"Triangular QRS-ST-T waveform ECG pattern" during SARS-CoV-2 infection in a paediatric case with multiple comorbidities.

Triangular QRS-ST-T waveform electrocardiography pattern, so-called "shark fin sign," is a rare and highly mortal electrocardiography finding, which usually occurs in adult patients with coronary occlusion. Here, we reported the first paediatric case occurring in a striking "triangular waveform electrocardiography pattern" due to myocarditis during COVID-19 infection.

The serial changes in myocardial functions after paediatric haematopoietic stem cell transplantation.

The aim of this study is to evaluate the changes in myocardial functions in children who underwent haematopoietic stem cell transplantation along with associated chemotherapy. Additionally, we evaluated the effect of baseline echocardiographic parameters on mortality. We evaluated 39 patients (mean age 7.4 years) who underwent haematopoietic stem cell transplantation owing to non-malignant disease. The control group included 39 healthy children who had normal cardiac findings. The myocardial functions were evaluated in all subjects by conventional echocardiography and tissue Doppler echocardiography before haematopoietic stem cell transplantation and in the 1st, 3rd, 6th, and 12th month after haematopoietic stem cell transplantation. All patients had normal left ventricular ejection fraction before haematopoietic stem cell transplantation, except one case. Before haematopoietic stem cell transplantation, the patient group had significantly greater mean pulmonary artery pressure and lower tricuspid valve annular plane excursion rate. Baseline E' velocities for mitral lateral annuli, septum, and tricuspid lateral annuli were lower in the patient group than the control group. The E' velocities for the left ventricle decreased in the patient group after haematopoietic stem cell transplantation, and then returned to baseline levels at the 6 months. E' and S' velocities for tricuspid lateral annuli also decreased after haematopoietic stem cell transplantation and were still depressed in the first year after haematopoietic stem cell transplantation. Baseline E' velocity for septum was significantly lower in patients who died after haematopoietic stem cell transplantation than patients who survived (p = 0.009). Subclinical impairment in both ventricular functions was observed after haematopoietic stem cell transplantation and the right ventricular functions were affected for longer periods than left ventricle after haematopoietic stem cell transplantation. The myocardial functions should be monitored after the first year of haematopoietic stem cell transplantation.

A rare complication of Behçet's disease in a 12-year-old girl: a large intracardiac thrombosis.

Behcet's disease is a multi-systemic inflammatory disease with a clinical spectrum as a triple complex of recurrent oral, genital ulcers, and uveitis. Cardiac involvement in patients with Behcet's disease is extremely rare and often associated with poor prognosis. Behcet's disease should be considered in the differential diagnosis of right ventricular mass especially in young adults, even there is no typical clinical features of Behcet's disease. In this case, a 12-year-old girl who admitted with chest pain and haemoptysis and then was diagnosed with intracardiac thrombus related to Behcet's disease during follow-up was described.

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.

Effects of vagus nerve stimulation on heart rate variability in children with epilepsy.

PURPOSE: The aim of this study was to evaluate the effects of vagus nerve stimulation (VNS) on heart rate variability (HRV) in children with epilepsy. METHODS: The subgroups of HRV, namely time domain (Standard deviation of NN interval (SDNN), SDNN index, Standard deviation of the averages of NN intervals (SDANN), Root mean square of successive differences (RMMSD), Adjacent NN intervals differing by more than 50 ms in the entire recording divided by the total number of all NN intervals (PNN50), triangular index) and frequency domain (Low-frequency (LF), High-frequency (HF), LF/HF), were investigated in 20 pediatric patients before and after 6 and 12months of VNS treatment during day and night by comparing their data with those of 20 control subjects. In addition, subgroups of age, epilepsy duration and localization, and antiepileptic drugs (AEDs) were also evaluated if they had further effects on basal HRV levels. RESULTS: Increased heart rates (HRs); decreased SDNN, SDANN, RMMSD, and PNN50; and increased LF/HF ratios were identified before VNS therapy (p<0.05). Even though remarkable improvement was seen after 6months of VNS treatment (p<0.05), no further changes were observed in 12-month compared with 6-month levels (p>0.05) in all parameters, still even significantly lower than those of controls (p<0.05). Longer duration of epilepsy and localization of epileptic focus, such as in the temporal lobe, were also found to further contribute to diminished basal HRV levels (p<0.05). CONCLUSION: The cardiovascular system is under deep sympathetic influence in children with epilepsy. Although VNS seems to provide a substantial improvement by achieving increased parasympathetic effects in short-term therapy, the levels were still lower than those of healthy children after either short- or long-term therapy. Therefore, impaired cardiovascular autonomic regulation may be associated with the epileptic process itself as well as with the contribution of some additional factors. Overall, different aspects such as age, epilepsy duration, epileptic focus, seizure frequency, and AEDs should also be considered for their further possible effects on HRV during VNS therapy.

The pre-participation screening in young athletes: which protocol do we need exactly?

The aim was to assess the utility and feasibility of a comprehensive cardiac screening protocol in young athletes before participation in sports. A total of 380 athletes referring before participation in sports, between April, 2014 and April, 2015, were included in this study. The mean age was 12.4 years. A screening protocol has been applied to all, including personal and family history, physical examination, 12-lead electrocardiography, transthoracic echocardiography, 24-hour rhythm Holter analysis, and treadmill exercise test. The most frequent complaints were chest pain in 19 (5%), dyspnoea in 13 (3.4%), and dizziness and fainting in five patients (1.3%) on exercise. There was sudden death and arrhythmia in 41 patients (10.7%) owing to family history. Heart murmur was present in 20 (5.2%) and hypertension in 10 patients (2.6%) on physical examination. The 12-lead electrocardiography was abnormal in 9 patients (2.4%). The findings of transthoracic echocardiography were insignificant in 47 patients (12.3%) and in five patients (1.3%) a haemodynamically important condition was detected. The 24-hour rhythm Holter analysis was abnormal in six patients (1.5%). There were significant ST changes in two patients (0.5%) on treadmill exercise test with normal findings on myocardial perfusion scans. No significant relation was present between findings of screening protocol and transthoracic echocardiography, 24-hour rhythm Holter analysis, or treadmill exercise test results. Pre-participation screening in young athletes should consist of a targeted personal history, family history, physical examination, and 12-lead electrocardiography. Other tests should be applied only if the screening indicates the presence of a cardiovascular disease.

Elasticity of ascending aorta and left ventricular myocardial functions in children with bicuspid aortic valve.

BACKGROUND: The aim of the study was to determine the aortic elasticity parameters (EPs) and myocardial function in children with bicuspid aortic valve (BAV). Additionally, we evaluated the effect of aortic leaflet phenotype (ALP) and aortic dilatation on elasticity parameters. METHODS: Sixty-two children diagnosed with isolated BAV (mean age 9.3 years old; patient group) and 63 healthy children (control group) have been evaluated for this study. Patient group was divided into three age subgroups: between 0-6, 7-11 and 12-17 age intervals. Distensibility (DI) and stiffness index (SI) of ascending aorta were calculated by M-mode echocardiographic data. The myocardial functions were evaluated by tissue Doppler echocardiography. RESULTS: Patient group exhibited significantly lower DI and higher SI than control group (SI: 3.8 ± 1.7 vs 2.4 ± 0.8, P < .0001). The aortic elasticity indexes in patient group with different age subgroups were different from those in control subgroups. Patient group had significantly lower E' velocity at mitral lateral annulus and septum than control group (P < .017 and P < .001). There was no statistically significant correlation between E' velocities and DI/SI values. We, however, found a weak correlation between septal E' velocities and strain values (r = .255, P = .046). EP did not show statistically significant difference with regard to ALP and presence of aortic dilatation. CONCLUSION: Abnormality of aortic elasticity and myocardial functions can be detected in children with BAV from infantile to adolescent. Myocardial functions are not related to SI and DI. We considered the possibility of intrinsic aortic wall abnormality in children with BAV.

Assessment of left ventricular functions and myocardial iron load with tissue Doppler and speckle tracking echocardiography and T2* MRI in patients with ß-thalassemia major.

BACKGROUND: The purpose of this study is to determine early myocardial dysfunction in ß-thalassemia major (BTM) patients. Where the myocardial dysfunction cannot be detected by conventional echocardiography, it could be detected by tissue Doppler imaging (TDI) or speckle tracking echocardiography (STE). METHODS: In this study, we analyzed 60 individuals, 30 of whom were BTM patients and the other 30 of whom were the control group. T2* magnetic resonance imaging (MRI) was used to measure cardiac iron deposition. The myocardial functions were evaluated by conventional echocardiography, TDI and STE. RESULTS: When basal lateral left ventricular and basal septal wall TDI values were compared between the patient group and control group, only isovolumic contraction time values were significantly longer in the patients. The global circumferential strain was significantly lower in the patients. When evaluated as segmental, longitudinal strain values of basal inferoseptum and circumferential strain values of anteroseptum, anterior, and inferolateral segments were significantly lower in the patients. In the patients, global longitudinal and circumferential strains in the group who had pathological T2* values were significantly lower than the group who did not. In addition, circumferential strain values in anteroseptum, anterolateral, inferior, and inferoseptum segments were significantly lower in the patients with T2* values<20 ms than those with T2* values≥20 ms. CONCLUSION: Although T2* MRI is the most sensitive test detecting myocardial iron load, TDI and STE can be used for screening myocardial dysfunction. The abnormal strain values, especially circumferential, may be detected as the first finding of abnormal iron load and related to T2* values.

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.

Evaluation of cardiotoxicity by tissue Doppler imaging in childhood leukemia survivors treated with low-dose anthracycline.

Improvement in long-term survival in patients with acute childhood leukemia has led to the need for monitorization of chemotherapy-related morbidity and mortality. This study included 60 patients with acute lymphoblastic leukemia that were in remission for at least 2 years and 30 healthy controls. Systolic and diastolic function of myocardium was evaluated using conventional echocardiography and tissue Doppler imaging of the left ventricle, interventricular septum and right ventricle. Median age of patients was 11.7 years (range 10-14.9 years), and the median duration of remission was 4 years (range 2.5-5 years). All patients were treated with a low cumulative dose of adriamycin (100 mg/m(2)) according to the St. Jude Total-XIIIA protocol. The ejection fraction (EF) and fractional shortening were normal in the patient and control groups, even though EF values were significantly lower in the patients (69.5 ± 2.3 vs. 72.7 ± 3 %, P < 0.01). Myocardial systole (S m), early diastole (E m) and late diastole (A m) velocities in all segments of the myocardium were significantly lower in the patient group (P < 0.01 for all segments). Cardiotoxicity was noted in all segments of the myocardium in the patient group, despite the fact that they were all treated with a low cumulative dose of adriamycin. Based on these findings, we think that there is no safe dose for anthracyclines and periodic echocardiographic evaluation of both the left and right ventricles must be performed in all patients treated with anthracyclines, even at low doses.

Reversible pulmonary arterial hypertension in cobalamin-dependent cobalamin C disease due to a novel mutation in the MMACHC gene.

UNLABELLED: Methylmalonic aciduria and homocystinuria, cobalamin C (CblC) disease (OMIM 277400), is the most frequent inborn error of vitamin B12 (cobalamin, Cbl) metabolism and is caused by an inability of the cell to convert Cbl to its active forms (MeCbl and AdoCbl). More than 75 mutations have been identified in the MMACHC gene which is responsible for CblC disease. We present a case with CblC disease and pulmonary arterial hypertension (PAH) as the main symptom. The patient improved dramatically with parenteral hydroxocobalamin treatment. Most cases of CblC disease have a multisystemic disease with failure to thrive, developmental delay, hypotonia, visual impairment, and hematologic manifestations. This patient had isolated pulmonary hypertension and hyperhomocysteinemia which is thought to be an important factor in the pathogenesis of PAH. Genetic analysis identified a novel homozygous mutation (c.484G > T; p.Gly162Trp) in the MMACHC gene. CONCLUSION: CblC disease should be considered in the differential diagnosis of pulmonary hypertension.

Three-dimensional echocardiographic evaluation of a patient with double-orifice mitral valve, bicuspid aortic valve, and coarctation of aorta.

A 16-year-old male patient was diagnosed with bicuspid aortic valve and coarctation of aorta, and had a history of a subclavian patch plasty operation at 1 year of age. In addition, we detected double-orifice mitral valve (DOMV) at a routine evaluation and demonstrated by both two-dimensional and three-dimensional (3D) echocardiographic examinations. DOMV should be kept in mind in cases with left ventricular obstructive lesions and 3D echocardiography may provide a more detailed assessment of mitral valve and subvalvular apparatus.

Multiple giant aneurysms and stenoses of the coronary and systemic arteries in an infant with kawasaki disease at the early stage of convalescent period.

Myocardial infarction and systemic arterial aneurysms are rarely seen during the course of the Kawasaki disease (KD). Herein, we report the case of a 4-month-old Turkish infant who was diagnosed with KD on the 17th day of the illness. On admission, echocardiogram showed multiple coronary arterial aneurysms (CAAs) and massive pericardial effusion. He was given intravenous immunoglobulin, aspirin and anticoagulant drugs. However, the aneurysms progressed to "super giant" CAAs, multiple huge coronary arterial thromboses developed recurrently and caused myocardial ischemia. Furthermore, the conventional angiography revealed multiple giant aneurysms and stenoses in the subclavian, celiac, and iliac arteries, besides CAAs.

Native aortic valve endocarditis complicated by pseudoaneurysm of mitral-aortic intervalvular fibrosa.

Pseudoaneurysm of mitral-aortic intervalvular fibrosa (PA-MAIVF) is a rare complication of native aortic valve endocarditis. This region is a relatively avascular area and prone to infection during endocarditis and subsequent aneurysm formation. The rupture into the pericardial cavity and left atrium or aorta, systemic embolism, myocardial infarction secondary to left coronary compression, and sudden death are the reported complications. Herein, we present a 9-year-old boy who was diagnosed with bicuspid aortic valve endocarditis complicated by PA-MAIVF, cerebral embolism, and hemorrhage. PA-MAIVF was visualized by both two- and three-dimensional transthoracic echocardiography and ruptured into pericardial space causing a fatal outcome.

Evaluation of cardiac functions and atrial electromechanical delay in children with adenotonsillar hypertrophy.

Deterioration of the right ventricular (RV) functions and the increase in the pulmonary arterial pressure (PAP) of children with moderate to severe adenotonsillar hypertrophy (ATH) have been well described. In addition to these complications, this study aimed to investigate the influence of ATH on the conduction system. The study investigated 46 patients with a diagnosis of ATH and 46 healthy control subjects. Conventional echocardiography, P-wave dispersion (Pd), tissue Doppler imaging (TDI) findings, and atrial electromechanical delay (AED) were compared between the patients and the control subjects before and after adenotonsillectomy. The maximum P-wave duration and Pd were significantly higher in the patients than in the control subjects (p < 0.001). The patient group showed significantly greater RV end-diastolic dimension (p = 0.01), right atrial area (p < 0.001), and mean PAP (p = 0.03) but lower E/A ratios for the mitral (p = 0.04) and tricuspid (p = 0.01) valves and a shorter pulmonary flow trace acceleration time (p = 0.03). The tricuspid annular-plane systolic excursion was similar between these groups (p = 0.21). In the patient group, TDI studies showed significantly lower E'/A' ratios for the tricuspid lateral (p = 0.006) and mitral septal (p = 0.003) segments than in the control group. The myocardial performance index was lower for the mitral lateral, mitral septal, and tricuspid lateral segments in patient group (p < 0.001). Similarly, AED was prolonged in the patient group at all three segments (p < 0.001). Also, the patient group showed a significantly longer interatrial (p = 0.03) and intraatrial (p = 0.04) electromechanical delay. However, all the electro- and echocardiographic parameters were similar between the patients and the control subjects after adenotonsillectomy (p > 0.05). The prolongations in P-wave duration, Pd, and inter- and intraatrial electromechanical delays were first shown in this population. The cardiac changes induced by ATH-associated hypoxia may facilitate arrhythmias during the long term.

Intracardiac thrombus in children: the fine equilibrium between the risk and the benefit.

The medical records of 16 patients diagnosed as intracardiac thrombus were searched. The size, location and outcome of thrombus together with demographic data of patients were assessed. The median age of the patients was 2.2 years. Six patients were newborn and two patients were infant. The median size of thrombus was 9 mm. The localization was right atrium in seven, right ventricle in five, left ventricle in one, pulmonary artery in one, and superior vena cava in two patients. There was prematurity in five, ciyanotic congenital heart disease in one, blood culture positivity in three, malignancy in four, nephrotic syndrome in one, indwelling catheters in 10, and acquired or genetic thrombophilia in six patients as risk factors. In the treatment, the first choice was tissue plasminogen activator in two patients, heparin infusion in one patient and low molecular weight heparin in remaining 12 patients. In nine patients, therapy included parenteral antimicrobials together with anticoagulants. The result was complete resolution in 15 patients and in one patient thrombus was surgically removed. The median time was 16 (2-70) days for 50% resolution and 26 (3-93) days for complete resolution. There was a statistically significant (P = .027 and r = 0.5) correlation between the size and the complete resolution time. There was no anticoagulant therapy related major complication. In patients with intracardiac thrombus, selection of anticoagulant therapy may decrease the risk of complications. Surgery is rarely required and thrombolytics are not usually necessary for resolution of thrombus.

Pediatric syncope: is detailed medical history the key point for differential diagnosis?

Syncope is a transient loss of consciousness as a result of global cerebral hypoperfusion. It is generally benign but may be a sign of pathology. The purpose of this study was to analyze the frequency of syncope due to cardiac, neurocardiogenic, neurologic, situational, psychiatric, and other causes and make a differential diagnosis of syncope types according to detailed medical history and further investigations. We examined prospectively 268 children presented to pediatric polyclinics as well as cardiology and neurology departments (age range, 1-18 years) with a primary complaint of syncope for the study. Cardiac syncope was diagnosed in 12 patients, neurocardiogenic syncope in 232, neurologic syncope in 9, psychiatric syncope in 9, situational in 4, and benign paroxysmal positional vertigo in 2. The neurologic syncope group consists of patients diagnosed with epilepsy after evaluation. Eight patients in the cardiac syncope group were found to have diseases such as long QT syndrome, and the remaining patients had hypertrophic cardiomyopathy, atrioventricular nodal reentry tachycardia, ventricular tachycardia, and a second-degree heart block that can cause sudden death. In conclusion, syncope is a common problem in childhood that requires hospitalization. Because it may be the first finding of an underlying malignant cardiac or neurologic disease, clinicians must be very careful during medical evaluation. An electrocardiogram and a medical history including the details of the event, chronic diseases, and familial diseases are among the most important steps for the right diagnosis and prognosis. Instead of a routine procedure, further diagnostic workup should be directed according to medical history for high yield. Convulsive movements may be defined in all types of syncope related with cerebral hypoxia, and this may lead to a misdiagnosis of seizure by the clinician.

The effect of recombinant human growth hormone therapy on left-ventricular chamber size and function in children with growth hormone deficiency.

This study was designed to assess the effect of recombinant human growth hormone (rhGH) therapy on left-ventricular (LV) chamber size and function in children with idiopathic isolated growth hormone deficiency (GHD) using conventional echocardiography and tissue Doppler imaging (TDI). Thirty patients (19 boys and 11 girls) with idiopathic isolated GHD were followed-up for 12 months. Mean age of patients was 11.0 ± 2.6 years (range 6.3-15.5). At baseline and at 3, 6, and 12 months of treatment, the structure of the left ventricle was assessed by conventional echocardiography and myocardial rates and time intervals by TDI. There was a significant increase in LV mass (LVM) compared with pretreatment values. Like LVM, relative wall thickness (RWT) was also increased significantly. The significant increase in LVM indexed to body surface area and RWT became apparent at month 3 of treatment with a significant increase in LVM indexed to height(2.7) at treatment month 6. Normalized LVM increased as early month 3 of treatment, and a steady increase was observed until month 12. However, no patient had LVM > +2 standard deviation scores at month 12 of treatment. No significant differences were observed in functional parameters of the left ventricle and the interventricular septum. The results of this study showed that rhGH therapy causes an increase in myocardial mass without changing the geometry or function of the myocardium. Therefore, the increase in myocardial mass appears to be concentric, thus causing remodeling instead of hypertrophy.

A large intracardiac thrombus in a child with steroid-resistant nephrotic syndrome.

Intracardiac thrombus is a rare complication of nephrotic syndrome in children, and only a few cases have been reported. We report the case of a 3.5-year-old child diagnosed with Henoch-Schönlein Purpura and steroid-resistant nephrotic syndrome for 2 months. The patient had massive oedema but no cardiovascular instability. Echocardiography displayed a large thrombus within the right atrium and the patient was successfully managed with enoxoparine.