Noncompaction-like membranous structure in left ventricle.

A 22-day-old boy born to a 21-year-old gravida 1, para 1 mother was admitted to our hospital for routine newborn examination. On physical examination, any clinical abnormality or malformations were not observed except 1/6 systolic murmur. A well-demarcated membranous structure was confirmed by echocardiography which was starting from left ventricular apex, extending to the mitral chords including papillary muscles in apical four-chamber and parasternal long-axis examination. This structure was considered as a variant of left ventricular noncompaction. The patient is still being followed up in our pediatric cardiology department.

Mitral arcade: A rare case presenting with fatigue and cough.

An 11-year-old girl was admitted to pediatric emergency unit with complaints of fatigue and cough. The patient had no previous complaints. There was no history of rheumatic heart disease. The height and duration of the P-wave was 4 mm and 0.16 seconds, respectively (p mitrale). Echocardiography showed enlarged left atrium (51×61 mm in diameter). Both the anterolateral and posteromedial papillary muscles were directly attached to the anterior and posterior mitral valve leaflets without tendinous chords. The patient was diagnosed with mitral arcade, severe mitral stenosis, and mitral regurgitation. The patient was referred to surgery for replacement of mitral valve.

Persistent left superior vena cava accompanying congenital heart disease in children: Experience of a tertiary care center.

BACKGROUND: This study focuses on determining concomitant persistent left superior vena cava (SVC) in patients with congenital heart disease (CHD). METHODS: Between 2005 and 2012, a total of 2.663 patients with CHD, 88 (3.3%) of whom were diagnosed with persistent left SVC, were evaluated retrospectively. The demographic characteristics of patients, clinical and radiographic findings, echocardiography, cardiac catheterization, and angiography results obtained from the patients' records were reviewed. RESULTS: The median age of the patients was 9.5 months, and 46 of the 88 (52.3%) patients were female. The most common concomitant CHD were ventricular septal defect, double outlet right ventricle (DORV), and tetralogy of Fallot (TOF). When the patients were compared according to their CHD, persistent left SVC frequency was significantly higher in those with DORV (P<.001), TOF (P=.04), patent ductus arteriosus (P=.01), and atrial septal defect (P=.03). Eighty-three of the 88 (94.3%) patients with persistent left SVC had right SVC, and 5 (5.7%) had absence of the right SVC. Twenty-seven of the 83 (32.5%) patients with double SVC had connected normal innominate vein. In all cases, right aortic arch association was seen in 14 (15.9%) patients. Eighty-four (95.4%) of the patients were diagnosed by echocardiography prior to catheter angiography. Persistent left SVC drained to the coronary sinus in all cases. CONCLUSION: Increased awareness about the association of certain CHD with persistent left SVC and a careful echocardiographic examination can facilitate the diagnosis of persistent left SVC. In addition, precise prior diagnosis of persistent left SVC can prevent complications during surgery.

Decreased Deformation in Asymptomatic Children with Isolated Left Ventricular Non-compaction and Normal Ejection Fraction.

Echocardiography is the mainstay of screening and disease surveillance in isolated left ventricular non-compaction (iLVNC). The aim of our study is to determine the early regional and global myocardial functional changes and whether the myocardial changes that cannot be detected by conventional echocardiography could be detected by tissue Doppler imaging (TDI) or two-dimensional speckle-tracking echocardiography (STE) in iLVNC cases without symptoms. Longitudinal and circumferential strain (S) and strain rates (SR) as determined by STE in 20 children aged 12.1 ± 3.3 years was compared with those in 20 controls. All children underwent echocardiographic assessment using two-dimensional, tissue Doppler and speckle-tracking echocardiography. iLVNC patients who had normal systolic function by ejection and shortening fractions were included in this study. According to the TDI in all three segments [the non-compacted (NC), neighboring NC (NNC) and compacted (C) segments], isovolumic contraction time, isovolumic relaxation time and myocardial performance index values were significantly higher, while ejection time were significantly lower in the iLVNC group. According to STE in two segments (NC and NNC-segments) longitudinal S and SR values and also circumferential S and SR values were significantly lower in the iLVNC group compared with the control group; whereas, in the global measurements both longitudinal and circumferential S and SR values in all three segments were significantly lower in the iLVNC group compared with the control group. We believe that TDI and STE that evaluates myocardial deformation can be used for the detection of early myocardial dysfunction in the iLVNC patients who are subclinical and whose left ventricular functions were detected as normal by conventional methods with normal ejection and shortening fractions.

Assessment of heart rate variability in breath holding children by 24 hour Holter monitoring.

PURPOSE: Previous studies have shown that the underlying pathophysiologic mechanism in children with breath holding may be generalised autonomic dysregulation. Thus, we performed cardiac rhythm and heart rate variability analyses using 24-hour Holter monitoring to evaluate the cardiac effects of autonomic dysregulation in children with breath-holding spells. METHODS: We performed cardiac rhythm and heart rate analyses using 24-hour Holter monitors to evaluate the cardiac effects of autonomic dysregulation in children during a breath-holding spell. Our study group consisted of 68 children with breath-holding spells - 56 cyanotic type and 12 pallid type - and 39 healthy controls. RESULTS: Clinical and heart rate variability results were compared between each spell type - cyanotic or pallid - and the control group; significant differences (p<0.05) in standard deviation of all NN intervals, mean of the standard deviations of all NN intervals for all 5-minute segments, percentage of differences between adjacent RR intervals >50 ms, and square root of the mean of the sum of squares of the differences between adjacent NN intervals values were found between the pallid and cyanotic groups. CONCLUSIONS: Holter monitoring for 24 hours and heart rate variability parameters, particularly in children with pallid spells, are crucial for evaluation of cardiac rhythm changes.

An evaluation of heart rate variability and its modifying factors in children with type 1 diabetes.

OBJECTIVE: To evaluate heart rate variability by Holter monitoring in type 1 diabetic children compared with a healthy control group and determine the factors modifying heart rate variability. METHODS: This was designed as a prospective study comparing 28 patients, diagnosed with type 1 diabetes and under follow-up, with 27 healthy control group subjects. RESULTS: The patients were aged 9.9 ± 4.2 years in the diabetic group, including 13 (46.5%) girls and 15 (53.5%) boys. The healthy control group comprised 20 (74%) girls and seven boys (26%) with an average age of 8.6 ± 3.7 years. The search for factors modifying heart rate variability yielded the following correlations: for the time-dependent variables, negative between age and both average and maximal heart rate (r = -0.263 and -0.460, respectively), negative between haemoglobin A1c and percentage of differences between adjacent RR intervals >50 ms, positive between diabetes duration and square root of the mean of the sum of squares of differences between adjacent NN intervals. The average heart rate and percentage of differences between adjacent RR intervals >50 ms was significantly higher in the girls than the boys in all groups. With regard to the frequency-dependent factors affecting heart rate variability, correlations were found between haemoglobin A1c level and both total power and very low frequency (r = -0.751 and -0.644) and between very low frequency and diabetes duration. CONCLUSION: A reduction in heart rate variability parameters was observed in type 1 diabetes mellitus patients who had a long disease duration or were poorly controlled, as compared with healthy controls.

Double outlet right ventricle with intact ventricular septum.

Double outlet right ventricle is a cardiac malformation in which both the aorta and pulmonary artery arise from the right ventricle. A double outlet right ventricle with intact ventricular septum is extremely rare. A nine-day-old boy born at 38 weeks with a birth weight of 3200 g was referred due to cyanosis and murmur. The history of the patient was nonsignificant. On the physical examination, oxygen saturation of the patient on room air was 84%, and cardiac activity and a 2/6 systolic murmur at the right sternal border were found. On electrocardiography, deep q's were determined in D2, D3, and aVF. On echocardiography, left atrial isomerism, dextrocardia, wide, high venosum atrial septal defect, double outlet right ventricle with intact ventricular septum, malposition of the great arteries, and moderate pulmonary stenosis were observed. Catheter angiography was performed for diagnostic confirmation. Herein, a newborn with cyanosis and murmur diagnosed as double outlet right ventricle with intact ventricular septum is reported.

Assessment of ventricular functions by tissue Doppler echocardiography in children with asthma.

Patients with asthma develop pulmonary hypertension due to recurrent hypoxia and chronic inflammation, leading to right heart enlargement with ventricular hypertrophy. Patients with severe asthma can experience cor pulmonale later in life, but little is known about ventricular function during the early stages of the disease. This study aimed to investigate ventricular functions in asymptomatic children with asthma as detected by conventional echocardiography and tissue Doppler echocardiography (TDE). Fifty-one pediatric patients (mean age 10.4 ± 2.2 years) with asthma and 46 age- and sex-matched healthy children (mean age 10.9 ± 2.4 years) were studied. All subjects were examined by conventional echocardiography and TDE, and they had pulmonary function tests on spirometry. The right-ventricular (RV) wall was statistically (p = 0.01) thicker among asthmatic patients (4.7 ± 1.5 mm) compared with healthy children (3.6 ± 0.4 mm). However, conventional pulsed-Doppler indices of both ventricles did not differ significantly between asthmatic patients and healthy children (p > 0.05). The results of TDE examining RV diastolic function showed that annular peak velocity during early diastole (E'), annular peak velocity during late diastole (A') (16.4 ± 1.8 and 5.1 ± 1.4 cm/s, respectively), E'/A' ratio (3.2 ± 0.7), isovolumetric relaxation time (67.7 ± 10.2 ms) and myocardial performance index (48.1 % ± 7.0 %) of the lateral tricuspid annulus among asthmatic patients differed significantly (p = 0.01) from those of healthy children (13.2 ± 2.3, 8.2 ± 2.0 cm/s, 1.6 ± 0.5, 46.2 ± 8.7 ms, and 42.0 % ± 5.7 %, respectively). Only peak expiratory flow (PEF) rate from the pulmonary function tests was negatively correlated with the E'/A' ratio of the tricuspid annulus (r = -0.38, p = 0.01). This study showed that although the findings of clinical and conventional echocardiography were apparently normal in children with asthma, TDE showed subclinical dysfunction of the right ventricle, which is negatively correlated with PEF. These findings signify the diagnostic value of TDE in the early detection and monitoring of such deleterious effects among asthmatic patients.

Strain and strain rate echocardiography findings in children with asymptomatic congenital aortic stenosis.

The aim of our study was to evaluate myocardial functions with strain/strain rate echocardiography in asymptomatic patients having congenital aortic stenosis (CAS) with normal cardiac functions as determined by conventional echocardiographic techniques and comparing them with those of healthy controls. A total of 58 patients with various degrees of isolated CAS and 52 healthy controls were enrolled in this study. Conventional and two-dimensional speckle tracking (2DSTE) echocardiography were performed. Global longitudinal strain (LS) (-23.1 ± 3.6 and -23.8 ± 4.7), and longitudinal strain rate (LSR) (-1.49 ± 0.32 and -1.76 ± 0.39) values were lower, whereas circumferential strain (CS) (-25.9 ± 4.7 and -22.8 ± 6.4) and circumferential strain rate (CSR) (-1.82 ± 0.46 and -1.69 ± 0.49) values were greater in the patient group than in the control subjects. The difference was significant for global LSR and CS (p < 0.05) values. Regional analysis showed lower LS values in the basal part of the left-ventricular (LV) free wall and lower LSR values in the basal parts of both of the septum and free wall in the patient group (p < 0.05). CS values in the anteroseptal, posterior, and inferior walls were significantly greater in the patients (p < 0.05). 2DSTE detects subtle alterations in myocardial function in asymptomatic children with CAS. Impairment of LV long-axis function occurred earlier and was more prominent in basal parts of the interventricular septum and the free wall of the left ventricle.

Heart failure after transvenous closure of atrial septal defect associated with atrial standstill and thiamine-responsive megaloblastic anemia.

Despite advances in device closure for atrial septal defect, post-closure heart failure remains a clinical problem in adult patients but is seen only rarely in children. An eight-year-old boy, who had been followed by a local pediatrician with the diagnosis of diabetes mellitus and congenital heart disease, was consulted to us for cardiac re-evaluation. Electrocardiography demonstrated absent P waves, and echocardiography revealed enlargement of the right ventricle and both atria and secundum atrial septal defect. With the diagnosis of atrial standstill, secundum atrial septal defect and thiamine-responsive megaloblastic anemia, acute heart failure developed after transvenous closure of the atrial septal defect, which improved dramatically with thiamine and supportive treatment.

Acute rheumatic fever in the Central Anatolia Region of Turkey: a 30-year experience in a single center.

UNLABELLED: The study was carried out in Dr. Sami Ulus Children's Hospital to investigate and to compare the incidence and findings of acute rheumatic fever patients (ARF) seen in the past 30 years. The medical records of 1,115 patients with ARF admitted to Dr. Sami Ulus Children's Hospital Department of Pediatric Cardiology during 1980-2009 were retrospectively analyzed. Twenty-one percent of those patients were admitted between 1980 and 1989, 44.6% between 1990 and 1999, and 34.2% between 2000 and 2009. The highest incidence was detected in the second decade with a rate of 60.0:100,000. Male/female ratio was 1:18. The age of patients ranged between 2 and 15 years. Carditis was detected in 64.7% of patients, arthritis in 59.1%, and chorea in 14.1%. Mitral regurgitation was the most common echocardiographic finding. Heart failure was detected in 13.8%. Recurrent attacks occurred in 8.1% of patients. The median follow-up was 6.8 years (range, 1.2-10.5 years). The prevalence of chronic rheumatic valvular disease was 58%. Mortality rate was 0.8%. CONCLUSION: Although the incidence of ARF has decreased in the last decade, it still continues to be an important public health problem in Turkish pediatric population.

Could arterial stiffness be early reversible target organ damage test in childhood hypertension?

OBJECTIVE: The recommended treatment for hypertension (HTN) in children has been revised recently. This study aimed to present the changes in target organ damage (TOD) and arterial stiffness parameters after treatment in children with primary HTN who were managed in accordance with the 2016 European Society of Hypertension Guidelines. METHODS: Patients with primary HTN included in this study were newly diagnosed, untreated, and were followed-up for a minimum of 6 months. HTN was confirmed by 24-h ambulatory blood pressure monitoring (ABPM). All patients underwent the following assessments: anthropometrical measurements of body mass index (BMI), carotid intima-media thickness (cIMT), left ventricular mass index (LVMI), plasma creatinine, urea, electrolytes, uric acid, fasting plasma glucose, blood lipids, urinalysis, urine culture, and first morning urine albumin tocreatinine ratio. The ABPM device performed measurements such as central blood pressure (cBP) and pulse wave velocity (PWV). RESULTS: Thirty-two of 104 patients were enrolled. Seventeen patients were male, and 53% were obese. Compared with pretreatment, creatinine, urea, systolic BP (SBP), diastolic BP (DBP), systolic load, diastolic load, central SBP (cSBP), cSBP z score, cDBP, and PWV z score decreased, whereas LVMI and BMI z scores were unchanged. CONCLUSION: After BP improvement, while LVMI did not regress, the cSBP, cSBP z, and PWV z score values, which are markers of arterial stiffness, regressed. This supports the corrective effect of BP control on the cardiovascular system even in a short-term follow-up. Further longitudinal studies are needed for the assessment of BP control on arterial stiffness in childhood.

Hypertrophic Cardiomyopathy After a Single Dose of Dexamethasone in a Preterm Infant.

Dexamethasone is widely used in preterm infants with severe pulmonary disease. Hypertrophic cardiomyopathy (HCM) is a transient side effect observed after multiple doses of dexamethasone. We report a preterm infant with myocardial hypertrophy after a single dose of dexamethasone (0.5 mg/kg) used to treat laryngeal edema secondary to prolonged intubation. A benign course was observed without left ventricular outflow tract obstruction and with recovery within 4 weeks. Myocardial effects of dexamethasone may be independent of dose and duration of treatment. The risk/benefit ratio must be carefully considered before using even a single dose of dexamethasone in preterm infants.

Assessment of left ventricular functions with tissue Doppler, strain, and strain rate echocardiography in patients with familial Mediterranean fever.

OBJECTIVE: This study assessed the early changes in regional and global systolic and diastolic myocardial functions in patients with familial Mediterranean fever without any cardiovascular symptoms using tissue Doppler and strain and strain rate echocardiography and compared them to the results of a control group. METHODS: This study has a cross-sectional and observational design. FMF patients with normal left ventricular function were included in the study. We excluded patients who had arrhythmia, acquired/congenital heart disease, pericarditis, or acute attack. We compared 45 children with familial Mediterranean fever on colchicine therapy and 45 age- and sex-matched healthy children. RESULTS: The 45 patients with familial Mediterranean fever included 24 (55.3%) girls and 21 (46.7%) boys with a mean age of 11.3 ± 3.7 (range 2-18) years. The mean disease duration was 4.6 ± 2.4 (range 0.5-10) years. In the patient group, the homozygous M694V mutation was the most common (64.4%) mutation. The patients with familial Mediterranean fever had statistically lower longitudinal global strain, radial global strain, and strain rates (-14.44 ± 4.77%, 14.80 ± 6.29%, and 0.59 ± 0.24 s, respectively) than the controls (-17.40 ± 1.79%, 17.53 ± 4.63%, and 0.83 ± 0.51 s) (p < 0.05). The circumferential global strain did not differ significantly between the groups. CONCLUSION: Patients with familial Mediterranean fever who are subclinical from a cardiac aspect might have normal left ventricular function as measured by conventional echocardiography. However, the disease affects their myocardial tissue, and these patients should be followed with conventional, strain, and strain rate echocardiography techniques regularly.

[Assessment of left ventricular functions with tissue Doppler, strain and strain rate echocardiography in children with juvenile idiopathic arthritis: An observational study]. / Juvenil idiyopatik artritli çocuklarda sol ventrikül fonksiyonlarinin doku Doppler, gerilme ve gerilme hizi ekokardiyografi ile degerlendirilmesi: Gözlemsel bir çalisma.

OBJECTIVE: In juvenile idiopathic arthritis (JIA) cardiac involvement is usually silent without typical symptoms. The purpose of this study was to assess left ventricular functions with tissue Doppler echocardiography (TDE), strain and strain rate in children with JIA. METHODS: Our study was designed as a cross-sectional observational study. Thirty pediatric patients with JIA and 30 age- and sex-matched healthy controls were studied. In addition to standard echocardiographic methods, tissue Doppler, strain and strain rate imaging's were performed to assess left ventricular functions in all participants. The means of variables that did not distributed normally were compared with Mann-Whitney U test. RESULTS: In patients with JIA, E' values of mid and apical regions of left ventricular lateral wall were significantly lower than those of the controls (15.76±3.24 vs 17.91±3.29 cm/s, 11.10±2.96 vs12.64± 2.42 cm/s, p<0.05). In longitudinal strain reflecting left ventricular regional systolic functions, apical-lateral, basal and mid-septum peak S values, lateral peak S values in circumferential strain (-17.30±6.22 vs -21.97±4.32, -18.23±4.62 vs -21.53±2.69, -20.35±3.75 vs -22.75±3.50, -9.68±7.12 vs -13.70±6.81 cm/s, p<0.05, r:0.42, 0.41, 0.42), in longitudinal strain reflecting diastolic functions, apical-lateral, mid-lateral, apical-septum, mid-septum peak E values (2.22±1.00 vs 3.17±0.87, 1.62±0.84 vs 2.15±0.72, 2.51±0.76 vs 3.31±0.87, 1.99±0.64 vs 2.47±0.57 cm/s, p<0.05, r:-0.39,-0.55,-0.43) and in circumferential strain lateral and posterior peak E values (1.32±0.83 vs 1.88±0.94, 1.31±0.71 vs 1.85±0.91 cm/s, p<0.05, r:-0.33, -0.22) were significantly lower than those of the controls. CONCLUSION: Although marked myocardial involvement was not detected with tissue Doppler imaging in JIA patients with subclinical cardiac disease, regional impairments in left ventricular strain and strain rates were found.