Subclinical Left Ventricular Longitudinal and Radial Systolic Dysfunction in Children and Adolescents with Type 1 Diabetes Mellitus.

INTRODUCTION: There are few studies of subclinical systolic dysfunctions in children and adolescents with type 1 diabetes mellitus (DM), and so the available data are limited. The aim of this study was to determine early echocardiographic signs of LV systolic dysfunction in children and adolescents with type 1 DM using two-dimensional speckle tracking echocardiography (2DSTE). MATERIAL AND METHODS: The study included 84 children and adolescents with type 1 DM and 32 sex-, age-, and body mass index-matched healthy subjects. The LV functions were assessed using conventional echocardiography, tissue Doppler imaging, and 2DSTE. RESULTS: The results showed LV diastolic dysfunction as reflected by significantly increased A-wave velocity, decreased E/A ratio, and increased early filling deceleration time in the patients with diabetes (P = 0.02, P = 0.029, and P = 0.04; respectively). Compared with the control group, patients with diabetes showed significantly lower values for longitudinal systolic strain and strain rate in most segments of the LV; for radial strain values of the LV; for lateral circumferential strain and posterior and anterolateral circumferential strain rate of the LV; and for global longitudinal and radial strain of the LV. Global longitudinal and radial strain values of the LV were significantly lower among patients with poor glycemic control than in the control group. CONCLUSION: In addition to diastolic dysfunction, LV longitudinal and radial function was found to be impaired in asymptomatic children and adolescents with type 1 DM who have normal LV ejection fraction by 2DSTE. Glycemic control may be the main risk factor for alteration of myocardial function.

Prevalence of overweight and obesity among patients with congenital and acquired heart disease in Kocaeli, Turkey.

BACKGROUND: Childhood obesity has increased in the last half of the century. The aim of this study was to evaluate the frequency of obesity in the children with congenital or acquired heart disease. METHODS: A total of 1410 children were assessed in this study. The study population was composed of 518 children (289 boys, 229 girls) as control group and 892 children (477 boys, 415 girls) as heart disease group. Patients were grouped into four categories: (I) "Clinic control subjects"; (II) "mild heart disease" that has not been treated with either surgical or catheter intervention; (III) congenital heart disease treated with surgical and/or catheter intervention; and (IV) "arrhythmias". A body mass index ⩾85th percentile was defined as overweight, ⩾95th percentile as obese, and <5th percentile was defined as underweight. RESULTS: We did not detect any association between heart disease and obesity. There was no difference in the rates of overweight, obesity, and underweight between the healthy control subjects and patients with heart disease (8.1%, 13.3%, and 5.0%; 9.0%, 10.7%, and 4.7%, respectively, p=0.145). All subgroups had a similar prevalence of underweight, overweight, and obesity as the healthy control population. Within the heart disease population, the overall prevalence rates for overweight, obesity, and underweight were similar between the boys and girls. CONCLUSION: Obesity is a common problem in children with heart disease, at least in general population. It is an important additional risk factor for long-term cardiovascular morbidity and mortality in children with heart disease. Precautions to prevent obesity should be a part of paediatric cardiologist's examination.

Crossed pulmonary arteries: a report on 20 cases with an emphasis on the clinical features and the genetic and cardiac abnormalities.

Crossed pulmonary arteries (CPAs) are a rare abnormality in which the ostium of the left pulmonary artery originates superior to the right pulmonary artery and to its right. Recognition of this rare pathology is important because it generally is accompanied by other congenital heart defects, extracardiac anomalies, and certain genetic problems. To date, only a few cases have been reported, and most of these cases have been associated with complex cardiac abnormalities. The authors detected 20 cases of CPA between June 2009 and November 2012 through their increasing awareness of this anomaly. Approximately 9,250 echocardiograms were performed during this period, and all of them also were checked for this anomaly. This report describes 20 cases of this CPA, with an emphasis on the clinical features and the genetic and cardiac abnormalities. The patients ranged in age from 1 day to 13 years at the time of the initial diagnosis. Four patients had complex cardiac pathologies such as tetralogy of Fallot, truncus arteriosus, transposition of the great arteries, and complete atrioventricular septal defect. Of the 20 patients, 11 had ventricular septal defects, and 12 had atrial septal defects. Pulmonary artery stenosis was detected in 12 (55 %) of the 20 patients. Aortic arch abnormalities such as interrupted aortic arch, right aortic arch, and coarctation of the aorta were detected in six patients. One patient had a left persistent superior vena cava. In 45 % of the cases, an associated genetic syndrome (DiGeorge-, Noonan-, Holt-Oram syndromes, vertebral, anal, cardiac, tracheal, esophageal, renal, limb anomalies [VACTERL] anomalies) was present. These syndromes were diagnosed based on their clinical features. Karyotype and fluorescent in situ hybridization (FISH) analyses for a 22q11 deletion were performed for 11 patients, with 10 patients found to have normal karyotype and FISH results. Only one patient had a 22q11 deletion. Six patients underwent successful operations. During the follow-up period, 3 of the 20 patients died. At this writing, the remaining patients are clinically stable and being followed without surgery. The authors believe that CPA is not a rare anomaly. If careful echocardiographic examination is performed, CPA will be diagnosed more frequently. Although this pathology usually is associated with genetic syndromes and other cardiac abnormalities, patients with CPA generally are asymptomatic.

Absence of the right pulmonary artery associated with a partial anomalous pulmonary venous connection.

Unilateral absence of a pulmonary artery is a rare anomaly. Patients may present with hemoptysis, recurrent respiratory infections, pulmonary hypertension, or congestive heart failure. This report describes the case of a missing right pulmonary artery associated with anomalous left pulmonary venous connection not previously described in the medical literature.

P-wave dispersion and heart rate variability in children with mitral valve prolapse.

Previous studies have reported that patients with mitral valve prolapse (MVP) may display autonomic dysfunction. Measurement of heart rate variability (HRV) and P-wave dispersion (PWD) may provide insights into the functional state of the autonomic nervous system. Heart rate variability (HRV) has been used as a noninvasive marker of autonomic activity. However, to the authors' knowledge, PWD has not been studied in the context of MVP. This study aimed to examine HRV and PWD in patients with MVP and to determine whether differences exist between symptomatic and asymptomatic patients. The study population consisted of 54 healthy children (17 boys and 37 girls) ages 6-18 years and 76 patients with MVP (20 boys and 56 girls) ages 6-18 years. The duration and dispersion of the P-wave were measured by surface 12-lead electrocardiograms (ECGs). Heart rate variability was quantified using both time-domain and frequency-domain analyses of Holter ECGs. The minimum duration of the P-wave was significantly lower in the MVP patients (42.4 ± 10.0 ms) than in the control subjects (54.4 ± 12.8 ms) (p < 0.01), and the PWD was significantly increased in the MVP group (42.7 ± 10.8 ms) compared with the control subjects (31.8 ± 10.9 ms) (p < 0.01). However, no significant differences were found between the symptomatic and asymptomatic patients. In addition, the HRV parameters were not statistically different between the two groups. In conclusion, although HRV parameters were not significantly different between the MVP and control groups, the findings show that PWD was increased for the children with MVP. However, no relationship could be established between PWD and clinical symptoms.

Management of an ascending aortic aneurysm associated with a bicuspid aortic valve and a coarctation in an adolescent.

Ascending aortic aneurysms are commonly associated with bicuspid aortic valves in adults but are rarely seen in childhood. We now report the management of an ascending aortic aneurysm in a 12-year-old male with an associated bicuspid aortic valve and a coarctation.

Preliminary results of propranolol treatment for patients with infantile hemangioma.

Propranolol, a non-selective beta-blocker, has recently been introduced as a treatment for infantile hemangiomas. In this study, we evaluated the effect of propranolol in 12 infants with hemangioma. Twelve infants (9 girls) with a median age of 4.5 months were included in the study. All of the patients in the study group received short-term (1-9 weeks, median: 4 weeks) systemic corticosteroids as a first-line therapy. All patients received propranolol 2 mg/kg/day, divided into three doses. They were treated in an inpatient setting for the first 72 hours of the treatment. Vital signs, blood pressure and blood glucose were monitored. Propranolol treatment was given for 4-9 months (median: 5 months). In the study group, regression rate of the mean dimension of the lesion was 38% +/- 15 (range 15%-50, median 45%) at the 2nd month of therapy. Over 9 months, which was the maximum follow-up period, the regression rate of the mean dimension of the lesion was 55% +/- 31 (range 20%-80, median 50%). One patient had transient bradycardia, which improved spontaneously. No other side effect was observed in the study population. Propranolol appears to be an effective drug for infantile hemangiomas with good clinical tolerance. We suggest that propranolol is the preferable drug as the first-line therapy for infantile hemangiomas.

Does the z-score value of the abdominal aorta predict recoarctation in an infant?

OBJECTIVE: We evaluated left ventricular dimensions and aortic arch z-scores in infants who underwent balloon angioplasty (BAP) or surgery for coarctation of aorta (CoA). We searched for risk factors predicting recoarctation. PATIENTS: Between 2007-2011, 27 male and 17 female infants (mean age 2.93 ± 4.78 months, range 2 days-24 months) with CoA were evaluated. Left ventricular dimensions, systolic functions, mitral and aortic annuli, transverse aortic arch, isthmus, coarctation site, and diaphragmatic aorta measurements were done and z-scores were determined before intervention. RESULTS: Six patients underwent primary operation, 38 patients had BAP (86.4%). Associated cardiac pathologies in operated patients were double outlet right ventricle (n = 2), atrioventricular septal defect (n = 1), Ebstein's anomaly (n = 1), arch hypoplasia (n = 2). Twelve patients (27.2%) had simple coarctation. Ventricular septal defect was the most frequent associated cardiac pathology (n = 20, 45.4%). The patients were followed for 10.22 ± 8.21 months. Among 33 primary successful BAP's, 14 had recoarctation (42%). Eleven patients were primarily operated (including 5 with unsuccessful BAP), two had recoarctation (18%). Abdominal and transverse aorta values and z-scores were significantly lower in the recoarctation group (7.15 ± 2.12 mm and 6.07 ± 1.86 mm respectively in the "no-recoarctation group"; vs. 5.53 ± 0.75 mm and 4.94 ± 1.53 mm in the "recoarctation group" P <.05). Abdominal aorta z-score of 0.42 was 88.9% sensitive and 53.8% specific to predict recoarctation (area under ROC curve: 0.618-0.902, P <.05). CONCLUSION: Although BAP for native coarctation is still a controversial treatment option due to frequent restenosis rates, abdominal aorta z-score of 0.42 could correctly eliminate recoarctation in 89% of these cases. This cutoff value might help us choose patients for primary BAP and decrease the recoarctation rate after BAP.

Crisscross heart with tricuspid atresia diagnosed in utero.

Crisscross heart is a rare congenital cardiac anomaly in which systemic and pulmonary venous streams cross without mixing at atrioventricular level. We report a case of crisscross heart with tricuspid atresia, double outlet right ventricle, and pulmonary stenosis, which was diagnosed prenatally.

Predictors of vasovagal syncope recurrence in children and adolescents and value of head-up tilt table test.

OBJECTIVE: The aim of the study was to define predictors of syncope recurrence in children and adolescents with vasovagal syncope and to determine the value of tilt test. METHODS: A retrospective observational study performed of prospective cohort of 150 patients aged between 8-18 years who were referred to our clinic because of fainting or who underwent tilt test with the pre-diagnosis of vasovagal syncope. The progress updated by telephone or face-to-face interview. Unpaired t-test, Mann-Whitney U test used for normal and non-normal distributed variables. Logistic regression analysis was used to determine the predictors of recurrence. RESULTS: Tilt test was positive in 97 and negative in 53 patients. Forty-eight patients had mixed, 34 had vasodepressor and 15 had cardioinhibitory type syncope. Recurrence found significantly higher in patients who had syncope in the first 20 minutes of the test (p=0.012). The number of the episodes decreased after the test; 3.86±4.75 vs 0.73±0.44, p<0.001). The recurrence was higher in patients who had more than 4 episodes. The recurrence was similar between positive and negative tilt groups. Age of syncope (OR 1.01, 95% CI 1.002, p=0.027) positive family history (OR 4.47, 95% CI 1.071-1.389, p=0.001) and the number of previous syncopal episodes (OR 1.22, 95% CI 1.882-10.623, p=0.003) were identified as risk factors for recurrence of vasovagal syncope. CONCLUSION: Age of syncope, positive family history and the number of previous syncopes are the predictors of recurrence of vasovagal syncope in children and adolescents. The number of recurrent episodes decreased after the test independently from Head-up tilt test results.

Functional pulmonary atresia in newborn with normal intracardiac anatomy: Successful treatment with inhaled nitric oxide and pulmonary vasodilators.

Functional pulmonary atresia is characterized by a structurally normal pulmonary valve that does not open during right ventricular ejection. It is usually associated with Ebstein's anomaly, Uhl's anomaly, neonatal Marfan syndrome and tricuspid valve dysplasia. However, functional pulmonary atresia is rarely reported in newborn with anatomically normal heart. We report a newborn with functional pulmonary atresia who had normal intracardiac anatomy, who responded to treatment with nitric oxide and other vasodilator therapy successfully.