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This study evaluates clinical and epidemiological features of acute rheumatic fever using the data of last 25 years in our hospital in south-east of Turkey. The medical records of 377 patients with acute rheumatic fever admitted to Pediatric Cardiology Department of Çukurova University during 1993-2017 were retrospectively analysed. Two hundred and six patients were admitted between 1993 and 2000, 91 between 2001 and 2008, and 80 between 2009 and 2017. The largest age group (52%) were between 9 to 12 years of age and approximately two-thirds of the patients presented in the spring and winter seasons (62.8%). Among the major findings, the most common included carditis 83.6% (n = 315), arthritis at 74% (n = 279), Sydenham's chorea at 13.5% (n = 51), and only two patients (0.5%) had erythema marginatum and two patients (0.5%) had subcutaneous nodule. Carditis was the most common manifestation observed in 315 patients (83.6%). The most commonly affected valve was the mitral valve alone (54.9%), followed by a combined mitral and aortic valves (34%) and aortic valve alone (5.7%). Of the patients with carditis, 48.6% (n = 153) had mild carditis, of which 45 had a subclinical. Sixty-two patients (19.7%) had moderate and 100 patients (31.7%) had severe carditis. At the follow-up, 2 patients died and 16 patients underwent valve surgery. Twenty-eight (7.4%) patients' valve lesions were completely resolved. Conclusion: Although the incidence of acute rheumatic fever decreased, it still is an important disease that can cause serious increases in morbidity and mortality rates in our country.

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OBJECTIVE: Management of the patients with transposition of the great arteries and intact ventricular septum may be challenging beyond the newborn period. Herein, we would like to present our alternative strategy for training the left ventricle in these patients. METHODS: Six patients with transposition of the great arteries and intact ventricular septum were evaluated in our clinic. Two of them were palliated with Glenn procedure and pulmonary banding as a definitive treatment strategy at other centers. Four patients were operated on and a bidirectional cavopulmonary anastomosis in combination with pulmonary artery banding was performed (stage-1: palliation and ventricular training) in our center. In four out of these six patients, arterial switch operation was performed with takedown and direct re-anastomosis of the superior vena cava to right atrium after an interstage period of 21-30 months (stage-2: anatomical repair). RESULTS: Any mortality was not encountered. The left ventricular mass indices increased from 18-32 to 44-74 g/m2 in patients undergoing the anatomical repair. All of the patients were uneventfully discharged following the second stage. The mean follow-up period was 20 months (9-32 months) following stage 2. All of the patients are doing well with trivial neoaortic regurgitation and normal biventricular function. CONCLUSIONS: Bidirectional cavopulmonary anastomosis with pulmonary artery banding may be a promising left ventricle training approach in ventriculoarterial discordance when compared to the traditional pulmonary artery banding with concomitant systemic-to-pulmonary artery shunt procedures which still carry a significant interstage morbidity and mortality.

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BACKGROUND: We present our experience with the double switch operation in sixteen patients with congenitally corrected transposition of the great arteries. METHODS: We enrolled 16 patients with congenitally corrected transposition of the great arteries operated by a single surgeon between 1995 and 2015. The mean age was 25 months (range 4 to 72 months) and the mean body weight was 8.9 kg (range 4.3 to 19 kg) at the time of operation. RESULTS: We encountered seven patients with moderate to severe tricuspid regurgitation, five of which had Ebstein anomaly. We performed a combination of atrial and arterial switch procedures in 11 cases, one of which had a concomitant coarctation of the aorta that was repaired along with the double switch procedure. Atrial switch and the Rastelli procedures were performed in three cases with concomitant pulmonary stenosis. A combination of arterial switch, Hemi-Mustard procedure, and bidirectional cavopulmonary anastomosis was performed in two cases. During a mean follow-up period of 67 months (range three months to 18 years), we encountered one early postoperative mortality related to intracerebral bleeding. All but one of the patients are now in NYHA class I-II. CONCLUSIONS: Congenitally corrected transposition of the great arteries is a rare congenital cardiac anomaly in which the results of the anatomical repair with double switch operation appear to be superior to that achieved by a physiological repair.

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OBJECTIVES: Cor triatriatum sinister (CTS) is a rare developmental cardiac abnormality resulting in left ventricular inflow obstruction. In this report, we aimed to present our mid-term results of early childhood patients operated for CTS and associated cardiac abnormalities. METHODS: We enrolled 15 patients with CTS who were operated by a single surgeon between 2001 and 2013. A retrospective analysis was performed in order to determine the demographics, operative and postoperative results of the patients. The median age was 14 months and the median body weight was 8.2 kg at the time of operation. RESULTS: Fourteen patients had concomitant cardiac pathology. Three of the patients had atrial septal defect and 1 of the patients had partial abnormal pulmonary venous connection, whereas 4 of the patients had both. In 2 cases of complete atrioventricular septal defect, 1 case with ventricular septal defect, 1 case with patent ductus arteriosus, 1 case with double outlet right ventricle and another case with tetralogy of Fallot, complete repair was performed together with membrane resection in the left atrium (LA). The mean preoperative left atrial gradient was 13.3 mmHg, whereas the mean LA pressure at the first postoperative year was 4.2 mmHg. There was 1 case with early mortality due to septic multiorgan failure secondary to pneumonia. CONCLUSIONS: CTS is a rare congenital cardiac anomaly in which the results of the corrective surgery are encouraging. Early and long-term outcomes may be variable according to the associated congenital heart defects.

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In heterotaxia syndrome with left atrial isomerism, the distinguishing feature is interrupted inferior vena cava with azygos continuation. We report using a transjugular approach to device closure of an atrial septal defect in an 8-year-old boy with heterotaxia syndrome. We found that device closure of the child's atrial septal defect through a jugular venous approach was safe when an inferior vena cava approach was not possible. To our knowledge, ours is the first report of the use of internal jugular vein access to close an atrial septal defect in a child.

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OBJECTIVES: We evaluated long-term follow-up results and prognosis of pediatric patients with isolated ventricular septal defects (VSD). STUDY DESIGN: The study included 799 patients (368 girls, 431 boys; mean age at diagnosis 24.3±37.4 months; median 6 months) who were monitored by the pediatric cardiology department for VSD. The mean follow-up period was 32.8±30.3 months (median 20 months). RESULTS: The VSDs were classified as perimembranous (n=610, 76.4%), muscular (n=171, 21.4%), doubly committed subarterial (n=10, 1.3%), and multiple (n=8, 1%). Spontaneous closure rates were 42.7%, 13.1%, and 25% in muscular, perimembranous, and multiple VSDs, respectively, which corresponded to a mean age of 18.6±19.9 months (median 12 months) in muscular and 30.2±33.7 months (median 14.5 months) in perimembranous VSDs. Before 2 years of age, 78.1% of muscular and 58.6% of perimembranous VSDs underwent spontaneous closure. Of 256 defects (32%) that required surgical closure, 91.4% were of perimembranous location. The mean age at surgery was 38.8±49.1 months (median 11 months) for muscular, and 43.7±40.9 months (median 24 months) for perimembranous defects. During the follow-up period, the following complications were noted: aortic valve prolapse (0.7%), aortic regurgitation (0.6%), left ventricle-to-right atrium shunt (2.6%), subaortic ridge (3.7%), and infundibular stenosis (1.2%). Aortic regurgitation developed in eight patients (3.7%) after surgical closure. CONCLUSION: Our data on the natural course and prognosis of VSDs may be of relevance with respect to patients' age, defect type, and complications encountered in the follow-up period.

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Atrioventricular (AV) block is a potential risk after transcatheter closure of perimembranous ventricular septal defect (VSD) with the Amplatzer perimembranous device. We present herein a case of a 6.5-year-old female patient who developed complete AV block six days after closure of VSD and recovered with steroid and salicylate treatment. It is important to be alert to the development of AV block after transcatheter perimembranous VSD closure. Very careful monitoring of rhythm is mandatory during the short- and long-term follow-up.

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The aim of this study was to evaluate the mid-term clinical status of patients following tetralogy of Fallot (TOF) repair. We performed a cross-sectional observational analysis of 57 postoperative TOF patients and 58 age-matched controls. Patients were examined with myocardial performance index (MPI), which was obtained by tissue Doppler imaging (TDI) in addition to the conventional methods. Compared with controls, patients had significantly higher right ventricular (RV) dimensions and volumes, RV MPI and left ventricular (LV) MPI, and significantly lower RV ejection fraction (EF), LV EF, exercise duration, and maximum heart rate with exercise (p<0.001 for all parameters). A positive correlation was found between RV MPI and LV MPI (r=0.541, p<0.001). There was a negative correlation between LV MPI and exercise duration (r=0.260, p=0.034). After total surgical correction of TOF, the patients had a good clinical status at the mid-term follow-up; decreased exercise capacity and impaired functions in both ventricles related to the degree of pulmonary regurgitation were found. By using MPI, impaired cardiac functions can identify such conditions before they become clinically symptomatic.

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OBJECTIVE: To establish and compare the frequency of intrauterine congenital heart defects in high-risk and low-risk pregnancies for congenital heart diseases. METHODS: Records of 3782 patients who underwent fetal echocardiography at the Pediatric Cardiology Unit were reviewed for reasons of referral and results of echocardiography retrospectively. The categorical variables between the groups were analyzed using a Chi-square test. RESULTS: Structural heart defects were found in 213 (5.6%) fetuses in both high and low risk groups. Most common defects were ventricular septal defect (36.2%) and atrioventricular septal defect (14.1%). Frequency of congenital heart diseases was 7.8% (169 fetuses) in high-risk group: 4.1% were complex, 2.3% significant and 1.4% were minor. In low-risk group, the frequency was 2.7% (44 fetuses): 0.6% were complex, 0.8% significant and 1.3% were minor. In this group, rates of congenital defects were high regarding particular reasons: intrauterine fetal death in previous pregnancy (6.3%), abnormal first or second trimester screening tests (4.3%), and multiple gestations (3.4%). The sensitivity and specificity of fetal echocardiography according to transthoracic echocardiography were found to be 86% and 99%, respectively. CONCLUSION: Congenital heart diseases rate, as expected, was found to be higher in high-risk group. However, regarding intrauterine fetal death in previous pregnancy, abnormal first or second trimester screening tests and multiple gestation, no statistically significant difference was observed between low-risk and high-risk groups. Therefore, we suggest the routine use of fetal echocardiography in cases we have mentioned above if the staff and equipment of the pediatric cardiology clinic are eligible.

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Diabetic ketoacidosis (DKA) is a widely known acute metabolic complication of diabetes mellitus (DM), which can be potentially fatal. It is not difficult to diagnose when a patient with DM comes with symptoms such as coma, fruity breath, hyperglycemia, acidosis, and tachypnea. If the patient has not been diagnosed with DM before, then other sicknesses characterized by an increased anion gap should be considered. A 12-year-old boy with type 1 DM and repeated earlier admissions for DKA was admitted to the emergency department in another apparent case of DKA with coma, hyperglycemia, and profound metabolic acidosis. When his condition did not improve with initial treatment, intoxication was suspected as an alternate cause of his condition. Further laboratory tests detected methanol and amitriptyline. The patient underwent hemodialysis and recovered completely. This case illustrates that a seemingly obvious medical condition can mask serious intoxication. This report is the only publication on two different entities characterized by an increased anion gap and at the end the patient has been cured completely without any complications.

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Primary persistent hyperinsulinaemic hypoglycaemia is characterised by clinical symptoms that occur when blood glucose levels drop below the normal range. Diazoxide treatment remains the mainstay of medical therapy. Tolerance of diazoxide is usually excellent, but several side effects of this drug have been described. We present a 4-month-old girl who developed pulmonary hypertension, heart failure and neutropenia during diazoxide therapy. Diazoxide toxicity was suspected and the drug was withdrawn on day 13. During the next 3 days, respiratory and haemodynamic status dramatically improved and she was weaned from mechanical ventilation. Control white blood cell count was 8800 cells/mm(3) and a new echocardiography showed modreduction of pulmonary artificial pressure to 20 mmHg and resolution of atrial and ventricular enlargement. Paediatric physicians should be in mind of pulmonary hypertension, heart failure and neutropenia developing during diazoxide therapy.

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Congenital aortico-cameral communications are rarely seen. We present an asymptomatic patient in whom there was a congenital vascular communication rising from the aortic root and terminating in the left atrium. She had an atrial septal defect (ASD). The diagnosis was made with echocardiography and confirmed by aortography. She was treated by closing the tunnel and the ASD. The outcome was satisfactory in this extremely rare case of a congenital cardiac lesion. Coincidental diagnosis could be made during careful echocardiographic examination.

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Direct communication between the right pulmonary artery and left atrium is a very rare vascular malformation. We report a patient with this anomaly. She presented with unexplained cyanosis and brain abscesses. The diagnosis was made with contrast echocardiography and angiography. We treated this anomaly successfully with surgery. Complete cure for this anomaly can be achieved by ligation.

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AIM: We aimed to evaluate the long-term results of endocardial pacing with Autocapture threshold tracking pacemakers in children. METHODS AND RESULTS: Implantation and follow-up data of 20 children with these pacemakers were retrospectively evaluated. The pacemakers were implanted subpectorally in five and subcutaneously in 15 patients. The indication for pacing was high-grade atrioventricular block in 18 cases. The mean age at implantation was 7+/-4.8 years. Four patients were pacemaker dependant (heart rate < 30 bpm). At implantation, the mean pacing threshold was 0.5 V at 0.5 ms. The mean evoked response (ER) signal was 8.5+/-3.6 mV, and the polarisation signal (PS) was <1 mV in 15 patients and 1-2 mV in five patients. During the mean follow-up period of 60 months, mean ER signal decreased significantly to 7.7+/-6.3 mV at 24 months and 6.5+/-2.5 mV at 60 months (P < 0.05). In four of 15 patients (26.6%), with a predischarge PS value of <1 mV, it increased between 1 and 2 mV over time. During follow-up, autocapture function was deactivated in six (30%) patients; due to inappropriate ER/PS values in four and due to severe muscle twitching in two with subpectoral implants. These problems occurred during a median period of 21 months after implantation. Generators were replaced in three patients with Microny pacemakers because of battery depletion at 54, 66 and 78 months. In two of them autocapture function had been working since implantation. In seven of 10 patients, who completed > or = 60 months of follow-up, battery impedances were still at the predischarge level. CONCLUSIONS: Autocapture function works well in most children at implantation. Mean ER signal significantly decreases over time despite stable pacing parameters. Autocapture function may become nonoperational due to decreased ER signal in some patients. Muscle twitching may be an important problem that may result in discontinuation of autocapture function in children with subpectoral implants.

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Mitral annular calcification and liquefaction necrosis of this lesion mimicking intracardiac tumor because of secondary hyperparathyroidism have been described in adult patients with chronic renal failure, but have not been reported in children. Chronic renal failure is one of the predisposing factors of this condition. We report the case of a 13-year-old patient with continuous ambulatory peritoneal dialysis with severe hyperparathyroidism who was found to have intracardiac and rib lesions considered to be brown tumors.

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Twenty-one preterm infants (with a mean gestational age and birth weight of 29.3 weeks and 1288.6 g) and nine pretem infants (with a mean gestational age and birth weight of 29.6 weeks and 1153.1 g) were treated with an enteral preparation of indomethacin and with intravenous indomethacin, respectively, for the closure of hemodynamically significant ductus arteriosus. The patients received three doses of either oral indomethacin capsule (Endol, Deva, Turkey) or intravenous indomethacin (Confortid, Dumex GmBH, Germany) in a dose of 0.2 mg/kg at 12-hour intervals. The ductus was closed in 17 (81%) and 7 (77%) of the babies in the orally and intravenously treated groups, respectively (p > 0.05). There was no significant difference in blood urea nitrogen, creatinine levels or thrombocyte counts in either group before and after treatment with indomethacin (p > 0.05). No side effect was reported in the oral indomethacin group. Oral indomethacin may be an alternative to the intravenous preparation in developing countries if the intravenous form is not available or not affordable.

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Anthracycline and cytosine arabinoside are used in combination as the standard therapy for remission induction of acute nonlymphoblastic leukemia. Idarubicin, a synthetic daunorubicin analogue, shows an improved spectrum activity and diminishes acute or chronic toxicity when compared with the other anthracyclines. This study has been carried out in our clinic in order to evaluate the efficiency of the acute nonlymphoblastic leukemia protocol which includes idarubicin. Thirty-eight patients admitted to our Department between 1992-1999 and diagnosed as acute nonlymphoblastic leukemia (ANLL) were included in the study. Their median age was 7 years 6 months (range, 8 months to 14 years). Induction therapy consisted of idarubicin plus cytosine arabinoside and etoposide. Consolidation therapy consisted of two courses, followed by maintenance therapy with thioguanine, cytosine arabinoside, vincristine and cyclophoshamide. The complete remission rate was found to be 71%. The overall survival estimate was found to be 40% for one year and 23% for three years. We established that the protocol with idarubicin reached a higher remission ratio when compared with the other protocols with anthracycline. However, the degree of the hematologic toxicity ratios related to the therapy increased the complication ratios, which affected the long-term life analyses directly. Therefore this protocol may be revised according to socioeconomical conditions, especially in the developing countries.

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