Hayrettin Hakan Aykan

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.

The efficacy of amiodarone-propranolol combination for the management of childhood arrhythmias.

The aim of this study was to present our data regarding the efficacy and safety of combining amiodarone and propranolol for the management of arrhythmias in infants.

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.

Transcatheter closure of antegrade pulmonary blood flow with Amplatzer muscular VSD occluder after Fontan operation.

The Fontan procedure refers to any operation that results in the flow of systemic venous blood to the lungs without passing through a ventricle. An accessory source of pulmonary blood flow in patients with the Fontan procedure may cause elevation of the pulmonary artery pressure, leading to ventricular failure (1). An 8-year-old-girl with double outlet right ventricle presented with marked pleural effusion on postoperative day 14 after a Fontan procedure with extracardiac conduit without ligation of the pulmonary trunk. Her immediate postoperative course was uneventful. She had prominent pleural effusion with 80% saturation. Echocardiography showed significant antegrade flow from the right ventricle into the pulmonary artery. The superior vena cava and main pulmonary artery pressures were markedly high [26/22 mm Hg (mean 25 mm Hg) and 27/23 mm Hg (mean 24 mm Hg), respectively]. The pulmonary artery angiogram showed normally branched pulmonary arteries with rapid wash-off of the contrast. The diameter of the stenotic pulmonary valve was measured as 11.2 mm. A 12 mm Amplatzer muscular VSD occluder (AGA Medical, MN, USA) device was deployed very close to the pulmonary valve at the infundibular stenotic region (Fig. 1). The pulmonary artery pressure was not decreased immediately [27/21 mm Hg (mean 24 mm Hg)] when compared with the pressure measured before the intervention. Antiaggregant and anticoagulant therapy were started. The patient recovered uneventfully, with an oxygen saturation >90%, and was discharged two weeks later. She has been symptom-free on sildenafil treatment for one year. Although several studies have reported that non-pulsatile pulmonary blood flow results in decreased capillary flow and increased vascular resistance (1), the preservation of antegrade pulmonary blood flow in a patient with a total cavopulmonary shunt may complicate the Fontan procedure, resulting in persistent pleural effusions or progressive ventricular failure (2). Therefore, the benefit of additional sources of systemic to pulmonary artery flow remains controversial. Desai et al. (2) and Petko et al. (3) showed that transcatheter closure of ventriculopulmonary artery communication was a safe and effective technique for the treatment of selected patients after cavopulmonary shunt. Our opinion is also that transcatheter closure of pulmonary flow is an alternative to surgery because it is less invasive, easy to perform, reliable, and more comfortable. Madan et al. (4) reported an 8 year-old boy with thrombus in the pulmonary artery stump after a Fontan operation that included ligation of the main pulmonary artery distally to the pulmonary valve. Devices can be placed across the pulmonary artery band, across pulmonary valve tissue, and above the valve in a relatively large homograft without any focal narrowing (3). However, a thrombus may form if a room remains between the pulmonary valve and the device that can create stasis after the intervention. In this case, our preference is to deploy the device in the infundibular area. Transcatheter closure of antegrade pulmonary blood flow seems a safe and effective alternative method to surgery in the treatment of selected patients who have undergone a Fontan procedure and show early complications such as pleural effusion due to excess pulmonary blood flow.

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.

NRG™ RF powered transseptal needle: a useful technique for transcatheter atrial septostomy and Fontan fenestration: report of three cases.

Transseptal puncture (TSP) is a frequently performed procedure for gaining access to the left atrium for catheter ablation, hemodynamic assessment of the left heart, left ventricular assist device implantation, percutaneous left atrial appendage closure or mitral valvuloplasty during childhood and adulthood. The standard technique for transseptal puncture applies mechanical pressure on the fossa ovalis with a Brockenbrough needle. However, this method is not feasible when the fossa ovalis is thick and aneurysmatic. In such patients, the radiofrequency ablation energy systems can be offered as a better alternative for TSP. Here, we aimed to demonstrate the outcome of transseptal puncture performed with an NRG™ RF powered transseptal needle in three patients.

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.

Evaluation of tissue Doppler echocardiographic imaging findings in children with pulmonary hypertension

Akın A, Alehan D, Aykan HH, Özkutlu S, Özer S, Karagöz T. Evaluation of Tissue Doppler Echocardiographic Imaging findings in children with pulmonary hypertension. Turk J Pediatr 2017; 59: 244-253. Tissue Doppler Imaging has become an important prognostic marker that can be used in follow-up and determination of the prognosis in pulmonary hypertension patients. We compared the Tissue Doppler imaging parameters of 34 patients with pulmonary hypertension and 43 healthy controls. In addition, Brain-Natriuretic Peptide levels, pulmonary artery systolic pressures measured with echocardiography, 6-minute walking tests and New York Heart Association functional classification were compared. Among patients with Eisenmenger syndrome and idiopathic pulmonary hypertension, Tissue Doppler imaging parameters were mostly similar. In patients with New York Heart Association functional class 3, mitral septal annulus E/Ea (p=0.050) and mitral lateral annulus myocardial performance index (p=0.009) were higher than class 2 patients. In patients with higher Brain Natriuretic Peptide level, mitral lateral annulus and tricuspid septal annulus Ea/Aa values were lower (p=0.046 and < 0.001 respectively); tricuspid septal annulus E/Ea and interventricular septum myocardial performance index values were higher than in patients with normal Brain-Natriuretic Peptide level (p=0.006). In conclusion tissue Doppler imaging findings were significantly impaired in children with pulmonary hypertension compared to the control group. Findings were similar in patients with idiopathic pulmonary hypertension and Eisenmenger syndrome. Mitral lateral annulus myocardial performance index value may have a prognostic importance due its significant association with poor functional class. Due to the significant associations between mitral lateral annulus, tricuspid septal annulus Ea/Aa, tricuspid septal annulus Ea and E/Aa, interventricular septum-myocardial performance index values and brain natriuretic peptide levels, these parameters may be used in evaluating response to therapy.

Recent Advances in Kawasaki Disease

Özet Kawasaki hastalığı, daha önceleri mukokütanöz lenf nodu hastalığı veya infantil poliarteritis nodoza olarak da bilinen çocuklarda görülen akut febril bir hastalıktır. Hastalık, nedeni tam olarak bilinmeyen sistemik bir vaskülit olarak orta çaplı damar tutulumu ile seyreder ve çoğunlukla 5 yaş altı çocuklarda görülür. Kan damarları, cilt, muköz membranlar ve lenf nodları başta olmak üzere birçok organ sistemi etkilenir. En Nadir fakat en önemli etkilerinden biri kalp üzerinedir, tedavi edilmeyen çocuklarda fatal koroner arter anevrizmalarına neden olabilmektedir. Bu nedenle tanının erken konması, tedavinin zamanında ve uygun bir şekilde planlanması oldukça önemlidir. Bu derlemede ülkemizde de önemli bir morbidite nedeni olan Kawasaki hastalığının tanı, tedavi süreçleri ve prognostik özellikleri tartışılmıştır. Abstract Kawasaki disease is an acute febrile disease inchildren formerly known as mucocutaneous lymphnode disease or infantile polyarteritis nodosa. The disease, a systemic vasculitis of unknown etiology, in which the medium-sized blood vessels are affected, is frequently seen in children under five years of age. Organ systems, mainly the blood vessels, skin, mucous membranes, and lymph nodes are involved. The rarest but the most serious effect of the disease is on the heart, where it can cause fatal coronary artery aneurysms in untreated children. Therefore, early diagnosis, timely and appropriate treatment approach is very important. In this review, diagnosis, treatment processes and prognostic features of Kawasaki disease, also an important cause of morbidity in our country, were discussed.