Congenital heart defects and postoperative follow-up of patients with Williams syndrome as a single center experience and review of the cases from Türkiye.

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.

Pre- and postoperative levels of serum brain-derived neurotrophic factor in neonates with congenital heart defects.

BACKGROUND: The objectives of this study were to assess the preoperative and postoperative serum brain- derived neurotrophic factor (BDNF) levels in neonates undergoing surgery for congenital heart defects (CHD). Also to explore the relationship between changes in BDNF levels and the impact of perioperative factors including intraoperative body temperature, aortic cross-clamp time, perfusion time, operation time, inotropic score, vasoactive inotropic score and lactate levels. METHODS: Forty-four patients with CHD and 36 healthy neonates were included in the study. Blood samples for serum BDNF levels were collected three times: preoperatively, and at 24 and 72 hours postoperatively from each patient in the operated group. Additionally, samples were collected once from each individual in the non-operated case group and the control group. Serum BDNF levels were analyzed using the Elabscience ELISA (Enzyme-Linked Immunosorbent Assay) commercial kit. Cranial ultrasonography (USG) was performed on all infants with CHD. Following cardiac surgery, patients underwent second and third cranial USG examinations at 24 and 72 hours postoperatively, respectively. RESULTS: Forty-four consecutive patients with CHD were divided into two groups as follows: the operated group (n=30) and the non-operated group (n=14). Although there were no differences in the baseline serum BDNF levels between the case and control groups, the preoperative serum BDNF levels were significantly lower in the patients operated compared to the non-operated patients. The serum BDNF levels at the 24th hour postoperatively were higher than the preoperative levels. However, no significant correlation was found between the serum BDNF levels at 24 and 72 hours postoperatively as well as the cranial USG findings at corresponding times. CONCLUSIONS: Serum BDNF levels were initially lower in neonates with CHD who underwent surgery, but increased during the early postoperative period. These results suggest that serum BDNF levels are influenced by CHD and the postoperative period.

Evaluation of acute kidney injury after surgery for congenital heart disease in neonates: a tertiary hospital experience.

PURPOSE OF THE ARTICLE: Acute kidney injury (AKI) after cardiac surgery in children with congenital heart disease (CHD) is a serious complication closely associated with high morbidity and mortality. Despite numerous studies on AKI in children, most studies have excluded neonates. We sought to characterize AKI associated with cardiac surgery in neonates, determine its incidence, perioperative and postoperative risk factors, and short-term results. MATERIALS AND METHODS: This retrospective study included 177 neonates who were operated on for CHD in our hospital between January 2015 and December 2019. Data of the patients were analyzed according to nKDIGO (neonatal Kidney Disease Improving Global Outcomes) and nRIFLE (neonatal Risk, Injury, Failure, Loss of function, End-stage kidney disease) criteria for evaluating AKI retrospectively. Data of groups with and without AKI were analyzed. RESULTS: The average age of 177 neonates were 8.2 ± 6.1 (1-28) days. Twenty-two (12.4%) neonates had CS-AKI defined according to nKDIGO criteria. Four (2.3%) neonates reached nKDIGO stage I, 1 (0.6%) reached stage II, 17 (9.6%) reached stage III. Thirty-eight (21.5%) neonates had CS-AKI defined according to nRIFLE criteria. Twenty-four (13.6%) neonates reached nRIFLE stage risk(R), 6 (3.4%) reached stage injury(I), 8 (4.5%) reached stage failure (F). The incidence of cardiac surgery-associated acute kidney injury (CS-AKI) in neonates was 12.5% and 21.5% for nKDIGO and nRIFLE, respectively. The percentage difference between nKDIGO and nRIFLE for AKI assessment was due to the criteria for nRIFLE stage risk(R) urine output < 1.5 mL/kg/h for 24 h. In both classifications, the duration of cardiopulmonary bypass, operation, inotropic treatment, and mechanical ventilation, length of intensive care unit (ICU), and hospital stay were significantly higher in the AKI group than those without AKI group (p˂.05). The mortality rate in the groups with AKI was found to be significantly higher (p˂.05) than in the groups without AKI. In Kappa analysis, when two classifications were compared according to AKI stages, a significant agreement was found between nKDIGO and nRIFLE classifications (p˂.05) (Kappa: 0.299). CONCLUSION: AKI and mortality rates were similar between groups according to the nKDIGO and nRIFLE criteria. For early prediction of AKI and adverse outcomes, diagnostic reference intervals might be specified in more detail in neonates undergoing cardiac surgery for CHD.

Neurologic Complications After Pediatric Heart Transplant: A Single-Center Experience.

OBJECTIVES: Neurologic complications that can lead to serious mortality and morbidity in pediatric heart transplant recipients have been reported to range from 23.6% to 45%. In this study, the frequency, time, cause, and characteristics of neurologic complications in pediatric heart transplant recipients were evaluated. MATERIALS AND METHODS: We retrospectively reviewed data of 37 pediatric heart transplant recipients aged <18 years who were seen at our hospital between 2007 and 2017. Medical records were reviewed to identify neurologic complications. Clinical features were compared between pediatric heart transplant patients with and without neurologic complications. RESULTS: The rate of posttransplant neurologic complications in pediatric heart transplant was 27% (10/37). Median age of patients with neurologic complications was 12 years (range, 11-18 years). Median time for neurologic complications was 3 days (range, 2-46 days). Primary diagnoses of these 10 recipients were dilated cardiomyopathy (n = 7) and restrictive cardiomyopathy (n = 3). There were no significant differences between recipients with and without neurologic complications (P > .05).The etiologies of neurologic complications were posterior reversible encephalopathy syndrome in 3 patients (8.1%), stroke in 2 patients (5.4%), peripheral neuropathy in 2 patients (5.4%), hypertensive encephalopathy in 1 patient (2.7%), and drug encephalopathy in 1 patient (2.7%). CONCLUSIONS: Neurologic complications may lead to serious mortality and morbidity in pediatric heart transplant patients. Seizures, posterior reversible encephalopathy syndrome, stroke, peripheral neuropathy, transient ischemic attack, and cerebral infections are the most common neurologic complications, which are seen in the perioperative period in particular. Careful follow-up of pediatric heart transplant patients, with detection and early treatment of neurologic findings, will contribute to lower rates of sequelae. To our knowledge, this is the largest study to show a detailed experience of neurologic complications in pediatric heart transplant patients from a single center in Turkey.

Results of balloon and surgical valvuloplasty in congenital aortic valve stenosis: A 19-year, single-center, retrospective study.

BACKGROUND: This study aims to compare the success, complications, and long-term outcomes of aortic balloon valvuloplasty and surgical aortic valvuloplasty in pediatric patients with congenital aortic valve stenosis. METHODS: Between March 2000 and October 2019, a total of 267 procedures, including 238 balloon valvuloplasties and 29 surgical valvuloplasties, in 198 children (135 males, 63 females; mean age: 57.4±62.6 months; range, 0.03 to 219 months) were retrospectively analyzed. The hospital records, echocardiographic images, catheterization data, angiography images, and operative data were reviewed. RESULTS: Aortic regurgitation was mild in 73 patients before balloon valvuloplasty, and none of the patients had moderate-to-severe aortic regurgitation. Compared to surgical valvuloplasty, the rate of increase in the aortic regurgitation after balloon valvuloplasty was significantly higher (p=0.012). The patients who underwent balloon valvuloplasty did not need reintervention for a mean period of 46±45.6 months, whereas this period was significantly longer in those who underwent surgical valvuloplasty (mean 80.5±53.9 months) (p=0.018). The overall failure rate was 8%. Moderate-to-severe aortic regurgitation was the most important complication developing due to balloon valvuloplasty in the early period (13%). All surgical valvuloplasties were successful. The mean length of hospitalization after balloon valvuloplasty was significantly shorter than surgical valvuloplasty (p=0.026). During follow-up, a total of 168 patients continued their follow-up, and a reinterventional or surgical intervention was not needed in 78 patients (47%). CONCLUSION: Aortic balloon valvuloplasty can be repeated safely and helps to eliminate aortic valve stenosis without needing sternotomy. Surgical valvuloplasty can be successfully performed in patients in whom the expected benefit from aortic balloon valvuloplasty is not achieved.

Coronary artery fistulae and treatment in children.

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.

Management of pediatric cardiac transplantation candidates with pulmonary hypertension and high pulmonary vascular resistance.

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.

Aortic balloon valvuloplasty and mid-term results in newborns: a single center experience.

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.

Early and late outcomes of surgical repair of double-chambered right ventricle: a single-centre experience.

OBJECTIVE: Double-chambered right ventricle is characterised by division of the outlet portion of the right ventricle by hypertrophy of the septoparietal trabeculations into two parts. We aim to report our experiences regarding the presenting symptoms of double-chambered right ventricle, long-term prognosis, including the recurrence rate and incidence of arrhythmias after surgery. METHODS: We retrospectively investigated 89 consecutive patients who were diagnosed to have double-chambered right ventricle and underwent a surgical intervention from 1995 to 2016. The data obtained by echocardiography, cardiac catheterisation, and surgical findings as well as post-operative follow-up, surgical approaches, post-operative morbidity, mortality, and cardiac events were evaluated. RESULTS: Median age at the time of diagnosis was 2 months and mean age at the time of operation was 5.3 years. Concomitant cardiac anomalies were as follows: perimembranous ventricular septal defect (78 patients), atrial septal defect (9 patients), discrete subaortic membrane (32 patients), right aortic arch (3 patients), aortic valve prolapse and/or mild aortic regurgitation (14 patients), and left superior caval vein (2 patients). The mean follow-up period was 4.86 ± 4.6 years. In these patients, mean systolic pressure gradient in the right ventricle by echocardiography before, immediately, and long-term after surgical intervention was 66.3, 11.8, and 10.4 mmHg, respectively. There were no deaths during the long-term follow-up period. Surgical reinterventions were performed for residual ventricular septal defect (2), residual pulmonary stenosis (1), and severe tricuspid insufficiency (1). CONCLUSION: The surgical outcomes and prognosis of double-chambered right ventricle are favourable, recurrence and fatal arrhythmias are unlikely in long-term follow-up.

Diagnosis and treatment of abnormal left coronary artery originating from the pulmonary artery: A single-center experience.

OBJECTIVE: We aimed to review symptoms, findings, surgical treatment options, short- and mid-term outcomes, and reoperation rate of patients diagnosed with of left coronary artery from the pulmonary artery (ALCAPA) of an anomalous origin in our institution. METHODS: From May 2000 to March 2018, 33 patients who had left coronary artery originating from the pulmonary artery were retrospectively examined. The clinical features of patients, diagnostic tools and their efficacy, outcomes of surgical repair, and problems during follow-up were evaluated. RESULTS: Thirty-three patients (22 females, 11 males) were included in the study. At the time of surgery, the median age and weight of patients were 6 months (minimum/maximum, 1-166 months) and 6.5 kg (minimum/maximum, 3-38.5 kg), respectively. The mean follow-up was 5±3.5 years (range, 1-16 years). Dyspnea, tachypnea, diaphoresis, prolonged feeding time, and developmental delay were common presenting signs and symptoms. It was determined that all the patients who were diagnosed at another center reached our center for surgical treatment within 1 month. Twenty-three (69.7%) patients had pathologic Q wave with anterior and/or anterolateral myocardial infarction signs on an electrocardiogram (ECG), whereas 22 (66.6%) patients had ST-T segment changes. Twenty-one (63.6%) patients had cardiomegaly on the telecardiogram. A reimplantation surgery was performed to 22 patients and 10 patients underwent the Takeuchi procedure. In addition to ALCAPA repair, 5 patients needed mitral valve plasty. Atrial septal defect (ASD) and ventricular septal defect (VSD) were closed in one patient, and Tetralogy of Fallot was totally corrected in another. At discharge, there was a significant improvement in left ventricular (LV) systolic functions. At the last visit, all patients had normal LV systolic functions except four who had mild dysfunction. The mean follow-up of the four patients was 2.8 years. In the early postoperative period, complications were seen in 10 patients. Five patients died in the early postoperative period, while one patient died 9 months after the ALCAPA surgery because of low cardiac output syndrome that developed after mitral repair. CONCLUSION: Patients with ALCAPA commonly present with congestive heart failure symptoms. When the diagnosis is confirmed in these patients, surgical treatment should not be delayed. The availability of surgical center and surgery outcomes for ALCAPA diagnosed patients are comparable with other countries, but the delay in the diagnosis of disease is still a problem in our country.

Asymptomatic giant congenital left atrial aneurysm.

Yakut K, Varan B, Erdogan I. Asymptomatic giant congenital left atrial aneurysm. Turk J Pediatr 2019; 61: 117-119. Congenital aneurysm of the left atrial appendage can be caused by congenital dysplasia of the pectinate muscles and may be accompanied by a congenital absence of the pericardium. Symptoms generally manifest after two decades and the most common symptom is atrial arrhythmia in the form of atrial fibrillation / flutter. A four year-old patient with no symptoms underwent an echocardiographic examination for the investigation of a heart murmur. Echocardiographic examination revealed a large cystic lesion occupying the left hemithorax and compressing the left ventricle. The patient was referred to our center. The lesion caused displacement of the heart rightward behind the sternum which made the examination difficult. We aimed to present this rare case of giant left atrial appendage aneurysm in the light of current literature.

Reversible cardiomyopathy-tachycardiomyopathy in children.

Arslan A, Erdogan I, Varan B, Yilmaz 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.

The results of interventional catheterization in infants weighing under 2,000 g.

BACKGROUND: The aim of this study was to evaluate the early and mid-term results of interventional cardiac catheterization and procedure-related complications in infants weighing <2,000 g. METHODS: Between May 1998 and April 2017, 22 patients (14 males, 8 females; mean age 14±8.4 days; range, 1 to 30 days) weighing <2,000 g who underwent a total of 23 interventional cardiac catheterization were retrospectively analyzed. Procedures were balloon coarctation angioplasty in 14, balloon atrial septostomy in five, balloon aortic valvuloplasty in one, balloon pulmonary valvuloplasty in one, patent ductus arteriosus closure in one, and stent placement in the ductus in one patient. Another patient underwent balloon coarctation angioplasty and balloon aortic valvuloplasty in the same session. RESULTS: The overall success rate of the interventional procedures was 95.6%. The mean follow-up was 3.2±1.6 years (range, 1 to 5.5) for 18 patients with available records. The rate of serious complications was 18%. The most frequent complications in the early period were low hemoglobin levels requiring erythrocyte suspension transfusion (54.5%) and vascular injury (54.5%). Two patients required reintervention, one patient required surgery after the second intervention, and three patients required only surgery. Six patients underwent palliative interventional procedures, and interventional procedures led to definitive treatment in five patients. CONCLUSION: The mortality and morbidity rate of surgery is high in premature under 2,000 g infants and interventional heart catheterization can be life-saving in this patient group, although it is associated with significant complications in low birth weight newborns.

[Case report and the surgical treatment of two cases with pulmonary atresia in which pulmonary arteries is circulated by coronary arteries]. / Pulmoner arter kanlanmasi koroner arterler yoluyla olan pulmoner atrezili iki olgu sunumu ve cerrahi tedavisi.

Pulmonary atresia (PA) and ventricular septal defect (VSD) can occur in a variety of ways, from simple valve atresia to a condition in which circulation to the pulmonary bed occurs through collateral arteries separated from the aorta and there are no real pulmonary arteries, or they are present but hypoplastic. The size of the pulmonary arteries and concomitant complex cardiac lesions are important in making decisions about treatment and correctional alternatives. While complete correction surgeries in the style of a correction of tetralogy of Fallot are performed in simpler cases, many very invasive procedures are also performed and the resulting quality of life is very variable. The size of the pulmonary artery and its branches and the presence of accompanying collateral vessels are determining factors in the management of the disease. In this report, 2 cases of VSD and PA, in which the circulation of the pulmonary arteries was through the coronary arteries, diagnosed as a result of echocardiography performed following murmurs heard during examination, are described. The first patient was 40 days old and the second was 2 days old. In the second case, diagnosis was confirmed by catheter angiography, and in the first case, the final diagnosis was made during surgery. The aim of this report is to emphasize the importance of pulmonary artery size, the presence of pulmonary confluence, the origin of circulation, and concomitant collateral arteries when considering treatment methods.

Complications of cardiac catheterization in children with congenital heart disease.

Tokel K, Gümüs A, Ayabakan C, Varan B, Erdogan I. 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.

Rituximab Therapy for Rejection in Pediatric Heart Transplant.

OBJECTIVES: Humoral rejection is the B-cell-mediated production of immunoglobulin G antibody against the transplanted heart. Antibody-mediated rejection may be resistant to standard immunosuppressive therapy and is associated with high mortality and graft loss. Rituximab can be used to treat antibody-mediated rejection in heart transplant recipients. This retrospective study describes our experience with rituximab treatment in children with heart transplants. MATERIALS AND METHODS: We present 7 pediatric patients with antibody-mediated rejection who were treated with plasma exchange and rituximab therapy. Rituximab was given at a dose of 375 mg/m2 by slow infusion in the intensive care unit after 5 days of plasmapheresis, in addition to a conventional regimen consisting of steroids, mycophenolate mofetil, and tacrolimus. The peripheral blood count and sodium, potassium, serum urea nitrogen, creatinine, aspartate aminotransferase, and alanine aminotransferase levels were measured in all patients before and after treatment. RESULTS: Seven patients were treated with plasma exchange and rituximab. We repeated this therapy in 5 patients because of refractoriness or recurrent rejection. After diagnoses of antibody-mediated rejection, 4 patients died within 6 months (mortality rate of 57.1%). We did not observe any adverse effects or complications related to rituximab. CONCLUSIONS: Rituximab can be used in humoral rejection after pediatric heart transplant. However, the success of the treatment is controversial, and further study is needed to find an effective treatment for antibody-mediated rejection and steroid-resistant cellular rejection in children.

Hypomagnesemia in Pediatric Heart Transplant Patients Treated with Tacrolimus.

OBJECTIVES: We aimed to investigate the frequency of hypomagnesemia and urinary magnesium excretion in pediatric heart transplant recipients. MATERIALS AND METHODS: In this study, 22 pediatric patients who underwent heart transplanted at a single center between March 2014 and April 2015 and who were treated with oral tacrolimus were analyzed prospectively. Serum magnesium, creatinine, and tacrolimus levels and total amount of urinary magnesium excretion were measured. Serum tacrolimus levels were measured 12 hours after the last dose of tacrolimus. RESULTS: Our patient group included 11 boys (50%) and 11 girls (50%) with a mean age of 16.72 ± 4.78 years. Serum tacrolimus levels were in the therapeutic range, with a mean of 1.48 ± 0.13 ng/mL (range, 1.2-1.69 ng /mL), mean fractional magnesium excretion was 8.59 ± 5.9% (range, 3%-22%), and 24-hour urinary magnesium excretion was 90.2 ± 62.95 mg/d. Hypermagnesuria was assessed in 80% of patients. We found 24-hour urinary magnesium excretion to be higher than normal in 27% of patients. There was no association between serum tacrolimus levels and serum magnesium levels or urinary magnesium excretion. CONCLUSIONS: Serum magnesium levels should be periodically measured in pediatric heart transplant patients treated with tacrolimus.

Diagnosis and surgical treatment of aortopulmonary window: Our single-center experience.

BACKGROUND: In this study, we aimed to report our single-center experience in aortopulmonary window and review clinical signs, symptoms, surgical correction techniques, and long-term outcomes. METHODS: We retrospectively reviewed the medical records of a total of 30 patients who were followed with the diagnosis of aortopulmonary window in our hospital between May 1998 and June 2016. The clinical characteristics of the patients, echocardiographic and angiographic findings, surgical treatment outcomes, and medical problems during follow-up were reviewed. RESULTS: The most common signs and symptoms were murmur, dyspnea, tachypnea, growth retardation, and signs of congestive heart failure. The mean age at the time of surgery was 8.2±14.4 months (7 days to 60 months). Eighteen patients (60%) had additional congenital cardiac anomalies. Eleven patients had simple congenital heart diseases, and seven patients had complex congenital heart diseases. Four patients were unable to be operated due to Eisenmenger syndrome (n=3) and complex congenital heart disease (n=1). No early or late postoperative death was observed. The mean follow-up was 6.4±4.8 years (range, 5 months to 16 years). In addition to aortopulmonary window repair, an additional cardiac anomaly modifying surgical intervention was corrected in nine patients (34.6%). One patient was reoperated for residual aortopulmonary window and another patient for pulmonary stenosis (valvular, supravalvar) after three years. One of these patients underwent pulmonary balloon valvuloplasty after two years. The reoperation rate was 7.7% (n=2) during follow-up. CONCLUSION: Aortopulmonary window is a rare cardiac anomaly which may be overlooked by echocardiographic study, and which is amenable for repair with low-surgical risk. It is, therefore, imperative to diagnose and treat this condition, before pulmonary vascular disease develops.