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Crossed pulmonary arteries (CPA) is a rare abnormality in which the ostium of the left pulmonary artery is located rightward and the ostium of the right pulmonary artery is leftward. CPA has been reported only once at prenatal period and has not been associated with CHARGE syndrome as yet. Prenatally left pulmonary artery was seen to pass beneath the ductus and directing towards the left side and pulmonary artery bifurcation could not be demonstrated at the same plane. Postnatal echocardiography reconfirmed the presence of CPA. Bilateral choanal atresia, genital hypoplasia, hearing loss with facial and external ear asymmetry and psychomotor retardation of the newborn led to clinical diagnosis of CHARGE syndrome and was confirmed by gene analysis.

Objective We aimed to evaluate fetal left modified myocardial performance index and renal artery Doppler parameter in fetuses with isolated oligohydramnios and normal amount of amniotic fluid. Material and methods In a prospective cohort study, 25 pregnancies with isolated oligohydramnios and 25 healthy gestational age matched controls between 24+0 to 36+6 weeks of gestation were recruited. Primary outcome was to compare left modified myocardial performance index and mean fetal renal artery PI and secondary outcome was to compare adverse perinatal outcomes between the groups. Results Mean modified myocardial performance index was significantly higher and isovolumetric relaxation time was longer in the isolated oligohydramnios group (p=0.001, p=0.009). Mean renal artery PI values were not different between the groups. Birthweight and gestational age at birth were significantly lower (p=0.041, p=0.001), and incidences of delivery before 37 weeks and Cesarean section due to non-reassuring fetal heart rate testing were significantly higher (p=0.034, p=0.021) in women with isolated oligohydramnios than the control group. We found no significant relationship between modified myocardial performance index and adverse perinatal outcomes. Conclusion Fetuses with isolated oligohydramnios have increased left modified myocardial performance index which may be due to mild cardiac diastolic dysfunction. Increased modified myocardial performance index is not associated with adverse perinatal outcomes and does not seem to help in the management of pregnancies before 37 weeks of gestation with isolated oligohydramnios.

Juvenile spondyloarthropathy is an umbrella term for a group of childhood rheumatic diseases that can cause chronic arthritis extending to the axial skeleton before the age of 16. Although ankylosing spondylitis has aortic involvement as one of its most important effects, this relationship has not been extensively studied in children with juvenile spondyloarthropathy. Here, a cross-sectional study of the elastic properties of the aorta of 43 patients with juvenile spondyloarthropathy and 19 healthy controls is reported. Aortic stiffness assessed by echocardiography was used to predict the presence of aortitis, supplemented by pulsed-wave tissue Doppler indices. The right ventricular fractional area change was found to be significantly lower in the patients with juvenile spondyloarthropathy than in the healthy controls; aortic strain and distensibility were also significantly lower, and aortic stiffness index β was significantly higher; and the aortic root diameter change was significantly lower. According to HLA-B27 positivity, there was no difference in the stiffness parameters between the two groups. There was a significant correlation between juvenile Ankylosing Spondylitis Disease Activity Index and aortic diameter change, between juvenile Ankylosing Spondylitis Disease Activity Index and aortic stiffness. Thus, juvenile spondyloarthropathy is linked to high aortic stiffness parameters.

The aim of this analysis was to study the postnatal short-term outcome of cases with aneurysmal septum primum (ASP) and non-restrictive foramen ovale in otherwise structurally normal hearts. This is a retrospective review of fetuses with ASP referred between 2016 and 2018 to the University Hospital of Cerrahpasa, Department of Fetal Cardiology for cardiac scanning. Prenatal and postnatal clinical features and outcomes for each case were ascertained from the departmental database and from individual clinical hospital records. We presented twenty-four cases which also had postnatal echocardiographic examination in our hospital. At the time of diagnosis of ASP, the mean maternal age was 31.1 ± 5.7 years, the mean gestational age was 28.9 ± 5.9 weeks, the mean birth week was 37.4 ± 3.1 weeks, and the mean birth weight was 2940.8 ± 736.6 g. The postnatal first 3 months prevalences of atrial septal aneurysm, patent foramen ovale and, secundum atrial septal defect (ASD) were 12.5%, 12.5%, 20.8% among fetuses with ASP, respectively. ASP may persist after birth and may increase the frequency of secundum ASD. These data suggest that fetuses with ASP should be followed by a pediatric cardiologist in postnatal life.

Objectives: Multi-system inflammatory syndrome in children (MIS-C) is a less understood and a rare complication of coronavirus disease-2019 (COVID-19). Given the scarce data regarding this novel disease, we aimed to describe the clinical features and outcomes of our patients with MIS-C and to evaluate the associated factors for the pediatric intensive care unit (PICU) admission. Methods: The MIS-C patients under 18 years old diagnosed and treated in three referral centers between July 2020 and March 2021 were included. Data of the patients were retrospectively obtained from their medical records. Results: Overall, 76 subjects (24 females) with a mean age of 8.17 ± 4.42 years were enrolled. Twenty-seven (35.5%) patients were admitted to the PICUs. The two most common systemic involvement patterns were cardiac and gastrointestinal. There was only one lethal outcome in a patient with underlying acute lymphoblastic leukemia. Those with higher procalcitonin levels at admission were found to stay longer in the hospital (r = 0.254, p = 0.027). The risk of PICU admission increased with age (aOR: 1.277; 95% CI: 1.089–1.498; p = 0.003) and with decreased initial serum albumin levels (aOR: 0.105; 95% CI: 0.029–0.378; p = 0.001). Conclusion: Although there is a wide clinical variability among the patients with MIS-C, we suggest that those with older age and lower initial serum albumin levels merit close monitoring due to their higher risk for PICU admission.Key Points• Although there is a wide variability regarding the management process among clinicians, MIS-C is a rare, severe, less understood complication of COVID-19 that may cause rapid clinical deterioration in the patients.• Clinicians should be aware of this condition in children with persistent fever and a family history of COVID-19.• Older age and low serum albumin levels are the independent predictors for the pediatric intensive care unit admission among MIS-C patients. © 2021, International League of Associations for Rheumatology (ILAR).

BACKGROUND/OBJECTIVE Juvenile spondyloarthropathies (JSpAs) are a group of inflammatory diseases characterized by asymmetric peripheral arthritis (especially in lower extremities), axial skeleton involvement, and enthesitis. Although cardiovascular findings of inflammatory diseases such as juvenile systemic lupus erythematosus (SLE) and juvenile scleroderma (SD) are well documented, there are only a few studies assessing the cardiovascular consequences of JSpA in the literature. METHODS Forty patients with JSpA and 20 healthy controls were included into this cross-sectional study. Cardiac functions of the participants were evaluated by conventional echocardiography and pulse-wave (PW) tissue Doppler. RESULTS The patients with JSpA had higher mitral lateral S (p = 0.005) and E' wave (p < 0.001), tricuspid A' wave (p = 0.03), ejection fraction (p = 0.03) and shortening fraction (p = 0.01) than the control patients. In contrast, the patients with JSpA had lower left ventricle MPI (p = 0.01) and the ratio of tricuspid E'/A' waves (p = 0.05). Patients with enthesitis detected on magnetic resonance imaging had lower ejection fraction (p = 0.05), the ratio of E/A waves (p = 0.03) and had higher Mitral lateral A' wave (p = 0.01) than those without. There was a significant inverse correlation between the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) and PW transmitral A velocity (r = -0.256, p = 0.03), the BASDAI score and tricuspid annular plane systolic excursion (r = -0.301, p = 0.04), the BASDAI score and the ratio of E/E' waves (r = -0.276, p = 0.02), and the Juvenile Spondyloarthritis Disease Activity Index and PW transmitral A velocity (r = -0.246, p = 0.04). CONCLUSIONS In this study, we report the possible early signs of RV diastolic dysfunction and possible association between magnetic resonance imaging-confirmed enthesitis and lower LV systolic functions. Early identification of cardiac dysfunctions can help with prevention of long-term cardiovascular complications.

Cardiac manifestations in juvenile scleroderma or systemic sclerosis (JSSc) have poor prognosis, begin in early stages of the disease, and remain clinically asymptomatic. New echocardiography modalities, such as 2D/3D speckle tracking (STE, strain analysis for regional and global ventricular functions), can detect cardiac involvement in early stages. We assessed 21 JSSc patients and 19 controls using 2D/3D STE. The left ventricular end diastolic volume, end systolic volume, and ejection fraction of the patient and control groups were significantly different (99.2 ± 23.8 vs. 52 ± 23.8, 40.6 ± 16.0 vs. 20.2 ± 17.4 and 59.2 ± 7.5 vs. 65.6 ± 5.2, respectively). Global longitudinal strain (GLS) and global circumferential strain (GCS) were lower in the patient group (18.4 ± 4.7 vs. 22.4 ± 3.7, 26.4 ± 5.8 vs. 31.4 ± 3.5), as were the peak systolic strain values of the right ventricular longitudinal strain (RVLS) septum and RVLS free wall (18.1 ± 6.8 vs. 24.8 ± 6.0 and 22.8 ± 5.9 vs. 28.0 ± 6.9, respectively). 3D measurements of RVEDV, RVESV, and RVSV were higher in the patient group (88.2 ± 31.3 vs. 50.8 ± 23.5, 43.1 ± 17.6 vs. 19.0 ± 12.2, and 45.0 ± 16.2 vs. 31.7 ± 12.6). RVLS freewall results were lower in the JSSc patients with interstitial lung fibrosis, arthritis, muscle weakness, weight loss, and anti-scl 70 antibodies than in the JSSc patients without these variables. We found that a GCS of

The medical records of 2283 patients with ventricular septal defect (VSD) were reviewed to determine spontaneous closure, left ventricular-to-right atrial shunt, subaortic ridge, and aortic valve prolapse. One thousand eight hundred and twenty-three patients had been followed 1 month to 26 years (median 4 years) by echocardiography. Most of 460 patients could not be followed due to transportation of the institution. VSD was perimembranous in 68.8% (1255), trabecular muscular in 21.7% (395), muscular outlet in 6% (109), muscular inlet in 2.6% (48), and doubly committed subarterial in 0.9% (16). Defect size was classified in 66.8% (1218) as small, in 15.7% (286) as moderate, and in 17.5% (319) as large. VSD closed spontaneously in 18.8% (343 of 1823 patients) by ages 40 days to 24.9 years (median, 1.8 years). One hundred fifty-seven of 1255 perimembranous defects (12.5%) and 167 of 395 trabecular muscular defects (42%) closed spontaneously (p 0.001). Defect size became small in 306 (16.8%) of patients with VSD at a median of 2.5 years. Aneurysmal transformation was detected in 32.9% (600), left ventricular-to-right atrial shunt in 9.7% (176), subaortic ridge in 2.6% (48) of 1823 patients who were followed. In 381 (20.9%) of the 1823 patients, the VSD had been closed by a surgical or transcatheter technique. Surgery is required in one-fifth of patients with subaortic ridge or aortic valve prolapse. In conclusion, isolated VSDs are usually benign abnormalities that tend to shrink and close spontaneously.

Speckle-tracking echocardiography is a recently developed technique for the evaluation of myocardial deformation or strain. Our objective was to examine strain through a mid-term follow-up of patients with Kawasaki disease.MethodsWe explored left ventricular mechanics using speckle-tracking echocardiography in 35 patients with a history of Kawasaki disease at least 6 months after the acute phase. We also included 30 healthy children as controls. Strain data sets were acquired for the quantification of left ventricular global strain, segmental strain, and left ventricular ejection fraction.ResultsThe mean age of our patients was 25.6±15.4 months. At a median follow-up of 57.5 months (16.5–98.2), although both values were in the normal range, the mean left ventricular ejection proportion of patients (57.3%) was a little lower than that of controls (p⩽0.05). Patient strain values at the basal inferoseptal (20.0), basal anterolateral (19.5), apical septal (23.3), and apical inferior (24.0) segments were lower compared with controls. In all, seven patients had coronary aneurysms during follow-up. Kawasaki disease patients with pyuria had lower left ventricular strain at the mid anterior, mid anteroseptal, apical anterior, and apical inferior segments and global longitudinal strain compared with patients with no pyuria (p⩽0.05).ConclusionIn children with a history of Kawasaki disease, impairment of left ventricular mechanics occurs especially within the left anterior descending artery territories.

Objective: To investigate the association between fetal left ventricular modified myocardial performance index (LMPI) and intrahepatic cholestasis of pregnancy (ICP) and to evaluate the value of LM...

Objective: To compare the fetal modified myocardial performance index (Mod-MPI) in appropriately grown, early (EO) and late onset (LO) fetal growth restricted (FGR) fetuses and to assess it...

Isotretinoin, a synthetic derivative of vitamin A, is one of the most potent human teratogens, and is mainly utilized for the treatment of severe recalcitrant nodular acne. Retinoic acid embryopathy is well defined in the literature.The mother was referred for a fetal posterior fossa abnormality, first detected at 20 weeks of gestation. The mother used isotretinoin until 18 weeks gestation. Ultrasound examination revealed hypertelorism, cerebellar hypoplasia, vermian agenesis, truncus arteriosus, anotia, thymic aplasia, corpus callosum hypoplasia. An intrauterine diagnosis of fetal retinoid syndrome was confirmed by fetopsy after termination of pregnancy.The typical findings of fetal retinoid syndrome can be visualized with ultrasound in early second trimester.

Aortic arch anomalies refer to congenital malformations of position or branching pattern of the aortic arch. To-date, only a few small studies have documented prenatal detection of aortic arch anomalies. In this article, we share our experience in detecting aortic arch anomalies. Foetal echocardiograms, clinic and genetic histories of 33 patients who had been diagnosed with aortic arch anomaly from 2007 to 2015 were reviewed. In 15 patients, right aortic arch with mirror image branching; in 13 patients, right aortic arch with left ductus arteriosus and aberrant left subclavian artery; in three patients, left aortic arch with aberrant right subclavian artery; in one patient bilateral ductus and right aortic arch with aberrant left subclavian artery and in one patient double aortic arch were detected. In a patient with isolated right aortic arch, 22q11 microdeletion had been revealed. Given this data, we strongly suggest foetal karyotype analysis when aortic arch anomalies are identified. Impact Statement What is already known on this subject: The data about the prenatal diagnosis of aortic arch anomalies are limited. What the results of this study add: In our study, 653 patients were examined by foetal echocardiography during the study period. Thirty three patients who had been diagnosed with aortic arch anomaly prenatally and confirmed after delivery were enrolled in the study. In 15 patients, right aortic arch with mirror image branching; in 13 patients, right aortic arch with left ductus arteriosus and aberrant left subclavian artery; in three patients, left aortic arch with aberrant right subclavian artery; in one patient bilateral ductus and right aortic arch with aberrant left subclavian artery and in one patient double aortic arch were detected. Trisomy 18 was detected in the patient with bilateral ductus arteriosus and Di George syndrome (22q11 microdeletion) was determined in two patients with right aortic arch. While in the first patient, there were no other intracardiac anomalies; in the second patient with 22q11 microdeletion, Fallot tetralogy accompanied the right aortic arch. What the implications are of these findings for clinical practice and/or further research: This results showed that aortic arch anomalies can be associated with genetic anomalies even when they are found without other congenital heart disease. Given these data, we strongly suggest foetal karyotype analysis and genetic testing when aortic arch anomalies are identified.

PubMed ID: 28704174 Juvenile systemic lupus erythematosus (jSLE) is a chronic multisystemic autoimmune disease. Previous studies among adults have shown impaired right ventricular (RV) function in patients with SLE. Also, these patients may develop pulmonary artery hypertension (PAH), which is one of the most threatening complications of SLE. Nevertheless, studies on PAH among jSLE patients are still rare. The aim of this study was to assess the RV function in jSLE patients by Doppler echocardiography (Echo Doppler). We also estimated pulmonary artery systolic pressure (PASP) and mean pulmonary artery pressure (mPAP) in these patients. A total of 38 jSLE patients and 40 sex- and age-matched controls were retrospectively analyzed. All patients underwent combined M-mode, cross-sectional echo, and Doppler Echo examination. The RV function was significantly impaired in jSLE patients compared to controls. PASP and mPAP were normal in 37 out of 38 patients (97.37%), however, the mean values of PASP and mPAP were significantly higher in jSLE patients compared to controls (26.90 mmHg versus 21.71 mmHg and 12.63 mmHg versus 9.89 mmHg, respectively) [p < 0.05]. Only one patient (2.6 %) had elevated mPAP (60 mmHg). The right cardiac catheterization confirmed PAH in this patient. Although PAH was detected only in one patient, there was a marked increase of PAP in our jSLE patients. Overall, PASP and mPAP were significantly higher in jSLE patients compared to healthy controls. Prospective studies with ethnically diverse cohorts could give more insight on the relevance of PAP and PHT in patients with jSLE. © 2018 ABMSFBIH.

Rhabdomyoma is the most common cardiac tumour in children often associated with tuberous sclerosis. Arrhythmia caused by cardiac rhabdomyomas may be the initial sign of tuberous sclerosis. Rhabdomyomas unresponsive to other treatments could be successfully managed with everolimus, which has demonstrated benefit in tuberous sclerosis. We report a case of rhabdomyoma causing severe arrhythmia in a newborn managed successfully with everolimus.

To evaluate the incidence, associated cardiac and extracardiac malformations and clinical outcome of fetuses with dextrocardia.A retrospective review of 3556 fetal echocardiograms between 2000 and 2011 revealed 39 cases of dextrocardia. Dextrocardia was defined as right-sided positioning of the fetal heart. Prenatal and postnatal records of the fetuses were reviewed.The incidence was 1.1%. Of the 39 fetuses, 22 were primary dextrocardia and 17 were dextroposition. Diaphragmatic hernia was the most common cause of dextroposition with the incidence of 76%. Of the fetuses with dextroposition 35.5% had a cardiac anomaly. The survival rate of dextroposition was 31.2% and none of the survivors had an associated cardiac anomaly. Primary fetal dextrocardia was most common with situs solitus (45.4%), followed by situs ambiguous (36.3%) and then situs inversus totalis (18.1%). Structural cardiac malformations were found in 100%, 80% and 25% of fetuses with situs ambiguous, solitus and inversus, respectively. Of the dextroposition, 47.6% terminated pregnancy, 14.2% resulted in intrauterine death, 9.5% died after birth, and 28.5% survived.A wide spectrum of complex cardiac malformations are associated with fetal dextrocardia. Fetal echocardiography enables detection of complex cardiac anomalies so that parents can be appropriately counselled.

Prenatal diagnosis of a congenital coronary artery fistula between the left coronary artery and the right ventricle was established at 28 weeks of gestation. Hydrops fetalis developed during follow-up and the baby died on the first day after delivery. It is rare for coronary artery fistulas to become symptomatic during fetal life.

ObjectiveWe aimed, in this study, to compare dispersion of the p wave in patients with type 1 diabetes to nondiabetic control subjects, and to investigate the relationship between the dispersion of the p wave and cardiac autonomic dysfunction in diabetic children.MethodsWe enrolled 49 patients with type 1 diabetes, and 32 age- and sex-matched healthy subjects, measuring the Valsalva ratio, resting heart rate, and orthostatic hypotension in all. The duration of the p wave was measured manually on a high-resolution computer screen. Dispersion, defined as the difference between maximum and minimum durations of the p waves, was also measured in the 12-lead electrocardiogram before and after the Valsalva maneuver.ResultsThe mean age of the patients and their controls were 14.2 ± 4.8 years, and 12.7 ± 4.5 years, respectively. The mean duration of diabetes had been 6.2 ± 4.6 years. Maximal and minimal values for the duration of the p wave were significantly decreased in the diabetic children, with the dispersion itself significantly increased. Values for the dispersion in the diabetic subjects were similar before and after the Valsalva maneuver, whereas dispersion was found significantly increased after this maneuver in the controls. The differences in the Valsalva ratio, resting heart rate, and orthostatic hypotension between the groups, on the other hand, were not found to be statistically significant.ConclusionThe noted increase in the dispersion of the p wave in diabetic children reveals the onset of cardiac electrophysiological heterogeneity before it is possible to detect parasympathetic and sympathetic dysfunction with other tests.

The purpose of this study was to evaluate the feasibility of multislice computed tomographic (MSCT) angiography as a noninvasive method for detecting ostial, proximal, and middle segment coronary stenosis or occlusion and anatomy in patients with transposition of the great arteries who had undergone arterial switch operation (ASO). Sixteen-detector-row MSCT angiography was performed in 16 patients treated with ASO for transposition of the great arteries. The median age was 10.3 years (range, 6.2-16.3 years). Sixteen-detector-row MSCT angiography was performed in 16 patients who had undergone ASO. CT imaging was performed in the craniocaudal direction from 2 cm above the carina up to the heart basis. Noninvasive assessment of coronary artery stenosis and anatomy were investigated by MSCT angiography. Two patients were excluded from the study because of artifacts. Of 14 evaluated patients, 1 patient had ostial stenosis (7.1%). A coronary artery anatomy variant was present in six patients: left main artery (LMA) and right coronary artery (RCA) originating from the right sinus as a single orifice (n = 2); left circumflex artery (LCX) originating from the RCA (n = 1); LMA and RCA, after branching to the LCX, originating separately from the right sinus (n = 1); and LMA (n = 1) and left anterior descending artery (LADA; n = 1) originating directly from the right sinus. Intramural bridging in the LAD (n = 2) was detected. Five patients were normal. In conclusion, MSCT angiography, as a noninvasive, feasible technique for assessing coronary stenosis or occlusion and anatomy, can be used in the follow-up of patients who have undergone ASO.

Postural orthostatic tachycardia syndrome (POTS) is an abnormal heart rate response to a positional change. Several potential mechanisms for pathophysiology of POTS are defined. This syndrome can coexist with different clinical situations. In our report, the first case was a 13-year-old female who has been followed up for diagnosis of homocystinuria. She was admitted to our outpatient clinic with complaints of dizziness after suddenly moving from supine to upright position and chest pain after exercise. Tilt table test was performed to evaluate dizziness. According to the tilt table test the patient was diagnosed with POTS. The second case was a 17-year-old female who had been evaluated in different centers with the complaints of fainting, bruising, redness, and swelling on the hands and feet after moving from supine position to upright position during the last 4 years. Postural orthostatic tachycardia syndrome was diagnosed by tilt table test and ivabradine was started. Herein, we aimed to point out the cooccurrence of different clinical entities and POTS.

Constriction of the ductus arteriosus in utero may be an ominous event and may lead to congestive heart failure, foetal hydrops, and even foetal or neonatal death (Levin et al. 1979, Harlass et al....

Juvenile scleroderma (JS) represents a rarely seen group of connective tissue diseases with multiple organ involvement. Cardiac involvement in JSS is well known and, although rare in children, it may be an important cause of mortality and morbidity. Therefore, an early determination of cardio-vascular and pulmonary involvement is of the most relevance to reduce the mortality in patients with juvenile scleroderma. The aim of the study was to explore the non-invasive methods (Doppler echocardiography, pulmonary function tests), Forced vital capacity (FVC) and Carbon monoxide diffusion capacity (DLCO) in the assessment of the cardiopulmonary involvement in patients with JS. The assessment of pulmonary arterial pressure (PAP) and risk factors for pulmonary arterial hypertension (PAH) were made by the measurement of maximum tricuspid insufficiency (TI), end-diastolic pulmonary insufficiency (PI), ratio of acceleration time (AT) to ejection time (ET) (AT/ET), right atrial pressure (RAP) and contraction of vena cava inferior during inspiration. Thirty-five patients with confirmed JS were included in the study. The mean age of onset of the disease was 9.57 years (median 10 years, range 2-18 years). The mean disease duration and follow-up time was 2 years (median 1 year, range 0.5-8 years) and 3.57 years (median 2 years, range 0.5-14.5 years), respectively.The values of all the analyzed parameters including TI, PI, AT/ET, PAP, FVC and DLCO were found to be within normal ranges in all the patients tested, confirming an uncommonness of cardiopulmonary involvement in patients with juvenile scleroderma.

Aim: To show the effects of a single course of antenatal betamethasone on cardiac measurements and systolic functions in premature newborn infants. Methods: Seventy six newborn infants with a gestational age of 25–33 weeks were included in the study. They were first classified according to their gestational age: 25–29 weeks (n = 28) and 30–33 weeks (n = 48). They were then reclassified as betamethasone positive (mother received one course of betamethasone) or betamethasone negative (mother did not receive any antenatal glucocorticoid treatment). Cross sectional M mode echocardiographic scans were performed during the first three postnatal days and at the end of the first and third weeks. Left interventricular septum (IVS), left ventricular posterior wall (LVPW), left ventricular end diastolic (LVED), and left ventricular end systolic (LVES) dimensions, aortic root (AO), and left atrial diameters (LAs) were measured. The IVS to LVPW ratio was calculated to identify asymmetrical septal hypertrophy. Results: In neither group was any statistically significant difference noted in IVS, LVED, LVES, LVPW, LA, and AO measurements during the three cardiac ultrasonography scans. Systolic function, as assessed by fractional shortening, was not significantly different in infants who received betamethasone antenatally, in either age group. There was no difference in the IVS/LVPW ratios between those who received antenatal steroid and those who did not for the 25–29 week and 30–33 week groups during these three consecutive scans. Conclusion: One course of antenatal betamethasone did not affect the cardiac wall thicknesses and systolic function in premature infants.

Right atrial appendage aneurysm is a very rare malformation that may be congenital or acquired (Gulati et al. 2011). Congenital-type atrial appendage aneurysms are thought to develop due to dysplas...

The medical records of 2283 patients with ventricular septal defect (VSD) were reviewed to determine spontaneous closure, left ventricular-to-right atrial shunt, subaortic ridge, and aortic valve prolapse. One thousand eight hundred and twenty-three patients had been followed 1 month to 26 years (median 4 years) by echocardiography. Most of 460 patients could not be followed due to transportation of the institution. VSD was perimembranous in 68.8% (1255), trabecular muscular in 21.7% (395), muscular outlet in 6% (109), muscular inlet in 2.6% (48), and doubly committed subarterial in 0.9% (16). Defect size was classified in 66.8% (1218) as small, in 15.7% (286) as moderate, and in 17.5% (319) as large. VSD closed spontaneously in 18.8% (343 of 1823 patients) by ages 40 days to 24.9 years (median, 1.8 years). One hundred fifty-seven of 1255 perimembranous defects (12.5%) and 167 of 395 trabecular muscular defects (42%) closed spontaneously (p

The aim of this study was to investigate subclinical systolic and diastolic dysfunction in juvenile-onset systemic lupus erythematosus (j-SLE) patients with speckle tracking echocardiography (STE) and the effects of disease activity on left ventricular (LV) regional functions. Thirty-five patients with j-SLE and 30 healthy children (control group) were evaluated between January and August 2015. STE was performed on all patients and controls. Medical records, including diagnosis criteria, age at diagnosis, and duration of disease, were evaluated. SLE disease activity was assessed using the SLE Disease Activity Index (SLEDAI). j-SLE patients had lower ejection fraction than did control subjects but still within normal range. LV end-diastolic and end-systolic dimensions were significantly larger in j-SLE patients (32.43 ± 3.2 vs 28.3 ± 3.1 and 21.1 ± 1.9 vs 18.9.0 ± 2.2, respectively; p = 0.001). There was a significant reduction in longitudinal strain of LV segments in the j-SLE patients compared with controls. J-SLE patients were further divided into subgroups. Group 1 comprised patients having SLEDAI scores >8 at the onset of disease but who improved with therapy during follow-up. Group 2 included j-SLE patients with SLEDAI scores >8 at diagnosis and persistently >4 at the end of follow-up. In the LV mid-inferior and mid-inferolateral segments, STE strain measurements of group 2 were significantly lower than those of group 1 (15.9 ± 6.4 vs 20.0 ± 4.4, 17.9 ± 7.2 vs 23.2 ± 3.8; p = 0.075, p = 0.055, respectively). Simple and non-invasive STE would be helpful in predicting cardiovascular prognosis with new therapeutic medications/interventions or in objectively comparing the effects of immunosuppressive drugs in comparison with preceding STE evaluation.

Generalised lymphangiomatosis is a rare disease that is characterized by widespread bony and soft tissue involvement of lymphangioma. Radiological evaluation is crucial because the site and extent of the lymphangioma are important prognostic factors. The computed tomography, ultrasonography and magnetic resonance images showed sharply defined, non-enhanced cystic lesions involving the mediastinum, bones, spleen, lung, and lower neck. We report here a case that was referred to us for investigation of marked cardiomegaly.

Objective: To emphasize the formation of fibrous aneurysmal structures in the setting of muscular ventricular septal defects Methods: Among patients with muscular ventricular septal defects followed in our institute, we described six who were diagnosed with formation of fibrous aneurysms. Of the patients, one was female and others were male. The age at diagnosis ranged from eight days to 16 years. In one, the defect opened between the ventricular inlets, but in the others the defects occupied the middle part of the apical trabecular septum. Results: The sizes of defects at initial echocardiographic evaluation ranged from 2.9 to 8 millimetres. The period of follow up for four patients was from 5 months to 7 years, one patient could not be followed up, and the other had already been diagnosed when first seen in our Institution. In our second and third patients, no aneurysm was seen during the initial echocardiographic evaluation, and it was recognized during follow-up. In these two patients, sequential echocardiographic examinations showed that the formation of the aneurysm made the defect smaller, with it almost closing in one. Conclusion: Although seen most frequently in the setting of perimembranous defects, fibrous aneurysms can also form adjacent to, and reduce the size of, muscular ventricular septal defects.

Aortic arch anomalies have been well described in the literature; the term refers to a variety of congenital abnormalities of the position or branching pattern, or both, of the aortic arch (Yoo et ...

Myocardial bridging is usually seen in the setting of hypertrophic cardiomyopathy or left ventricular hypertrophy. It is rarely reported in an asymptomatic patient with an otherwise structurally normal heart. Familial subaortic stenosis is also a rare entity, and its mode of inheritance is still unknown. Here, we described the case of a 13-year-old asymptomatic girl with a positive family history of sudden cardiac death and subaortic stenosis who was diagnosed with severe myocardial bridging concomitant with familial subaortic stenosis.

Prenatal diagnosis of a congenital coronary artery fistula between the left coronary artery and the right ventricle was established at 28 weeks of gestation. Hydrops fetalis developed during follow-up and the baby died on the first day after delivery. It is rare for coronary artery fistulas to become symptomatic during fetal life.

Background: Assessment of right ventricular function is a key point in the follow-up of operated patients with tetralogy of Fallot. Cardiac magnetic resonance assessment of right ventricular function is considered the gold standard. However, this technique is expensive, has limited availability, and requires significant expertise to acquire and interpret the images. Myocardial performance index and isovolumic acceleration have recently been studied for the assessment of right ventricular function and are shown to be simple yet powerful tools for assessing patients with right ventricular dysfunction of various origins. Methods: In this study, the integrity of myocardial performance index and isovolumic acceleration obtained by tissue Doppler imaging echocardiography to quantify right ventricular function was assessed in 31 patients operated for tetralogy of Fallot. Myocardial performance index and isovolumic acceleration measurements were compared with the parameters derived by cardiac magnetic resonance imaging. Results: In this study, a significant correlation has not been detected between cardiac magnetic resonance-originated right ventricular ejection fraction, pulmonary regurgitation fraction and myocardial performance index, isovolumic acceleration obtained by tissue Doppler imaging echocardiography from the lateral tricuspid annulus of the right ventricle. Conclusion: We have concluded that when evaluated separately, myocardial performance index and isovolumic acceleration obtained from tissue Doppler imaging echocardiography can be used in the long-term follow-up of patients who have been operated for tetralogy of Fallot, but that they do not show correlation with cardiac magnetic resonance-originated right ventricle ejection fraction and pulmonary regurgitation fraction.

Cervical aortic arch is a rare congenital anomaly. We present a case of left-sided cervical aortic arch, found in a patient with mitral regurgitation, mitral stenosis, and a regurgitant bicuspid aortic valve. There was atypical obstruction proximally within the arch. The obstructive segment was resected, and corrected by performing an end-to-side anostomosis. Mitral valvoplasty was performed in the same surgical procedure. To the best of our knowledge, a cervical arch has not previously been described with such atypical obstruction, and in association with multiple lesions involving the left heart.

Background Myocardial dysfunction due to coronary arterial lesions is an important complication after Kawasaki disease in childhood (1,2). Speckle-tracking echocardiography (STE) is a recently developed technique for evaluation of myocardial deformation (3). Objectives Our objective was to examine myocardial deformation at long term follow-up of Kawasaki patients. Methods We assessed regional myocardial function in 32 asymptomatic Kawasaki patients and 30 age matched healthy child as control group. STE examination were recorded and the acquired raw data were saved for offline analysis The inferoseptal, anterolateral, inferior, anterior, and inferolateral (posterior) walls were investigated for longitudinal systolic peak values for strain (LV-SR) imaging. Results Mean age was 21±12 months, mean follow up time 61 months, Left ventricular ejection fraction was 57,373±8,338 in patients and 61,100±3,804 in control group. LV-SR of Kawasaki patients at basal-inferoseptal (20,000±6,046 vs 25,200±5,074), basal-anterolateral (19,595±5,713 vs 22,733±3,494), apical-septal (23,378±5,609 vs 26,267±4,334), apical-inferior (24,054±3,696 vs 25,200±2,396) segments had lower values than control group. Conclusions We demonstrated subclinical systolic impairment of function especially at left ascending artery territories with STE. STE has the potential to detect these subtle abnormalities, and help management during long term follow up. References Printz BF, et al. Noncoronary cardiac abnormalities are associated with coronary artery dilation and with laboratory inflammatory markers in acute Kawasaki disease. Journal of the American College of Cardiology 2011;57(1): 86–92. Xu QQ, et al. Evaluation of left ventricular systolic strain in children with Kawasaki disease. Pediatric cardiology 2014;35(7): 1191–1197. Marwick TH, et al. Myocardial strain measurement with 2 dimensional speckle-tracking echocardiography: definition of normal range. JACC: Cardiovascular Imaging 2009;2(1): 80–84. Disclosure of Interest None declared

Background Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by vasculitis and inflammation in various organs. Cardiovascular involvement, although not frequent in juvenile-onset SLE (j-SLE), if present, is a significant cause of morbidity and mortality (1). Particularly, involvement of the myocardium layer may lead to ventricular dysfunction since its progression is insidious (1,2). Speckle tracking echocardiography (STE) could demonstrate subclinical myocardial deformations (strain) that globally seems normal in conventional echocardiography (2). Objectives The aim of this study is early detection of subclinical systolic dysfunctions in j-SLE with STE and if present, then to investigate whether this is disease-related or it is a result of other predisposing conditions. Methods 35 patients with j-SLE and 30 healthy children as a control group were evaluated between January and August 2015 at outpatient clinics of Cerrahpasa Medical Faculty. STE was performed on all patients and controls. Medical records that are including age at diagnosis, duration of the disease, diagnostic criteria, laboratory tests and cumulative clinical manifestations were evaluated. SLE disease activity was assessed using the SLE Disease Activity Index (SLEDAI). A SLEDAI score >4 was arbitrarily designated as a sign of moderate/severe disease activity. Results j-SLE patients had lower EF values than control subjects. Left ventricular end diastolic dimension (LVEDD) and left ventricular end systolic dimension (LVESD) were significantly greater in j-SLE patients (42,278±4,530 vs. 37,314±5,535; 28,108±3,344 vs. 24,055±3,290 p=0.001, respectively) than in the control group. There was a significant reduction in systolic parameters of longitudinal strain in the j-SLE group (p SLE patients were divided into two subgroups. Group 1 included patients having SLEDAI scores >8 at the beginning of the disease but who improved with therapy during follow up, with resulting SLEDAI scores less than or equal to 4 points. Group 2 included j-SLE patients with SLEDAI scores >8 at diagnosis but with SLEDAI scores still greater than 4 at the end of follow up. In comparisons of two groups, mid inferior and mid inferolateral LV segment STE strain measurements of Group 2 were significantly lower than those in Group 1, (15.9000±6.47130 vs. 20.0714±4.49725 mid inferior; 17.9000±7.23341 vs. 23.2308±3.87629 mid inferolateral, p=0.075, 0.055, respectively). Conclusions We can say that prevention of long-term cardiovascular complications in j-SLE begins with noticing the iceberg early, before irreversible changes take place. In this regard, STE is an accurate method for detecting subclinical systolic dysfunction. References Apte M, McGwin G Jr, Vila LM, Kaslow RA, Alarcόn GS, Reveille JD. Associated factors and impact of myocarditis in patients with SLE from LUMINA, a multiethnic US cohort. Rheumatology (Oxford) 2008;47: 362–367. Huang BT, Yao HM, Huang H. Left ventricular remodeling and dysfunction in systemic lupus erythematosus: A three-dimensional speckle tracking study. Echocardiography 2014; 31: 1085–1094. Disclosure of Interest None declared

In a 4-year-old boy with severe mitral regurgitation, cross sectional echocardiography combined with Doppler interrogation confirmed the presence of isolated perforation of the aortic leaflet of the mitral valve. The perforation was closed with a patch of fresh autologous pericardium. Serial echocardiograms taken postoperatively showed no regurgitation across the mitral valve.

Objective Heart rate variability (HRV) measures are altered in various cardiac and non-cardiac situations in children. The autonomic nervous system is assumed to have a role in the pathophysiology of atrial septal defect (ASD). In this study, we evaluated the autonomic system by measuring HRV in children with ASD. Methods Twenty-eight patients with ASD and 32 healthy children (mean ages: 6.6 ± 2.1 years and 6.4 ± 2.2 years, respectively) were enrolled in the study. Twenty-four-hour ambulatory electrocardiographic recordings were obtained and the seven time-domain (SDNN, SDANN, rMSSD, SD, SDNN index, PNN50, and mean RR) and four frequency-domain (VLF, LF, HF, and LF/HF ratio) indices of HRV were analyzed. Results A significant decrease in calculated HRV variables was observed in children with ASD as compared to controls. The HRV alteration was found in both time-domain and frequency-domain parameters. Conclusions Our results indicate that HRV is decreased in children with ASD, which implies parasympathetic withdrawal and sympathetic predominance.

Macrocephaly-capillary malformation syndrome is characterized by cutaneous vascular malformations with associated anomalies as macrocephaly, macrosomia, hemihypertrophy, hypotonia, developmental delay, lax joints, loose skin, polysyndactyly, and neuroimaging abnormalities. We present a newborn with a prenatal diagnosis of macrosomia and tetralogy of Fallot. He also had macrocephaly; a high forehead; capillary hemangioma on the forehead, upper lip, and philtrum; generalized loose skin; postaxial polydactyly of both hands and feet, with neuroimaging findings of polymicrogyria and thrombosis in sagittal sinus and sinus rectus. His condition was diagnosed as macrocephaly-capillary malformation syndrome in the neonatal period and he died suddenly during sleep at 6 months of age. The clinical course in this syndrome is not as benign as was previously thought. Careful follow-up of these patients with particular emphasis on neuroradiologic and cardiologic evaluation might help decrease the risk of sudden death and to improve long-term outcome.

Address for Correspondence/Yaz›sma Adresi: Dr. Bulent Koca, Istanbul Universitesi Cerrahpasa Tip Fakultesi, Cocuk Sagligi ve Hastaliklari Anabilim Dali, Cocuk Kardiyoloji Bolumu, Istanbul-Turkiye Phone: +90 212 530 69 19 Fax: +90 212 632 86 33 E-mail: bkoca78@yahoo.com Accepted Date/Kabul Tarihi: 07.09.2011 Available Online Date/Cevrimici Yayin Tarihi: 03.12.2011 ©Telif Hakk› 2011 AVES Yay›nc›l›k Ltd. Sti. Makale metnine www.anakarder.com web sayfas›ndan ulas›labilir. ©Copyright 2011 by AVES Yay›nc›l›k Ltd. Available on-line at www.anakarder.com doi:10.5152/akd.2011.197 Bulent Koca, Ayse Guler Eroglu, Selman Gokalp, Levent Saltik, Funda Oztunc

Early recognition of congenital cardiac pathologies and their treatment by means of palliative or corrective surgery at birth or infancy has vital importance. Successful repair of congenital cardiac defects by surgical methods has gained importance especially during the last twenty years. As the scope of the surveillance increased so did the interest in the outcomes of these treatments when the patients had reached puberty and adulthood. The purpose of our research was to study the psychological framework of the adolescents who had experienced these surgeries by listening both the children and the parents talk about their feelings and experiences. Our data was accumulated through interviews with 17 adolescents and their families, using qualitative methods. The main theme at the end of the analysis was "to be strong and resistive". We reached the conclusion that this condition was not a pathological build up but an attitude of coping, as it did not cause loss of functionality. The defensive psychological mechanisms used by these adolescents consisted of repression, compensation and reaction formation. We believe that this information is important to understand the real meaning of the manners displayed when these adolescents and their families pursue their daily lives, communicate and make relationships with their environment and especially professionals in the health services.

Familial Mediterranean fever is a hereditary disease characterised by recurrent and self-terminated attacks of fever and polyserositis. An earlier study found that adult patients of Familial Mediterranean fever had an abnormally longer QT dispersion and corrected QT dispersion, markers for ventricular arrhythmogenicity. QT dispersion is a simple non-invasive arrhythmogenic marker that can be used to assess homogeneity of cardiac repolarisation; however, it has not been studied in children with Familial Mediterranean fever before. The aim of this study was to assess QT dispersion and corrected QT dispersion, and their relationship with systolic and diastolic function of the left ventricle in a group of children with Familial Mediterranean fever. We performed electrocardiography and Doppler echocardiography on patients and controls. Maximum QT, minimum QT, QT dispersion, corrected QT, maximum corrected QT, minimum corrected QT, and corrected QT dispersion intervals were measured from standard 12-lead electrocardiography. No statistically significant differences were found between the groups in QT dispersion, corrected QT dispersion, and systolic–diastolic function of the left ventricle parameters. During the 12 months of follow-up, no ventricular arrhythmias were documented in either group.