Transcatheter Closure of Atrial Septal Defect in Children: Single-Center Experience, Mid-Term Follow-up Results.

OBJECTIVE: Atrial septal defect is a congenital heart disease usually diagnosed in childhood. This study aimed to evaluate the mid-term follow-up results of patients who underwent trans- catheter closure of atrial septal defect by comparing the devices and methods used in the procedure and investigating the complications of this procedure in children. MATERIALS AND METHODS: This study evaluated 232 patient files retrospectively. Of the 232 patients, 24 were excluded from the study due to missing files or data. Also, patients with multi-fenestrated atrial septal defect and aneurismatic septal tissue were excluded from the study. The following data were evaluated: follow-up time, patient complaints, symptoms, trans- thoracic echocardiography, and transesophageal echocardiography findings (if performed), the size of the defect as measured by balloon-sizing, the size of the device used in the proce- dure, and major and minor complications. RESULTS: The study included 208 children who were diagnosed with atrial septal defect. The mean age of the patients was 88.0 ± 56.5 months. Of the patients, 170 (81.7%) had no com- plaints. The success rate of the procedure was found to be 95.7%. While device embolization was the most common major complication, arrhythmia was the most common minor complica- tion. The complication rate was statistically different according to the device type used in the procedure. CONCLUSION: Transcatheter closure of atrial septal defect is a safe method for atrial septal defect closure in pediatric patients. The study found that defect diameters measured by differ- ent methods were not correlated with each other. The procedure complication rates differed according to device type.

Balloon angioplasty of severe aortic coarctation in a newborn, using axillary artery access: The first experience in Turkey.

Axillary artery access for demanding interventions in newborns is rarely described, in spite of the many clear advantages. This access route in newborns with critical congenital heart disease is a real alternative to the more commonly used femoral or carotid artery routes. In brief, axillary access is an attractive alternative approach in newborns with complex heart diseases. It is advisable to stop an elective procedure if axillary access is not successful. Presently described is the use of axillary artery access for balloon angioplasty in a newborn with critical aortic coarctation. To the best of our knowledge, it was the first instance in Turkey.

Cardiovascular abnormalities in Williams syndrome: 20 years'experience in Istanbul.

AIMS: Williams syndrome (WS) is a microdeletion syndrome affecting cardiovascular and connective tissue as well as the endocrine and central nervous systems in 1 in 10,000 live births. This study aims to identify and evaluate cardiovascular abnormalities (CVAs) in 45 WS patients. PATIENTS AND METHODS: We retrospectively reviewed a cohort of WS patients who were followed at our institution from January 1, 1990 through December 31, 2010. WS was clinically diagnosed by an experienced medical geneticist and confimed by fluorescence in situ hybridization. CVAs were assessed using electrocardiography, echocardiography or cardiac catheterization. RESULTS: Twenty-seven patients (60%) were male; 18 were female (40%).The mean age at presentation was 4.6 +/- 3.1 years (3 months-13 years); the follow-up period was 6.9 +/- 4.4 years (6 months-18 years). CVAs were found in 86% of patients, the most common one being supravalvar aortic stenosis (SVAS) in 73% (isolated in 48%), peripheral pulmonary artery stenosis (PAS) in 42%, and mitral valve prolapse (MVP) in 22%. Less common were aortic insufficiency (15%), ventricular septal defect (11%), valvular pulmonary stenosis (11%), and aortic arch hypoplasia (8%) and coarctation (2%). Hypertension was present in 22% of patients. Surgical or catheter-based interventions were performed in 22% of cases. Two patients were lost in the postoperative period. CONCLUSION: CVAs were found in more than four out of five patients, the most common ones being SVAS and PAS. Although surgery was performed in more patients with SVAS than with PAS, SVAS was minimal or mild in most patients and improved in few cases.

[A rare cause of recurrent wheezing and respiratory distress: Scimitar syndrome]. / Tekrarlayan hisilti ve solunum sikintisinda nadir bir neden: Scimitar sendromu.

Scimitar syndrome is characterized by partial or total anomalous pulmonary venous return from the right lung. We present a 15-month-old boy who was diagnosed with Scimitar syndrome after examinations for recurrent wheezing and respiratory distress. The chest radiograph showed a scimitar sign in the right hemithorax, obscuring the contours of the right atrium. Echocardiography showed dilatation of the right heart cavities and increased flow in the inferior vena cava, without a cardiac abnormality. The patient underwent cardiac catheterization for radiographic and hemodynamic evaluations, during which a scimitar vein was detected, draining the right pulmonary veins to the inferior vena cava. Coil occlusion was performed on the abnormal artery arising from the infradiaphragmatic aorta. The patient was referred to surgery for repair of the anomalous pulmonary venous return and resection of the sequestered pulmonary segment.

[The comparison a 16-year follow-up results of balloon angioplasty for aortic coarctation in children of different age groups: a single-center experience]. / Aort koarktasyonu tanisiyla balon anjiyoplasti uygulanan farkli yas gruplarindaki çocuklarin 16 yillik izlem sonuçlarinin karsilastirilmasi: Tek merkez deneyimi.

OBJECTIVE: Pediatric patients with different age groups who underwent balloon angioplasty for aortic coarctation were evaluated for recoarctation, aneurysm, peripheral arterial injuries and concomitant diseases. METHODS: From January 1994 to 2010, 80 patients with aortic coarctation (native/recoarctation) were evaluated, retrospectively. According to age at angioplasty, patients were divided into three groups: Group A (0-3 months, n=29, 25 male/4 female, average weight 4±1.2 kg), Group B (3-12 months, n=20, 15 male/5 girls, average weight 6.5±1.9 kg) and Group C (> 1 year, n= 31, 15 male/16 girls, average weight 22.8±16 kg). The patients were followed with echocardiography and clinical signs. The data of the native and recoarcted patients and also those in three different age groups were analyzed by using Chi-square, Kruskal-Wallis, and Student t tests. RESULTS: Peak systolic pressure gradient was reduced from 42±17 mmHg to 6.2±6 mmHg after balloon angioplasty (p<0.001) [n=80, 56 (70%) native, 24 (30%) recoarctation]. There was no difference between groups for early success. None of them did require immediate surgery. There were ventricular septal defect in 23 (28.7%), bicuspid aorta in 18 (22.5%), patent ductus arteriosus in 11 (13.7%) patients. Two patients had Turner's syndrome. Mean follow-up period was 74±56 months. While recoarctation developed in 20 (25%), [12 (60%) in Group A, 5 (25%) in Group B and 3 (15%) in group C, incidence was higher in the 0-3 months age group (p=0.018). Femoral artery occlusion and aneurysm were developed in 6 (7.5%) and 4 (5%) patients, respectively, and all of them were under 1-year-old. CONCLUSION: Balloon angioplasty can be used a method in treatment of native aortic coarctation and postoperative restenosis. Especially, children under 3 months should be monitored closely after the procedure for recoarctation, aneurysms and peripheral artery problems.

Transcatheter closure of a fistula between the right pulmonary artery and left atrium using the Amplatzer septal occluder.

A congenital fistula between the right pulmonary artery (RPA) and left atrium (LA) is a rare condition that results in central cyanosis. An 11-year-old boy was admitted with exertional dyspnea and easy fatigability. He had severe cyanosis of the lips and limbs with clubbing of the fingers. Systemic oxygen saturation was 70%. There was no abnormal finding on electrocardiography, chest radiography, and echocardiography. Agitated saline injection showed early appearance of contrast bubbles in the LA. A pulmonary arteriovenous fistula was suspected and diagnostic cardiac catheterization was performed. Angiography demonstrated a large fistula between the proximal RPA and LA. The narrowest part of the fistula was 13.8 mm in balloon sizing. A 14-mm Amplatzer septal occluder was deployed at the narrowest site; however, the device migrated to the LA and then to the aortic arch. The device was removed and was successfully reimplanted to the fistula. After the procedure, arterial oxygen saturation increased from 70% to 96% and control angiography demonstrated complete occlusion of the fistula. The patient was symptom-free on follow-up evaluations at 6, 12, and 18 months, with a mean oxygen saturation of 96%. This case represents the first pediatric patient in whom a septal occluder was used.

Electrocardiographic findings at initial diagnosis in children with isolated left ventricular noncompaction.

BACKGROUND: The aim of this study was to comprehensively evaluate electrocardiographic (ECG) findings of isolated left ventricular noncompaction (IVNC) patients at initial diagnosis and to explore the correlation between them and the clinical, echocardiographic, and magnetic resonance imaging (MRI) findings. METHODS: Twenty-three patients diagnosed with IVNC by echocardiography and cardiac MRI between January 2006 and June 2010 were enrolled in this study. The patients were examined with standard ECG and 24-hour Holter ECG. For comparison purpose, ECGs of 50 healthy children of similar ages and demographic characteristics were taken. RESULTS: In 87% of patients, ECG abnormalities were found. The most frequently seen ECG findings were left ventricular hypertrophy, ST-segment depression, and negative T wave related to abnormal repolarization particularly in DII, DIII, and V(4-6) leads, as well as prolonged PR and QTc intervals. No ECG features or patterns were found that were specific to the disease. In contrast to adult patients, while no intraventricular conduction defects (particularly in the left bundle brach) were found in any of our patients, 13% had considerable bradycardia and one required a pacemaker. The Holter ECG recordings showed supraventricular tachycardia attacks in two patients and a short ventricular tachycardia attack in one. Patients whose echocardiograms and MRI showed left ventricular systolic dysfunction and left ventricular dilatation had signs of left ventricular hypertrophy and repolarization abnormality on their ECGs, but there was no significant difference in PR, QRS, and QTc intervals. CONCLUSION: Regardless of how frequently left ventricular hypertrophy and repolarization abnormalities are found on IVNC patients' initial ECGs, we think that they are not unique to the disease but are related to the severity of the cardiomyopathy.

Left ventricular non-compaction in children and adolescents: clinical features, treatment and follow-up.

BACKGROUND: Left ventricular non-compaction (LVNC) is a specific cardiomyopathy that occurs following a disruption of endomyocardial morphogenesis. This study presents clinical findings, diagnostic features, treatment and follow-up of pediatric patients diagnosed with LVNC. METHODS: Patients with LVNC who were followed from January 2006 to March 2010 were included in this study. Diagnosis was made with the use of characteristic findings of magnetic resonance imaging and echocardiography. Holter electrocardiography and metabolic screening tests were also performed in all patients. RESULTS: A total of 24 patients were studied (18 male, six female). Patient age at diagnosis was 50 ± 60 months (eight days to 15 years). Average follow-up period was 22 ± 12 months (four months to four years). Findings at diagnosis were as follows: eight (33%) patients had heart failure, five (20%) had rhythm abnormalities, five (20%) had cardiomegaly, two had murmurs, two had cyanosis, and two presented with fatigue. Ten (41%) patients had been followed previously with other diagnoses. In 21 (87.5%) patients, electrocardiographic abnormalities were noted, especially left ventricular hypertrophy and ST-T changes. Patients had an average ejection fraction of 46% (18-73%) and three of them had additional congenital heart disease (patent ductus arteriosus, aortopulmonary window and complex cyanotic heart disease). Scanning for metabolic diseases revealed fatty acid oxidation disorder in one patient, and mitochondrial disease in another. During follow-up, a permanent pacemaker was implanted in a patient with severe bradycardia and ventricular dysfunction, and three patients died. CONCLUSION: LVNC can be diagnosed at any age from newborn to adolescent and has a variable clinical course. Closer study of patients with cardiomegaly and heart failure can reduce delays in diagnosis of LVNC.

Marcadores eletrocardiográficos para detecção precoce de doença cardíaca em pacientes com talassemia beta maior / Electrocardiographic markers for the early detection of cardiac disease in patients with beta-thalassemia major

OBJETIVO: Analisar comparativamente a dispersão da onda P (DOP) em pacientes com talassemia beta maior (β-TM) e indivíduos saudáveis (controles) para a detecção precoce do risco de arritmias. MÉTODOS: Oitenta e uma crianças com β-TM, com idades entre 4 e 19 anos, e 74 crianças saudáveis (grupo controle) foram submetidas a exame eletrocardiográfico e ecocardiograma transtorácico de rotina para avaliação cardíaca. A DOP foi calculada como a diferença entre as durações máxima e mínima da onda P. RESULTADOS: Houve uma diferença estatisticamente significativa entre o grupo de estudo e o grupo controle no pico de velocidade do fluxo transmitral no início da diástole (E) e na razão E/fluxo transmitral tardio (A). A duração máxima da onda P e a DOP foram significativamente maiores nos pacientes com β-TM do que nos indivíduos controles. CONCLUSÕES: O aumento da DOP em nossos pacientes com β-TM pode estar relacionado à depressão na condução intra-atrial, devido à dilatação atrial, e ao aumento da atividade simpática. Estes pacientes devem ser acompanhados atentamente devido à possibilidade de ocorrência de arritmias com risco de vida.

OBJECTIVE: To comparatively evaluate P-wave dispersion (PWD) in patients with β-thalassemia major (TM) and healthy control subjects for the early prediction of arrhythmia risk. METHODS: Eighty-one children with β-TM, aged 4-19 years, and 74 healthy children (control group) underwent routine electrocardiography and transthoracic echocardiography for cardiac evaluation. PWD was calculated as the difference between the maximum and the minimum P-wave duration. RESULTS: There was a statistically significant difference between study and control groups in peak early (E) mitral inflow velocity and E/late (A) velocity ratio. Maximum P-wave duration and PWD were found to be significantly higher in β-TM patients than in control subjects. CONCLUSIONS: Increased PWD in our β-TM patients might be related to depression of intra-atrial conduction due to atrial dilatation and increased sympathetic activity. These patients should be closely followed up for risk of life-threatening arrhythmias.

Congenital heart disease in children with Down's syndrome: Turkish experience of 13 years.

BACKGROUND: Down's syndrome (DS) is the most common chromosomal abnormality due to a trisomy of chromosome 21 commonly associated with congenital heart defects (CHDs). This study aimed to evaluate the frequency and types of CHD patterns in Turkish children with DS. METHOD: The data relate to paediatric patients with DS who underwent cardiologic screening between 1994 and 2007 and were reviewed in our Paediatric Cardiology unit. RESULTS: Four hundred and twenty-one out of the 1042 paediatric patients with DS studied over a 13-year period had associated CHD. Of these, 320 (77.6%) had a single cardiac lesion, while the remaining 92 patients (22.4%) had multiple defects. The most common single defect was an atrioventricular septal defect (AVSD) found in 141 patients (34.2%), followed by 69 patients (16.7%) showing secundum type atrial septal defect, and ventricular septal defect in 68 patients (16.5%). AVSDs were the leading type, isolated or combined with other cardiac anomalies with an overall occurrence of 19.8% of paediatric patients with DS, and 49.2% of paediatric patients with both DS and CHD. CONCLUSION: This is the first study concerning the frequency and type of CHD observed in Turkish children with DS. The high frequency of AVSD in Turkish children with DS implied that early screening for CHDs by echocardiography is crucial. The correction of AVSDs in paediatric patients with DS should be performed in the first 6 months of life to avoid irreversible haemodynamic consequences of the defect.

Long term follow-up results of 139 Turkish children and adolescents with rheumatic heart disease.

We aimed to evaluate the predictors of the severity of chronic rheumatic valvar disease. The long term follow-up records of 139 patients with chronic rheumatic carditis were reviewed. Children were followed-up on an outpatient basis for a period ranging from 1-16 years (5.0 +/- 3.7 years). Mitral regurgitation either isolated (51%, n=71) or combined with aortic regurgitation (AR) (49%, n=68) was observed in all cases of the initial attack of rheumatic carditis. AR at initial attack of the rheumatic carditis was found to be affected by gender (AR was more associated with males, p = 0.032), combined mitral and aortic regurgitation (CMAR), and presence of MR at initial attack (p = 0.000 and p = 0.012, respectively) with univariate analysis. The effect of CMAR on AR at initial attack was also significant by multivariate analysis (p = 0.000). CMAR, MR, and AR at initial attack had significant effects on CMAR at final evaluation (p = 0.000, p = 0.020, and p = 0.000, respectively) in univariate analysis. Multivariate analysis revealed the significant effects of CMAR and MR at initial attack on CMAR at final evaluation (p = 0.000 and p = 0.005, respectively). Univariate analysis showed that MR and AR at initial attack, and CMAR at final evaluation, had significant effects on MR at final evaluation (p = 0.000, p = 0.029, and p = 0.000, respectively). MR at initial attack and CMAR at final evaluation had significant effects on MR at final evaluation with multivariate analysis (p = 0.001 and p = 0.003, respectively). AR at final evaluation was affected by CMAR and AR at initial attack (p = 0.000 and p = 0.000, respectively), and CMAR and MR at final evaluation (p = 0.000 and p = 0.000, respectively) with both univariate and multivariate analysis. Mitral valve prolapsus was more common in patients with a longer duration (37.5%, 6 out of 16) than those with a shorter duration (11%, 14 out of 123) and the difference was significant (p = 0.020). In conclusion, the initial severity of valve involvement and the presence of CMAR at initial attack were found to be the best predictors for the severity of chronic rheumatic valvar disease in this study.

The effects of surgical repair on P-wave dispersion in children with secundum atrial septal defect.

INTRODUCTION: Atrial septal defect (ASD) is one of the most common congenital heart diseases in children. P-wave dispersion has been reported to be associated with non-homogeneous propagation of sinus impulses. The heterogeneity of atrial conduction time may predispose the atria to arrhythmias. The aim of this study was to determine the impact of surgical repair on P-wave indices in children with isolated secundum ASD. METHODS: Children with isolated secundum ASD undergoing surgical repair (n=50; mean age, 7.0+/-3.0 years) and healthy controls (n=51; mean age, 7.6+/-2.7 years) were compared. Maximum P-wave duration (Pmax), shortest duration (Pmin) and P-wave dispersion (Pd) were measured using 12-lead surface electrocardiography. RESULTS: Mean Pmax was found to be significantly higher in children with ASD compared with controls (95.2+/-10.8 vs 84.1+/-9.2 msec; P<0.001), and Pd before surgery was significantly higher compared with controls (47.4+/-12.0 vs 38.8+/-9.7 msec; P<0.001). Both P-wave indices were significantly decreased within the first year after surgical closure - the values decreased to those comparable to healthy controls (Pmax, 86.2+/-9.7 msec; Pd, 39.8+/-10.7 msec; P>0.05). CONCLUSION: Surgical closure of ASD in children decreases Pmax and P-wave conduction time. We speculate that earlier closure of the defect may play an important role in avoiding permanent changes in the atrial myocardium and atrial fibrillation in adulthood.

Electrocardiographic and echocardiographic findings in street children known to be substance abusers.

Substance abuse is prevalent in adolescent street children, and death is reported as secondary to aspiration, accidental trauma, asphyxia, cardiac arrhythmia, anoxia, vagal inhibition and respiratory depression. In this study, we examined electrocardiographic and echocardiographic findings from 53 street male adolescents, comparing our findings to those obtained from 61 controls in the same age group. The street children smoked cigarettes (98.1%) and had used, or were using, thinner (73.6%), glue (75.5%), hashish (79.2%), morphine or its products (24.5%), ecstasy (37.7%), anti-emetics (13.2%) and alcohol (60.4%). On examination, their blood pressures were lower than the control group. Electrocardiographically, PR, QRS, QT were found to be longer (p less than 0.05) than the values for healthy controls. Although it was not statistically significant, QTc duration was also longer than the control group. Echocardiography revealed increased diameters of the left ventricle and atrium, the aorta, and the coronary arteries as compared to the healthy children (p less than 0.05).

Fish oil supplementation improves left ventricular function in children with idiopathic dilated cardiomyopathy.

Fish oil has a cardioprotective effect in adults with ischemic heart disease. The authors examined the effects of fish oil in children with idiopathic dilated cardiomyopathy (DCM). Eighteen DCM patients (group I) and 12 healthy children (group III) were given fish oil (10 mL/d). Their cardiac findings were compared with those of 11 patients with DCM who did not receive fish oil (group II). After 6.62+/-1.70 months, left ventricular ejection fraction had increased by 8.44%+/-3.80% (P<.05), in group I; 2.48%+/-3.85% (not statistically significant) in group II; and 0.84%+/-2.34% (not statistically significant) in group III. Left ventricular internal diastolic diameter (mm) was reduced by 4.36+/-4.86 (P=.001) in group I and 1.92+/-5.37 (P=.263) in group II, but increased by 0.22+/-2.54 (not statistically significant) in group III. The results suggest that fish oil leads to accelerated improvement of left ventricular function. The authors believe that if these results are confirmed in larger studies, fish oil should be added to the standard anticongestive therapy of children with DCM.

Left-sided aortic arch, right-sided descending aorta, and right-sided arterial duct associated with interruption of the aortic arch and presence of Abbott's artery.

We describe a male infant, seen at the age of 10 days, with a very rare form of vascular ring. The aortic arch was left-sided, but the aorta descended on the right, with a right-sided persistently patent arterial duct associated with interruption of the aortic arch and presence of Abbott's artery. We performed end-to-side anastomosis of the descending aorta to the ascending aorta, divided the Abbott's artery and the right-sided arterial duct, and banded the pulmonary trunk. Despite our best efforts, the patient died on the eighth day after the surgical intervention.

Epitheloid hemangioendothelioma of the heart in infancy.

We report a case of primary cardiac epithelioid hemangioendothelioma arising from the right atrium of a 2-month-old infant. The tumor was found incidentally during exploratory sternotomy for recurrent pericardial effusion. This case represents a very rare situation, because this is the youngest patient found in relevant literature, and because it involves extensive infiltration by the tumor without any development of intracardiac mass appearance.