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Sabaz MN, Akin A, Bilici M, Demir F, Ture M, Balik H. Factors affecting mortality in children with dilated cardiomyopathy. Turk J Pediatr 2019; 61: 485-492. Dilated cardiomyopathy (DCMP) is a heart disease with high mortality rates that is often seen in children. Genetic and infectious reasons are primary in the etiology. The aim of this study was to investigate the etiology of DCMP and the parameters predicting mortality. A retrospective examination was made of 37 patients diagnosed with DCMP between January 2012 and October 2016. Data were recorded from the patient files of age, gender, complaints on presentation, findings of the physical examination, laboratory test results, echocardiography and electrocardiography findings at the time of diagnosis. These parameters were then compared between the surviving and nonsurviving patients. The patients comprised 21 males with a mean age of 27.50±50 months. Diagnosis was made at the age of < 12 months in 67.6% patients. Within mean 8 months of diagnosis, 16.2% of the patients were lost to mortality and 83.8% of the patients survived. In 83.3% of the non-surviving patients and in 29% of the surviving patients, sinus tachycardia was present at the time of diagnosis (p=0.023). Corrected QT (QTc) at the time of diagnosis was longer in the non-surviving patients (p=0.007). On ECG, the rate of ST-T wave change was higher in the non-surviving patients (80% vs. 17.8%, p=0.012). In conclusion, a significant proportion of the patients were diagnosed below the age of one year. In the non-surviving patients, as sinus tachycardia and ischaemic changes on ECG were seen more often and the QTc was longer, these findings could be considered to be predictors of mortality.

Bilici M, Fidanci-Dedeoglu Z, Demir F, Akin A, Ture M, Balik H, Tan I, Ertugrul S. Prolonged QT dispersion is associated with pediatric syncope. Turk J Pediatr 2019; 61: 85-91. We aimed to find out whether QT dispersion can be used as a diagnostic marker in terms of syncope type, and recurrence risk. Fifty-two patients (28 male, 53.8%) admitted to the pediatric cardiology clinic with syncope were compared with 50 healthy controls (28 male, 56%; mean age: 13.8±2.3 years, range: 11-17 years) for QT dispersion (QTd) and other electrocardiographic findings. Gender, age, echocardiography, blood pressure while standing and sitting, electrolyte levels, liver and kidney function tests, and QTd and QTcd (calculated with corrected QT: QTc) in electrocardiography (ECG) of patients were all evaluated. Patients` ages varied between 7-17 years and the mean age at first syncope was 13.9±2.4 years. Mean follow-up duration of the patients was 10±5 months (range: 5-18 months). Mean number of syncope attacks was 2.8±2.2. QTd (72±46 ms vs. 34±14 ms) and QTcd (77±45 ms vs. 33±14 ms) values of the patients were significantly longer, compared to control group (p < 0.001). The age, gender, QTd, and QTcd values did not differ between the subtypes of syncope based on pathogenetic mechanism. Additionally, these parameters did not differ in terms of the number of syncope recurrence and tilt test. Patients` complaints reduced after cardiologic evaluation and advice regarding prevention of syncope. We think that in follow-up period, education and preventive precautions that can be taken in the daily life must be emphasized, and drug treatment can be started in unresponsive cases.

Aim Over the past 2 decades, transcatheter occlusion of patent ductus arteriosus (PDA) with coils and the duct occluders evolved to be the procedure of choice. A new device, the Occlutech PDA® occluder (ODO) device has been designed. Herein, we aimed to evaluate the characteristics and short-term results of patients who underwent transcatheter closure of PDA using the ODO. Methods We reviewed the clinical records of 60 patients from different centers in Turkey between December 2013 and January 2016. The medical records were reviewed for demographic characteristics and echocardiographic findings. Device size was selected on the narrowest diameter of PDA. Results The median patient age was 2.5 years (6 months–35 years), and median PDA diameter was 2.5 mm (1.2–11 mm). Fifty-eight of 60 patients (96.6%) had successful ODO implantation. The occlusion rates were 37/58 (63.7%) at the end of the procedure, 51/58 (87.9%) at 24–48 hours post-procedure, and 57/58 (98.2%) on echocardiography at a median follow-up of 7.6 months. Conclusion Our results indicate that transcatheter closure of PDA using the ODO is effective. Larger studies and longer follow-up are required to assess whether its shape and longer length make it superior to other duct occluders in large, tubular, or window-type ducts. (J Interven Cardiol 2016;29:325–331)

Kawasaki disease (KD) is a systemic febrile condition leading to the inflammation of vessels including coronary arteries (1). Coronary artery aneurysms, occuring in approximately 15–25% of the patients, are the most important and life-threatening complication of KD (2). KD mainly affects children younger than five years old. Although it has been known for years, the etiology of the disease remains largely unexplained (3,4). Due to the lack of specific tools, the diagnosis of KD is still based on clinical features. At least four of the following five clinical criteria together with fever lasting equal to or longer than five days should be present for the diagnosis: bilateral non-purulent conjunctivitis; extremity findings including erythema and/or indurative edema on the hands and feet, and membranous desquamation starting in the periungual regions; changes in the lips and oral cavity; polymorphous exanthema; and cervical lymphadenopathy (usually unilateral with a diameter of 1.5 cm or more). However, those patients with prolonged fever (≥5 days) together with two or three clinical criteria may be diagnosed as incomplete KD in the presence of some supplemental laboratory findings (5). It has been reported increasingly in recent years, especially among young infants and children. Because incomplete KD also results in coronary artery lesions (CALs), it should be diagnosed promptly (5,6). However, the diagnosis is still difficult and further laboratory studies are needed. Neutrophil to lymphocyte ratio (NLR) is a novel parameter, studied in the conditions with inflammatory pathogenesis including ischemic heart disease, stroke, and infections (7–9). It was reported to be a strong predictor of adverse cardiovascular outcome and severity of coronary artery ectasia in adult population (10–12). In this study, the usefulness of NLR in the diagnosis of KD or incomplete KD and the correlation between NLR and the presence of coronary artery lesions were evaluated. The usefulness of NLR has not been studied in patients with KD so far.

Although influenza is an acute and uncomplicated disease, that limits itself in the healthy children, it may lead to death by rarely forming the sickness. The most common complication of influenza is pneumonia and it is a rare complication which is developed together with myocarditis by influenza A and B viruses. A 32 months-old male patient was admitted for rapidly developed respiratory distress and tachycardia after fever, cough, vomiting, malaise and runny nose. His general status was medium, he had conscious and had hepatomegaly, tachycardia, dyspnea, tachypnea, intercostal-subcostal retractions and bilateral rhonchus. Cardiac enzyme levels and other laboratory parameters were found normal. Myocarditis and ejection fraction was determined as 42% in echocardiography. However, hospitalization hours between 24 and 48, the patient, whose significant respiratory compromise developed, was intubated and fastened to a mechanical ventilator. H1N1 is produce in nasopharyngeal swab culture at the sixth day of follow-up. Because we think H1N1 virus was responsible from current myocarditis, oseltamivir treatment was initiated. In the fourth day of the treatment the patient’s fever returned to normal, in the ninth day a dramatic recovery was observed. In tracking echocardiography, a significant improvement was observed in the ejection fraction and myocarditis picture compared with admission time. This case was presented in order to remind that in a patients, who present with influenza findings but have respiratory distress and tachycardia in addition to lower respiratory tract infection, myocarditis should also be considered in the differential diagnosis and to remind that promising results could be obtained with the early diagnosis and treatment.

Pediatric dysrhythmias are rare but important causes of admission to pediatric clinics and emergency departments. Due to the development of successful surgical treatment of congenital heart diseases and improvements in the diagnostic tools, pediatric dysrhythmias are more frequently diagnosed. Although pediatric dysrhythmias are may be asymptomatic, they may manifest with weakness, dizziness, decrease in the effort capacity, easy fatigability, irregularity in heartbeats, palpitations, syncope and cardiac arrest. Since dysrhythmias may give rise to significant hemodynamic outcomes, their recognition by pediatricians and family physicians is vital for the patients. This review aims to contribute to the correct diagnosis and management of the cases with frequently encountered pediatric dysrhythmias.

Karadeniz, Cem/0000-0003-0529-2391; Guvenc, Tolga/0000-0003-1468-3415 WOS: 000340836500006 PubMed: 25098616 Cardiac contusion is a potentially fatal complication of blunt chest trauma. The effects of a combination of quercetin and methylprednisolone against trauma-induced cardiac contusion were studied. Thirty-five female Sprague-Dawley rats were divided into five groups (n = 7) as follows: sham, cardiac contusion with no therapy, treated with methylprednisolone (30 mg/kg on the first day, and 3 mg/kg on the following days), treated with quercetin (50 mg.kg(-1).day(-1)), and treated with a combination of methylprednisolone and quercetin. Serum troponin I (Tn-I) and tumor necrosis factor-alpha (TNF-alpha) levels and cardiac histopathological findings were evaluated. Tn-I and TNF-alpha levels were elevated after contusion (P = 0.001 and P = 0.001). Seven days later, Tn-I and TNF-alpha levels decreased in the rats treated with methylprednisolone, quercetin, and the combination of methylprednisolone and quercetin compared to the rats without therapy, but a statistical significance was found only with the combination therapy (P = 0.001 and P = 0.011, respectively). Histopathological degeneration and necrosis scores were statistically lower in the methylprednisolone and quercetin combination group compared to the group treated only with methylprednisolone (P = 0.017 and P = 0.007, respectively). However, only degeneration scores were lower in the combination therapy group compared to the group treated only with quercetin (P = 0.017). Inducible nitric oxide synthase positivity scores were decreased in all treatment groups compared to the untreated groups (P = 0.097, P = 0.026, and P = 0.004, respectively). We conclude that a combination of quercetin and methylprednisolone can be used for the specific treatment of cardiac contusion.

We present a rare case of spontaneous closure of a fistula between the left coronary artery and the right ventricle (RV) within a few days of newborn period. A 14-day-old male newborn was referred to our clinic for investigation of tachypnea and cardiac murmur. A color flow Doppler echocardiography revealed turbulent flow of a large coronary artery fistula (CAF) between the left coronary artery and the RV. Tachypnea was regressed and repeat echocardiogram showed no CAF related to cardiac chambers after 4 days of admission. The spontaneous closure of CAF was found to be more likely in cases younger than 2 years with small-sized fistulas opening into the right-sided structures, especially into the RV. Nevertheless, the spontaneous closure is very rare in cases with significant shunt. To the best of our knowledge, this is the first case with documented spontaneous closure of CAF just within the newborn period.

Objective: To investigate the effects of carbamazepinetherapy on thyroid function tests in childrenMethods: The carbamazepine group consisted of 58 childrenunder observation for epilepsy, and the control groupof 54 healthy children. Age of onset, length of drug use,drug dosage and laboratory parameters including free triiodothyronine(FT3), free thyroxin (FT4) and thyrotropin(TSH) were recorded. These data were then comparedagainst those from the control group.Results: In the carbamazepine group, FT3 was 3.86 ±0.43 pg/mL, FT4 was 1.15 ± 0.18 mg/dL and TSH: 2.58 ±1.33 ml U/L. In the control group, FT3 was 4.13 ± 0.59 pg/mL, FT4 1.34 ± 0.13 mg/dL and TSH was 2.06 ± 0.89 mlU/L. No statistically significant difference between rates ofsubclinical hypothyroidism was determined between thetwo groups (p=0.196).Conclusion: Although, carbamazepine reduces thyroidhormone concentrations, rarely causes hypothyroidism.Key words: Child, carbamazepine, thyroid function tests

Background: The clinical spectrum of Brucella infection is quite diverse and characterized by multi-system involvement. Patients present with myocarditis, endocarditis, or pericarditis. Infective endocarditis is the most common cardiovascular complication in patients with brucellosis. Although conduction abnormalities are seen in cases with endocarditis, they are reported very rarely in the setting of cardiac Brucella infection. Case Report: An eight and a half-year-old male patient was referred to our clinic due to inadequate response to cotrimaxazole plus streptomycin treatment at the 15th day of admission. Although local hospital records on the patient showed a heart rate of 80 bpm, we determined a heart rate of 46 bpm. The electrocardiogram showed complete atrioventricular (AV) block. The average heart rate was determined as 48 bpm with 24-hour Holter electrocardiogram (ECG) monitoring. The echocardiographic examination showed normal-sized heart chambers and the absence of valvular involvement. An agglutination test for brucellosis was found to be positive with a titer of 1/320. High fever, arthralgia, and splenomegaly regressed following doxycycline plus rifampicin therapy, but there was no improvement in the AV block. A permanent pacemaker was implanted because of the detection of an average heart rate of 48 bpm. Conclusion: Because cardiac failure and rhythm abnormalities are reported in the course of Brucella infection and may be associated with significant outcomes, cases with brucellosis should be evaluated carefully in terms of cardiac involvement. This report aims to draw attention to complete AV block as an extremely rare complication of Brucella infection.

Over the past 2 decades, transcatheter occlusion of patent ductus arteriosus (PDA) with coils and the duct occluders evolved to be the procedure of choice. A new device, the Occlutech PDA® occluder (ODO) device has been designed. Herein, we aimed to evaluate the characteristics and short-term results of patients who underwent transcatheter closure of PDA using the ODO.We reviewed the clinical records of 60 patients from different centers in Turkey between December 2013 and January 2016. The medical records were reviewed for demographic characteristics and echocardiographic findings. Device size was selected on the narrowest diameter of PDA.The median patient age was 2.5 years (6 months-35 years), and median PDA diameter was 2.5 mm (1.2-11 mm). Fifty-eight of 60 patients (96.6%) had successful ODO implantation. The occlusion rates were 37/58 (63.7%) at the end of the procedure, 51/58 (87.9%) at 24-48 hours post-procedure, and 57/58 (98.2%) on echocardiography at a median follow-up of 7.6 months.Our results indicate that transcatheter closure of PDA using the ODO is effective. Larger studies and longer follow-up are required to assess whether its shape and longer length make it superior to other duct occluders in large, tubular, or window-type ducts. (J Interven Cardiol 2016;29:325-331).

Ture M, Bilici M, Akin A, Demir F, Balik H, Darakci SM. Complete atrioventricular block associated with clozapine intoxication: case report. Turk J Pediatr 2019; 61: 618-621. Clozapine is one of the atypical anti-psychotic drugs used in the treatment of resistant schizophrenia. Although cardiac side-effects are rare, it has been reported that there may be development of myocarditis, dilated cardiomyopathy, postural orthostatic hypotension and prolonged QT duration. Complete atrioventricular (AV) block is characterized by the inability to transmit all of the atrial signal to the ventricles. Causes may be congenital, idiopathic or acquired which are associated with surgery, infection, or muscle disease. AV block is extremely serious and permanent pacemaker insertion is usually necessary for all patients. Complete AV block may develop due to clozapine intoxication through increase in vagal tonus, sinoatrial node (SN) and the inhibition of atrioventricular node signalling. The case presented here is of a 15-year old female patient who developed AV total cardiac block associated with the taking of clozapine in a suicide attempt.

Kardiyak fibrom, rabdomiyomdan sonra en sık görülen benign kardiyak tümör olmasına rağmen insidansı oldukça düşüktür. Çıkış yolu obstrüksiyonuna, aritmiye veya ciddi kapak disfonksiyonuna yol açtıklarında cerrahi müdahale gerektirirler. Bu yazıda oldukça büyük olmasına rağmen asemptomatik seyir gösteren fibromlu bir süt çocuğu sunuldu. Although fibroma is the second most common benign cardiac tumor following rabdomyoma, its incidence is quite low. Fibromas causing ventricular outflow tract obstruction, arrhythmia, and/or severe valvular dysfunction necessitate surgical resection. An infant with asymptomatic clinical course, despite having a giant right ventricular fibroma was reported herein.

Electric shock is a condition that may affect various organ systems and potentially cause death. Cardiac findings vary from asymptomatic mild injury to fatal myocardial involvement. Herein we present a five-year-old boy with a cardiac thrombus developing after an accidental electrical shock. Cardiac arrhythmias and evidence of ischemia have been reported after electric shock; we were, however, unable to identify an earlier case report of intracardiac thrombosis related to electric shock. Findings such as elevated cardiac enzymes and systolic dysfunction, which indicate myocardial damage following electric shock, were present in our patient. We think that the cardiac thrombus might have resulted from the myocardial damage and the slowed intracardiac blood flow related to systolic dysfunction. As the thrombus was thought to have been formed through known mechanisms, it was treated traditionally. However, further data regarding the etiology and management of such thrombi is needed.

Lightning strikes may cause injury to the heart, ranging from slight electrocardiographic changes to fatal damage. As heart injury is the most important cause of mortality in these patients, cardiac monitoring is crucial. Even though various ECG changes have been reported, published data on pathologic ST-segment changes is scarce. Herein, we present a seven-year old patient with ST-segment elevation following a lightning strike. There is not sufficient data regarding lightning-related myocardial ischemia. However, because of the similar effects of lightning strikes and high-voltage electric shocks, we believe myocardial injury related to lightning may be managed in the same manner as is cardiac involvement associated with electric shock.

Although influenza is an acute and uncomplicated disease, that limits itself in the healthy children, it may lead to death by rarely forming the sickness. The most common complication of influenza is pneumonia and it is a rare complication which is developed together with myocarditis by influenza A and B viruses. A 32 months-old male patient was admitted for rapidly developed respiratory distress and tachycardia after fever, cough, vomiting, malaise and runny nose. His general status was medium, he had conscious and had hepatomegaly, tachycardia, dyspnea, tachypnea, intercostal-subcostal retractions and bilateral rhonchus. Cardiac enzyme levels and other laboratory parameters were found normal. Myocarditis and ejection fraction was determined as 42% in echocardiography. However, hospitalization hours between 24 and 48, the patient, whose significant respiratory compromise developed, was intubated and fastened to a mechanical ventilator. H1N1 is produce in nasopharyngeal swab culture at the sixth day of follow-up. Because we think H1N1 virus was responsible from current myocarditis, oseltamivir treatment was initiated. In the fourth day of the treatment the patient’s fever returned to normal, in the ninth day a dramatic recovery was observed. In tracking echocardiography, a significant improvement was observed in the ejection fraction and myocarditis picture compared with admission time. This case was presented in order to remind that in a patients, who present with influenza findings but have respiratory distress and tachycardia in addition to lower respiratory tract infection, myocarditis should also be considered in the differential diagnosis and to remind that promising results could be obtained with the early diagnosis and treatment, İnfluenza, sağlıklı çocuklarda genellikle kendi kendini sınırlayan, akut ve komplikasyonsuz bir hastalık olsa da nadiren ciddi hastalık tablosu oluşturup ölüme neden olabilir. Enfeksiyonun en sık komplikasyonu pnömoni olup, miyokardit influenza A ve B virüsüne bağlı olarak gelişebilen nadir bir komplikasyondur. 32 aylık erkek hasta ateş, öksürük, kusma, halsizlik, burun akıntısı, sonrasında hızlı gelişen solunum sıkıntısı ve taşikardi şikâyetleri ile kabul edildi. Genel durumu orta, bilinci açık, hepatomegali, taşikardi, dispne, takipne, interkostal-subkostal çekilme ve bilateral ronküs mevcuttu. Kardiak enzim düzeyleri ve diğer laboratuvar parametreler normaldi. Ekokardiyografide miyokardit ve ejeksiyon fraksiyonu %42 olarak bulundu. Ancak takiplerinin 24-48. saatinde belirgin solunum sıkıntısı gelişen hasta entübe edilip mekanik ventilatöre bağlandı. Hastanın nazofarinks sürüntü kültüründe takiplerinin altıncı gününde H1N1 üredi. Mevcut myokardit tablosunun H1N1virüsüne bağlı olabileceği düşünülerek Oseltamivir tedavisi başlandı. Tedavinin dördüncü gününde ateşi normale dönen hastanın dokuzuncu gününde kliniğinde dramatik bir iyileşme görüldü. Ekokardiyografi takiplerinde ise ejeksiyon fraksiyonu ve myokardit tablosunda başvuru anına göre belirgin düzelme gözlendi. Gribal enfeksiyon bulgularıyla başvurup solunum sıkıntısı ve taşikardi gelişen hastalarda, alt solunum yolu enfeksiyonu yanında miyokarditin de ayırıcı tanıda düşünülmesi ve erken tanı ve tedavi ile yüz güldürücü sonuçların alınabileceğini hatırlatmak amacıyla sunuldu.Anahtar Kelimeler: Çocuk, H1N1, influenza virüsü, myokardit, oseltamivir

Objective To evaluate the epidemiologic, clinical and laboratory characteristics of a group of children with type 1 diabetes mellitus (T1DM) living in a Turkish city. Methods The records of 395 (boys/girls: 199/196) children with newly diagnosed T1DM hospitalized in the years 1985-2004 were evaluated retrospectively. The data were assessed by gender and age subgroups (≤5, 6-10 and ≥11 years). Results Mean age of children at diagnosis was 8.1±4.1 years. At T1DM onset, the number of children ≤5, between 6-10 and ≥11 years old was 110 (27.9%), 147 (37.2%) and 138 (34.9%), respectively. The patients were mostly diagnosed at ages 6-8 years (24.1%), followed by cases aged 3-5 years (22.0%). Polyuria and polydipsia were the most common symptoms (94.7%). Mean duration of symptoms was 21.5±18.6 days. Although the patients mostly presented in autumn (30.7%), no season-related significant differences were found. The frequency of ketoacidosis was relatively high (48.5%). When compared to boys, the girls experienced higher rates of ketoacidosis (55.1% vs. 41.7%, p=0.042); had a higher frequency of anti-thyroid peroxidase antibodies (11.7% vs. 4.2%, p=0.049) and higher insulin requirement (0.89±0.41 vs. 0.77±0.36 IU/kg, p=0.005). Cases with a family history of T1DM were more likely to have anti-endomysial antibodies (42.9% vs. 8.1%, p=0.027) and higher initial blood glucose levels (510.5±145.0 vs. 436.1±156.5 mg/dL, p=0.005). Conclusion The findings possibly indicate a decreasing age of T1DM onset. The high frequency of ketoacidosis at presentation is noteworthy. Girls had higher rates of ketoacidosis, higher frequency of anti-thyroid antibodies and higher insulin requirements as compared to boys. Patients with a family history of T1DM had higher initial glucose levels and higher frequency of anti-endomysial antibodies.

PMID: 30325127 Akın A, Bilici M, Demir F, Yılmazer MM, İpek MŞ, Kara H. Percutaneous retrieval of umbilical vein catheter fragment in an infant two months after embolization. Turk J Pediatr 2018; 60: 191-193. Umbilical vein catheterization is frequently preferred and a safe route of venous access especially in newborns. However, some cases with breaking and embolization of those catheters have been rarely reported. Herein we present a two-and-a-half-month-old infant being catheterized within first postnatal week and diagnosed to have embolization of the catheter fragment to conjunction of hepatic vein and right atrium. Percutaneous withdrawal of broken catheter was achieved despite several months after the embolization took place. We suggest that transcatheter removal of catheter fragment embolizations may be safe even in late diagnosis cases.

Reports state that Down syndrome (DS) patients with congenitally normal hearts might experience the development of cardiac abnormalities such as cardiac autonomic dysfunction, valvular lesions, bradycardia, and atrioventricular block. However, the presence of any difference in terms of P-wave dispersion (PWd) and QT dispersion (QTd) was not evaluated previously. This study prospectively investigated 100 DS patients with structurally normal hearts and 100 age- and sex-matched healthy control subjects. Standard 12-lead electrocardiograms were used to assess and compare P-wave and QT durations together with PWd and QTd. The median age of the DS patients and control subjects was 48 months. Heart rates and P-wave and QT dispersions were significantly greater in the DS group than in the control group (113 +/- A 22.9 vs 98.8 +/- A 16.6 bpm, p < 0.001; 31.3 +/- A 9.5 vs 24 +/- A 8.6 ms, p < 0.001; and 46.6 +/- A 15.9 vs 26 +/- A 9.1 ms, p < 0.001, respectively). A positive correlation was found between PWd and age in the DS patients (p < 0.05; r = 0.2). All children with DS should be followed up carefully with electrocardiography in terms of increased P-wave and QT dispersions even in the absence of concomitant congenital heart disease for management of susceptibility to arryhthmias.

Guvenc, Tolga/0000-0003-1468-3415 WOS: 000343078300003 PubMed: 25318911 Background: Cardiac contusion is an important cause of mortality after blunt chest trauma (BCT). The aim of this study was to investigate the therapeutic efficacy of the usage of aminoguanidine (AG), in myocardial damage occurring after BCT, alone and in combination with methylprednisolone (MP). Methods: Thirty-five female Wistar albino rats were randomly assigned to five groups (n = 7) including: sham controls (S); only cardiac contusion (CONT); cardiac contusion treated with methylprednisolone (CONT+MP); cardiac contusion treated with aminoguanidine (CONT+AG); and cardiac contusion treated with methylprednisolone and aminoguanidine (CONT+MP+AG). Seven days following the treatments, heart and serum specimens were evaluated histopathologically, immunohistochemically, and biochemically in all groups. Results: Serum AOPP and Tn-I levels increased significantly after cardiac contusions. Haemorrhage, tissue degeneration, and necrosis development was evident following contusions. Increased iNOS expression in myocardial tissue was significantly decreased in the CONT+AG+MP group compared to CONT+AG and CONT+MP groups (p = 0.001 and p = 0.011, respectively). The combined treatment of AG and MP increased Bcl-2 expression significantly after contusions compared to the other treatment groups. Conclusions: Combined usage of AG, a selective iNOS inhibitor, with MP, in cardiac contusions, showed a more powerful cardioprotective effect by increasing Bcl-2 expression and reducing iNOS expression (Tab. 3, Fig. 4, Ref. 33). Text in PDF www.elis.sk.

We present a rare case of spontaneous closure of a fistula between the left coronary artery and the right ventricle (RV) within a few days of newborn period. A 14-day-old male newborn was referred to our clinic for investigation of tachypnea and cardiac murmur. A color flow Doppler echocardiography revealed turbulent flow of a large coronary artery fistula (CAF) between the left coronary artery and the RV. Tachypnea was regressed and repeat echocardiogram showed no CAF related to cardiac chambers after 4 days of admission. The spontaneous closure of CAF was found to be more likely in cases younger than 2 years with small-sized fistulas opening into the right-sided structures, especially into the RV. Nevertheless, the spontaneous closure is very rare in cases with significant shunt. To the best of our knowledge, this is the first case with documented spontaneous closure of CAF just within the newborn period.

Fontan operation is the connection of the systemic venous return to the pulmonary arteries, performed in patients with single ventricle physiology. The Fontan circuit is commonly fenestrated because of early postoperative risks such as high systemic venous pressure and low cardiac output. As it causes progressive cyanosis and increased risk of paradoxical embolism in the follow-up period, occlusion of these fenestrations is generally suggested. Successful closure of extracardiac Fontan fenestration with atrial septal occluder in a 10-year-old girl is reported herein. To our knowledge, this is the first such report from Turkey.

Noonan syndrome (NS; MIM #163950) is an autosomal dominant syndrome characterized by hypertelorism, downward slanting of the palpebral fissures, ptosis, low-set posteriorly angulated ears, short stature, and congenital heart disease, most commonly pulmonary valve stenosis, hypertrophic cardiomyopathy (HCM), and atrial septal defects (ASDs). We report a 6-day-old girl who had an unusual combination of supravalvular pulmonary stenosis (PS), obstructive HCM, secundum ASD, and abnormal mitral chordae. Diagnosis was based on characteristic dysmorphic features and presence of HCM. Subsequent cardiac catheterization confirmed the presence of hemodynamically significant PS, and surgical correction was planned. Supravalvular PS is an unusual cardiac phenotype in NS and its association with HCM and ASD has not been reported previously. A detailed echocardiographic examination is mandatory in NS patients for accurate diagnosis of cardiac phenotype and for further treatment plans.

The aim of this study was to investigate the endothelial dysfunction (ED) and carotid intima-media thickness (IMT) in patients with glycogen storage disease (GSD) types Ia and III. In 22 patients with GSD (13, type Ia; 9, type III) and 18 healthy subjects, endothelial functions of the brachial artery and carotid IMT were evaluated by high-resolution ultrasound. Endothelial-dependent dilatation (EDD) was assessed by establishing reactive hyperemia. EDD and carotid IMTs were compared between the three groups. Mean cholesterol level was slightly higher in GSD type III patients but the difference was not significant. Triglyceride levels and cholesterol to high density lipoprotein (HDL) ratio were significantly higher in GSD type Ia patients. EDD was significantly impaired in GSD type Ia (13% +/- 8%, P = .001) and type III (15% +/- 6%, P = .005) patients when compared with the healthy subjects (22% +/- 4%). The carotid IMT was significantly higher in both GSD type Ia (0.23 +/- 0.03 mm, P =.005) and type III (0.26 +/- 0.05 mm, P = .001) patients when compared with the healthy subjects (0.20 +/- 0.02 mm). Both GSD type Ia and type III patients show significant ED and increased IMT, which are predictors of atherosclerosis.

Background Familial Mediterranean fever (FMF) is an inflammatory disease, which is suggested to be associated with increased risk of atherosclerosis. Epicardial adipose tissue (EAT) thickness and the mean platelet volume (MPV) are parameters used in prediction of atherosclerotic risk in various conditions. These parameters were evaluated in children with FMF and compared with healthy controls. Methods Forty-five patients with FMF and 54 age- and gender-matched healthy controls were assessed. Duration of symptoms, age at diagnosis, duration of delay in diagnosis, frequency and duration of FMF attacks, disease severity scores, response to colchicine therapy, MEditerraneanFeVer (MEFV) gene mutations, and MPV values were recorded. EAT thicknesses were measured by echocardiography. Results Epicardial adipose tissue thicknesses of the children with FMF were found to be significantly greater than that of controls (5.1 ± 1.4 vs. 4.5 ± 0.9 mm, p = 0.036). FMF patients had significantly higher MPV values compared with the controls (7.8 ± 1.1 vs. 7.3 ± 1.4 fl, p = 0.044). Age at diagnosis, duration of delay in diagnosis, and MPV values were found to be correlated with EAT thickness in the patient group (r = 0.49, p = 0.001 for the former parameters and r = 0.32, p = 0.04 for MPV). Conclusion Epicardial adipose tissue thickness and MPV values seem to be increased in children with FMF. These findings may indicate an increased risk of atherosclerosis in FMF patients.