Standart Cordes tekniğine göre kalbin sağ/sol ekseninin ve malpozisyonlarının prenatal ekokardiyografik tanısı

Objective: The aim of this study was to evaluate distinguishing the right/left side of the fetus, cardiac axis and position according to the standardized Cordes technique in 20 cases with cardiac malposition. Methods: We studied retrospectively 1536 cases whose fetal echocardiographic examinations were performed between 1999 and 2006 in prenatal cardiology unit. Among these, cardiac malpositions were determined in 20 cases. The cardiac axis and position were determined according to the Cordes technique. All cases were followed-up by serial fetal echocardiograms until birth or intrauterine death occurred. In cases of intrauterine death, an autopsy was performed. After birth, physical and echocardiographic examinations were done and prenatal and postnatal diagnoses were compared. Results: Of 1536 fetal echocardiograms performed, 144 revealed congenital heart diseases (9.4%), among these cases 20 were diagnosed with cardiac malposition. Of cases with cardiac malposition, 16 had congenital heart disease, and four had extracardiac malformation. There were six cases of isolated dextrocardia, three cases of situs inversus totalis, six cases of situs ambiguous, and one case of situs inversus with isolated levocardia. Of four cases with extracardiac malformations, two cases had mesoposition, one had dextroposition, and one had extreme levoposition. In six cases the autopsy findings were the same as that their prenatal echocardiographic findings. When postnatal echocardiographic results of the remaining cases with cardiac malposition due to congenital heart disease were compared with prenatal diagnoses, the same echocardiographic findings were verified. Conclusion: The fetal right/left axis must be determined correctly for the accurate diagnosis of cardiac malpositions. Therefore, we recommend that Cordes technique provides a simple and reliable determination of the fetal right/left axis and fetal situs.Bu çalışmada, standart Cordes tekniğine göre fetüsün sağ/sol tarafının, kalbin aksının ve pozisyonunun ayrımı ve bu teknikle kardiyak malpozisyon tanısı konulan 20 olgunun değerlendirilmesi amaçlandı. Yöntemler: Çalışmamızda, 1999-2006 yılları arasında prenatal Kardiyoloji ünitesinde fetal ekokardiyografi yapılan 1536 olguyu retrospektif olarak değerlendirildi. Bu olguların 20’sinde kardiyak malpozisyon saptandı. Kalbin aksı ve pozisyonu Cordes tekniğe göre saptandı. Tüm olgular seri fetal ekokardiyografik çalışmalar ile doğuma veya intrauterinde ölüm meydana gelene kadar izlendi. İntrauterin ölen olgulara otopsi yapıldı. Doğumdan sonra fizik muayene ve ekokardiyografik değerlendirme yapıldı, prenatal ve postnatal tanılar karşılaştırıldı. Bulgular: Fetal ekokardiyografi yapılan 1536 olgunun 144’ ünde konjenital kalp hastalığı saptandı ve bu olguların 20’sine kardiyak malpozisyon tanısı konuldu. Kardiyak malpozisyonlu olguların 16’sında konjenital kalp hastalığı ve 4’ünde kalp dışı nedenlere bağlı malpozisyon mevcuttu. Altı olguda izole dekstrokardi, 3 olguda situs inversus totalis, 6 olguda situs ambigus ve 1 olguda izole levokardi ile birlikte situs inversus saptandı. Kalp dışı nedenlere bağlı malpozisyonlu 4 olgunun 2’sinde mezopozisyon, 1’inde dekstropozisyon ve 1’inde ileri levopozisyon mevcuttu. Altı olguya yapılan otopsi bulguları prenatal ekokardiyografik bulgular ile benzerdi. Konjenital kalp hastalığına bağlı kardiyak malpozisyonlu diğer olguların doğum sonrası ekokardiyografik sonuçları prenatal tanılarıyla karşılaştırıldığında aynı sonuçlar elde edildi. Sonuç: Kardiyak malpozisyonun doğru tanısı için fetüsün sağ/sol aksı doğru saptanmalıdır. Bu nedenle fetal situs ve fetüsün sağ/sol aksının saptanmasında kolay ve güvenilir bir teknik olan Cordes tekniğini öneriyoruz

Fetal Arrythmia; Maternal Systemic Lupus Erythematosus

Congenital heart block may develop in the fetus during pregnancy in anti-Ro/La antibodies positive women, leading to heart failure of fetus in utero and even mortality in the first 3 years of life. In this study, we report a fetal arrhythmia case who manifested in 21 weeks of gestational age. Diagnosis of Systemic Lupus Erythematosus (SLE) was confirmed with the positive blood samples for antinuclear antibodies, anti-Ro/SSA and anti-La/SSB and ultrasonography. There was no hidrops in the fetus when arrhythmia was detected. After steroid therapy, patient`s symptoms concerning SLE regressed. Following the delivery of baby, permenant pacemaker had been applied and currently, the baby has no cardiac problem. Therefore, it could be concluded that, SLE can be asymptomatic till pregnancy and the control of the disease is essential for the delivery of a healthy baby

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.PubMedWoSScopu

UNCORRECTED PROOF-IN PROCESS Original Investigation Clinical and electrophysiological evaluation of pediatric Wolff- Parkinson-White patients

Objective: Wolff-Parkinson-White (WPW) syndrome presents with paroxysmal supraventricular tachycardia and is characterized with 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 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 and 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, 14 with syncope; 13 were asymptomatic. Atrial fibrillation (AF) was induced in 6 of the tachycardia patients, 5 of the syncope patients and 1 of the asymptomatic patients. Induced AF degenerated to ventricular fibrillation (VF) in 2 patients. Of the two patients with VF one was asymptomatic and the other had syncope, the accessory pathway effective refractory period was ≤180 ms in both. Intracardiac electrophysiologic study was performed in 92 patients and ablation was not attempted for risk of atrioventricular block in 8 (8.6%). Success rate of ablation was 90.5%. Conclusion: 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 it was thought. All patients with WPW pattern on ECG should be electrophysiologically assessed and risk stratified. Ablation of patients with risk factors can prevent sudden death in this population. (Anadolu Kardiyol Derg 2014; 14(0): 000-000

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