Assessment of cardiac function and electrocardiographic findings in patients with Wilson's disease.

BACKGROUND: This study evaluated cardiac function using tissue Doppler echocardiography and assessed electrocardiographic findings in children diagnosed with Wilson's disease. METHOD: Asymptomatic patients with a diagnosis of Wilson's disease (n = 43) were compared to healthy controls (n = 37) that were age and gender matched. RESULTS: The standard electrocardiographic and conventional echocardiographic examinations were similar in both groups. The left ventricular ejection fraction, shortening fraction, and diastolic function were not significantly different between the two groups. The Tei index for mitral lateral, mitral septal, tricuspid lateral, tricuspid septal, and inter-ventricular septum on tissue Doppler echocardiography was higher in the patient group, yet it did not reach statistical significance. Mitral lateral and septal systolic annular velocity values were significantly lower in the patient group when compared to the control group (p = 0.02 and 0.04, respectively). Also, mitral lateral and septal isovolumetric contraction time values were higher in the patient group (p = 0.04). Although the left ventricular values were not significantly different, relative left ventricular wall thickness was higher in the patient group when compared to the control group, and concentric remodelling in the left ventricle was found in 7 (16%) of 42 patients. QT interval (p = 0.02) and P-wave dispersion values (p = 0.04) were significantly higher in the patient group compared to the control group, and these tend to predict arrhythmias. CONCLUSION: Our study based on the tissue Doppler echocardiography assessment indicated a subclinical systolic, rather than diastolic, dysfunction in the myocardium with increased QT interval and P-wave dispersion, despite the young age of the patients and short disease duration.

Transcatheter closure of the patent foramen ovale in children: intermediate-term follow-up results.

The patent foramen ovale is almost a normal anatomical hole between the atria with ~30% incidence in the general population. It has been suggested that the patent foramen ovale is the cause of some neurological events, which is explained by paradoxical embolism. Transcatheter closure of the patent foramen ovale is a common procedure in adult patients with cerebral ischaemic events, but there are limited data investigating the results in children. Between January, 2005 and February, 2014, 17 patients' patent foramen ovales were closed by the transcatheter approach in our department. The indications for closure were transient ischaemic attack in 10 patients, stroke in four patients, and migraine in three patients. The mean age and mean weight at the time of the procedure were 11.1±3.7 years and 42.1±15.4 kg, respectively. We asked our patients whether their previous ailments continued. All patients responded to the study survey. In 15 patients, ailments did not continue after patent foramen ovale closure and they significantly decreased in two of them. We suggest that under the right conditions device closure of the patent foramen ovale is a safe solution for these cryptogenic ischaemic events and migraine.

Unusual Presentations of Childhood Systemic Lupus Erythematosus to the Emergency Department.

INTRODUCTION: Systemic lupus erythematosus (SLE) is a multisystemic autoinflammatory disease that can involve any organ system; therefore, diagnosis can be challenging. Hereby, we present 4 cases that presented to pediatric emergency department with unusual clinical pictures of SLE. CASES: Case 1 presented with inability to walk or talk for the last 1 week as well as intermittent pain and swelling in her joints. Case 2 presented with generalized edema and severe dyspnea. Case 3 and 4 presented to pediatric emergency department with rashes on the legs. DISCUSSION: Systemic lupus erythematosus may mimic many clinical entities, and differential diagnosis may be difficult, especially if presentation is atypical. In every emergency physician, right diagnosis and prompt treatment are very important especially in life-threatening conditions such as cardiac involvement in SLE.

Midterm Results of Implantable Cardioverter Defibrillators in Children and Young Adults from a Single Center in Turkey.

BACKGROUND: 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. METHODS: This retrospective study included patients who were implanted with an ICD between 2001 and 2014. Demographic characteristics, clinical information, shock features, and complications for all patients with ICD were analyzed. The study population was divided into two groups: early-era patients implanted before 2008, and late-era patients implanted after 2008. RESULTS: Sixty-nine patients (median age: 12 years, median follow-up: 52 months) were implanted with an ICD. Diagnostic categories were channelopathy (56.6%), cardiomyopathy (36.2%), congenital heart disease (5.8%), and other (1.4%). We performed implantation for primary prevention in 66.6% (39.3% in early-era patients and 85.4% in late-era patients). Thirty-one (44.9%) received 139 appropriate shocks (66% of total shocks) while 14 (20.2%) received 71 inappropriate shocks. However, there was no statistically significant difference in the use of appropriate shocks in the primary (66.7%) versus the secondary (72.2%) prevention groups. The incidence of appropriate and inappropriate shock was 66.7% and 33.3% in the primary prevention group, and 72.2% and 27.8% in the secondary prevention group, respectively. Two patients died, although only one death was the result of a lead problem. CONCLUSIONS: Although lead integrity problems, inappropriate shocks, and infections are significant issues, ICD therapy appears to be a safe, effective, and necessary option for the prevention of SCD in both children and young adults.

Fetal aorta larger than the main pulmonary artery on the three-vessel view: Correlation with postnatal echocardiographic findings.

PURPOSE: This study investigated postnatal cardiac anomalies determined by postnatal echocardiography in fetuses with the ascending aorta (AA) diameter larger than that of the main pulmonary artery (MPA) on the three-vessel view (3VV). METHODS: The study included 17 pregnancies. The diameters of the AA and MPA were assessed on the 3VV in second-trimester sonographic screening, and all the patients underwent postnatal echocardiography to assess the cardiac outcome. RESULTS: In the study population, the mean AA diameter was 3.7 mm (range, 2.2-5.6 mm), and the mean MPA diameter was 3.2 mm (range, 1.8-5.2 mm). The mean AA/MPA ratio was 1.2 (range, 1.1-1.9). According to the postnatal echocardiograms, one of the patients had tetralogy of Fallot. This patient had the highest prenatal AA/MPA ratio (1.9). Among the remaining 16 cases, five had secundum atrial septal defects, with two having concomitant dilatation of the AA. There was one case of isolated dilatation of the AA. CONCLUSIONS: Although an AA with a diameter larger than that of the MPA on the 3VV does not usually indicate severe congenital heart disease involving the ventricular outflow tract and/or great arteries, careful prenatal and postnatal echocardiographic examinations are mandatory to determine the presence of congenital heart disease. © 2016 Wiley Periodicals, Inc. J Clin Ultrasound 44:423-428, 2016.

Serum Bcl-2 values in children with pulmonary hypertension.

In this study, we aimed to assess levels of serum B cell lymphoma 2 (sBcl-2) in children, which has been implicated in the etiopathogenesis of pulmonary hypertension (PH), as well its association with tissue Doppler echocardiographic imaging (TDI) data and parameters used in the follow-up of PH. The sBcl-2 level was assessed in 35 children with PH (24 had eisenmenger syndrome, and 11 had idiopathic PH) and in 38 healthy children as controls. TDI was performed on 25 patients whose cardiac anatomy allowed the test. The respective sBcl-2 values in patients and controls were 35.69 ± 18.83 and 2.66 ± 7.95 ng/ml (p < 0.001). The sBcl-2 levels were significantly greater in the New York Heart Association (NYHA) functional class 3 patients than those in the NYHA class 2 patients (p = 0.033). The sBcl-2 value in patients who walked <475 m in the 6-min walk distance (6MWD) test was significantly greater than in those who walked ≥475 m (p = 0.038). The sBcl-2 level showed a negative correlation with ejection time measured at the septal anulus (p = 0.026) and a positive correlation with interventricular septum-Tei (p = 0.018). The results of this study showed for the first time that there is an increase in the levels of sBcl-2 as an inflammatory marker and that the sBcl-2 levels are associated with prognostic parameters in children with PH. Because sBcl-2 levels were greater in patients who walked <475 meters during the 6MWD test, we suggest 475 ms as the cut-off value for the 6MWD test to differentiate between a good and a bad prognosis.

Comparison of Transesophageal and Intracardiac Electrophysiologic Studies for the Diagnosis of Childhood Supraventricular Tachycardias.

In this study, we aimed to compare the results of transesophageal electrophysiologic studies (TEEPS) and intracardiac electrophysiologic studies (IEPS) in a cohort of pediatric patients with SVTs. The medical records of children aged between 0 and 18 years who underwent TEEPS between January 2007 and June 2012 were systematically reviewed, and those without pre-excitation and who underwent subsequent IEPS were identified. Post-procedural diagnoses were compared for compatibility. A total of 162 patients were included in the study with a mean age at diagnosis 11.6 ± 3.6 years. Tachycardia was induced in 152 patients by TEEPS and in 154 patients by IEPS. Overall, in 147 patients, tachycardia was induced by both TEEPS and IEPS. Diagnoses were compatible in 135 out of 147 patients (91.8 %). Nine out of the 12 patients with discrepant results were diagnosed with atrioventricular-reentrant tachycardia (AVRT) and three with atrioventricular nodal reentrant tachycardia (AVNRT) after TEEPS. Following IEPS, TEEPS diagnosis of AVRT was revised to typical AVNRT in 5 patients and atypical AVNRT in 4 patients. Two of the 3 patients who were diagnosed as having AVNRT following TEEPS were confirmed to have atrial tachycardia after IEPS, while the other patient was diagnosed with AVRT. Tachycardia terminated spontaneously in 3 patients, while overdrive pacing was attempted to terminate the tachycardia in 149 patients, with a success rate of 93.2 % (139/149). The diagnostic compatibility between TEEPS and IEPS is quite high. A diagnostic discrepancy mostly occurs in patients diagnosed with AVRT by TEEPS, and the possibility of atypical AVNRT should be considered in patients with a VA ≥70 ms to avoid such discrepancies.

Sensitivity of transesophageal electrophysiologic study in children with supraventricular tachycardia on electrocardiography.

INTRODUCTION: The aim of this study was to evaluate the inducibility of tachycardia by transesophageal electrophysiologic study (TEEPS) in patients with documented supraventricular tachycardia (SVT) on electrocardiography and to investigate the accuracy of TEEPS records by comparing with intracardiac electrophysiologic study (IEPS). MATERIAL AND METHODS: The TEEPS records of patients having documented electrocardiography during SVT were reviewed. The results of TEEPS in 43 of 85 patients were compared with results of IEPS for compatibility of diagnosis. RESULTS: A total 85 patients, 46 male and 39 female, mean weight 35.1 kg (36-87), aged 1 month-17 years, were included. Tachycardia was induced by TEEPS in 79 of 85 patients with documented electrocardiography (sensitivity 92.9%). IEPS for diagnosis or ablation was conducted in 40 patients having inducible tachycardia and three of six who had no inducible tachycardia by TEEPS. Tachycardia was induced by IEPS in 39 of 40 (97.5%) patients who had inducible tachycardia and two of three who had no inducible tachycardia by TEEPS. Mechanisms of tachycardias were similar in 97.5% of patients (37/39) who had inducible tachycardia in TEEPS and IEPS. One of the patients with atrioventricular reentry tachycardia by TEEPS was diagnosed as atrioventricular nodal reentry tachycardia (AVNRT) and the other one was diagnosed as atypical AVNRT and atrial tachycardia by IEPS. CONCLUSION: The rates of inducibility and mechanisms of tachycardias by TEEPS in children having documented SVT were similar with those obtained from IEPS.

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

BACKGROUND: 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. METHODS: Children aged between 0 and 18 years who received combination therapy with amiodarone and propranolol for persistent monotherapy resistance tachyarrhythmia between 2007 and 2011 were included in the study. Treatment efficacy and adverse effects were evaluated by review of clinical signs and symptoms, 12-lead electrocardiogram, 24-hour Holter monitorization, liver enzymes, thyroid function tests, chest x-ray, and ophthalmologic examination. RESULTS: A total of 25 patients (15 male and 10 female) were enrolled in this study with a mean age of 17.9 months (0-132) and a mean weight of 8.65 kg (2.2-25). In 18 patients, treatment was started before their first age. Tachyarrhythmia persisted in two patients despite combination therapy, and treatment regimen was changed. Complete control of the arrhythmia was achieved within the first 2 months of combination treatment in 20 patients, whereas in the remaining three patients short attacks of tachycardia who responded to dose increases were controlled beyond 2 months of treatment and no recurrence were observed. Overall, success of amiodarone-propranolol combination treatment was 92%. Mild elevation in thyroid-stimulating hormone level was observed in one patient that required discontinuation of treatment. CONCLUSION: Our results suggest that a combination of amiodarone and propranolol is an effective and safe option for the treatment of persistent arrhythmias in neonates and infants, and may serve as a bridge to ablation therapy in older children.

Cardiac rhabdomyomas in childhood: six cases from a single institution.

Primary cardiac tumors are rare during childhood. The most frequently encountered tumors are rhabdomyomas. We reviewed the clinical characteristics, treatment results, and outcomes of six pediatric patients with primary cardiac rhabdomyomas. The mean age was 16.8 days. Only one patient was symptomatic. The tumors mostly originated from the left ventricle. The diagnosis was established by magnetic resonance imaging (MRI) plus echocardiography with or without histopathology. Total tumor resection was performed in two patients. After a median follow-up of 39 months, one patient had a stable tumor, two patients had marked tumor regression and one had complete tumor regression. Considering the fact that rhabdomyomas often show spontaneous regression, close follow-up may be sufficient in hemodynamically stable cases. Although rhabdomyomas do not cause any symptoms at the time of diagnosis, they may lead to sudden death; thus, further studies may be required for the decision of surgery and/or followup. The localization and infiltrative characteristics of the tumor are critical factors for decision-making in children with symptomatic rhabdomyoma even if surgery is indicated in such cases.

Early-mid term follow-up results of percutaneous closure of the interatrial septal defects with occlutech figulla devices: a single center experience.

BACKGROUND: Percutaneous closure of secundum type atrial septal defect (ASD) and patent foramen ovale (PFO) has gained widespread use in recent years. Herein, we evaluated the safety and efficacy of the Occlutech® Figulla devices for PFO and ASD closure in a reference tertiary center. METHODS: All 143 patients (46.9% male, mean age 39.3 ± 12.2 years) who underwent transcatheter PFO (n = 85) and ASD (n = 58) closure with Occlutech® Figulla devices between February 2009 and October 2011 were included in this study. An echocardiographic follow-up examination was performed at the 1st, 6th, and 12th month visits. RESULTS: The devices were successfully implanted in all 143 patients (100%). In-hospital periprocedural complications were device embolization (0.7%; 1 ASD patient), atrial fibrillation (1.4%; 1 ASD and 1 PFO patients), supraventricular tachycardia (0.7%; 1 PFO patient), and vascular access hematoma (0.7%; 1 ASD patient). Among ASD patients, 2 patients had trivial (jet width <1 mm in diameter) and 1 patient had small (1-2 mm) residual shunts before hospital discharge, which disappeared after the 6-month visit. During the mean 15.4 ± 9.6 months follow-up, all patients were asymptomatic and no ischemic stroke, cardiac perforation, device erosion, embolization, thrombus formation, or malposition of the device was observed. CONCLUSIONS: Percutaneous PFO and secundum type ASD closure with the novel Occlutech® Figulla Occluder devices without left atrial central pin and with significantly reduced meshwork was safe, feasible, and effective.

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.

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.

Clinical features and mid- and long-term outcomes of pediatric patients with subclinical carditis.

A study to examine mid- and long-term outcomes in patients with subclinical carditis was conducted. Data obtained at the time of diagnosis and during the follow-up of 158 patients diagnosed with subclinical carditis were retrieved and analyzed. Most patients had isolated mitral insufficiency. Frequency of morphological changes in the mitral valve was significantly lower in patients with at least one additional Jones criterion both at baseline (10.3% vs. 48.8%, p < 0.01) and at the end of the follow-up (27.8% versus 43.5%, p = 0.11). Mean jet size for mitral (12.0 +/- 8.8 versus 18.2 +/- 5.5 mm, p < 0.01) and aortic (4.1 +/- 4.0 versus 14.0 +/- 5.8 mm, p = 0.008) insufficiency were decreased compared to baseline. Improvement in mitral insufficiency was more frequent among patients with more than five years of follow-up (82.6% versus 60.0%, p = 0.039). Subclinical carditis due to acute rheumatic fever is not a benign and temporary condition. These patients should be given secondary prophylaxis.

Analysis of idiopathic ventricular tachycardia in childhood.

Idiopathic ventricular tachycardia (VT) is a relatively benign and rare form of VT. It is seen in young people without demonstrable cardiac pathology. The aim of our study was to review the clinical picture of idiopathic VT, before evaluating the indications for antiarrhythmic treatment and the efficacy of radiofrequency ablation (RFA). The notes of patients diagnosed with idiopathic VT in the last 13 years (n: 22) were included in the study. The median age of onset was 11 years (1 month-16 years). We evaluated the findings regarding the diagnosis, treatment and prognosis of these patients. The most common initial symptom was palpitation, in 15 cases. Five children with idiopathic VT were symptom-free. VT was of right ventricular origin in 10 patients and left ventricular origin in 8 patients. Beta-blockers were the mainstay of medical treatment in right VT and calcium channel blockers (Ca-channel blocker) were mostly used in left VT cases. The success rate of RFA was 57% in right VT and 100% in left VT. The median follow-up was 41 months (9 months-60 months), and all patients are alive currently with no symptoms. VT without demonstrable cardiac pathology is associated with a good prognosis. Treatment is unnecessary for asymptomatic non-sustained VT. RFA is useful in patients with symptomatic drug-refractory idiopathic VT arising from the left or right ventricle.

Transthoracic echocardiography guidance during percutaneous closure of patent foramen ovale.

BACKGROUND: Percutaneous closure of patent foramen ovale (PFO) has been increasingly performed for several indications; mostly due to cryptogenic stroke. In this study we aimed to evaluate the safety and efficacy of transthoracic echocardiographic (TTE) guidance during percutaneous closure of PFO in using the Amplatzer and Occlutech Figulla PFO occluder devices. METHODS: Between October 2005 and March 2011, 139 patients (74 male, mean age: 40.4 ± 10.3) underwent transcatheter PFO closure. In all patients transesophageal echocardiography performed subsequently to diagnose, assess the size and evaluate for suitability of the defect for percutaneous closure. During the procedure fluoroscopy and TTE were used for guidance. RESULTS: Among 139 patients, Amplatzer PFO occluder was used in 74 patients and in 65 of them Occlutech Figulla device was selected for occlusion. The indications for PFO closure were ischemic stroke in 98 (70.5%), recurrent transient ischemic attacks (TIA) in 40 (28.7%), peripheral embolism in 1 (0.8%) of the patients. In all patients, percutaneous intervention was performed successfully under TTE guidance. There have been no neurologic (recurrent strokes or TIAs) and cardiovascular complications during the immediate and long-term follow-up period (2-67 months, median 29). There was significant difference between the mean fluoroscopic time from the beginning which is 8.6 ± 3.4 min in the former versus 3.4 ± 1.9 min in the latter (P < 0.05). CONCLUSION: Our study confirms the efficacy and safety of TTE guidance during percutaneous closure of PFO, which shortens the procedural time and obviates the need for general anesthesia or endotracheal intubation.

Transcatheter closure of atrial septal defects with transthoracic echocardiography.

OBJECTIVES: The aim of this study is to evaluate our clinical experience using an Amplatzer septal occluder for catheter closure of a secundum atrial septal defect under transthoracic echocardiography guidance without general anaesthesia. METHODS: Patients eligible for transcatheter atrial septal defect closure were selected using transthoracic echocardiography. The largest defect diameter measured in different views was selected as the reference diameter. All procedures were performed under conscious sedation with fluoroscopic and transthoracic echocardiographic guidance. RESULTS: Between November, 2006 and December, 2009 a secundum-type atrial septal defect was closed with the Amplatzer septal occluder in 40 patients with transthoracic echocardiographic guidance. The mean age and weight were 7.9 years and 26.9 kilograms, respectively. The mean atrial septal defect diameter was 11.4 millimetres, total septal diameter was 38.5 millimetres, and the mean device diameter and the difference between device and atrial septal defect diameter were 12.6 and 1.2 millimetres, respectively. There were no major complications. The mean follow-up time was 14.8 months. CONCLUSION: In selected cases, in which the defects are small and the rims are adequate and transthoracic echocardiography provides high image quality, transthoracic echocardiography can be substituted with transoesophageal echocardiography. The ratio of defect size to total septal diameter can be used as a guide for patient selection; those that have a value of 0.33 or greater can be considered eligible for closure with transthoracic echocardiography. However, transthoracic echocardiography should not be used when there are large or multiple defects, or the rims are thin and soft and the image resolution is inadequate.

Myocardial performance index by tissue Doppler in bronchopulmonary dysplasia survivors.

Bronchopulmonary dysplasia (BPD) survivors from the surfactant era were evaluated by echocardiography in a few studies and no significant differences were found between BPD and non-BPD children. In this study, we evaluated these children with myocardial performance index (MPI), which was obtained by tissue Doppler echocardiography (TDE) in addition to the conventional methods. Fifteen children with BPD who did not have any cardiopulmonary symptoms at the time of the study were examined. All children were studied with M-mode, two-dimensional and DE. Pulmonary artery systolic pressures (PAPs) were estimated from tricuspid regurgitant velocity, and MPI for both ventricles were obtained by TDE. Results were compared with those of term-born, age- and sex-matched control children. While the variables obtained by M-mode and DE did not differ between the groups, the right and left ventricular MPI were found to be significantly higher in the BPD group compared with the control group (mean right ventricular MPI 0.48 +/- 0.04 vs. 0.41 +/- 0.05; mean left ventricular MPI 0.47 +/- 0.05 vs. 0.39 +/- 0.06). In addition, mean PAPs values of the patients were found to be significantly higher than those of the controls (30.4 +/- 6.9 mmHg vs. 23.3 +/- 5.3 mmHg), and there was a positive correlation between PAPs and right ventricular MPI values in the BPD group (r = 0.5). While routine echocardiographic examinations revealed no difference between the groups, MPI measurements by TDE technique yielded significantly higher values in the BPD group. To this extent, our study is the first to show that survivors of BPD may, in fact, have a subclinical ventricular dysfunction.

[Percutaneous closure of interatrial septal defects: mid-term follow-up results]. / Interatriyal septal defektlerin perkütan yolla kapatilmasi: Orta dönem izlem sonuçlari

OBJECTIVES: We evaluated short- and mid-term results of percutaneous closure of atrial septal defects (ASD) and patent foramen ovale (PFO) using various closure devices. STUDY DESIGN: The study included 278 patients (128 men, 150 women; mean age 39±12 years; range 17 to 66 years) who underwent percutaneous closure of secundum ASD (n=134) or PFO (n=144). All the patients were assessed by transthoracic (TTE) and transesophageal (TEE) echocardiography before the procedure. Percutaneous closure was performed with the Amplatzer (n=180), Occlutech Figulla (n=92) or BioSTAR (n=6) devices. Closure of ASDs was performed under general anesthesia with TEE guidance, and closure of PFOs was performed under local anesthesia with TTE guidance. Follow-up controls were at 1, 6, and 12 months, and annually thereafter. The mean follow-up period was 35.1 ± 24.6 months for ASD cases, and 24.9 ± 15.5 months for PFO cases. RESULTS: The mean device size was 20.2 ± 5.6 mm for ASD cases and 24 ± 2.9 mm for PFO cases. The mean procedural and fluoroscopy times were 28.4 ± 6.1 min and 4.9 ± 2.1 min for ASD closure, and 18.2 ± 5.3 min and 3.6 ± 1.2 min for PFO closure, respectively. Procedural device embolization occurred in only one patient. No recurrent embolic events occurred after PFO closure, and no residual shunts were seen after ASD closure. Device thrombosis developed in two patients during the procedure and in one patient at one-year follow-up. CONCLUSION: Percutaneous closure of secundum ASDs and PFOs using the Amplatzer, Occlutech Figulla, and BioSTAR devices is an effective and safe treatment option.

Midterm experience with implantable cardioverter-defibrillators in children and young adults.

AIMS: This single-centre study was undertaken to review our experience with implantable cardioverter-defibrillator (ICD) implantation in children with relatively different aetiologies. METHODS AND RESULTS: We retrospectively reviewed the records of the paediatric patients who underwent ICD implantation between October 2001 and December 2008. The data of these patients were collected by reviewing the patients' medical records and computerized departmental pacemaker databases. A total of 28 patients who underwent ICD implantation during this period were included in this study. The median age was 12 years and median weight was 32 kg. Most of the patients had ion-channel diseases (n = 13) or cardiomyopathy (n = 11). Devices were implanted for either secondary (n = 22) or primary (n = 6) prevention. The selected ICD generator type was single chamber in 22 patients, dual chamber in 5 patients, and biventricular in 1. Nineteen patients received 122 shocks. Fifteen of 22 patients (68.2%) from the secondary prevention group and 2 of 6 patients (33.3%) from the primary prevention group experienced at least one appropriate shock during a median period of 11.3 months (range: 4 days-6.5 years). Forty-two inappropriate shocks were delivered in seven (31.8%) patients from the secondary prophylaxis group during a median period of 11.3 months. The most important reason for inappropriate shocks was T-wave oversensing. In six patients, lead-related acute or chronic complications occurred. CONCLUSION: The ICD was safe and effective in interrupting malignant arrhythmias in children and adolescents with a high risk of sudden cardiac death. However, the occurrence of lead complications is significant. The incidence of therapies delivered by the device, with appropriate and inappropriate shocks, was high and interfered with the quality of life. The most important reason for inappropriate shocks was T-wave oversensing. Careful programming is mandatory to reduce the inappropriate shocks.