of coarctation of the aorta.

Address for Correspondence/Yaz›sma Adresi: Dr. Tevfik Karagoz, Section of Pediatric Cardiology, Department of Pediatrics, Faculty of Medicine, Hacettepe University, Sihhiye, 06100, Ankara-Turkey Phone: +90 312 305 11 57 Fax: +90 312 309 02 20 E-mail: tkaragoz@hacettepe.edu.tr Accepted Date/Kabul Tarihi: 01.02.2011 Available Online Date/Cevrimici Yayin Tarihi: 18.05.2011 ©Telif Hakk› 2011 AVES Yay›nc›l›k Ltd. Şti. Makale metnine www.anakarder.com web sayfas›ndan ulas›labilir. ©Copyright 2011 by AVES Yay›nc›l›k Ltd. Available on-line at www.anakarder.com doi:10.5152/akd.2011.089 Isil Yildirim, Tevfik Karagoz, Murat Şahin, Dursun Alehan, Sema Ozer, Suheyla Ozkutlu, Alpay Celiker1

OP-036 TRANSCATHETER CLOSURE OF CORONARY ARTERY FISTULAS

Objective: Coronary artery fistulas (CAF) are rare congenital or acquired malformations in which a direct vascular connection from a coronary artery to a cardiac chamber or a great vessel, bypassing the myocardial capillary network exists. Large fistulas may cause heart failure, myocardial ischemia, arrhythmias, endocarditis, potential aneurismal dilatation and rupture; and require closure, if symptomatic, upon recognition. METHODS and Results: Patients evaluated in our clinic with murmur, heart failure, history of infective endocarditis or suspected cardiac anomaly who were diagnosed with CAVF and cardiac catheterization was performed are included in this study. Between 2003 and 2009, 12 patients aged between 3 days and 204 months (median 42 months) were diagnosed with coronary artery fistulas and transcatheter closure of CAVF was accomplished in nine patients, whereas it was not possible to close the fistula in three patients. In the nine patients that TCC could be achieved, the fistula originated from the left coronary artery in six patients and from the right coronary artery in three; and ended in right side of the heart in seven and left side in two. A narrowing in the distal segment of the fistula was observed in almost all of the patients, and the diameter of this narrow segment was between 1-5 millimeters. The fistula was closed retrogradely in seven and anterogradely in two. Multiple microcoils, vascular plugs, PFM coils, fibrin glue and ADO II were used during the procedure. Control angiography was performed in eight patients. Seven patients had complete occlusion; one had further aneurismatic dilatation of left coronary artery and a residual shunt, and was referred to surgery. Patients were followed up for 13 -48 months (median 35 months) during which time no device related complication was observed and patients presented with heart failure showed significant improvement. Conclusions: Coronary artery fistulas can be safely and effectively closed using transcather techniques. Anatomical variations and different sizes of coronary artery fistulas necessitates availability of different sizes and types of devices at the time of catheterization for successful closure. Patients need to be closely followed up for complication such as residual shunts, new fistula formation and coronary artery stenosis.