Surgical and transcatheter pulmonary valve replacement in patients with repaired tetralogy of Fallot: cardiac magnetic resonance imaging characteristics and morphology of right ventricular outflow tract.

BACKGROUND: Pulmonary valve replacement is recommended in patients with repaired tetralogy of Fallot based on cardiac magnetic resonance imaging (MRI) criteria. This procedure is performed by surgical or transcatheter approaches. OBJECTIVE: We aimed to investigate the differences in preprocedural MRI characteristics (volume, function, strain) and morphology of the right ventricular outflow tract and branch pulmonary arteries in patients for whom surgical or transcatheter pulmonary valve replacement was planned. MATERIALS AND METHODS: Cardiac MRI of 166 patients with tetralogy of Fallot were analyzed. Of these, 36 patients for whom pulmonary valve replacement was planned were included. Magnetic resonance imaging characteristics, right ventricular outflow tract morphology, branch pulmonary artery flow distribution and diameter were compared between surgical and transcatheter groups. Spearman correlation and Kruskal-Wallis tests were performed. RESULTS: Circumferential and radial MRI strain for the right ventricle were lower in the surgical group (P=0.045 and P=0.046, respectively). The diameter of the left pulmonary artery was significantly lower (P=0.021) and branch pulmonary artery flow and diameter ratio were higher (P=0.044 and P = 0.002, respectively) in the transcatheter group. There was a significant correlation between right ventricular outflow tract morphology and right ventricular end-diastolic volume index and global circumferential and radial MRI strain (P=0.046, P=0.046 and P= 0.049, respectively). CONCLUSION: Preprocedural MRI strain, right-to-left pulmonary artery flow, diameter ratio and morphological features of the right ventricular outflow tract were significantly different between the two groups. A transcatheter approach may be recommended for patients with branch pulmonary artery stenosis, since both pulmonary valve replacement and branch pulmonary artery stenting can be performed in the same session.

Persistent left superior vena cava draining into the unroofed coronary sinus in a patient with tetralogy of Fallot and coronary sinus orifice atresia.

INTRODUCTION: In this study, we present a case of directing persistent left superior vena cava drainage into the unroofed coronary sinus to the right atrium in a patient with tetralogy of Fallot and coronary sinus orifice atresia without innominate vein. CASE REPORT: A 16-month-old boy diagnosed with tetralogy of Fallot was admitted. Following intracardiac repair, the left superior vena cava was divided from the left atrial junction, passed through under the ascending aorta and anastomosed to the right superior vena cava. CONCLUSION: To our knowledge, this is the first patient reported with persistent left superior vena cava, unroofed coronary sinus, coronary sinus orifice atresia, and tetralogy of Fallot. The extracardiac direct anastomosis technique was used successfully to direct persistent left superior vena cava to the right atrium without any post-procedure complications.

Surgical repair of the anomalous aortic origin of the coronary arteries: a single-center experience.

OBJECTIVE: Anomalous aortic origin of the coronary artery is a rare congenital cardiac anomaly. The aim of this study was to present our experience with patients who underwent surgery for the anomalous aortic origin of the coronary artery. METHODS: This was a retrospective review of our experience with patients who had anomalous aortic origin of the coronary artery from 2019 to 2022. RESULTS: Seven patients were managed for anomalous aortic origin of the coronary artery, including five males and two females. The median age of the patients were 16 years (IQR, 14.5-26 years). Five patients had anomalous aortic origin of the right coronary artery and two patients had anomalous aortic origin of the left coronary artery. Five patients were treated surgically, one patient refused surgical treatment despite myocardial ischaemia symptoms, and the other one was not operated because she had no symptoms. Two patients underwent pulmonary root anterior translocation and left main coronary artery unroofing procedure, one patient underwent right coronary artery unroofing procedure, one patient underwent pulmonary artery lateral translocation procedure, and the last patient underwent right coronary artery osteal translocation procedure. The post-operative mortality or myocardial infarction was not observed in any patient. Patients were followed for a median of 10 months (IQR, 6.75-20.5 months) after repair. CONCLUSION: The data suggest that surgical repair of anomalous aortic origin of the coronary artery can be performed confidently and can be very effective for relieving myocardial ischaemia symptoms. Different surgical techniques can be used in anomalous aortic origin of the coronary artery according to the course and origin of the coronary arteries. To the our knowledge, pulmonary root anterior translocation and coronary artery unroofing procedure were performed for the first time in the literature.

Rare association of absent pulmonary valve syndrome, complete atrioventricular canal defect, double outlet right ventricle, right aortic arch, and aberrant right subclavian artery in a fetal case.

Absent pulmonary valve syndrome (APVS) is a rare congenital cardiac anomaly. It is often associated with TOF, but may also be associated with an intact ventricular septum or, more rarely, with tricuspid atresia. It's combination with a complete atrioventricular septal defects and double outlet right ventricle has been reported extremely rare, even in the postnatal period. Herein, we report a 20-week-old fetus with a right aortic arch and an aberrant left subclavian artery with this rare combination. We report a case of a 20-week fetus diagnosed having this rare combination of right aortic arch and aberrant left subclavian artery. The APVS with complete atrioventricular septal defects may represent another type of APVS.

Unifocalization with pericardial roll technique in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.

OBJECTIVE: This study aims to compare both the pericardial roll technique with the patch augmentation technique of the unifocalization, and single-stage complete repair with the unifocalization and shunt for the repair of the ventricular septal defect, pulmonary atresia, and major aortopulmonary collateral arteries. METHODS: This was a retrospective review of the 48 patients undergoing unifocalization of the ventricular septal defect, pulmonary atresia, and major aorticopulmonary collateral arteries from a single center. Our cohort had two surgical pathways, including single-stage midline unifocalization (n = 40), unifocalization after pulmonary artery rehabilitation by creating an aortopulmonary window or central shunt (n = 8). There were two surgical techniques in single-stage midline unifocalizaton, including widening of the pulmonary arteries with a patch (n = 30), and connecting pulmonary arteries with a pericardial roll (n = 10). RESULTS: A total of 14 (29.2%) of 48 patients underwent single-stage complete repair, 26 patients underwent shunt palliation with unifocalization. Combined early and late mortality was seen in seven patients in those who underwent shunt palliation with unifocalization, while it was seen in one patient in those who underwent a single-stage complete repair (mortality ratio 26.8% vs. 7.1%, p = .22). There was no statistically significant difference between the pericardial roll and patch augmentation techniques in terms of pulmonary artery reintervention (p = .65). Although all pulmonary artery reinterventions were for unilateral pulmonary artery in the roll technique group, 41.7% of reinterventions were for bilateral pulmonary arteries in the pericardial augmentation group. CONCLUSION: Single-stage complete repair of the ventricular septal defect, pulmonary atresia, and major aorticopulmonary collateral arteries has better results than unifocalization with a shunt. In terms of nonvaluable raw material, the use of the pericardial roll technique is a considerable alternative for unifocalization.

Evaluation of midterm outcomes after Shone's complex surgery: Analysis of reoperation and mortality risk factors.

OBJECTIVE: The midterm results of patients who underwent biventricular repair surgery for Shone's complex were examined, and mortality and reoperation risk factors were evaluated. METHODS: This retrospective study included 34 patients with Shone's complex who underwent mitral valve (MV) surgery between 2005 and 2020. RESULTS: A total of 19 patients (56%) had coarctation, 10 (29%) patients had subaortic stenosis, 9 (26.5%) patients had a hypoplastic aortic arch (AA), and 9 (26.5%) patients had aortic valve (AV) stenosis. Twenty-four (70.6%) patients had bileaflet AV. Associated left-sided in-flow stenotic lesions included parachute MV in 19 (56%) patients and supramitral ring in 18 (53%) patients. The estimated freedom from reoperation rate on the 6th month, 1 year and 2 years after surgery was 84.4%, 79.5%, and 71.5%, respectively. The overall mortality rate was 20.6% (seven patients) with a median follow-up of 10 months (0-41). The estimated survival rate on the 6th month, 1 year, and 3 years after surgery was 83.8%, 79.4%, and 79.4 respectively. Bicuspid aortic valve (p = .017) (HR (95% CI) = 0.130 (0.025-0.695) and hammock mitral valve (p = .038) (HR (95% CI) = 11,008 (1,146->100) were associated with mortality. CONCLUSION: The presence of a bicuspid aortic valve hammock mitral valve might have an effect on negative effect on the outcome.

Coronary artery-pulmonary artery fistula in pulmonary atresia with ventricular septal defect: report of two surgical cases.

Pulmonary atresia with ventricular septal defect is a complex congenital cardiac anomaly. The blood is supplied to the lungs through a patent ductus arteriosus, a major aortopulmonary collateral artery, or in very rare cases from a coronary artery-pulmonary artery fistula. We present two cases with coronary artery-pulmonary artery fistula which underwent surgical intervention. In our first patient, the main pulmonary artery was supplied from the left main coronary artery. In the second patient, the right pulmonary artery originated from the left main coronary artery and continued to the right lung posteriorly to the aorta, while the left pulmonary artery originated from the patent ductus arteriosus. The difference in our cases is that the coronary artery pulmonary artery fistulas behave like major aortopulmonary collateral arteries originating from the coronary arteries. These fistulas were the main source of pulmonary blood flow.

Anomalous left coronary artery from the pulmonary artery repair outcomes: Preoperative mitral regurgitation persists in the follow-up.

OBJECTIVE: This study aims to present the midterm outcomes of surgical correction of the anomalous left coronary artery from the pulmonary artery (ALCAPA) with a focus on mitral regurgitation (MR). METHODS: Medical records of 36 ALCAPA patients who underwent surgery in a single center were retrospectively reviewed. RESULTS: There were one (2.7%) neonate, 19 (52.7%) infants, 15 (41.6%) children, and 1 (2.7%) adult patient operated. Coronary reimplantation, tube reconstruction, and coronary artery bypass surgery techniques were performed in 29 (80.5%), 6 (16.6%), and 1 (2.7%) patient, respectively. Sixteen (44.4%) patients had a low ejection fraction and 13 (36.1%) patients had at least moderate MR preoperatively. None of our patients underwent a concomitant mitral surgery during the initial repair. Three and two patients died in the early and late postoperative period, respectively. Two patients underwent reoperation due to MR and pulmonary stenosis, separately. Preoperative MR was the only factor associated with at least moderate MR at the final follow-up (p < .01) and the presence of preoperative moderate or over-moderate MR estimated at least moderate MR at the final follow-up with 100% sensitivity and 80% specificity. CONCLUSION: Although the mitral valve was not repaired in the first operation, the reoperation rate is low. However, a moderate or higher preoperative MR predicted MR at the last follow-up. Performing annuloplasty in such patients can be a strategy to be researched.

The choice of palliative arterial switch operation as an alternative for selected cases in a single center: Experience and mid term results.

INTRODUCTION AND OBJECTIVE: There are various management options for newborns with single ventricle physiology, ventriculoarterial discordance and subaortic stenosis, classically involving the early pulmonary banding and aortic arch repair, the restricted bulboventriculer foramen enlargement or the Norwood and the Damus-Kaye-Stansel procedures. The aim of this study is to evaluate our preferred technique and comment on the midterm results of our clinical experience with palliative arterial switch operation (pASO) for a certain subset of patients. METHOD: We hereby retrospectively evaluate the charts of patients who went through pASO, as initial palliation through Fontan pathway, starting from 2014 till today. RESULTS: Ten patients underwent an initial palliative arterial switch procedure. Eight of 10 patients survived the operation and discharged. Seven of 10 patients completed Stage II and 1 patient reached the Fontan completion stage and the other six of ten (6/10) patients are doing well and waiting for the next stage of palliation. There are two mortalities in the series (2/10) and one patient lost to follow-up (1/10). CONCLUSION: In our opinion, the pASO can be considered as an alternative palliation option for patients with single ventricle physiology, transposition of the great arteries and systemic outflow tract obstruction despite longer cross clamp times compared to other methods, but It not only preserves systolic and diastolic ventricular function, but also provides a superior anatomic arrangement for following stages.

High incidence of fever in patients after biointegral pulmonic valved conduit implantation.

OBJECTIVE: To describe our short- and medium-term outcomes using the BioIntegral pulmonic conduit. METHODS: Between August 2018 and September 2019, the BioIntegral pulmonic valved conduit was used for right ventricular outflow tract reconstruction in 48 patients. The data were retrospectively retrieved from the patient charts. RESULTS: The median age at surgery was 36 months (interquartile range [IQR] = 18-62 months). The diagnoses were pulmonary atresia-ventricular septal defect in 28 patients, absent pulmonary valve in four patients, truncus arteriosus in six patients, TGA-VSD-PS in five patients, conduit stenosis in three patients, and left venticular outflow tract obstruction requiring a Ross operation in two patients. In the postoperative short-term follow-up, 15 patients out of 48 had a high fever. Of these, 12 patients had concomitantly elevated C-reactive protein levels. There were no patients with blood culture positivity. The median postoperative length of hospital stay was 14 days (IQR = 8-21 days). The overall mortality was 4% in two patients, one died of right ventricular failure and multiple organ failure and one died of pulmonary embolism. The two patients who died were not among the 15 patients with fever. However, four patients with fever underwent balloon angioplasty for conduit restenosis in their medium-term follow-up. CONCLUSION: There was a high incidence of fever and adverse outcomes in the short-term postoperative follow-up of patients in whom the BioIntegral pulmonic valved conduit was implanted. Caution is advisable in using these conduits until there is convincing evidence about the sterilization and storage standards of these grafts.

Ligation of patent ductus arteriosus through anterior thoracotomy in preterm infants: a 10-year experience.

OBJECTIVE: The aim of this study is to report on the short-term and mid-term outcomes of preterm infants who underwent patent ductus arteriosus ligation through anterior mini-thoracotomy. METHODS: Data for 103 preterm infants who underwent patent ductus arteriosus clipping through an anterior mini-thoracotomy at the 2nd intercostal space between 2009 and 2019 were retrospectively reviewed. The patients were divided into two groups according to their weight at the time of surgery. The complications, morbidity, and mortality rates of each group were compared at postoperative day 30 and at the end of 1 year after surgery. RESULTS: During the operation, the median weight of the patients was 900 g (IQR 800-1125 g), the median age was 21 days (IQR 14.5-29 days). The lowest body weight was 460 g. In three patients (3%), there was intraoperative bleeding from the patent ductus arteriosus that required transition to median sternotomy. In one patient (1%) a residual patent ductus arteriosus that required reoperation was observed. Twelve patients (12%) died in the first 30 days postoperatively. Six patients (6%) died between the postoperative day 30 and 1 year. There was no statistically significant difference in the rates of mortality, morbidity, and complication between the groups. CONCLUSIONS: Based on our observations of over a hundred preterm infants with patent ductus arteriosus over a decade, ligation through anterior mini-thoracotomy is the main surgical procedure of choice for this patient group in our clinic. Our findings demonstrate the safety of this approach and we believe that it can be successfully replicated in other institutions.

Comparison of Doty and McGoon techniques for surgical reconstruction of congenital supravalvular aortic stenosis.

BACKGROUND: We aimed to compare the results of two surgical methods for the treatment of congenital supravalvular aortic stenosis. METHODS: From May 2004 to January 2020, 29 patients underwent surgical repair for supravalvular aportic stenosis in a single centre. The perioperative evaluation of the patients was retrospectively reviewed. RESULTS: Fifteen (51.7%) and 14 (48.2%) patients were treated with the Doty and the McGoon methods, respectively. The median age of our cohort was 4.5 (3.0-9.9) years. Ten (34.5%) patients had Williams-Beuren syndrome, and pulmonary stenosis was observed in 12 (41.3%) patients. The median follow-up time was 2.5 (0.7-7.3) years. On follow-up, five patients had residual stenosis with the McGoon technique and one with the Doty technique (p = 0.05). One patient died early in the post-operative period in the Doty group, and three patients were re-operated on due to restenosis in the McGoon group. Freedom from re-operation in the Doty group at 1, 3, 5, and 10 years was 100%. In the McGoon group, freedom from re-operation rates at the 1-, 3-, and 7-year follow-up were 100, 88.9, and 44.4%, respectively (p = 0.08). CONCLUSION: Our results with both surgical techniques suggest that supravalvular aortic stenosis can be treated with good results. The Doty method provided better relief for the supravalvular aortic segment, considering the residual stenosis and the re-operation rates.

The impact of COVID-19 pandemic on congenital heart surgery practice: An alarming change in demographics.

BACKGROUND: The aim of this study is to investigate the effect of COVID-19 outbreak on congenital cardiac surgery practice in a single center. METHODS: The first case of COVID-19 in our country was seen on March 11th, 2020. The patients operated between March 11th, 2019-and March 10th, 2020 were taken as the pre-COVID group, and those operated between March 11th and May 11th, 2020 were taken as the COVID group. The data was retrospectively collected, and the two periods were compared. RESULTS: Monthly average number of cases which was 52 patients/month (626 patients in 12 months) before COVID decreased to 35 patients/month (70 patients in 2 months) during COVID periods (P < .01). During the pre-COVID period the median postoperative length of hospital stay was 3 (IQR: 1-5) days. During the COVID period, this decreased to 1 (IQR: 1-3) day (P < .01). During the pre-COVID period, the hospital expenses of 17% (8/47) of the foreign nationals were covered by their homeland. The remaining 83% (39/47) were paid from the asylum seekers' fund. The proportion of foreign nationals operated significantly decreased during the COVID period ([7%; 47/632 vs 1%; 1/70]; P = .04). No significant difference was observed in terms of STAT mortality scores and categories and postoperative results of the operations performed between the two periods. CONCLUSIONS: Congenital cardiac surgery practice can be safely maintained with restricted case volume during the pandemic period. It is alarming that patients in the deprived areas cannot access pediatric cardiac surgery and possibly other health services because of closure of the borders between countries.

Early outcomes of usage of cryoFORM® probe for cryomaze procedure in congenital heart surgery.

OBJECTIVES: This study aimed to evaluate the early outcomes of patients who underwent a concomitant therapeutic maze procedure for congenital heart surgery. MATERIALS AND METHODS: Between 2019 and 2020, eight patients underwent surgical cryoablation by using the same type of cryoablation probe. RESULTS: Three patients had atrial flutter, two had Wolf-Parkinson-White syndrome, two intra-atrial reentrant tachycardia, and one had atrial fibrillation. Four patients underwent electrophysiological study. Preoperatively, one patient was on 3, two were on 2, five were on 1 antiarrhythmic drug. Six patients underwent right atrial maze and two underwent bilateral atrial maze. Five out of six right atrial maze patients underwent right atrial reduction. Nine different lesion sets were used. Some of the lesions were combined and applied as one lesion. In Ebstein's anomaly patients, the lesion from coronary sinus to displaced tricuspid annulus was delicately performed. The single ventricle patient with heterotaxy had junctional rhythm at the time of discharge and was the only patient who experienced atrial extrasystoles 2 months after discharge. Seven of the eight patients were on sinus rhythm. No patient needed permanent pacemaker placement. CONCLUSION: Cryomaze procedure can be applied in congenital heart diseases with acceptable arrhythmia-free rates by selecting the appropriate materials and suitable lesion sets. The application of cryomaze in heterotaxy patients can be challenging due to differences in the conduction system and complex anatomy. Consensus with the electrophysiology team about the choice of the right-left or biatrial maze procedure is mandatory for operational success.

Aortic translocation procedure: Early outcomes from a single center.

BACKGROUND/AIM: The data on the Nikaidoh procedure is limited in the literature. This study presents our experiential findings with aortic translocation (Nikaidoh) procedure and its modifications. METHODS: We retrospectively reviewed the data of all the patients who underwent aortic translocation in our institution. RESULTS: Between September 2014 and November 2018, eight patients underwent aortic translocation surgery. The median age was 3.2 years (14 months and 9 years). The diagnosis was transposition of the great arteries (TGA), ventricular septal defect (VSD), and left ventricular outflow tract obstruction (LVOTO) in five patients (63%); double-outlet right ventricle (DORV), VSD, and LVOTO in two patients (25%) and DORV and remote VSD in one patient (12%). Additionally, two patients had tricuspid straddling. In terms of surgical procedures, six patients underwent standard Nikaidoh procedures, while one patient underwent double root translocation and one patient underwent a half-turned truncal switch operation. Hospital mortality was recorded for one patient (12.5%). Median intensive care and hospital stay duration was 3 days (interquartile range [IQR]: 2-5 days) and 11 days (IQR: 8-17 days). Median follow-up duration was 10 months (IQR: 10-24 months). One of the patients, who underwent standard Nikaidoh procedure, died in the late follow-up. CONCLUSIONS: Aortic translocation procedure is an option for TGA, VSD, and LVOTO patients with a distal VSD or tricuspid straddling and DORV patients with a small VSD. Avoiding the use of a conduit through modifications, such as double root translocation and half-turned truncal switch operation, may decrease the risk of long-term recurrent interventions.

Closure of a patent ductus arteriosus in pre-term neonates using a left anterior mini-thoracotomy.

OBJECTIVES: We present a surgical technique for closure of a patent ductus arteriosus (PDA) in pre-term neonates. Left anterior mini-thoracotomy is a surgical procedure that consists of an anterior mini-thoracotomy (~1.5 cm) below the clavicle, clipping the PDA, and closing the thorax without a tube thoracotomy. METHODS: Using this method between 2009 and 2012, we performed PDA closure in 32 pre-term neonates. Logistic regression analysis of potential risk factors for mortality was determined. RESULTS: The mean weight of the patients at the operation was 822.81 ± 24.59. The mean age at the operation was 28.97 ± 2.20 days. No surgery-related mortalities occurred. Four short-term mortalities occurred after the operation (12.5%) before the patients could be discharged. Three were due to sepsis, and one was due to necrotizing enterocolitis. According to the linear regression analysis, no other variables were found to be statistically significant for predicting mortality. A negative linear correlation was found between the weight of the patients at the operation and extubation time (p = 0.39; r = -0.39). CONCLUSION: The surgical outcome of anterior mini-thoracotomy for PDA closure in neonates is compatible with good results. This technique may be advantageous for extremely low birth weight infants and results in less traction on the lungs.

Results for surgical closure of isolated ventricular septal defects in patients under one year of age.

BACKGROUND: This study evaluated the outcomes of patients undergoing surgical repair of isolated ventricular septal defect (VSD) in the first year of life with particular attention to age and severity of pulmonary hypertension (PH). METHODS: Between July 1, 2002 and May 31, 2012, 282 patients aged less than one year underwent isolated VSD closure at a median age of five months (range, 21 days to 1 year) and a median weight of 5.3 kg (range, 2.9 to 12.5 kg). Patients were divided into three groups according to the age at surgery (0-3, 3-6, and 6-12 months), and groups were compared in regard to severity of PH associated with morbidity and mortality. RESULTS: Four (1.4%) early and four (1.4%) late deaths occurred postoperatively. All mortalities were patients with severe PH, aged between 3 and 12 months. Although hemodynamic studies revealed a higher incidence of persistent postoperative PH in patients above three months of age, there was no statistically significant difference in morbidity associated with prolonged mechanical ventilation, and long intensive care unit and hospital stays between age-related groups. CONCLUSION: In this study, the incidence of mortality was higher in patients over three months of age undergoing repair of isolated VSD; the data suggest that the mortality may be decreased in patients with severe PH who were operated on earlier in life. We conclude that in infants with severe PH, early surgical repair (less than three months) of isolated VSDs is strongly advised to achieve more favorable results.

Experiences in Patent Ductus Arteriosus Closure of Preterm Infants Transported to Another Center for Surgical Ligation.

OBJECTIVE: The timing of surgical patent ductus arteriosus ligation in preterm infants remains controversial. Early ligation (<2 or 3 weeks of life) benefits preterm infants with a lower incidence of bronchopulmonary dysplasia and necrotizing enterocolitis. We present patent ductus arteriosus ligation experience in premature infants who had been transported for closure to an outside centre other than the hospital they were hospitalized. MATERIALS AND METHODS: We retrospectively evaluated 17 consecutive patients who had undergone surgery of premature infant patent ductus arteriosus closure during the period of March 2009-December 2020. Patent ductus arteriosus had been clipped in 17 patients. RESULTS: The median birth weight and age were 930 g and 28 gestation weeks, respectively. The birth age of the sub-groups were A: ≤28 weeks and B: >28 weeks and birthweight were group I: ≤800 g and group II: >800 g. The median day of PDA ligation wasa 20 days, and patients with birthweight ≤800 g were ligated later than patients weighing >800 g. Two patients had intracranial hemorrhage, 6 had bronchopulmonary dysplasia, and 2 were dead. We found that exposure to large patent ductus arteriosus and low birth age in preterm babies was associated with longer hospitalization duration, preoperative mechanical ventilation time, and sepsis. CONCLUSION: Infants exposed to moderate-to-large patent ductus arteriosus requiring intubation and resistant to medical therapy for more than 2 weeks should have surgical ligation as soon as possible.

Experience of 500 cardiovascular magnetic resonance imaging and systematic analysis of cases.

OBJECTIVE: Cardiovascular magnetic resonance imaging (MRI) is a widely accepted reference imaging technique in routine cardiology clinics in many centers due to its advantages in providing preferable functional, morphologic information. However, there is little information about national experience in clinical application and findings of cardiovascular MRI. The objective of this study was to demonstrate the clinical and demographic characteristics of patients admitted to our cardiac imaging department. METHODS: A total of 500 cardiovascular MRI examinations performed between 2016 and 2019 were enrolled in this retrospective study. Clinical indications, demographic, and cardiovascular MRI findings of the patients were retrospectively evaluated. RESULTS: Five hundred patients (M/F=301/199) were included in this retrospective, single center study. The majority of the examinations were performed for the assessment of congenital heart disease (n=254, 50.8%). The other indications were for myocardial disease (n=160, 32%), cardiac mass (n=44, 8.8%), valvular heart disease (n=20, 4%), magnetic resonance angiography (n=12, 2.4% for aorta and pulmonary artery [n=9, 1.8%] and for coronary arteries [n=3, 0.6%]), and vasculitis (n=7, 1.7%), pericardial disease (n=3, 0.6%). Minor complication was seen during the contrast agent injection in three patients (0.06%). CONCLUSION: Cardiovascular MRI is a reliable and accurate imaging tool in identifying the various cardiac pathology with widely accepted use in the clinical area. Our single-center experience of 500 cases demonstrates the varieties of clinical indications in daily practice that may contribute to the national data pool.

Successful surgical repair of critical ascending and descending aortic aneurysms at nine-year intervals in Wiskott-Aldrich syndrome: A case report.

Wiskott-Aldrich syndrome is an uncommon X-linked inherited disorder related to primary immunodeficiency, infections, eczema, and thrombocytopenia. A 21-year-old male patient with this syndrome underwent descending aortic aneurysm repair at the age of 12. The patient had ascending aortic aneurysm with aortic valve regurgitation and surgical aortic root replacement was performed. To the best of our knowledge, this is the first case with Wiskott-Aldrich syndrome operated due to aneurysms development in different segments of the thoracic aorta in both childhood and young adult periods.