Çocuklarda Transkatater Atriyal Septal Defekt ve Patent Foramen Ovale Kapatılması Deneyimleri, Kısa, Orta ve Uzun Dönem Sonuçların Değerlendirilmesi

In this study, we aimed to evaluate transcathater atrial septal defect (ASD) and patent foramen ovale (PFO) closure experiences, short, intermediate and long term results and investigate the effects of different methods on procedural success and complications between 2000-2013, in our department. After the archive scanning, 506 cases included for study, of 90 cases files that containing data about follow-up and diagnosis before the procedure could not be reached. Male:female ratio was 1:1,3, mean age and weight was 8,6±5,8 years and 28,9±16,4 kg respectively during procedure, mean follow-up period was 6,05±3,7 years (1 month – 13,5 years). Mean procedure and flouroscopy duration was 61,5±23,5 and 7,8±6,7 minutes respectively. The procedures were performed under TTE guidance in 90 (17,8%) cases, balloon sizing used in 214 (42%) cases. Pulmonary hypertension was observed in 67 (16%) cases. Within closure attempted 416 cases, the procedure was successful in 401 (96,3%). Procedure-fluoroscopy durations were shorter, defect and device sizes were smaller in successful group than unsuccessful group (p<0,05). The presence of deficient rim and use of “balloon sizing” were not influential on procedural success. In balloon sizing group, longer procedure-fluoroscopy duration and lower total septum/device ratio were observed (p<0,05). In selected cases, it was found that TTE guidance shorten the procedure-fluoroscopy duration(p<0,05). Rezidual shunt was seen in 32,4% and 0,9% of patients immediately after procedure and at the end of the follow-up respectively. Major (rescue surgery, thrombus, erosion) and minor (most frequently rhythm disorders) complications rate were %1,4 and %1,8 respectively. No embolisation and mortality were observed. Benign holter abnormalities were observed in 7,7% of patients. İn conclusion, transcathater ASD and PFO closure is a safe and effective method. Reducing the fluoroscopy time and not requiring general anesthesia are the additional advantages of the defect closure with TTE guidance in the presence of sufficient rim and centrally localized defect.Bu çalışmada, bölümümüzde 2000-2013 yılları arasında transkatater yöntem ile kapatılan sekundum atriyal septal defektli (ASD) ve patent foramen ovaleli (PFO) vakalarımız retrospektif olarak değerlendirilip kısa, orta ve uzun dönem sonuçların ve işlemde kullanılan farklı yöntemlerin işlem başarısı ve komplikasyonları üzerine etkilerinin araştırılması amaçlandı. Arşiv taraması sonrası 506 vaka çalışma kapsamına alındı, bunlardan 90 vakanın işlem öncesi takip ve tanı verilerini içeren dosyalarına ulaşılamadı. Çalışmamızda erkek:kız oranı 1:1,3, işlem sırasındaki ortalama yaş ve ağırlık sırasıyla 8,6±5,8 yıl ve 28,9±16,4 kg, ortalama izlem süresi 6,05±3,7 yıl (1 ay – 13,5 yıl) idi. İşlem ve skopi süreleri sırasıyla 61,5±23,5 dk ve 7,8±6,7 dk olarak bulundu. İşlem 90 (%17,8) hastada TTE eşliğinde yapıldı, 214(%42) hastada “balloon sizing” kullanıldı. Hastaların 67’sinde(%16) PH saptandı. Girişim yapılan 416 hastadan 401’inde(%96,3) başarı sağlandı. Başarılı işlem yapılan grupta başarısız gruba göre işlem-skopi süreleri kısa, defekt çapı ve kullanılan cihaz boyutları daha küçük bulundu (p<0,05). Eksik rim varlığı ve “balloon sizing” kullanımı başarı üzerine etkili bulunmadı. “Balloon sizing” kullanılan grupta işlem ve skopi süresi daha uzun, total septum/cihaz oranı ise daha küçük idi (p<0,05). Seçilmiş vakalarda, TTE eşliğinde kapatma girişiminin işlem ve skopi süresini kısalttığı görüldü (p<0,05). Katater odasında görülen %32,4’lük rezidü oranının izlem sonunda %0,9 olduğu saptandı. Major komplikasyon (kurtarıcı cerrahi, trombüs, erozyon) ve minör komplikasyon (en sık ritm bozuklukları) oranları %1,4 ve %1,8 idi. Embolizasyon ve mortalite izlenmedi. Hastaların %7,7’sinde izlemde tedavi gerektirmeyen holter bozukluğu saptandı. Çalışma sonucunda transkatater ASD ve PFO kapatma işlemlerinin deneyimli uygulayıcılar tarafından yapıldığında güvenli ve etkili bir yöntem olduğu, defektin santral yerleşim gösterdiği ve rimlerinin yeterli olduğu durumlarda TTE kılavuzluğunda yapılan işlemlerin skopi süresini azaltması ve genel anestezi gerektirmemesinin ek bir avantaj olduğu görüldü

Nrg (Tm) 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 tsansseptal 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 (TM) RF powered transseptal needle in three patients.WoSScopu

Analysis of Gene Copy Number Variations in Patients with Congenital Heart Disease Using Multiplex Ligation-Dependent Probe Amplification

Objective: At the molecular and cellular levels, heart development entails the precise orchestration of genetic events such as the interplay of master transcriptional regulators, signaling pathways, and chromatin remodeling. Recent studies among patients with congenital heart disease (CHD) have shown the importance of recurrent copy number variations (CNVs) in a significant number of patients. Recurrent CNVs that span several genes may affect other important organs, besides the heart. Because CHD may be the first presenting symptom in such patients, the analysis of recurrent CNVs in the genomic regions containing genes associated with CHD in patients referring to cardiology clinics may lead to an early diagnosis and the treatment of extracardiac symptoms in these patients. In this study, we aimed to screen CNVs of genomic regions including GATA4, NKX2-5, TBX5, BMP4, and CRELD1 genes and to analyse the 22q11.2 chromosomal region in apparently nonsyndromic patients with cardiac septal defects. Methods: Genomic regions including GATA4, NKX2-5, TBX5, BMP4, and CRELD1 genes and the 22q11.2 chromosomal region were analyzed in apparently nonsyndromic 45 patients with cardiac septal defects using the MLPA P-311 A2 Congenital Heart Disease kit. Multiplex ligation-dependent probe amplification (MLPA) is an established technique for the detection of known CNVs. MLPA is substantially less expensive than array CGH and is relatively simple to use for clinicians without specific expertise in genomic technology; thus, MLPA could be used as a first-tier screening assay. Results: We screened 45 patients with cardiac septal defects for CNVs using the MLPA P-311 A2 kit. We identified three CNVs (n=3/45, 6.66%) and three 22q11 deletions. The CNVs were confirmed using fluorescence in situ hybridization. Conclusion: Our study confirmed that the analysis of recurrent CNVs using the MLPA assay within pediatric cardiology clinics can led to an early syndrome diagnosis in nonsyndromic patients with CHD.PubMedWoSScopu

Electrical Storm In An Adolescent With Arrhythmogenic Right Ventricle Cardiomyopathy Treated With Cardiac Transplantation

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Evaluation Of Tissue Doppler Echocardiographic Imaging Findings In Children With Pulmonary Hypertension

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.WoSScopu

Results of Radiofrequency Ablation in Children with Tachycardia-Induced Cardiomyopathy

Objective: Tachycardia-induced cardiomyopathy (TIC) is a rare but potentially curable cause of dilated cardiomyopathy. Data on radiofrequency ablation (RFA) for TIC is limited. The aim of this study is to present our experience with RFA in children with TIC, retrospectively. Methods: The medical records of patients with TIC and those underwent RFA between 2000-2011 were systematically reviewed. Demographics and echocardiographic features were recorded. Tachycardia-induced cardiomyopathy is defined as dilatation of the heart chambers or heart failure with chronic or very frequent cardiac arrhythmia. Diagnosis was confirmed with normalization of functions after sinus rhythm has been achieved. Results: A total of 12 patients with a mean age of 6.3 +/- 4.1 years (in utero-11.5 years) at diagnosis were enrolled in the study consisting of 7 patients with permanent junctional reciprocating tachycardia and 5 patients with focal atrial tachycardia. The mean pre-RFA left ventricular end-diastolic diameter and left ventricular ejection fraction (LVEF) values were 49.1 +/- 9.6 mm (32-66) and 40.8 +/- 13.4% (23-57%), respectively. One month after RFA, the mean LVEF value was 62 +/- 4.9% (52-69), with only 2 out of 12 patients' LVEF values were still lower than 58%. Tachycardia recurrence was observed in 4 patients, 3 of which had successful repeated RFA procedure. Overall, cure for arrhythmia was achieved in 11 patients (92%), while reversal of heart failure achieved in all patients. In 1 patient permanent complete AV block was developed after the procedure. Conclusion: Treatment of tachycardia with RFA, particularly in patients with arrhythmia refractory to medical therapy, is a feasible and effective treatment option regardless of age.WoSScopu

Is Transesophageal Electrophysiologic Study Valuable in Children with Successful Radiofrequency Ablation of Supraventricular Tachycardia on Follow-Up for Recurrence?

Objective: The aim of this study was to evaluate the efficacy of transesophageal electrophysiologic study (TEEPS) for the determination of supraventricular tachycardia (SVT) recurrences in symptomatic and asymptomatic children after successful radiofrequency ablation (RFA) for SVT. Methods: A total of 66 patients who underwent TEEPS after successful RFA were included. The demographic features, symptoms of the patients, and the characteristics of the recurrences induced by TEEPS were evaluated. The arrhythmia types induced during RFA were compared with those induced by TEEPS in terms of the compatibility of the diagnosis. Results: Forty-two (63.6%) girls and 24 (36.4%) boys with a mean age of 11.8±3.4 years were followed-up for 44.1±15.7 months. The average time between RFA and TEEPS was 5.2±5.9 months. The diagnoses during RFA were atrioventricular nodal reentrant tachycardia (AVNRT) in 47 of 66 patients, atrioventricular reentrant tachycardia (AVRT) in 18 of 66 patients, and ectopic atrial tachycardia in 1 of 66 patients. SVT was induced by TEEPS in 2 of 25 symptomatic and 5 of 41 asymptomatic patients. The SVT inducibility rate was 5.5% (1/18) and 12.7% (6/47) in patients with AVRT and AVNRT, respectively. In addition, 85.7% (6/7) of all recurrences occurred within 3.5 months. The recurrences as AVNRT in 2 of 25 symptomatic patients occurred in the first month after RFA. AVNRT in 4 of 41 and AVRT in 1 of 41 asymptomatic patients were induced within 3.5 months and 15 months, respectively. Conclusion: TEEPS seems to be a valuable screening and diagnostic method for the determination of recurrence in symptomatic and asymptomatic children who underwent successful RFA.PubMedWoSScopu