Ergun Cil

Double-chambered right ventricle: experience with 52 cases

The presence of anomalous muscle bundles may produce a pressure gradient between the inflow and outflow portions of the right ventricle, thus resulting in double-chambered right ventricle bearing troublesome clinically in its diagnosis. The aim of the present study was to review the diagnostic criteria. Fifty-two patients with a double-chambered right ventricle were seen during an 8-year period. They ranged in age at the catheterization from 4 months to 17 years (mean 7.5 +/- 4.4 years). Diagnosis was confirmed in 51 patients at cardiac catheterization and in other one on operation. The majority of the patients had associated cardiac anomalies: there were 33 ventricular septal defect (63%), 21 pulmonary valve stenosis (40%), nine atrial septal defect (17%), and four double-outlet right ventricle. The electrocardiograms revealed upright T waves alone in right precordial leads suggesting right ventricular hypertrophy in 33% of the patients. At cardiac catheterization, there was a pressure gradient of 20-160 mmHg between the right ventricular inflow and outflow portions. Forty patients have had surgery and four have undergone balloon pulmonary valvuloplasty. Surgical treatment was planned for two patients and other six had no indication for treatment.

The effect of age and gender on the electrocardiogram in children.

Our aim was to determine, using a computer program for measurement and analysis, the effects, if any, of age and gender on the electrocardiographic measurements in a large cohort of Turkish children. We analyzed standard simultaneous 12-lead electrocardiograms from 2241 healthy Turkish children, aged from 1 day to 16 years, to calculate limits of normality of a variety of electrocardiographic measurements for 12 age groups. Clinically significant differences were shown to exist, and the results compared with previously established normal limits. Differences were demonstrated for gender in measurements of both amplitude and duration, particularly in the adolescent period. We have provided tables and figures showing age and gender-dependent means and upper limits of normal electrocardiographic parameters for the important leads.

Dilated cardiomyopathy in childhood: prognostic features and outcome.

The natural history of dilated cardiomyopathy in children is difficult to predict due to the heterogeneous character of the disease. The outcome in infants and children is highly variable from complete recovery to death. In this study, 40 children diagnosed with dilated cardiomyopathy between 1995 and 2004 in our paediatric cardiology unit were reviewed with respect to clinical course and outcome, retrospectively. The medical history of these patients with dilated cardiomyopathy was reviewed to determine age, gender, family history, preceding viral illness, duration of symptoms before the diagnosis, symptoms and signs at presentation, treatment and outcome. The diagnosis was made on the basis of cardiomegaly and evidence of poor left ventricular function by echocardiography. Median age at diagnosis was 14 months, ranging from 2 months to 8 years. At presentation, 28 patients (70%) were under and twelve (30%) were above the age of two years.Twenty-eight (70%) patients had signs of congestive heart failure. Mean duration of follow-up was 40 ± 24 months (ranging from 6 months to 9 years), 21 patients (52.5%) recovered, 17 patients (42.5%) had residual disease and two (5%) died. The cause of death in both patients was progressive cardiac failure. Sixteen of 28 patients (57%) who were below the age of two years and five of 12 patients (42%) who were above the age of two years at presentation recovered.The rate of recovery was significantly different between the two age groups (p < 0.05). Seventeen of 21 (81%) patients with a history of recent viral illness at presentation recovered. The mean duration of the disease among those who recovered was 11 ± 8.3 months. Five of 19 (26%) patients without recent viral illness recovered.The mean duration of the disease in this group was 22 ± 12 months.There was a significant difference between the two groups with respect to recovery and recovery time (p < 0.05). During the first 6 months after diagnosis, there was a significant difference between the patients who recovered and the patients who had residual disease with respect to improvement in the left ventricular FS (22 ± 3.5%, 15.2 ± 2.8%, respectively) (p < 0.05). In conclusion, in this study, the rate of recovery and survival is higher than in previous studies. A good outcome is related to age at presentation (≤ two years old), a history of viral disease within three months of presentation and improvement in ventricular function during the first 6 months after diagnosis. Intractable heart failure has an adverse effect on the outcome.

Risk of vasovagal syncope and cardiac arrhythmias in children with mitral valve prolapse

Objective — Postural phenomena, cardiac arrhythmias and autonomic dysfunction are responsible for presyncope and syncope in patients with mitral valve prolapse (MVP). In this study, arrhythmia and vasovagal syncope incidence were investigated in children with MVP. Methods — Between-04-2005 and-12-2006, 37 children with MVP and 26 healthy children were evaluated.Telecardiography, electrocardiography (ECG), echocardiography, Holter monitoring, exercise test and head-up tilt test were performed. Results — The MVP group consisted of 19 boys and 18 girls with a mean age of 11.8 years. The control group was similar with respect to age and gender.Telecardiography, ECG, Holter monitoring, exercise test and QTc of all children were within normal limits.There was a statistically significant difference between the two groups in terms of QT dispersion. The tilt table test was positive in 11 of 37 (29.7%) children with MVP and in 1 of 26 (3.8%) normal healthy children.This difference was statistically significant (P < 0.01). Conclusion — Arrhythmia and syncope frequency was found to be higher in children with MVP than in the normal population.The risk of vasovagal syncope indicated by a positive tilt test was found to be increased in children with MVP.Therefore, patients and families must be informed about the conditions that may predispose to vasovagal syncope and caution should be recommended in these patients.

Outcomes of Kawasaki Disease A Single-Center Experience

Objectives: Kawasaki disease (KD) is an acute systemic vasculitis of unknown etiology. Coronary artery lesions (CAL) develop in 15% to 20% of untreated cases. Our objective was to evaluate demographic, clinical, and laboratory features and short-intermediate coronary artery outcomes of children with KD. Patients and Methods: Medical records of patients with KD were retrospectively identified. Clinical information and echocardiography, laboratory, and angiographic results were noted using a standardized form. Results: The study included 44 patients with a mean age of the 29.72 ± 21 months (ranging from 1 month to 9.5 years). There were 28 male and 16 female patients; 20 patients were diagnosed as having had incomplete KD. Four cases with atypical presentation were significantly older than children with complete and incomplete KD; 17 patients (38.6 %) had coronary artery aneurysm (CAA), which declined to 6.8% after intravenous immunoglobulin (IVIG) treatment. Time between fever and diagnosis and abnormal levels of hemoglobin and platelets were all associated with CAA. The children were followed up for a mean of 36.39 ± 19 months (with a maximum of 16 years). Angiographic evolution and regression of CALs have been observed in 14 (82.3%) patients. Three patients in whom CALs persisted did not receive IVIG therapy because of delayed diagnosis. Conclusions: Awareness of KD in children has led to an increase in the number of cases. Utility of IVIG treatment to reduce the coronary artery involvement in patients with delayed diagnoses should be discussed and considered. Long-term results are required to assess whether the KD represents a risk factor for coronary artery diseases seen during adulthood.

Coarctation of Persistent 5th Aortic Arch: First Report of Catheter-Based Intervention

Persistent 5th aortic arch, originally called double-lumen aortic arch, is a rarely reported cardiac developmental anomaly that results in systemic-to-systemic or systemic-to-pulmonary shunting. When this malformation occurs, other intracardiac defects are almost always present. We report the case of a 7-month-old girl who presented with a heart murmur; she was found to have an interrupted 4th aortic arch and coarctation of a persistent 5th aortic arch, with no other defects. To our knowledge, this is the 3rd report of a pediatric patient with this condition in isolation, and the first report of its diagnosis and treatment by means of cardiac catheterization and balloon angioplasty.

FISH investigation of 22q11.2 deletion in patients with immunodeficiency and/or cardiac abnormalities

DiGeorge anomaly/velocardiofacial syndrome (DG/VCFS), called 22q11.2 deletion syndrome in general, is the most common chromosomal deletion syndrome found in humans. Typical facial features, palatal defects, conotruncal abnormalities of the heart, aplasia/hypoplasia of the parathyroid glands and of thymus are characteristics of this syndrome. Deletions of chromosome 22q11.2 (del22q11.2) are the leading causes of DG7VCFS. We report on a systematic search by fluorescence in situ hybridization (FISH) for deletions of chromosomes 22q11.2 in patients with a clinical suspicion or diagnosis of DG/VCFS. Using FISH we studied a series of 43 patients with suspected DG/VCFS. In this study, a total of 43 patients were investigated for the presence of a 22q11.2 deletion over a two-year period. Del22q11.2 was detected in 5 of the 43 patients tested. All patients with deletion had hypocalcemia, 80% had cardiac defects, 40% had facial dysmorphism, 40% had immunodeficiency , and 20% had otolaryngeal abnormalities. Chromosome 22q11.2 deletion is a relatively common condition and is readily diagnosed by FISH. We suggest that FISH analysis of 22q11.2 deletion should be performed in the presence of combined of hypocalcemia and congenital cardiac malformations, with or without any characteristics of the disease. This may facilitate an early diagnosis in such patients.

Determination of reference values for tricuspid annular plane systolic excursion in healthy Turkish children.

Objective: Tricuspid annular plane systolic excursion (TAPSE) is an echocardiographic measurement used for evaluating right ventricular systolic function. While established reference values of TAPSE exist for the adult population, only a limited number of studies have attempted to evaluate reference values for the pediatric population. The aim of the present study was to determine the reference values for TAPSE in healthy children in Turkey. Methods: A total of 765 healthy children aged between 0 and 18 years, all of whom were referred to our clinic with cardiac murmurs, were evaluated prospectively. Patients with no cardiac pathologies or other disorders were excluded from the study. The measurement of TAPSE was obtained using a 2D-guided M-mode technique with echocardiography, and the relationship between age and surface area with TAPSE was investigated. The statistical analysis was carried out using the SPSS 20.0 software package (SPSS Inc., Chicago, IL, USA, 2012). Results: The mean TAPSE value was found to be 19.56±5.54 mm, and no significant difference was identified between male and female children. TAPSE values showed a positive correlation with increasing age and surface area. The mean TAPSE value was 9.09±1.36 mm in newborns and 25.91±3.60 mm in the 13-18 years age group. A negative correlation was seen between TAPSE and heart rate. Conclusion: In the present study, the reference values for TAPSE in healthy Turkish children were presented in percentile tables and the corresponding z-scores were determined. These reference values may be useful in daily practice for the evaluation of right ventricular systolic function in children.

Bilateral septic arthritis of the knee joint in three children caused by unusual infectious agents

reports in adults with an underlying or predisposing factor such as joint prostheses, collagen vascular disease, arthropathy and/or their therapies.1–6 As far as we know, there has been only one report describing bilateral septic arthritis of the shoulder in children.7 In this report, three children were presented with septic arthritis of both knee joints mimicking juvenile rheumatoid arthritis (JRA). They all had important underlying or predisposing factors, such as panhypogammaglobulinemia (common variable immune deficiency), severe congenital heart disease (operated transposition of the great arteries) and systemic type JRA. All of the children also had anemia. The causes were unusual microorganisms such as Proteus mirabilis and Candida albicans (both agents were yielded from both synovial fluids). In the third case, Gram stains of two separate synovial fluids demonstrated Gram-positive cocci located inside and around the neutrophils with negative bacterial cultures.

Delayed hemopericardium due to trivial chest trauma.

We report two cases of hemopericardium occurring in seven-month-old and 12-year-old boys, who had no history of major trauma. The possible cause of the hemopericardium for the infant was falling from a bed which was 75 cm high two weeks prior to the admission. The 12-year-old boy had fallen from a chair and damaged his chest 4 weeks previously. Their coagulation tests were all normal. By means of pericardiotomy, we drained 120 ml and 1200 ml of blood, respectively. The boys have now been well over follow-up periods of 24 and 18 months, respectively.