Murat Çiftel

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.

Dilated cardiomyopathy secondary to rickets-related hypocalcaemia: eight case reports and a review of the literature.

INTRODUCTION Dilated cardiomyopathy is usually idiopathic and may arise secondary to infections or metabolic or genetic causes. Another rare cause is hypocalcaemia. Owing to the fact that calcium plays an essential role in excitation and contraction of myocardial muscle, myocardial contractility may decline in patients with hypocalcaemia. MATERIALS AND METHODS Patients with symptoms of congestive heart failure and rickets-related hypocalcaemia were assessed clinically and by echocardiography in a paediatric cardiology clinic. Echocardiography was performed for all patients. Rickets was diagnosed according to the clinical, laboratory, and radiologic findings. Maternal lifestyle and living conditions were investigated, and the maternal 25-OH vitamin D3 blood level was measured. RESULTS We evaluated eight patients who developed heart failure as a result of severe hypocalcaemia associated with rickets between August, 1999 and June, 2012. The age distribution of the patients was 3-12 months. Laboratory results were consistent with advanced-stage rickets. Severe hypocalcaemia was detected in all patients. The maternal 25-OH vitamin D3 levels were low. Echocardiography revealed increased pre-treatment left ventricle end-systolic and end-diastolic diameters for age and reduced ejection fraction and fractional shortening. After clinical improvement, the patients were discharged. CONCLUSIONS Severe hypocalcaemia associated with rickets must always be kept in mind among the causes of dilated cardiomyopathy and impaired cardiac function in infants. If diagnosed and treated in time, dilated cardiomyopathy and severe heart failure related to rickets respond well.

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.

Evaluation of carotid intima-media thickness and carotid arterial stiffness in children with adenotonsillar hypertrophy.

BackgroundAdenotonsillar hypertrophy can produce cardiopulmonary disease in children. However, it is unclear whether adenotonsillar hypertrophy causes atherosclerosis. This study evaluated carotid intimamedia thickness and carotid arterial stiffness in children with adenotonsillar hypertrophy.MethodsThe study included 40 children with adenotonsillar hypertrophy (age: 5-10 years) and 36 healthy children with similar age and body mass index. Systolic blood pressure, diastolic blood pressure, and pulse pressure were measured in all subjects. Carotid intima-media thickness, carotid arterial systolic diameter, and carotid arterial diastolic diameter were measured using a high-resolution ultrasound device. Based on these measurements, carotid arterial strain, carotid artery distensibility, beta stiffness index, and elasticity modulus were calculated.ResultsCarotid intima-media thickness was greater in children with adenotonsillar hypertrophy (0.36±0.05 mm vs. 0.34±0.04 mm, P=0.02) compared to healthy controls. Beta stiffness index (3.01±1.22 vs. 2.98±0.98, P=0.85), elasticity modulus (231.39±99.23 vs. 226.46±83.20, P=0.88), carotid arterial strain (0.17±0.06 vs. 0.17±0.04, P=0.95), and carotid artery distensibility (13.14±3.88 vs. 12.92±3.84, P=0.75) were similar between children with adenotonsillar hypertrophy and the healthy controls.ConclusionsThe present study revealed increased carotid intima-media thickness in children with adenotonsillar hypertrophy. The risk of subclinical atherosclerosis may be higher in children with adenotonsillar hypertrophy.

Rapid response to treatment of heart failure resulting from hypocalcemic cardiomyopathy.

Hypocalcemia is a rare condition that causes dilated cardiomyopathy and can result in heart failure. Patients with hypocalcemia have been reported to recover in 3 to 12 months after calcium and vitamin D replacement therapy as well as treatment of heart failure. A 6-month-old male patient who presented with dyspnea was admitted to the intensive care unit with severe heart failure and dilated cardiomyopathy. Blood biochemistry revealed hypocalcemia and vitamin D deficiency. After administration of anticongestive treatment, positive inotropic support, as well as vitamin D and calcium supplementation, cardiac function returned to normal in a week. Our case is the first report of such a rapid improvement in cardiac morphology and function in a patient with hypocalcemic dilated cardiomyopathy and heart failure.

Assessment of heart rate variability in breath holding children by 24 hour Holter monitoring.

PURPOSE Previous studies have shown that the underlying pathophysiologic mechanism in children with breath holding may be generalised autonomic dysregulation. Thus, we performed cardiac rhythm and heart rate variability analyses using 24-hour Holter monitoring to evaluate the cardiac effects of autonomic dysregulation in children with breath-holding spells. METHODS We performed cardiac rhythm and heart rate analyses using 24-hour Holter monitors to evaluate the cardiac effects of autonomic dysregulation in children during a breath-holding spell. Our study group consisted of 68 children with breath-holding spells - 56 cyanotic type and 12 pallid type - and 39 healthy controls. RESULTS Clinical and heart rate variability results were compared between each spell type - cyanotic or pallid - and the control group; significant differences (p<0.05) in standard deviation of all NN intervals, mean of the standard deviations of all NN intervals for all 5-minute segments, percentage of differences between adjacent RR intervals >50 ms, and square root of the mean of the sum of squares of the differences between adjacent NN intervals values were found between the pallid and cyanotic groups. CONCLUSIONS Holter monitoring for 24 hours and heart rate variability parameters, particularly in children with pallid spells, are crucial for evaluation of cardiac rhythm changes.

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.

Penicillin Therapy in Children with Acute Rheumatic Fever: Side Effects, Malpractice and Anaphylactic Reactions

Akut romatizmal ates ARA gelismekte olan ulkelerde daha siktir. ARA sistemik bir hastalik olup cocuklarda grup A streptokok GAS farenjitinden sonra gelisir. Romatizmal kapak hastaligi otoimmun cevap sonucu olusur. Penisilin GAS ile olusan tonsillofarenjitin tedavisinde, ARA’nin primer ve sekonder proflaksisinde kullanilmaktadir. Primer proflakside tek doz intramuskuler IM benzatin penisilin veya oral penisilin V fenoksimetilpenisilin 10 gun sure ile verilir. Romatizmal kapak hastaligi mevcut ise en az 40 yasina kadar veya yasam boyu benzatin penisilin ile sekonder proflaksi uygulamak gerekir. Penisilin IM enjeksiyonu cocuklarda korkuya ve agriya neden olabilir. IM enjeksiyon sirasinda agriyi azaltmak icin lidokain veya lidokain-prilokain EMLA krem kullanilabilir. Penisilinin en ciddi yan etkisi anaflaktik reaksiyondur. Anaflaktik reaksiyon penisilinin major veya minor determinanti ile olusabilir. Penisiline bagli anaflaksiyi onlemek icin penisilin alerjisini iyi sorgulamak ve deri testini uygun olarak yapmak ve yorumlamak gerekir. Penisilin alerji suphesi varsa deri testi yapmadan hastalarin pediatrik alerji tarafindan major ve minor determinant kullanilarak yapilan testler ile degerlendirilmesi ve penisilin alerjisinin aydinlatilmasi gerekir. Penisilin allerjisi olanlarda alternatif ilac yoklugunda desensitizasyon yapilabilir. Penisilin uygulanirken deri testinin uygun sekilde yapilmasi ve yorumlanmasi, olasi anaflaksi reaksiyona mudahale icin gerekli malzemenin ve ilaclarin hazirda bulundurulmasi ve anaflaksiye uygun mudahalenin yapilmasi tibbi ve yasal acidan onemlidir. Penisilin pediatri ve pediatrik kardiyolojide sik kullanilan bir ilactir

Congenital Subclavian Steal Syndrome Associated With Heterotaxy Syndrome and Atrioventricular Septal Defect

An infant under follow-up evaluation since the fetal stage was detected to have subclavian steal syndrome (SSS) associated with heterotaxy syndrome and atrioventricular septal defect. Such a complex association has not been reported previously. Congenital SSS occurs as a result of a subclavian artery originating from the main pulmonary artery through the ductus instead of the aortic arch. In rare cases, as in the reported case, it may occur due to proximal segment atresia of the left subclavian artery.