Transient second-degree high-grade atrioventricular block caused by myocarditis.

Myocarditis is an inflammatory disease of the heart muscle that most commonly occurs after infectious diseases in childhood. The clinical picture of acute myocarditis ranges from asymptomatic infection to fulminant heart failure and sudden death (1). Most of the patients may present with nonspecific symptoms such as respiratory distress, chest pain, nausea, and vomiting (2). While rhythm abnormalities such as ventricular and supraventricular rhythm disorders can be observed in these patients, various degrees of atrioventricular blocks may rarely develop (3). In this article, we aimed to present a patient who developed second-degree, high-grade atrioventricular block after myocarditis and recovered completely after treatment.

Assessment of Myocardial Mechanics in Acute Rheumatic Fever Using Speckle-Tracking Echocardiography.

BACKGROUND: This study aims to evaluate the role of speckle-tracking echocardiography to identify myocardial deformation in acute rheumatic fever. METHODS: Twenty-seven patients and 27 healthy children were prospectively evaluated. The patient group was divided into 2 subgroups based on echocardiographic findings, with or without carditis. The left ventricular global longitudinal strain and strain rate, left ventricular global circumferential strain and strain rate, and right ventricular global lon-gitudinal strain and strain rate were assessed by speckle-tracking echocardiography. RESULTS: In the acute phase of the disease, all values except the right ventricular global longitudinal strain were found to be significantly below the control group in the patient cohort. No significant difference was found between the patients grouped as carditis and non-carditis in the acute period. Comparison of the acute period with the post-treatment period revealed a significant increase in all strain values of the patients with carditis and significant increases observed in all values except left ventricular global longitudinal strain rate, left ventricular global circumferential strain rate, and right ventricular global longitudinal strain rate values in patients without carditis. Apart from the right ventricular global longitudinal strain rate, which was significantly lower in the non-carditis group compared to the control group, there was no significant difference in strain values between the patient and control groups following treatment. CONCLUSION: In the present study, we found that all patients, including patients in whom no valvular involvement was detected by echocardiography in the acute phase of acute rheumatic fever, had a lower right and left ventricular strain and strain rate measurements and that these findings improved after treatment, suggesting that strain echocardiography may be a helpful diagnostic method, especially in patients without valvular involvement.

Changing face of acute rheumatic fever in childhood and our clinical results.

OBJECTIVE: This study aims to evaluate the demographic and clinical findings of acute rheumatic fever (ARF) patients followed up in our clinic, their responses to treatment, and prognoses and to determine the clinical utility of echocardiography (ECHO) in the diagnosis of ARF. METHODS: We retrospectively evaluated the data of 160 patients with ARF (6-17, mean 11.7±2.3 years, F/M: 88/72) that was diagnosed according to the Jones criteria and followed up in the pediatric cardiology clinic between January 2010 and January 2017. RESULTS: About 29.4% (n=47) of 104 patients with rheumatic heart disease (RHD) had subclinical carditis. It was observed that subclinical carditis was most common in patients with polyarthralgia (52.2%); in contrast, clinical carditis was most commonly observed together with chorea (39%) and polyarthritis (37.1%). It was found that 60% (n=96) of the patients with rheumatic fever were between the ages of 10-13 and 31.3% (n=50) presented arthralgia most frequently in the winter months. The most common concomitant major symptoms were carditis + arthritis (35%) and carditis + chorea (19.4%). In patients with carditis, the most affected valves were mitral (63.8%) and aortic (50.6%) valves, respectively. The prevalence of monoarthritis, polyarthralgia, and subclinical carditis increased in cases diagnosed during and after 2015. The cardiac valve involvement findings of 71 of 104 patients (68.2%) with carditis improved during the approximately 7 years of follow-up. The regression of heart valve symptoms was significantly higher in patients with clinical carditis and those that complied with prophylaxis compared to patients with subclinical carditis and those that did not comply with prophylaxis. CONCLUSION: We conclude that ECHO results should be included in the diagnostic criteria of ARF, and that subclinical carditis is associated with a risk of developing permanent RHD. Secondary prophylaxis non-compliance is significantly associated with recurrent ARF, and early prophylaxis can reduce the prevalence of RHD in adults and potential associated complications.

A case series of intracardiac thrombi and vascular involvement in pediatric Behçet's disease.

To evaluate the general characteristics of pediatric Behçet's disease (BD) patients with thrombus and to present the clinical features, treatment responses and prognosis of patients with intracardiac thrombus. The clinical characteristics and outcomes of 15 patients with thrombus among 85 pediatric BD patients followed in the Department of Pediatric Rheumatology were evaluated retrospectively. Of the 15 BD patients with thrombus, 12 (80%) were male, 3 (20%) were female. The mean age at diagnosis was 12.9 ± 1.1 years. Thrombus was present at the time of diagnosis in 12 patients (80%), while thrombus developed in three patients within the first three months after diagnosis. The most common site of thrombus was the central nervous system (n = 9, 60%), followed by deep vein thrombus (n = 6, 40%) and pulmonary artery thrombus (n = 4, 26.6%). Three male patients (20%) developed intracardiac thrombus. The overall intracardiac thrombus rate in the 85 patients was 3.5%. Two of the three patients had thrombus in the right, and one had thrombus in the left heart cavity. In addition to steroids, 2 of the 3 patients received cyclophosphamide, while the patient with thrombus localized in the left heart cavity was given infliximab. In the follow-up, the two patients with thrombus in the right heart cavity were switched to infliximab because of resistance to cyclophosphamide. Complete resolution was observed in 2 of the 3 patients on infliximab; a significant reduction in the thrombus of the other patient was achieved. Intracardiac thrombus is a rare presentation of cardiac involvement in BD. It is usually observed in males and in the right heart. Although steroids and immunosuppressive agents such as cyclophosphamide are recommended as first-line treatment, favorable outcomes can be achieved with anti-TNFs in resistant cases.

Cardiac involvement in a case of juvenile dermatomyositis with positive anti-melanoma differentiation associated protein 5 antibody.

Juvenile dermatomyositis (JDM) is an autoimmune disease characterized by muscle weakness and specific skin lesions, as well as non-muscular involvement such as interstitial lung disease (ILD), cardiac involvement and arthritis. Anti-melanoma differentiation-associated protein 5 (anti-MDA5)-positive JDM patients are typically characterized by the presence of skin ulcers and rapidly progressing ILD (RP-ILD). Although cardiac involvement is not an expected involvement in anti-MDA5-positive JDM cases, it is significant because it can be fatal. In this report, an anti-MDA5 myositis-specific autoantibody-positive JDM case referred with the diagnosis of psoriatic arthritis in whom cardiomyopathy and arrhythmia were detected in follow-up is presented. Since cardiac involvement is associated with mortality, it would be useful to follow up anti-MDA5 positive patients for cardiac involvement in addition to lung involvement. Tofacitinib is a promising treatment option in treatment-resistant JDM.

Urgent aortic valve neocuspidization using the Ozaki procedure in an infant with native aortic valve endocarditis.

Native valve aortic endocarditis is rarely seen in the paediatric population. Although, the first-line of treatment is medical, surgical intervention may be indicated in patients with unrepairable valvular and subvalvular disease. Recently, the aortic valve neocuspidization (AVNeo) procedure has gained popularity both in adult and children in whom other repair techniques are not feasible. In this case report, we present an urgent aortic valve replacement using the AVNeo technique in a critically ill infant with a small annulus, severe left ventricular outflow tract stenosis, and severe aortic regurgitation.

Mid-cavitary hypertrophy after myocarditis in a patient with operated medulloblastoma.

A 16-month-old girl was referred for tachycardia and upper respiratory tract infection. Echocardiographic examination revealed pericardial effusion, mild mitral regurgitation, and left ventricle systolic dysfunction. Patient was positive for Parainfluenza type 4 virus. Her laboratory tests revealed increased troponin I level. The patient was treated with intravenous immunoglobulin considering acute viral myopericarditis. Two weeks after treatment, midventricular hypertrophy was detected.

Tetralogy of Fallot with double aortic arch and aortopulmonary window: a very rare trifecta.

Tetralogy of Fallot with an aortopulmonary window and double aortic arch is very rare. This complex coexistence may be over a wide clinical spectrum. Herein, we present an asymptomatic 8-day-old infant who was diagnosed as having tetralogy of Fallot, double aortic arch, and an aortopulmonary window using transthoracic echocardiography while being examined for microcephaly.

Successful occlusion of a feeding artery with Amplatzer Piccolo Occluder in a patient diagnosed with Scimitar syndrome.

Scimitar syndrome is a congenital anomaly in which some or all of right pulmonary veins drain into inferior caval vein. It is associated with anomalous systemic arteries arising from descending aorta supplying to right lung. Transcatheter embolisation of this artery prevents complications. We present a 2.5-year-old girl in which anomalous artery was embolised using Amplatzer PiccoloTM Occluder, for first time.

Investigation of the prevalence of cardiovascular risk factors in obese patients diagnosed with metabolic syndrome in childhood and examination of left ventricular function by echocardiography.

OBJECTIVES: The objective of this study is to investigate the cardiovascular risk factors associated with metabolic syndrome (MetS), which is increasingly becoming prevalent in childhood obesity. METHODS: A total of 113 patients, 76 of whom were between the ages of 10 and 17 (mean age: 14.5 ± 1.8 years) and diagnosed with obesity (30 non-MetS and 46 MetS using IDF) and 37 of whom constituted the control group, participated in the study. Echocardiographic examination and atherogenicity parameters (Atherogenic index of plasma [AIP: logTG/HDL], total cholesterol/HDL, and TG/HDL ratio and non-HDL) were evaluated. RESULTS: The most common component accompanying obese MetS was found to be hypertension and low HDL. While obesity duration, body mass index (BMI), blood pressure, fasting insulin, insulin resistance, atherogenicity parameters were determined to be significantly higher in the obese-MetS group. Echocardiography showed that while the thickness, volume, and diameter of LV end-diastolic wall, left ventricular mass (LVM), LVM index (LVMI g/m2) and relative wall thickness (RWT) were significantly high in the MetS group, however, mitral E/A ratio was significantly lower (p<0.05). Change in LV geometry consistent with concentric remodeling (increased RWT, normal LVMI) was visible in obese groups. LVM were positively significantly related to BMI, waist circumference, insulin resistance, blood pressure, LDL level, and negative to mitral E/A ratio. In the obese-MetS group, LVMI was positively correlated to office systolic BP, left atrium end-diastolic volume/index. CONCLUSIONS: LVMI and atherogenicity parameters that were found to be significantly higher in obese MetS exhibit increased cardiovascular risk in childhood.

The assessment of treatment outcomes in patients with acute viral myocarditis by speckle tracking and tissue Doppler methods.

OBJECTIVES: The aim was to evaluate the role of tissue Doppler imaging (TDI) and speckle tracking echocardiography (STE), to identify myocardial dysfunction, and to evaluate myocardial segmental deformation in acute viral myocarditis. METHODS: Twenty-one patients and twelve healthy children were studied prospectively. The TDI and STE were performed before and after treatment. The myocardial velocities (Sm , Em , and Am ) and time intervals (isovolumic contraction, isovolumic relaxation, and ejection times [ET]) at interventricular septum (IVS), left, and right ventricular basal segments were examined by TDI. The left ventricular global longitudinal strain (LVGLS) and strain rate (LVGLSR), left ventricular global circumferential strain (LVGCS) and strain rate (LVGCSR), and right ventricular global longitudinal strain (RVGLS) and strain rate (RVGLSR) were examined by STE. RESULTS: Sm and Em at IVS and at LV, ET at IVS, ET at RV, ET at LV were significantly lower in patients before treatment than controls. LVGLS, LVGLSR, LVGCS, LVGCSR, RVGLS, RVGLSR were significantly decreased in patients before treatment than controls. There was significant improvement for LVGLS, LVGLSR, LVGCS, LVGCSR, and RVGLS in patients after treatment. Sm , Em , and Am at LV were significantly lower in patients before treatment than in patients after treatment. In spite of improvements, Sm , Em , and ET at IVS, LVGLS, LVGLSR, LVGCS, LVGCSR were significantly lower in patients after treatment than controls. CONCLUSIONS: The TDI and STE were useful methods for detection of early myocardial dysfunction and evaluation of treatment outcomes in acute viral myocarditis.

Subclinical Myocardial Dysfunction Demonstrated by Speckle Tracking Echocardiography in Children with Euthyroid Hashimoto's Thyroiditis

Objective: Thyroid hormones have an important role in the regulation of the cardiovascular system. The aim of this study was to investigate the presence of subclinical myocardial dysfunction in children with euthyroid Hashimoto's thyroiditis (eHT) without evident heart disease using tissue doppler imaging (TDI) and speckle tracking echocardiography (STE) methods. Methods: TDI and STE were peformed in 50 children with eHT and in 35 healthy children. To assess myocardial velocities and time intervals, including peak systolic velocity (Sm), peak early diastolic velocity (Em), peak late diastolic velocity (Am), isovolumetric contraction time (IVCT), isovolumetric relaxation time (IVRT) and ejection time (ET), TDI was performed at the base of the interventricular septum (IVS) and in the left and right ventricles (LV and RV, respectively). Analysis of myocardial deformation by STE including strain (S) and strain rate (SR) was performed globally in two planes, longitudinal (L) and mid-circumferential (C) in LV [LV global longitudinal strain (LVGLS), LV global longitudinal strain rate (LVGLSR), LV global circumferential strain (LVGCS), LV global circumferential strain rate (LVGCSR)] and RV [(RV global longitudinal strain (RVGLS), RV global longitudinal strain rate (RVGLSR)]. Results: Among TDI parameters, ET at LV and IVS were significantly lower, IVRT and myocardial performance index at LV and IVS were significantly higher in the eHT group compared to controls (p=0.001). There were no significant differences in Sm, Em, Am and IVCT values between patients and controls. LVGLS, LVGLSR, LVGCS and LVGCSR values were significantly lower in patients than controls (p=0.01). There was a negative correlation between thyroid antibody levels and LV global longitudinal and circumferential strain and strain rate values (TPO-Ab and Tg-Ab between LVGLS, LVGLSR, LVGCS and LVGCSR; r=-411, p<0.001; r=-541, p<0.001; r=-430, p<.0.001; r=-502, r<0.01 and r=-397, p<0.001; r=-473, p<0.001; r=-519, p<0.001; r=-421, p<0.00, respectively). Conclusion: The results show that myocardial function in children with eHT is impaired in the absence of any clinical symptoms and that conventional echocardiography is inadequate to determine these changes.

Evaluation of right ventricular function in operated tetralogy of Fallot patients with tissue Doppler imaging before and after pulmonary valve replacement.

BACKGROUND: This study aims to investigate the efficacy of tissue Doppler echocardiography for the diagnosis of right ventricular dysfunction and for the evaluation of the response to pulmonary valve replacement. METHODS: Between December 2008 and December 2010, a total of 15 patients (8 males, 7 females; mean age: 14.5±4.5 years; range, 7 to 22 years) with pulmonary valve insufficiency in the follow-up of total correction of tetralogy of Fallot who underwent pulmonary valve replacement were included in the study. Echocardiographic imaging was performed before and one, three, and six months after replacement. Using pulse wave Doppler, early diastolic E and late diastolic A waves were measured in the flow velocity curve of tricuspid valve. Tissue Doppler measurements were made from the basal segment of right ventricular free wall and interventricular septum. S, e, and a myocardial velocities, isometric contraction time, isovolumetric relaxation time and ejection time were measured. Myocardial performance index was calculated for each patient. RESULTS: In pre-pulmonary valve replacement phase, s, e, a velocities and ejection time were lower in the interventricular septum compared to the basal segment of right ventricular free wall, while isometric contraction time, isovolumetric relaxation time and myocardial performance index values were higher. In the first post-replacement month, there was a significant decrease in the s value of interventricular septum, compared to pre-pulmonary valve replacement period; isometric contraction time decreased and myocardial performance index increased in the first postpulmonary valve replacement month in the free wall of right ventricle, while isovolumetric relaxation time decreased in the postoperative third month, and s velocity, isometric contraction time and myocardial performance index increased in the postoperative sixth month. Pre-pulmonary valve replacement tissue Doppler values at the septum showed that myocardial systolic and diastolic dysfunction developed earlier than the basal segment of right ventricular free wall. This result showed that right ventricular recovery was not complete in the sixth post-pulmonary valve replacement month. CONCLUSION: Tissue Doppler can show right ventricular dysfunction before replacement, but it may be insufficient to visualize right ventricular recovery until six months after treatment.

A rare cause of facial nerve palsy in a young infant: Kawasaki disease.

Orgun A, Karagöl C, Pamuk U, Gürsu HA, Çetin I. A rare cause of facial nerve palsy in a young infant: Kawasaki disease. Turk J Pediatr 2018; 60: 433-435. Kawasaki disease (KD) is a vasculitis in which the most common complication is development of coronary aneurysms. Neurological complications rarely occur in KD patients such as facial nerve palsy (FNP). FNP associated with KD may indicate increased risk of coronary artery aneurysm. Infants with facial nerve paralysis and unexplained-prolonged febrile period should be evaluated with echocardiography. Here in, we present a 4-month-old female with FNP and unexplained fever who was diagnosed KD due to echocardiographic findings.

A rare cause of fatal cardiac arrhythmia: Inhalation of butane gas.

Pamuk U, Gürsu HA, Emeksiz S, Özdemir-Sahan Y, Çetin I. A rare cause of fatal cardiac arrhythmia: Inhalation of butane gas. Turk J Pediatr 2018; 60: 755-756. Butane gas, especially available in lighters, is commonly misused among adolescents and its side effects are rarely observed but serious. A 14-yearold male was brought to our emergency department. The electrocardiographic (ECG) examination showed biphasic T waves in leads V4-V6, and long QTc at 481ms. Echocardiographic study showed left ventricular systolic dysfunction. Troponin I level was found to be high at 9.1 ng/ml. Taking into consideration the patient`s history, clinical and laboratory findings, ventricular fibrillation and myocardial injury resulting from butane gas inhalation was diagnosed.

Evaluation of Acute Rejection by Measuring Strain and Strain Rate in Children With Heart Transplant: A Preliminary Report.

OBJECTIVES: Asymptomatic rejection after heart transplant is difficult to detect by noninvasive methods. The present study investigated the efficacy of echocardiographic strain and the strain rate imaging method in detecting rejection after pediatric heart transplant. MATERIALS AND METHODS: Fourteen pediatric patients with heart transplant were examined both with endomyocardial biopsy and strain imaging. Patients were divided into 2 groups: group 1 included patients with rejection, and group 2 included patients without rejection. Patients underwent endomyocardial biopsy at regular intervals. Regional systolic function was evaluated by longitudinal myocardial peak systolic values of strain and of strain rate. Differences between the 2 groups were assessed with unpaired t test or Mann-Whitney U test. RESULTS: Acute rejection was detected in 7 patients (4 were female patients). Cardiac diagnosis was restrictive cardiomyopathy in 3 patients, dilated cardiomyopathy in 3 patients, and complex congenital heart disease in 1 patient. After heart transplant, 6 patients had rejection once and 1 patient had rejection twice. Evaluation of biopsy samples revealed grade IB rejection in all patients in group 1. Systolic functions of the 6 patients were determined as normal in standard echocardiographic examination. There were no significant differences in deformation and deformation rates between group 1 and 2 except in midseptal region (P < .05). CONCLUSIONS: One of the most significant complications in patients with heart transplant is rejection. Our results suggested that myocardial strain imaging may be valuable in defining low-grade rejection.

Analysis of right ventricle function with strain imaging before and after pulmonary valve replacement.

BACKGROUND: Pulmonary valve insufficiency may develop after surgical treatment of tetralogy of Fallot (ToF). Severe pulmonary valve insufficiency may result in right ventricular dysfunction. We aimed to compare cardiac magnetic resonance (CMR), with echocardiography. METHODS: Patients who developed severe pulmonary valve insufficiency after total correction for ToF, were included in the study. CMR was used to measure end-diastolic, end-systolic volumes and ejection fraction of the right ventricle before and 6 months after replacement, and echocar-diographic strain imaging was obtained before, and 1, 3, and 6 months after replacement. RESULTS: There were significant differences between pre- and post-replacement QRS durations, and right ventricle end-diastolic and end-systolic volumes measured with CMR (p < 0.05). However, right ventricular ejection fraction (RVEF) did not change. Therefore, CMR determined that right ventricle size and volume increased, and right ventricular function deteriorated before replacement. After replacement, no significant improvement was seen in RVEF. Lower-than-normal right ventricle strain and strain rate before replacement indicated that healthy and dysfunctional myocardium could be differentiated by this method. Pre-replacement strain and strain rate of asymptomatic and symptomatic patients were similar. Strain and strain rate values increased 6 months after replacement (p < 0.05). CONCLUSIONS: We suppose that increased experience with strain imaging, and further studies on a larger patient group with a longer follow-up period would show that this method is quite advantageous, and it will take its place in the literature as a non-invasive technique that may be used instead of magnetic resonance.

A case of neonatal arterial thrombosis mimicking interrupted aortic arch.

Neonatal arterial thrombosis is a very rare entity with clinical findings resembling coarctation of aorta or interrupted aortic arch. A two day-old male newborn was admitted to a different hospital with difficulty in sucking and sleepiness. On echocardiographic examination, a diagnosis of interrupted aortic arch was made and he was treated with prostoglandin E2. When the patient presented to our center, physical examination revealed that his feet were bilaterally cold. The pulses were not palpable and there were ecchymotic regions in the lower extremities. Echocardiography ruled out interrupted aortic arch. Computerized tomographic angiography revealed a large thrombosis and total occlusion of the abdominal aorta. Since there was no response to treatment with tissue plasminogen activator, we performed thrombectomy. Homozygous Factor V Leiden and Methylenetetrahydrofolate reductase mutations were found in this patient. Neonatal aortic thrombosis which is observed very rarely and fatal should be considered in the differential diagnosis of coarctation of aorta and interrupted aortic arch.

Oral budesonide as a therapy for protein-losing enteropathy in children after the Fontan operation.

BACKGROUND AND AIM: Protein-losing enteropathy is a rare complication of the Fontan palliation surgery. Budesonide is an effective treatment option for protein-losing enteropathy. We reviewed our retrospective experience in four patients who were treated with oral budesonide. METHODS: Four patients with refractory protein-losing enteropathy after the Fontan operation were started on oral budesonide 9 mg/daily. After achieving normal serum albumin the dose was tapered to 3 mg. Response to oral budesonide, side effects, and serum albumin levels before the treatment and at first, fourth, and ninth months of the budesonide course were recorded. Efficacy was measured based on serum albumin levels and clinical symptoms. RESULTS: Mean pretherapy albumin was 2.25 g/dL (range 1.7 to 2.5 g/dL) and nine months after therapy it was 4.15 g/dL (range 3.9 to 4.4 g/dL) (p < 0.05). All patients had at least a transient improvement in serum albumin levels and clinical findings. Systemic side effects included cushingoid features and oral moniliasis. All patients had improvement in side effects after tapering budesonide to 3 mg. The treatment was terminated in one case as soon as serum albumin level exceeded 3 g/dL. One death occurred from respiratory arrest six months after budesonide discontinuation. CONCLUSION: Budesonide can be used to treat protein-losing enteropathy in selected patients with cardiac diseases.