Echocardiographic Findings in Children with Multisystem Inflammatory Syndrome from Initial Presentation to the First Years after Discharge.

OBJECTIVE: We aimed to evaluate clinical and echocardiographic features of the children diag- nosed with multisystem inflammatory syndrome related to severe acute respiratory syndrome coronavirus-2 infection and determine early and mid-term cardiovascular outcomes. MATERIALS AND METHODS: We retrospectively evaluated 38 children who were diagnosed with multisystem inflammatory syndrome in our hospital between November 2020 and November 2021. Cardiovascular evaluations were performed during hospitalization, at the first, the second, and the third months after discharge, and then cardiac evaluation was repeated at 3-month intervals until a median of 24 weeks (range: 9-56 weeks). RESULTS: The mean age of patients was 9.6 years and 25 patients had cardiovascular involve- ment. Echocardiography showed that there was left ventricular dysfunction in 11 cases and any coronary abnormalities in 11 cases on admission. Cardiovascular involvement was most fre- quently seen in patients older than 10 years and of male sex. Severe clinical courses occurred in half of them. The mortality rate was 2.6% during hospitalization. At discharge, complete recovery was achieved in 30 cases and partial recovery was seen in 6 cases; there were 1 case with ventricular dysfunction and 5 cases with coronary abnormalities. At the last polyclinic visit, there was no case with symptoms or myocardial dysfunction, there was only 1 case with persist- ing coronary aneurysms. CONCLUSION: Cardiovascular abnormalities in patients with multisystem inflammatory syndrome show rapid resolution within the first month. We recommend long-term follow-up evaluation for coronary arteries.

A newborn with aortico-left ventricular tunnel mimicking sinus of valsalva aneurysm.

Aortico-left ventricular tunnel (ALVT) is a rare congenital cardiac anomaly and constitutes less than 0.1% of all congenital cardiac defects (1). ALVT is described as an abnormal connection between the ascending aorta and the left ventricle which originates commonly above the right sinus of valsalva. Most patients are diagnosed with an ALVT during early infancy (2). Although transthoracic echocardiography (TTEAQ5) is more effective in diagnosis of ALVT, misdiagnosis rate was 17.1% (3). Sinus of valsalva aneurysm (SVA) is frequently confused with ALVT (3). We report a term female newborn with SVA in echocardiographic examination, but in surgery, she was diagnosed with ALVT.

Evaluation of left ventricular functions with two-dimensional speckle-tracking echocardiography (2D-STE) and N-terminal ProBNP in diabetic children.

Simsek A, Turan Ö, Çiftel M, Kardelen F, Durmaz E, Özdem S, Akçurin G, Ertug H. Evaluation of left ventricular functions with twodimensional speckle-tracking echocardiography (2D-STE) and N-terminal ProBNP in diabetic children. Turk J Pediatr 2018; 60: 633-641. The purpose of this study was to examine the existence of subclinical left ventricular dysfunction by using 2D-STE and NT-ProBNP levels in children and adolescent patients with type 1 diabetes mellitus. Furthermore, it was also aimed to investigate the effects of the diabetes duration and the metabolic control of the disease on cardiac functions. The patient group was composed of 63 children who were being followed up for the type 1 diabetes mellitus. The control group was composed of 36 healthy children who were of the similar age. Patients with type 1 diabetes mellitus were divided into groups; according to the duration of the disease; group 1: 3-5 years, group 2: 6-10 years of follow-up. The conventional echocardiography and 2D-STE were applied to all of the patients and control individuals. NT-Pro BNP level was measured in the diabetes group. In the conventional echocardiographic examination; there was no difference between the patient and control groups in terms of left ventricular systolic functions, left ventricular diastolic functions; late-diastolic flow velocity in mitral valve (A) values increased and E-wave/A-wave ratio (E/A ) values decreased in diabetes mellitus patients. According to the 2D-STE results; global longitudinal strain, (-17.28±2.24 vs. -19.49±2.22; p < 0.05) and circumferential strain (-12.86±3.19 vs. -17.71±4.62; p < 0.05) were lower in diabetic patients compared to the parameters of control group individuals. There was no difference between levels of NT-ProBNP of the group 1 and group 2 diabetes mellitus patients. Our study showed that there was a dysfunction on the left ventricular systolic functions of the patients with type 1 diabetes mellitus. NT-Pro BNP levels were not considered as a distinguishing factor for the early stages of diabetes mellitus.

Premature Coronary Artery Disease due to Homozygous Familial Hypercholesterolemia in a 12-Year-Old Girl.

BACKGROUND: Homozygous familial hypercholesterolemia is a rare inherited metabolic disease caused by low-density lipoprotein receptor abnormality. Patients with homozygous familial hypercholesterolemia have an increased risk of cardiovascular complication that usually occurs in the first decade of life. Here, we report a 12-year-old girl with an unpredicted presentation for coronary artery disease and found to have homozygous familial hypercholesterolemia. CASE REPORT: A 12-year-old girl was admitted to our unit with syncope. Chest X-ray showed bilateral diffuse pneumonic consolidation and mild cardiomegaly. We detected stable ST depression by electrocardiography. Echocardiography showed normal systolic functions. Troponin-1 levels were high (66 mcg/dL, upper limit: 0.04 mcg/dL). Influenza A virus DNA was detected by the respiratory viral panel. After her successful treatment for acute pneumonia and myocarditis due to Influenza A virus, her syncope attacks persisted. Marked ST elevation was observed during exercise electrocardiography. Coronary angiography showed severe occlusions in the coronary arteries. High serum levels of total cholesterol (756 mg/dL) and low-density lipoprotein-C (556 mg/dL) were noticed. She had no tendon xanthomas. Medical histories revealed that her family members were diagnosed with heterozygous familial hypercholesterolemia. A coronary bypass surgery was performed. Statin and ezetimibe treatments were started. We also planned lipid apheresis. CONCLUSION: Children with homozygous familial hypercholesterolemia may present with symptoms of premature coronary heart disease requiring a routine lipid test and careful anamnesis.

Cardiac Autonomic Dysfunction in Patients With Infantile Spasm and the Effect of Adrenocorticotropic Hormone Treatment.

Infantile spasm is an age-dependent epileptic-encephalopathy syndrome. Cardiac autonomic function is frequently altered in epilepsy. In this study, we examined heart rate variability in patients with infantile spasm before and after treatment. Nineteen patients with infantile spasm and 13 healthy comparisons were enrolled in the study. Cardiac rhythm was recorded with a Holter device for 24 hours before adrenocorticotropic hormone (ACTH) (Synacthen depot) and B6 vitamin administration and 1 month after treatment. Heart rate variability analysis found lower heart rate variability parameters in patients with infantile spasm at the onset of the syndrome, prior to treatment with ACTH. The time domain parameters of heart rate variability values showed a statistically significant increase following ACTH treatment. Our data suggest that patients with infantile spasm exhibit lower heart rate variability parameters, and the treatment of spasms with ACTH and B6 together diminished the autonomic dysfunction in our cohort.

Assessment of left ventricular function by tissue Doppler echocardiography in pediatric chronic kidney disease.

BACKGROUND: Cardiovascular (CV) disease remains the most common cause of mortality in chronic kidney disease (CKD). METHODS: In this cross-sectional study, 43 pediatric patients with CKD were divided into two groups according to their estimated glomerular filtration rate (eGFR): groups 1 and 2 (eGR; 29-75 and 15-29 mL/min/1.73 m(2), respectively). M - mode, conventional pulsed wave Doppler (cPWD) echocardiography and tissue Doppler imaging (TDI) were performed in all patients and 16 healthy controls. Maximal early (E wave) and late (A wave) diastolic flow velocities were assessed by cPWD. Using TDI, the early (E') and late (A') diastolic filling velocities were recorded. Early and late diastoles were evaluated using E' values and E/E' ratios, respectively. RESULTS: Left ventricular hypertrophy (LVH) was determined in 19/43 (44.2%) patients. The E/E' ratio was significantly higher in group 2 than in group 1 and controls. E/E' was found to be positively correlated with left ventricular mass (LVM) index, and negatively with hemoglobin (Hb) levels. Low Hb levels were only independent predictor of E/E' (p = 0.001, ß: -0.470, 95% CI: -0.764; -0.196). E' ratio was significantly lower in both patient groups compared to the controls. CONCLUSIONS: LVH and diastolic dysfunction are already present in early stages of CKD. Treatment of risk factors, such as anemia, is important to improve the clinical outcome.

Assessment of atrial electromechanical delay using tissue Doppler echocardiography in children with asthma.

Right ventricular (RV) dysfunction, pulmonary hypertension, atrial enlargement, and cor pulmonale may be associated with asthma. These pathological conditions may disturb the electrophysiological properties of the right atrium. This study investigated the atrial electromechanical delay and P-wave dispersion (PWD) in patients with asthma. Thirty-four children aged 8-16 years who were being followed up for asthma constituted the patient group, and 34 age- and body mass index-matched patients constituted the control group. Both groups underwent RV tissue Doppler measurements, intra-right atrial conduction time (IRCT-echo) determination, intra-left atrial conduction time (ILCT-echo) determination, inter-atrial conduction time (IACT-echo) determination, and PWD measurement. The IRCT-echo (14.38 ± 5.46 and 8.20 ± 3.90 ms; p < 0.001) and IACT-echo (28.97 ± 6.58 and 22.79 ± 6.28 ms; p < 0.001) were higher in patients with asthma than in the control group. The PWD (44.58 ± 17.51 and 38.11 ± 13.50 ms; p = 0.09), maximum P-wave duration (87.94 ± 18.20 and 82.44 ± 16.36 ms, p = 0.19), minimum P-wave duration (43.58 ± 9.95 and 44.79 ± 9.13 ms; p = 0.60), and ILCT-echo (15.88 ± 5.40 and 14.58 ± 4.94 ms; p = 0.30) were similar between the two groups. The IRCT-echo was positively correlated with the isovolumetric relaxation time of the lateral tricuspid annulus (r = 0.60; p < 0.001) and with the myocardial performance index of the lateral tricuspid annulus (r = 0.59; p < 0.001) in patients with asthma. The IRCT-echo and IACT-echo were higher in patients with asthma than in the control group. The deterioration of the electrophysiological properties of the right atrium may result in a risk of atrial fibrillation in patients with asthma.

Investigation of endothelial dysfunction and arterial stiffness in children with type 1 diabetes mellitus and the association with diastolic dysfunction.

OBJECTIVE: The objective of this study is to investigate endothelial dysfunction (ED) and arterial stiffness (AS) and determine the association with diastolic dysfunction in children with type 1 diabetes mellitus (DM). METHODS: A total of 42 patients without diabetic complications (mean age: 13.21 years) and 40 healthy (mean age: 13.07 years) children were included in this study. AS was assessed with ascending aorta M-mode measurements, diastolic dysfunction with pulsed wave (PW) Doppler and tissue Doppler echocardiography measurements and flow-mediated dilatation (FMD) and carotid intima-media thickness (CIMT) with high-resolution ultrasonography. RESULTS: Results of diabetic group and healthy children were compared. In diabetic group, aortic strain (8.40 ± 2.98, 20.12 ± 5.04; p < 0.001), aortic distensibility (7.36 ± 2.92, 16.59 ± 4.25; p < 0.001) and FMD% (7.70 ± 2.83, 11.33 ± 2.85; p < 0.001) were found decreased, and CIMT (0.52 ± 0.09 mm, 0.47 ± 0.08 mm; p < 0.05) was found increased. Additionally, left ventricular lateral segment and right ventricular free-wall isovolumic relaxation time (IVRT) and myocardial performance index (MPI) were found increased. Correlation analyses demonstrated a negative correlation between FMD and IVRT and MPI. CONCLUSIONS: ED and AS were found in type 1 DM patients without diabetic complications. Additionally, correlation was shown between increased AS and ED and right and left ventricular diastolic dysfunctions.

Assessment of early-onset chronic progressive anthracycline cardiotoxicity in children: different response patterns of right and left ventricles.

We aimed to assess early-onset chronic progressive cardiotoxicity in the left and right ventricles with increasing cumulative anthracycline doses. We evaluated 72 patients within the first year after doxorubicin and/or daunorubicin treatment (median 1.3 months; range 0.3-11.5) and 31 healthy controls. Pretreatment and posttreatment QT interval analyzes were performed in 27 newly diagnosed patients. The echocardiographic data of all examinations of 72 patients were classified into three groups according to instant cumulative anthracycline doses: treatment group (TG)-I (≤120 mg/m(2); n = 26), TG-II (120-240 mg/m(2); n = 39), and TG-III (≥240 mg/m(2); n = 40). Diastolic and systolic parameters were analyzed by conventional echocardiography and tissue Doppler imaging (TDI) and compared with those of healthy controls. The mean age for patients and controls was 8.2 ± 4.5 and 9.6 ± 4.2 years, respectively (p > 0.05). QTc dispersion significantly increased after anthracycline treatment (p = 0.02). TDI showed decreased E' velocity (p < 0.001) and E'/A' ratio (p < 0.001) at lateral tricuspid annulus segment in TG-I, and these findings continued in TG-II and -III. In addition, S' velocity decreased in TG-I, -II, and -III at lateral mitral annulus (10.5 ± 2.6 cm/s, p < 0.05; 9.9 ± 2.2 cm/s, p < 0.001; and 10.1 ± 2.3 cm/s, p < 0.01, respectively). However, decrease in left-ventricular ejection fraction was statistically significant in TG-II and -III (p < 0.001). Although myocardial performance index was significantly increased in all treatment groups in both segments, it was primarily due to significant increases in isovolumic relaxation time at the lateral tricuspid annulus and isovolumic contraction time at the lateral mitral annulus. Abnormalities in diastolic function in right ventricle and systolic function in the left ventricle were observed even with a cumulative anthracycline dose <120 mg/m(2) by TDI. In addition, anthracycline treatment led to an increase in QTc dispersion.

Carotid intima media thickness and arterial stiffness in children with acute rheumatic fever.

Acute rheumatic fever (ARF) and rheumatic heart disease (RHD) constitute important public health problems in developing countries. Inflammation is present both in the early and late stages of the diseases. Chronic inflammation is known to be associated with atherosclerosis. We hypothesize that subclinical atherosclerosis and arterial stiffness may increase due to the ongoing inflammation as well as the increased pulse pressure and left-ventricular systolic dysfunction in RHD. The purpose of the present study was to investigate carotid intima media thickness (CIMT) and carotid artery stiffness in patients with ARF. Forty patients in follow-up due to ARF in the age group of 7-16 years (disease duration 1-10 years) and 36 volunteered subjects with similar body mass index were included in the study. The subjects included in the present study were compared regarding M-mode echocardiographic parameters and CIMT as well as carotid arterial strain (CAS), carotid artery distensibility (CAD), beta stiffness index (ßSI), and pressure-strain elasticity modulus (Ep) as carotid artery stiffness parameters. CIMT (0.52 ± 0.08 and 0.48 ± 0.07 mm, p = 0.01), ßSI (5.29 ± 2.98 and 3.02 ± 1.30, p < 0.001), and Ep (426.53 ± 210.50 and 254.44 ± 104.69 p < 0.001) were increased, whereas CAS (0.11 ± 0.01 and 0.19 ± 0.09, p < 0.001) and CAD (10.27 ± 4.69 and 17.76 ± 14.41, p < 0.001) were decreased in patients with ARF compared with the control group. There was a positive correlation between pulse pressure and ßSI (r = 0.25, p = 0.02) and Ep (r = 0.28, p = 0.01) in addition to a correlation between left atrial dilatation and CIMT (r = 0.55 p < 0.001) in patients with ARF. CIMT and carotid artery stiffness were increased in patients with ARF. Patients with ARF may have an increased risk of subclinical atherosclerosis and cardiovascular events.

Assessment of atrial electromechanical delay in children with acute rheumatic fever.

PURPOSE: There may be an increase in the risk of atrial arrhythmia due to left atrial enlargement and the influence on conduction system in acute rheumatic fever. The aim of this study is to investigate atrial electromechanical delay and P-wave dispersion in patients with acute rheumatic fever. PATIENTS: A total of 48 patients diagnosed with acute rheumatic fever and 40 volunteers of similar age, sex, and body mass index were included in the study. The study groups were compared for M-mode echocardiographic parameters, interatrial electromechanical delay, intra-atrial electromechanical delay, and P-wave dispersion. RESULTS: Maximum P-wave duration, P-wave dispersion, and interatrial electromechanical delay were significantly higher in patients with acute rheumatic fever compared with the control group (p < 0.001). However, there was no difference in terms of intra-atrial electromechanical delay (p > 0.05). For patients with acute rheumatic fever, a positive correlation was identified between the left atrium diameter and the P-wave dispersion and interatrial electromechanical delay (r = 0.524 and p < 0.001, and r = 0.351 and p = 0.014, respectively). Furthermore, an important correlation was also identified between the P-wave dispersion and the interatrial electromechanical delay (r = 0.494 and p < 0.001). CONCLUSION: This study shows the prolongation of P-wave dispersion and interatrial electromechanical delay in acute rheumatic fever. Left atrial enlargement can be one of the underlying reasons for the increase in P-wave dispersion and interatrial electromechanical delay.

Facial nerve palsy and Kawasaki disease.

Kawasaki Disease (KD) is a vasculitic disease and can affect any organ system in the body. The development of coronary artery aneurysms is the most common and life threatening complication of KD and makes this disease the leading cause of acquired heart disease in children in the developed world. Facial nerve palsy has been reported as a possible marker of more severe disease and increased risk of coronary artery involvement in KD. Herein, the authors report an 8-mo-old infant who had left sided facial nerve palsy and multiple coronary aneurysms associated with KD.

Chest pain with myocardial ischemia in a child: should we think about coronary slow flow phenomenon?

The coronary slow flow phenomenon (CSFP) is an angiographic finding characterized by delayed opacification of epicardial coronary arteries in the absence of stenotic lesion. Herein, we present a 13-year-old boy with recurrent chest pain who was diagnosed with acute ST-segment elevation myocardial infarction associated with CSFP, which has not been reported previously in the pediatric age group. Coronary angiography revealed only the presence of slow flow in the left anterior descending (LAD) coronary artery. Myocardial perfusion scintigraphy revealed a reversible perfusion defect in the LAD territory, which regressed partially at rest and showed complete improvement after dipyridamole infusion. All the symptoms, electrocardiogram abnormalities and cardiac markers returned to normal after dipyridamole treatment during the follow-up. We conclude that CSFP should be kept in mind in the differential diagnosis of chest pain with myocardial ischemia in the pediatric age group.

Possible autosomal recessive inheritance in an infant with acrofacial dysostosis similar to Nager syndrome.

The acrofacial dysostosis syndromes, which are characterized by malformations of the craniofacial region and limbs, are a clinically heterogeneous group of disorders. Based primarily on the of the pattern of limb defects two major groups have emerged: Nager syndrome with predominantly preaxial malformations plus mandibulofacial dysostosis (severe micrognathia and malar hypoplasia) and Miller syndrome with postaxial malformations plus mandibulofacial dysostosis. Among these syndromes, Nager syndrome is a rare condition but the most common form of acrofacial dysostosis. Most cases are sporadic, while autosomal dominant and autosomal recessive inheritance patterns have been reported. Recently, heterozygous mutations in the SF3B4 gene on chromosome 1q12-q21 were found to be responsible for a subset of sporadic and autosomal dominant cases. We present a female infant born to consanguineous parents with craniofacial features resembling Nager syndrome and a unilateral preaxial limb malformation. Mutation analysis of coding exons of SF3B4 did not identify any mutations. This couple also had a deceased child who had similar clinical features. We conclude that, the presence of consanguinity and absence of mutation in SF3B4, provides evidence in support of a recessive form of Nager syndrome.

The efficacy of cardiac findings in assessing the outcome in preterms with bronchopulmonary dysplasia.

OBJECTIVES: To evaluate if cardiac dysfunctions are important in assessing the outcome in newborns with Bronchopulmonary Dysplasia (BPD), by evaluating cardiac functions with N-terminal prohormone of brain natriüretic peptide (NT-proBNP) levels, M-mode and tissue doppler echocardiography at 6-12 mo of age. METHODS: Twenty eight patients were retrospectively classified as mild, moderate and severe according to the diagnostic criterias for BPD. All cases were assessed with standard M-mode, tissue doppler echocardiography and NT-proBNP levels. Control group consisted of 28 healthy infants, having similar postnatal ages as patients and were assessed with standard M-mode and tissue doppler echocardiography. RESULTS: The age of patients with BPD was 9.8 ± 2.3 mo and control group was 9.5 ± 2.6 mo. There was no significant difference between the postnatal ages of two groups (p > 0.05). Neither pulmonary hypertension nor pulmonary/tricuspid regurgitation was detected. The M-mode echocardiography measurements did not differ between patients and control group (p > 0.05). Tissue doppler echocardiography, tricuspid valve medial segment early diastolic myocardial relaxation velocity (TME') measurements of patients were found significantly lower, peak transtricuspid filling velocity in the early diastole (TE)/TME' ratios and isovolumetric relaxation time (IVRT) measurements were found significantly higher than control group (p < 0.05). Tricuspid E, TE/TLE' (Tricuspid valve lateral segment early diastolic myocardial relaxation velocity), TE/RVLE'(Right ventricular lateral segment early diastolic myocardial relaxation velocity), TE/TME' levels were also found as significantly abnormal in patients with severe BPD. A significant correlation was found between right ventricular diastolic disfunctions and severity of BPD (p < 0.05). No statistically significant difference was found between NT-proBNP levels, BPD stages and tissue doppler echocardiography measurements (p > 0.05). CONCLUSIONS: This is the first study evaluating cardiac findings in patients with BPD by tissue doppler echocardiography and NT-proBNP at the same time. On the basis of cardiac evaluations, tissue doppler echocardiography measurements were found as significant and specific for the early assessment of right ventricular diastolic disfunctions.

Endothelial dysfunction and atherosclerosis in children with irreversible pulmonary hypertension due to congenital heart disease.

OBJECTIVE: To assess endothelial dysfunction and the risk for coronary atherosclerosis in children with irreversible pulmonary hypertension due to congenital heart disease (CHD). METHODS: The study included 18 cyanotic patients (the mean age was 12.28 ± 3.26 years) who developed irreversible pulmonary hypertension due to cyanotic and acyanotic CHDs, and 18 control patients (the mean age was 11.78 ± 3.00 years). Study groups were compared for flow-mediated dilatation (FMD), carotid intima media thickness (CIMT) and atherosclerotic risk factors. RESULTS: Compared to the control group, the mean FMD was significantly reduced in the cyanotic group (5.26 ± 2.42% and 9.48 ± 2.60%, respectively; P-value < 0.001). No significant difference was observed between the groups in CIMT (0.41 ± 0.08 mm and 0.39 ± 0.06 mm, respectively; P-value = 0.299). The levels of total cholesterol, low-density lipoprotein-cholesterol and very low-density lipoprotein-cholesterol were statistically significantly lower compared tothe control group (P-value = 0.001, 0.006 and 0.014, respectively), whereas no statistically significant difference was found in the levels of high-density lipoprotein-cholesterol and triglycerides (P-value = 0.113 and 0.975, respectively). CONCLUSIONS: Systemic endothelial dysfunction in children with irreversible pulmonary hypertension due to CHD was noted but there was no increased risk for atherosclerosis.

Can troponin T levels be useful in the diagnosis of rheumatic carditis?

OBJECTIVE: Acute rheumatic fever (ARF) is an endemic disease observed in children of developing countries. The purpose of this study was to test if it was possible to identify myocardial involvement in cases with rheumatic carditis by the measurement of serum cardiac TnT. METHODS: 30 patients diagnosed as ARF underwent echocardiography and their cardiac troponin T (cTnT) serum levels were measured. Patients were divided into group 1: Arthritis alone, group 2: carditis, and group 3 carditis with congestive heart failure (CHF). RESULTS: cTnT serum levels were normal in all except one patient with in group 3. Two patients in carditis (group 2) and three patients in CHF (group 3) had dilation in left ventricular end diastolic diameter. CONCLUSIONS: Normal cTnT levels in our patient group suggests that inflammation rather than myocardial necrosis is predominant in ARF carditis.

Prenatal diagnosis of mobile flap-like tissue on the ventricular septal defect in a newborn with trisomy 18.

Trisomy 18 is the second most common autosomal trisomy in liveborn infants. Various congenital malformations, mental retardation, and high rate of infant mortality in the first year of life are characteristic features of trisomy 18. Congenital heart disease occurs in over 90% of these patients and the most common cardiac lesions are ventricular septal defect, patent ductus arteriosus and atrial septal defect. This is a case report of a baby born with trisomy 18 (postnatal diagnosis) in whom there was an unusual echocardiographic appearance of a mobile structure ("flap-like") around the area of a VSD-which was imaged prenatally.