A Rare Treatable Cause of Cardiomyopathy: Primary Carnitine Deficiency.

Introduction: Primary carnitine deficiency (PCD) is a rare autosomal recessive disorder caused by loss of function mutations in the solute carrier family 22 member 5 (SLC22A5) gene that encodes a high-affinity sodium-ion-dependent organic cation transporter protein (OCTN2). Carnitine deficiency can result in acute metabolic decompensation or, in a more insidious presentation, cardiomyopathy. Cardiomyopathy associated with PCD often presents with life-threatening heart failure. This presentation also usually includes skeletal muscle myopathy. Early recognition of this disorder and treatment with carnitine can avoid life-threatening complications related to cardiomyopathy. Case Presentation: Herein, we present a 10-month-old male patient with PCD, which was diagnosed while investigating the etiology of dilated cardiomyopathy and confirmed by molecular genetic analysis. Conclusion: Homozygous c.254_265 insGGCTCGCCACC (p.I89Gfs) pathogenic variant of the SLC22A5 gene was detected. With oral L-carnitine supplementation, the free carnitine level increased up to 14 µmol/L and the symptoms disappeared. LVEF increased by 45-70%. We would like to emphasize that this problem is a combination of the metabolic decompensation and the cardiac phenotypes, which are usually separated to either phenotype.

The Effects of Vitamin D on Myocardial Function Demonstrated by Speckle-Tracking Echocardiography in Children with Beta Thalassemia.

OBJECTIVE: Beta thalassemia major is an inherited hemoglobin disorder resulting in chronic hemolytic anemia. Cardiac involvement is the main cause of death in patients. Speckle-tracking echocardiography is a feasible method for the evaluation of cardiac function via an assessment of the longitudinal deformation of the myocardium through the cardiac cycle. The aim of our study is to evaluate the association between vitamin D deficiency and deformation of the left ventricular myocardium measured by speckle-tracking echocardiography in children with thalassemia major. METHODS: In this prospective study, 33 thalassemic patients with vitamin D deficiency were enrolled. Cardiac magnetic resonance T2* value, conventional echocardiography, and speckle tracking, and also left ventricular longitudinal and circumferential strain values were measured. Myocardial functions of the patients with vitamin D deficiency or insufficiency were evaluated by speckle-tracking echocardiography before and after vitamin D replacement. RESULTS: The mean age of the patients was 15.4 ± 3.09 years. Vitamin D level was deficient in 30 (90%) and insufficient in 3 (10%) of them. Speckle-tracking analysis showed a significantly decreased absolute value of the left ventricular global longitudinal strain before vitamin D replacement. A significant improvement in the global longitudinal strain was detected after vitamin D replacement (P < 0.05). A statistically significant increase was observed in parameters showing left ventricular systolic and diastolic functions after vitamin D replacement. CONCLUSION: Vitamin D deficiency is frequently observed and causes decreased contractility in thalassemic patients. In our study, we observed that our patients' cardiac functions had improved after vitamin D replacement therapy.

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.

The Relationship Between Hypothyroidism and Cardiac Findings in Children With and Without Down Syndrome.

OBJECTIVE: Down syndrome is a genetic syndrome characterized with various dysmorphisms and congenital malformations such as congenital heart diseases. We aimed to evaluate the relationship between Down syndrome, hypothyroidism, and cardiac ���ndings. METHODS: Thyroid hormone pro���les and echocardiographic ���ndings were evaluated. Patients with hypothyroidism and Down syndrome were named group 1; patients with hypothyroidism without Down syndrome group 2 and group 3 was control. The echocardiographic parameters (interventricular septum and left ventricular systolic, diastolic posterior wall thickness, left ventricular end-diastolic diameter, ejection fraction) were indexed to body surface area. Left ventricular mass index and relative wall thickness were calculated. Patients with relative wall thickness equal to or below 0.42 were classi���ed as eccentric hypertrophy or normal geometry, while those over 0.42 as concentric remodeling or concentric hypertrophy. RESULTS: Thyroid stimulating hormone values of groups 1 and 2 were signi���cantly higher than those of group 3. There were no signi���cant di���erences for fT4 between the groups. Interventricular septum and left ventricular posterior wall end-diastolic and end-systolic thickness were signi���cantly higher in group 1 than groups 2 and 3. There was no statistically signi���cant di���erence in left ventricular mass index between groups 1 and 2. In terms of relative wall thickness, 16 out of 29 patients in group 1 were revealed as concentric remodeling, 12 as normal geometry, 1 patient as eccentric hypertrophy. In group 2, 6 patients were revealed as concentric remodeling, 14 as normal geometry. There was no statistically signi���cant di���erence of left ventricular end-diastolic thickness between 3 groups. CONCLUSION: Cardiac morphology and functions were signi���cantly a���ected by hypothyroidism in patients with Down syndrome. Hypertrophy in Down syndrome may be caused by the cellular changes in myocardium.

Can Right Infra-Axillary Vertical Thoracotomy Make a Big Difference in Surgical Technique Preference?

AIM: Minimally invasive techniques in the surgical treatment of congenital heart diseases have gained popularity recently. Right infra-axillary vertical thoracotomy (RIAVT) is one of these techniques. The aim of this study is to investigate the postoperative morbidity and cosmetic results of the right infraaxillary thoracotomy technique and compare them with the results in the literature. METHOD: All patients who had undergone cardiac surgery using RIAVT at the Ankara City Hospital Pediatric Cardiovascular Surgery Clinic between 2019 and 2021 were enrolled in the study. Preoperative echocardiographic diagnoses, intraoperative surgical data and postoperative morbidity data of the patients was obtained. In addition, surgical incision length was measured and recorded for the evaluation of cosmetic outcome. This data was then compared with data from similar studies in the literature. RESULT: Between January 2019 and December 2021, 59 patients were operated on using RIAVT. Of the RIAVT patients, 45 (76%) were female and 14 (24%) were male. The mean weight of the patients was 22.38±12.48 kg. Although the youngest patient was a 5-month-old infant weighing 4 kg, the oldest one was 15-years-old weighing 57 kg. Isolated secundum atrial septal defect (ASD) repair (primary or patch) was performed in 47 patients. One (1) patient with Primum ASD and mitral cleft, 1 patient with secundum ASD and perimembranous ventricular septal defect (VSD) repair, nine patients with high venosum ASD and PAPVC, and one patient with a discrete subaortic membrane were operated on. The surgical incision length was between 4 to 5 cm. CONCLUSION: RIAVT is an important surgical alternative to median sternotomy in selected cases with certain pathologies. It can be performed safely in various congenital heart anomalies and provides excellent functional and cosmetic results in the long-term when compared to other surgical methods.

Incidental detection of accessory mitral valve in adolescent with atrial septal defect: Diagnosis with 3D transesophageal echocardiography and excision with transaortic method.

BACKGROUND: Accessory mitral valve tissue (AMVT) is an extremely rare causes left ventricular outflow tract (LVOT) obstruction and is usually incidentally detected in childhood. It is often associated with other cardiac and vascular congenital malformations. CASE PRESENTATION: In this case, we present a 15-year-old girl was diagnosed with AMVT by transesophageal echocardiography, resulting in LVOT obstruction during systole. Interestingly enough, the patient's accessory mitral valve remained undetected for years until he became symptomatic for wide ASD. Successful closure of the ASD with resection of the AMVT was performed with a transaortic approach. The patient was hemodynamically stable postoperatively. There were no abnormalities in the mitral valves and LVOT. CONCLUSION: It was also unusual to see AMVT with ASD instead of other frequently associated other congenital anomalies. Accessory mitral valve should be considered a rare but important cause of left ventricular outflow tract obstruction in childhood.

Efficacy of flecainide in bidirectional ventricular tachycardia and tachycardia-induced cardiomyopathy with Andersen-Tawil syndrome.

Andersen-Tawil syndrome is a rare autosomal dominant genetic or sporadic disorder characterized by periodic paralysis, ventricular arrhythmias and dysmorphic features. Ventricular arrhythmias can include frequent premature ventricular complex, polymorphic ventricular tachycardia, and less frequently bidirectional ventricular tachycardia. Left ventricle function has been reported in only a few individual cases of Andersen-Tawil syndrome. A 14-year-old female patient was referred to our clinic from another center with documented arrhythmia and left ventricular systolic dysfunction. Andersen-Tawil syndrome was suspected and the diagnosis was confirmed after detection of a previously unreported mutation in children. We report the successful use of flecainide in bidirectional ventricular tachycardia and tachycardia-induced cardiomyopathy in a case of Andersen-Tawil syndrome associated with a novel mutation.

Low serum 25-Hydroxy (OH) vitamin D levels are associated with increased arterial stiffness in healthy children: An echocardiographic study from Turkey.

OBJECTIVE: Arterial stiffness refers to arterial wall rigidity, particularly in central vessels, and is an independent predictor of cardiovascular disease in many chronic diseases. 25-Hydroxy (OH) vitamin D has beneficial effects on blood pressure, vascular endothelial function, and arterial stiffness; most importantly, its deficiency is common worldwide. Therefore, we aimed to elucidate the role of 25-OH vitamin D deficiency in arterial stiffness development and its relationship with arterial stiffness in healthy children. METHODS: This study included 80 patients with low levels of 25-OH vitamin D and 40 healthy control subjects. The study participants were then divided into three groups: group 1 consisted of patients with a deficient 25-OH D level of < 19.9 ng/ml, group 2 with an insufficient 25-OH D level between 20 and 29.9 ng/ml; group 3 were considered control group with a sufficient serum 25-OH vitamin D level of ≥30 ng/ml. Aortic strain, distensibility, stiffness index, and standard left ventricular measurements were calculated using M-mode echocardiographic data. RESULTS: Left ventricular mass index (LVMI) and inter-ventricular septal diastolic thickness (IVST) appeared to increase in group 1 compared to groups 2 and 3. Aortic strain and distensibility were significantly decreased in group 1, whereas aortic stiffness index and elastic modulus were significantly increased. The aortic stiffness index was negatively correlated with serum 25-OH vitamin D levels; however, aortic strain, aortic distensibility, and LVMI were positively correlated. CONCLUSIONS: Our study results revealed a significant relationship between 25-OH vitamin D levels indicative of a deficiency and aortic stiffness. Hence, we suggest that arterial stiffness may also occur in healthy children with a 25-OH vitamin D deficiency. Future in-depth studies are needed to understand the exact mechanisms underlying the aortic stiffness development associated with 25-OH vitamin D deficiency.

Premature cardiac beats in children with structurally normal heart: autonomic dysregulation.

BACKGROUND: Premature ventricular contractions and supraventricular contractions are common rhythm disorders requiring comprehensive investigation in children. The aim of the study was to evaluate the heart rate variability (HRV) in premature ventricular contractions (PVCs) and supraventricular contractions (PSVCs) in children. METHODS: The study compared the characteristics of HRV in 175 children with PVCs and 160 children with PSVCs who underwent 24-h Holter monitoring, with 101 healthy children. RESULTS: Significant differences were found between standard deviation of all normal RR intervals (SDNN), standard deviation of average RR intervals in all 5 min segments of registration (SDANN), root mean square of the successive differences (rMSSD), and the proportion of NN50 divided by total number of NNs (pNN50) values of the patient and control groups. The PVCs group had a significantly lower high frequency (HF) and higher low frequency (LF)/HF ratio and the PSVCs group had a significantly higher LF and higher LF/HF ratio compared to the control group. No significant correlation existed between frequency and the SDNN index, rMSSD, and pNN50 values of the PVCs group. The receiver operating characteristics analysis showed a significantly changed LF/HF ratio when premature contractions exceeded 60 beats per hour in children with PVCs. CONCLUSIONS: This study indicated an increased sympathetic tone and a significantly decreased vagal tone in children with PVCs and PSVCs. HRV can be used increasingly as a non-invasive method in the follow-up of children with premature cardiac contractions.

A rare association of left pulmonary artery sling with Scimitar syndrome: Recurrent wheezing and respiratory distress in a pediatric patient.

Congenital anomalies of the heart and great vessels may lead to localized recurrent pulmonary infections through different mechanisms. Pulmonary artery sling (left pulmonary artery originating from the right pulmonary artery) and Scimitar syndrome are rare causes of wheezing in infants. An 18-month-old female infant with left pulmonary artery sling, Scimitar syndrome, and an anomalous connection of left pulmonary veins to the left atrium was admitted to our clinic. She successfully underwent transcatheter embolization using the Vascular Plug-II on the anomalous systemic arterial supply and repair of pulmonary arterial sling and Scimitar anomaly via a median sternotomy. In conclusion, diagnosis of left pulmonary arterial sling accompanied by abnormalities can be missed in some cases using echocardiography. It may be necessary to conduct more advanced imaging methods before deciding the treatment method to be performed in these patients.

A case of infective endocarditis due to Herbaspirillum Huttiense in a pediatric oncology patient.

Infective endocarditis (IE) is an infection of the endocardium and/or heart valves that involves thrombus formation (vegetation). This condition might damage the endocardial tissue and/or valves. An indwelling central venous catheter is a major risk factor for bacteremia at-risked pediatric populations such as premature infants; children with cancer and/or connective tissue disorders. Herbaspirillum huttiense is a Gram-negative opportunistic bacillus that may cause bacteremia and pneumonia rarely in this fragile population. Herein we report the very first case of bacteremia and IE in a pediatric oncology patient caused by H. huttiense.

Miocarditis aguda tras una infección por el virus de la varicela-zóster en un adolescente inmunocompetente: una complicación poco frecuente / Acute myocarditis following varicella zoster infection in an immunocompetent adolescent: An uncommon complication

La miocarditis es una complicación grave de la infección por el virus de la varicela-zóster. Un varón de 15 años ingresó a la sala de emergencias debido a dolor torácico, taquicardia e hipotensión. En el electrocardiograma se observó taquicardia sinusal. Los biomarcadores cardíacos estaban elevados. En el ecocardiograma se notó hipocinesia apical, septal, e inferolateral del ventrículo izquierdo e insuficiencia mitral. Los anticuerpos IgM en suero para el virus de la varicela-zóster eran positivos. El paciente recibió el alta sin secuelas.

Myocarditis is a serious complication of varicella zoster virus infection. A 15 year-old boy was admitted to the Emergency Department for chest pain, tachycardia and hypotension. An electrocardiogram showed sinus tachyicardia. Cardiac biomarkers were elevated and echocardiography revealed left ventricular apical, inferolateral, septal hypokinesis, and mitral regurgitation. Varicella zoster virus serum immunoglobulin M antibody was positive. The patient was discharged without any sequelae.

Acute myocarditis following varicella zoster infection in an immunocompetent adolescent: An uncommon complication. / Miocarditis aguda tras una infección por el virus de la varicela-zóster en un adolescente inmunocompetente: una complicación poco frecuente.

Myocarditis is a serious complication of varicella zoster virus infection. A 15 year-old boy was admitted to the Emergency Department for chest pain, tachycardia and hypotension. An electrocardiogram showed sinus tachyicardia. Cardiac biomarkers were elevated and echocardiography revealed left ventricular apical, inferolateral, septal hypokinesis, and mitral regurgitation. Varicella zoster virus serum immunoglobulin M antibody was positive. The patient was discharged without any sequelae.

La miocarditis es una complicación grave de la infección por el virus de la varicela-zóster. Un varón de 15 años ingresó a la sala de emergencias debido a dolor torácico, taquicardia e hipotensión. En el electrocardiograma se observó taquicardia sinusal. Los biomarcadores cardíacos estaban elevados. En el ecocardiograma se notó hipocinesia apical, septal, e inferolateral del ventrículo izquierdo e insuficiencia mitral. Los anticuerpos IgM en suero para el virus de la varicela-zóster eran positivos. El paciente recibió el alta sin secuelas.

Catecholamine-induced Myocarditis in a Child with Pheochromocytoma

Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors. The clinical presentation of pediatric PPGLs is highly variable. In cases with pheochromocytoma (PCC), excess catecholamine may stimulate myocytes and cause structural changes, leading to life-threatening complications ranging from stress cardiomyopathy (CM) to dilated CM. Herein, we report the case of catecholamine-induced myocarditis in a child with asymptomatic PCC. A 12-year-and-2-month-old male patient with a known diagnosis of type-1 neurofibromatosis was brought to the emergency department due to palpitations and vomiting. On physical examination, arterial blood pressure was 113/81 mmHg, pulse was 125/min, and body temperature was 36.5 °C. Laboratory tests showed a leucocyte count of 12.8x103 µL/L and a serum C-reactive protein level of 1.1 mg/dL (Normal range: 0-0.5). Thyroid function tests were normal, while cardiac enzymes were elevated. Electrocardiogram revealed no pathological findings other than sinus tachycardia. The patient was diagnosed with and treated for myocarditis as echocardiography revealed a left ventricular ejection fraction of 48%. Viral and bacterial agents that may cause myocarditis were excluded via serological tests and blood cultures. Blood pressure, normal at the time of admission, was elevated (140/90 mmHg) on the 5th day of hospitalization. Magnetic resonance imaging revealed a 41x46x45 mm solid adrenal mass. The diagnosis of PCC was confirmed by elevated urinary and plasma metanephrines. The patient underwent surgery. Histopathology of the excised mass was compatible with PCC. It should be kept in mind that, even if there are no signs and symptoms of catecholamine elevation, CM may be the first sign of PCC.

[Cardiovascular findings and effects of enzyme replacement therapy in patients with mucopolysaccharidosis type VI]. / Mukopolisakkaridoz tip VI'li çocuklarda kardiyovasküler bulgular ve enzim replasman tedavisinin etkisi.

OBJECTIVE: The mucopolysaccharidoses (MPS) are an important group of lysosomal storage diseases. Commonly reported cardiac involvement includes mitral leaflet thickening and accompanying prolapsus, regurgitation, and rarely, stenosis. The aim of this study was to evaluate cardiac involvement in patients with MPS type VI. METHODS: The study included a total of 13 children with MPS type VI who were admitted to a single pediatric department between 2016 and 2018. Cardiac status was evaluated prospectively with clinical findings, electrocardiography, and echocardiography. The age of the patients (8 boys, 5 girls) ranged between 2 and 14 years (median: 9 years). RESULTS: No arrhythmia was observed in any patient. Thickening of the mitral valve with or without regurgitation and prolapsus was the most common lesion seen. Additional involvement of the aortic valve was detected in 12 (92.3%) patients, and additional involvement of the tricuspid valve in 4 (30.8%). Isolated septal hypertrophy was found in 2 patients, and congestive heart failure in another. CONCLUSION: Cardiac involvement is frequent in MPS. Mitral valve deformation is the most frequent finding. An echocardiographic examination should be performed periodically even if the patient has no clinical signs of cardiac disease, and any cardiac involvement should be kept under control with medical treatment.

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.

Recovery of myocardial mechanics in Kawasaki disease demonstrated by speckle tracking and tissue Doppler methods.

AIM: To investigate myocardial deformation and function during treatment for Kawasaki disease (KD) in children. METHODS: We performed speckle tracking echocardiography (STE) and tissue Doppler imaging (TDI) in 15 children with KD and 15 healthy children during treatment for KD. STE was performed for longitudinal and circumferential strain (S) and strain rate (SR) at the left ventricle (LV) and for longitudinal S and SR at the right ventricle (RV). TDI was performed at the base of interventricular septum (IVS), LV, and RV. RESULTS: Among TDI parameters, Em and ejection time (ET) at IVS, ET at LV and ET at RV obtained obtained before treatment were significantly lower in patients with KD compared to controls. After treatment, in spite of improvements, ET at IVS and ET at RV remained significantly lower in patients with KD compared to controls. Left ventricular global longitudinal and circumferential S and SR values obtained before treatment were significantly lower in patients with KD compared to controls. Left ventricular S and SR values were found to be increased after treatment. However, left ventricular global circumferential S value remained significantly lower in patients with KD compared to controls. There were no significant differences in right ventricular global longitudinal S and SR values between patients and controls before treatment. CONCLUSION: During acute phase, patients with KD have reduced global left ventricular S and SR which may be more sensitive indicators of myocardial inflammation. This study showed gradual improvements in left ventricular myocardial function during treatment for KD.

[A rare cause of 2:1 atrioventricular block and congestive heart failure in preterm infants: Hypocalcemia]. / Prematüre yenidoganda 2:1 atriyoventriküler blok ve konjestif kalp yetersizliginin nadir bir nedeni: Hipokalsemi.

Atrioventricular (AV) block in the neonatal period is a rare disorder. It is frequently associated with underlying structural congenital heart disease and maternal lupus. Presently described is premature baby who developed 2:1 AV block and congestive heart failure due to hypocalcemia. Dramatic clinical improvement was observed following treatment of intravenous 10% calcium gluconate. Therefore, it is suggested that serum calcium level of newborns with AV block and congestive heart failure be measured.