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Background: Cardiovascular system involvement is quite common and the leading cause of morbidity and mortality in patients with Williams syndrome (WS), most of whom need surgery. The present study aimed to provide a detailed evaluation of the features of surgical procedures and outcomes of patients with WS given as single-center experience, and additionally to make a detailed review from Türkiye., Materials and Methods: Thirty-five children with WS diagnosed between the years 1992 and 2021 were evaluated retrospectively including cardiovascular data, surgical treatment features, and outcomes. A total of six articles from Türkiye were evaluated., Results: A total of 35 patients with Williams Syndrome (24 male) with a median age of cardiologic diagnosis of 6 months (range, 2 days-6 years) were evaluated. The cardiac defects of the patients with WS were found as supravalvular aortic stenosis (SVAS) (n=30, 85%) and peripheral pulmonary stenosis (PPS) (n=21, 65%). Additional cardiac anomalies were seen in 71% patients. The rate of SVAS and PPS surgery in all patients with WS was 77.1%. The median surgical age of the patients was 2.5 years (range, 7 months-15.5 years). No patients died due to surgery. But one patient died because of ventricular tachycardia due to anesthesia at the beginning of angiography. A total of 138 (63% male) patients with WS were evaluated from the articles published in Türkiye. Of 138 patients, 64.4% had SVAS, 52.1% had PPS, and 39.8% had additional cardiac anomaly. The median follow-up period ranged from 17 months to 18 years, and six (4.3%) patients died in the early postoperative period., Conclusion: Cardiovascular system involvement is extremely common and is the leading cause of morbidity and mortality in patients with WS, often requiring surgical intervention. As seen in our study including 35 patients with WS and in publications from Türkiye, SVAS in patients with WS generally requires surgery, especially in the first year of life. PPS, on the other hand, requires surgery less frequently than SVAS, and pulmonary stenosis appears to decrease over time., Competing Interests: The authors declare that there is no conflict of interest.

Background and Objectives: Right ventricular failure is an important cause of mortality and morbidity after orthotopic heart transplantation (OHT). The right ventricle of the donor may fail to accommodate to the high pulmonary vascular resistance (PVR) of the recipient. Pulmonary hypertension (PH) due to chronic heart failure with PVRi > 4 Wood units.m2, transpulmonary gradient > 15 mmHg adversely affect the outcome of OHT. In this study we aimed to evaluate management strategies in our pediatric cardiac transplantation candidates with PH and high PVR prior to OHT., Method: Twenty-six cardiac transplantation candidates (age: 10.2 ± 4.6, 1-17 years) underwent cardiac catheterization for the determination of PVR and pulmonary arterial pressure. They were admitted to the hospital and received 1-3 days of intravenous (IV) vasodilator therapy; 0.5-3 μg/kg/min nitroglyserin and/or 0.5-3 μg/kg/min nitroprusside, 5-15 μg/kg/min dobutamin and/or dopamin to keep systolic blood pressure above 80 mmHg., Results: Thirteen patients had dilated cardiomyopathy (CMP), 11 had restrictive CMP, one had hypertrophic CMP and one had congenital heart disease (CHD). Nineteen of the 26 patients underwent OHT. Mean pulmonary arterial pressure of the patients ranged between 11 and 82 mmHg (30.4 ± 16 mmHg) and PVRi between 0.41-21.4 Wood units.m2 (5.3 ± 5.7). Nine patients had PVRi above 4 Wood units.m < sup > 2 < /sup > . Six of these patients had IV treatment for longer than three days and some received specific anti-PH treatment. Eventually they underwent a pulmonary vasoreactivity test with IV iloprost and six had PVRi < 4 Wood units.m < sup > 2 < /sup > . Five of them underwent OHT., Conclusion: Cardiac transplantation candidates with PH and high PVR should be evaluated after conditioning with vasodilator and inotropic treatment. Specific treatment for PH and vasoreactivity testing may help selected patients reenter the transplantation list.

Background and Objectives: Aortic balloon valvuloplasty (ABV) has become the first-line treatment for critical aortic valve stenosis in infants. We aimed to evaluate the short- and mid-term results of patients who underwent ABV during neonatal period, the factors affecting the success and complications of the procedure., Methods: We retrospectively examined 65 patients who underwent ABV during the neonatal period between 1998 and 2017. All hospital records including cardiac catheterization reports, echocardiographic information, and angiographic views were reviewed., Results: Forty five (69.2%) of the patients were male and mean follow-up was 6.2 ± 4.9 years (range: 6 months - 19 years). The mean age of the patients at the first ABV was 14.5 ± 10.6 days (range: 1-30 days) and body weight was 3.25 ± 0.6 kg (range: 1.5-4.8 kg). The peak systolic gradient measured during pre-valvuloplasty cardiac catheterization was 73.3 ± 22.7 mmHg (range: 30-142 mmHg), and it decreased to 29.2 ± 12.2 mmHg (range: 5-55 mm Hg) after the procedure. Valvuloplasty was successful in 59 (90.7%) patients. There was no more than mild aortic regurgitation in any patient before valvuloplasty. There was mild aortic regurgitation in 21 patients before the valvuloplasty. In the acute phase after valvuloplasty, 30 patients had mild, 15 had moderate and two had severe aortic regurgitation. There was a significant increase in the degree of aortic regurgitation related to valvuloplasty (p < 0.05). The most important complication of ABV was increased aortic regurgitation (26.2%). Another important complication was femoral artery occlusion; and was detected early after valvuloplasty (61.6%). There was no serious complication or death in the acute phase., Conclusions: In newborns with valvular aortic stenosis, balloon valvuloplasty has become the first choice in many centers due to its high success rate, low mortality and morbidity, and increased clinical experience. Aortic regurgitation and femoral artery occlusion were the most important complications. Although reintervention for residual or recurrent aortic valve stenosis is common during the first year after valvuloplasty, these patients are able to reach advanced ages without the need for surgical intervention. Surgical valvotomy is a good alternative treatment for a small number of patients in whom valvuloplasty fails.

Background and Objectives: In this study, we aimed to review the treatment options and long-term problems of patients who were diagnosed with coronary artery fistulae (CAF) in our institution. We also tried to determine the most appropriate time for treatment of this condition., Method: From 2000 to 2018, the medical records of 56 patients (33 males and 23 females) who had CAF diagnoses were retrospectively reviewed., Results: The mean age of the patients at the time of diagnosis was 3.9 ± 4.6 years (range, 1 month to 18 years) and the mean duration of the follow-up period was 7.4 ± 4.5 years (range, 1 year to 17.5 years). The right coronary artery (RCA) was the most common origin site for CAF, the left main coronary artery (LMCA) was the second most common origin site whereas the left anterior descending coronary artery (LAD) was the third most common origin site. Catheter angiography showed that right ventricle (RV) was the site of termination for CAF in 23 patients (41.1%) while the CAF drained to the pulmonary artery in 16 patients (28.6%). Transcatheter intervention was performed in ten patients, while CAF were corrected surgically in five patients. Transcatheter intervention was initially attempted in two out of the five surgically-treated patients, but the procedure was unsuccessful. A vascular plug was deployed in six patients, a platinum coil was used in three patients, and a platinum coil with tissue adhesive was placed in one patient using a catheter. Early complications were seen in two patients during transcatheter intervention and in one patient during surgery. There were no instances of death or late complications in patients treated surgically or via transcatheter., Conclusions: Coronary artery fistulae are usually asymptomatic, and medical therapy with long term follow up is the first line treatment. Fistulae that cause hemodynamically significant shunting, chamber enlargement, or visible symptoms should be closed at an early age. This study shows that transcatheter closure is a safe treatment option for CAF that may be performed with high success. Also, it should be known that surgery may be performed effectively with low rates of complications. Because complications can develop in treated and untreated patients of all ages, follow-up should occur during the patient`s lifetime.

Arslan A, Erdoğan İ, Varan B, Yılmaz M, Özin MB, Tokel NK. Reversible cardiomyopathy-tachycardiomyopathy in children. Turk J Pediatr 2019; 61: 552-559. Tachycardia-induced cardiomyopathy (tachycardiomyopathy) is defined by the presence of a sustained tachycardia that results in left ventricular systolic dysfunction. Restoration of cardiac function is dependent on the control of tachyarrhythmias. We report a series including ten children with tachycardia-induced cardiomyopathy with different etiologies. The medical records of patients with tachycardiomyopathy who were managed in a Pediatric Cardiology Clinic between the years of 2014-2017 were reviewed retrospectively. Ten children (3 female, 7 male) were diagnosed with tachycardiomyopathy. The median age of the patients was 12 years (range: 4-15.8). Five had atrial tachycardia, two had ventricular tachycardia, the others had Mahaim fiber tachycardia, permanent junctional reciprocating tachycardia and atrioventricular reentrant tachycardia. Seven patients had catheter ablation and three patients who had previous heart surgery were treated with antiarrhythmic drugs. Median ejection fraction was 33% (range: 10-48), median left ventricle end-diastolic diameter was 55 mm (range: 30-78). All showed complete recovery with median ejection fraction 60% (range: 55- 78). Two patient with severe heart failure required extracorporeal membrane oxygenation support, one of them had ventricular assist device support but the device was removed after successful ablation. After two years this patient required permanent pacemaker implantation due to complete atrioventricular block. Tachycardia-induced cardiomyopathy is a rare and treatable cause of heart failure. Early recognition is critical, aggressive treatment aimed at controlling the arrhythmia results in symptom resolution and recovery of ventricular function.

Varan B, Yakut K, Harman A. Transcatheter retrieval of embolized catheter using venovenous loop in a neonate. Turk J Pediatr 2018; 60: 113-115. Umbilical venous catheters are reliable in providing adequate medical care for premature babies however complications include infections, bleeding, thrombosis, catheter occlusion and migration of a piece of catheter to other parts in the body. Here, we present a case with migration of a piece of umbilical venous catheter which has been trapped between hepatic vein and right upper pulmonary vein causing apnea episodes, recurrent respiratory symptoms, need for mechanical ventilation and increase of acute phase reactants. The embolized catheter was retrieved successfully via percutaneous route.

Tokel K, Gümüş A, Ayabakan C, Varan B, Erdoğan İ. Complications of cardiac catheterization in children with congenital heart disease. Turk J Pediatr 2018; 60: 675-683. Catheterization procedures for congenital heart disease include a broad range of procedures with a large spectrum of potential adverse outcomes. We aimed to determine the incidence of various complications during pediatric cardiac catheterizations and to designate the relative risk factors for such complications. All pediatric patients undergoing cardiac catheterizations between January 2005-December 2010 were included. Data are collected prospectively by filling out computerized catheterization reports. Patient records were scanned for potential risk factors retrospectively. Groups were divided based on cardiac diagnosis; type of procedure. Adverse events were categorized into major or minor events. A total of 2662 cardiac catheterizations were performed during this period. The mean age of patients was 53.2±64.3 months. Diagnostic catheterizations were done for 1797 (67.5%) patients, and interventional procedures were done in 865 (32.5%) cases. Adverse events were observed in 688 patients (26%) during 941 procedures. Minor and major events were seen in 21.4% and 7.1% of the procedures respectively. Most frequent major complications were anesthesia related (6%), most frequent minor complications were vascular complications (45.2%). Complications were more frequent in younger patients (p=0.0001), during interventional procedures (p=0.0001). Thirteen patients died after a cardiac catheterization; they were younger and had longer procedures compared to those who survived (p=0.0001). Vascular complications were frequent among younger patients, with prolonged procedure time and vessel access (p < 0.0001). Cyanotic patients had more complications (p < 0.05; OR for major and minor complications: 3.5 and 2 respectively). Minor complications were 2.7 times more likely in ventricular outflow obstructions (p < 0.05). The complication rates of cardiac catheterization in children are low, but not negligible. Defining risk factors will help anticipate adverse events, which will guide in preparation for rescue procedures and improvement of patient safety systems in catheterization laboratories.

We present a premature infant with large intracardiac mass. She had a history of sepsis and umbilical venous catheterization in the neonatal period. Twenty-seven days after withdrawal of the catheter, a precordial murmur was noted. A large right atrial highly mobile mass suspected to be thrombus was detected by echocardiography. C-reactive protein was elevated. Three blood cultures were negative. Anticoagulation treatment was started. After one week, no resolution of the thrombus was observed. The mass was surgically resected and diagnosis of thrombus infected by fungi was made on histopathological examination. Early screening of cardiac chambers by echocardiography is recommended in all preterms with intravascular catheterization.

Despite the relatively recent introduction of propranolol in the treatment of infantile hemangiomas, there can be little doubt of its efficacy. With regard to safety issues, there are no prior data for very low weight infants. In this study, we used propranolol in preterm and very low weight infants. We used clinical criteria to assess the response to the therapy. We noted all side effects expected from beta-adrenergic blocking drugs, and followed the patients' weight gain during propranolol treatment. Objective, clinical evidence of hemangioma regression was seen after two months in all patients. None of the patients required treatment discontinuation due to adverse side effects. During the propranolol treatment, weight gain was normal in all patients. To the best of our knowledge, this is the first report on the use of propranolol in preterm and very low weight infants, and also the first report from Turkey on the use of propranolol in infantile hemangiomas.

We report the somatic growth characteristics of 60 infants who underwent corrective surgery for congenital heart disease. Patients were assigned to the following groups: Group 1, cyanosis with pulmonary hypertension (PH); Group 2, cyanosis without PH; Group 3, large left-to-right shunt and PH; and Group 4, left-to-right shunt or obstructive heart lesion and no PH. Weight, length, and head circumference measurements and z scores were obtained before the operation, at 45 days, and 3, 6, and 12 months after the operation. Details about dietary intake, socioeconomic status at presentation, length of stay in the intensive care unit, hospitalization period, and perioperative events were noted. The endpoint was reaching a z score > -1 for all anthropometric measurements. At presentation, 51 patients (85%) had malnutrition. The family income, dietary intake, and presence of preoperative chronic malnutrition were interrelated and influenced the weight of the patient at all times during the postoperative follow-up (p < 0.05 for all values). The severity of the heart defect had no significant influence on the postoperative anthropometric measurements (p > 0.05). The lowest preoperative z scores for weight and height were observed in Group 3. Seven patients could not achieve the endpoint at the end of 12 months (4 in Group 3 and 3 in Group 2). Catch-up growth is attained mostly in the first year after corrective surgery. Delays in reaching z scores > -1 are observed in the chronically malnourished children. If adequate calories are provided and early corrective surgery is performed, the normal growth potential may be fulfilled.

Rhythm disturbances that develop after pediatric surgery for heart disease significantly increase mortality and morbidity risk. The aim of this study was to determine incidence rates of different arrhythmias and identify risk factors for these disturbances in this patient group. The study involved 580 children in the pediatric cardiovascular intensive care unit who had undergone cardiac surgery between May 2001 and December 2002. Each was followed until discharge. The patient who sustained arrhythmia was recorded. Fifty-one of the patients (8.8%, mean age 1.7+/-2.3 years) developed arrhythmias. Twenty-one (41.1%) had supraventricular tachycardia, 12 (23.5%) had junctional ectopic tachycardia, 10 (19.6%) had complete atrioventricular block, 3 (5.8%) had ventricular arrhythmias, and 5 (9.8%) had atrial fibrillation and atrioventricular dissociation. There was a trend toward higher incidence of arrhythmia (rate, 43.1%) in the 0-6 months age group. The incidence rates of arrhythmia after certain procedures were as follows: 75% after Rastelli operation, 16.7% after total anomalous pulmonary venous return repair, 13.8% after ventricular septal defect repair, 12.8% after the arterial switch operation or arterial switch with ventricular septal defect closure for transposition of the great arteries, 12.5% after atrioventricular septal defect repair, 12.1% after total correction of tetralogy of Fallot, 9.1% after bidirectional cava-pulmonary connection and Fontan procedure, and 6.6% after other miscellaneous procedures. The mean cardiopulmonary bypass time was 105.4+/-54.1 min. At the time of arrhythmia appearance, the mean values for electrolyte (sodium 144+/-5 mEq/L, potassium 3.78+/-0.91 mEq/L, ionized calcium 1.15+/-0.33 mmol/L) and arterial blood gas parameters (pH 7.40+/-0.12 and HCO3 24.7+/-6.3 mmol/L) were all in the normal range. Fifteen (29.4%) of the patients with arrhythmias died and in 7 of these cases, the death was directly linked to resistant arrhythmia. Arrhythmias can be life-threatening especially in the early period after pediatric heart surgery. The incidence of arrhythmia in this series was 8.8%. The results identified type of operation as a major risk factor for arrhythmia after pediatric heart surgery and they also suggest that age may be important as well.

Rheumatic fever is still one of the major public health problems in developing countries. Recurrences have been reported during continuous regular prophylaxis. Here, we describe a 13-year-old girl who had four apparent recurrences of carditis while on a regimen of intramuscular benzathine penicillin G (BPG) every three weeks. The interesting and special features of this case were the presence of frequent recurrences of carditis with no evidence of arthritis despite regular BPG injections every three weeks, the requirement of long-term corticosteroid therapy due to these recurrences, and the shortness of time periods between attacks after withdrawal of steroid therapy. In this case, the administration of supraphysiologic doses of glucocorticoids caused iatrogenic Cushing syndrome with decreased bone mineral density and bilateral avascular tibial necrosis mimicking the symptoms of arthritis. Thus, this is a rare case. In similar cases with recurrences of either arthritis or carditis, instead of long-term corticosteroid therapy, we suggest a regimen of BPG prophylaxis every two weeks rather than every three weeks or monthly BPG injections because of the superiority of the every two weeks schedule in the adequate control of rheumatic fever recurrences, as shown in some previous studies.

Pulmonary hemosiderosis may rarely be associated with juvenile rheumatoid arthritis or can develop during the course of the disease. We present a three-year-old boy with severe iron deficiency anemia (without any pulmonary symptoms) and arthralgia at the time of diagnosis. Two years after the initial diagnosis he developed pulmonary hemosiderosis and pauciarticular type of juvenile rheumatoid arthritis which progressed to seronegative polyarticular juvenile rheumatoid arthritis. He responded very well to prednisolone and was maintained well on low-dose alternate-day prednisolone and naproxen sodium treatment. This is the only case of association of these two diseases in our experience in both the Pediatric Rheumatology and Pediatric Respiratory Diseases Departments.

We report a neonate who presented with hypotonia, hypoglycemia, and severe lactic acidosis. The patient's acidosis did not respond to bicarbonate replacement and dialysis. Postmortem liver samples revealed portal dilatation, fibrosis, canalicular proliferation, cholestasis, and hepatocellular hemosiderosis. Vacuolization of bone marrow precursors suggested a diagnosis of Pearson syndrome. A common mitochondrial DNA deletion of 4,978 bp was found. We emphasize that Pearson syndrome should be considered in neonates with lactic acidosis despite absence of anemia.

The efficacy of oxybutynin, pseudoephedrine and indomethacin treatment was investigated in 29 patients with primary nocturnal enuresis. Patients were randomly assigned to either oxybutynin (1st group, n = 9), pseudoephedrine (2nd group, n = 11) or indomethacin (3rd group, n = 9) treatments. Oxybutynin and indomethacin did not cause a statistically significant difference in the number of dry nights (p > 0.05), but patients treated with pseudoephedrine had a significant increase in the number of dry nights (p < 0.05). Five patients in the oxybutynin and one patient in the indomethacin group experienced side effects. None of the patients in the pseudoephedrine group had any complaints with the drug. We therefore conclude that pseudoephedrine can be an alternative in the treatment of primary nocturnal enuresis.