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.

Surgical management of cardiac cystic echinococcosis in a paediatric patient: a case report.

Cystic echinococcosis, a zoonotic parasitic disease, is endemic to many countries worldwide. This slowly progressing disease is seen rarely in the paediatric age group. In terms of cyst localization, cardiac involvement is infrequent. We report the case of a successful surgical and medical management of a paediatric hydatid disease patient with involvement of the heart.

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.

Redirection of Persistent Left Superior Vena Cava Using a Turned-In Left Atrial Appendage.

Persistent left superior cava mostly drains into the right atrium via the coronary sinus. It rarely drains into the left atrium. Extracardiac and intracardiac repair techniques have been described for the repair of persistent left superior vena cava draining into the left atrium. Herein, we report the successful application of a new intracardiac repair technique by using a turned-in left atrial appendage in a 3-year-old male patient with a persistent left superior vena cava draining into the left atrium.

Single-center outcomes after surgical creation of aortopulmonary window in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.

Background: The aim of this study is to present our experience regarding the creation of an aortopulmonary window as the initial palliative procedure. Methods: Between February 2016 and February 2021, a total of eight patients (3 males, 5 females; median age: 2 months; range, 0.7 to 6 months) who underwent aortopulmonary window creation were retrospectively analyzed. Data collection was performed by review from our institution. Results: There was no occurrence of early or late mortality in any patient. The median postoperative duration of mechanical ventilation and length of hospital stay were five and eight days, respectively. No postoperative reperfusion injury or severe pulmonary overcirculation was observed in any of the patients. Four patients achieved complete repair with unifocalization of the major aortopulmonary collateral arteries, one patient had a second procedure, and the remaining three patients waited for complete repair. The median right ventricle-toaortic pressure ratio after complete repair was 0.6 (range, 0.4 to 0.7). The median follow-up after complete repair was 1.4 (range, 0.9 to 2.8) years, and the median follow-up period for all survivors was 2.7 (range, 0.9-5.8) years. Conclusion: Our study results suggest that aortopulmonary window operation can be safely performed in selected patients with good early and mid-term outcomes. Although the central pulmonary arteries are very small, half of the patients underwent complete repair and achieved acceptable right ventricle-to-aortic pressure ratios. Patient selection criteria and early postoperative interventions are of utmost importance to prevent postoperative pulmonary overcirculation.

Effect of Atrioventricular Valve Repair on Multistage Palliation Results of Single-Ventricle Defects.

BACKGROUND: The presence of significant atrioventricular valve (AVV) regurgitation results in unfavorable conditions that affect the success of single-ventricle (SV) multistage palliation. We report our institution's AVV repair experience. METHODS: We examined incidence of AVV repair in 603 infants who underwent initial SV palliative surgery from 2002 to 2012. We explored patients' characteristics and anatomic and operative details associated with death, transplantation, and AVV reoperation. RESULTS: AVV repair was performed in 60 patients during first-stage (n = 10), Glenn (n = 27), and Fontan (n = 23). Median age at AVV repair was 6.9 months (interquartile range, 4.2-24.1 months). Underlying SV anomaly was hypoplastic left heart syndrome (n = 30), heterotaxy (n = 15), and other (n = 15). The AVV was tricuspid (n = 34), mitral (n = 6), or common (n = 20). Preoperatively, all patients had AVV regurgitation moderate or higher, and 7 (12%) had ventricular dysfunction moderate or higher. After the repair, AVV regurgitation was none/trivial in 21 (35%), mild in 21 (35%), and moderate or higher in 17 (30%). Competing risks analysis showed that 10 years after AVV repair, 18% of patients had undergone AVV reoperation, 26% had died or undergone transplantation, and 56% were alive without subsequent reoperation. Transplant-free survival was 38%, 65%, and 100% for AVV repair at first-stage, Glenn, or Fontan (P = .0011), respectively, and was 74%, 83%, and 56% for tricuspid, mitral, and common AVV repair (P = .344), respectively. Factors associated with transplant-free survival were timing of AVV repair, underlying SV anomaly, and systemic ventricle function. CONCLUSIONS: AVV repair at the first-stage operation and reduced systemic ventricle function are associated with poor outcomes. In those high-risk patients, different approaches that involve initial palliation mode, timing of AVV repair, or listing for transplantation might be warranted.

Single-Stage Fontan Operation in a Patient With Coronary Sinus Ostium Atresia.

An eight-year-old boy with tricuspid atresia was found to have atretic coronary sinus ostium during cardiac catheterization. Single-stage extracardiac fenestrated Fontan operation was performed with surgical unroofing of the coronary sinus into the left atrium to avoid the risk of cardiac congestion.

Intraoperative Flow Study Predicted the Postoperative Pulmonary Artery Pressure in the Bidirectional Glenn Operation.

BACKGROUND: The aim of this study was to evaluate the predictability of postoperative pulmonary artery pressure (PAP) using intraoperative flow study in patients undergoing bidirectional Glenn operation. METHODS: Patients who underwent Glenn operation under cardiopulmonary bypass (CPB) were included in the study. During the operation, after the completion of additional procedures under CPB, an intraoperative flow study was performed prior to Glenn anastomosis. After the completion of bidirectional Glenn, the patient was separated from the CPB and PAP was measured. The relationship between this pressure and flow study measurement was analyzed. RESULTS: Nine patients who underwent bidirectional Glenn operation with additional procedures under CPB between July 2018 and January 2019 were included in the study. The median PAP was 9 mm Hg (interquartile range [IQR]: 7-10 mm Hg) in the flow study and 10 mm Hg (IQR: 8-11 mm Hg) after CPB, and the median difference between these pressures was 1 mm Hg (IQR: 1-3 mm Hg). There was a strong correlation between these two measurements (r = 0.732; P = .025). CONCLUSION: The results of this study show that PAP after the Glenn procedure can be estimated using an intraoperative flow study. We believe that this method may be useful in intraoperative decision-making for Glenn operation in single ventricular patients who require extensive pulmonary artery (PA) reconstruction due to limited PA development, branch PA stenosis, or nonconfluent PAs. Also, this method can be used as a sort of intraoperative pulmonary resistance reversibility study in patients with high preoperative pulmonary vascular resistance due to surgically correctable pulmonary venous hypertension.

Management outcomes of primary pulmonary vein stenosis.

OBJECTIVES: Primary pulmonary vein stenosis (PPVS) is increasingly diagnosed in children with no prior pulmonary vein intervention history, and management is challenging. We describe characteristics of patients who underwent surgical repair of PPVS at our center, and examine factors associated with treatment failures. METHODS: A retrospective review of all patients who underwent surgical intervention for PPVS (2002-2016) was completed. Patients who had undergone prior cardiac surgery involving the pulmonary veins or atrial switch were excluded. Regression analyses were performed to examine characteristics, PPVS features, including severity score, and surgical details associated with treatment failures. RESULTS: Thirty-four children underwent initial surgical intervention for PPVS. Median age was 8.9 months (interquartile range, 5.9-18.4 months). Most patients (n = 31; 91%) had unilateral pulmonary vein involvement and the median PPVS severity score was 3.5 (interquartile range, 3-5). On competing risk analysis, 1 year following surgical repair, 9% of patients had died, 14% had undergone reintervention, and 77% were alive without reintervention; at 5 years the numbers were 9%, 30%, and 61%, respectively. Factors associated with mortality included bilateral disease and PPVS severity score >6. Bilateral disease and PPVS severity score >5 were associated with reintervention risk. CONCLUSIONS: Multidisciplinary management strategy is required for PPVS. Despite satisfactory early repair, patients continue to be at risk for recurrence and subsequent mortality, especially those with extensive primary involvement. The disappointing results underscore the need for multi-institutional collaborations to better understand this complex disease, establish management and follow-up protocols, and explore investigational treatment modalities that could modify the unfavorable outcome of this uncommon and challenging disease.

Ross operation early and mid-term results in children and young adults.

OBJECTIVE: The Ross procedure has been cited as the procedure of choice for young patients requiring aortic valve replacement. However, potential for reintervention requirement in both left and right ventricular outflow tracts can be a source of concern. The aim of the present study was to describe our experience with this procedure. METHODS: A retrospective chart review of all the patients who underwent the Ross procedure in a single institution was performed. National death registry records were used for late mortality. RESULTS: Eighteen Ross procedures between May 2003 and May 2018 were performed. The median age of the cohort was 15 [interquartile range (IQR): 12-18] years. The pulmonic conduit was a homograft in 11 patients, Labcor in 5 patients, Contegra in 1 patient, and Medtronic Freestyle Valve in 1 patient. There were three early deaths. The median follow-up of 15 hospital survivors was 11 (IQR: 3-14) years. Any late mortality was not observed. In the two surviving patients with infective endocarditis, there was no recurrent infective endocarditis. Freedom from reintervention was 80% at 8 years and onward. Any risk factors associated with reintervention could not be identified. However, freedom from autograft dilatation at 10 years was 45%. CONCLUSION: Autograft failure is a potential problem in the long-term follow-up of Ross patients. Freedom from reintervention was satisfactory, and the type of pulmonic conduit did not affect the mid-term outcomes. In patients with infective endocarditis, the Ross procedure has a low recurrence rate, but it might have an increased risk of mortality.

Analysis of congenital heart surgery results: A comparison of four risk scoring systems.

BACKGROUND: This study aims to evaluate the surgical results of our clinic according to presumption systems of Risk Adjustment in Congenital Heart Surgery, Aristotle Basic Complexity score, Aristotle Comprehensive Complexity score, and Society of Thoracic Surgeons and European Association for Cardiothoracic Surgery mortality categories and to compare the efficiency of these systems in predicting morbidity and mortality. METHODS: In the study, classification and the risk scoring were performed with the four different systems for 1,950 patients (1,038 males, 912 females; mean age 5.5 months; range, 1 day to 18 years) who were administered congenital heart surgery between 1 October 2012 and 31 December 2016. The hospital mortality and morbidity were calculated for each category from the four models. The discriminatory ability of the models was determined by calculating the area under the receiver operating characteristic curve and the receiver operating characteristic curves of the four models were compared. RESULTS: Median weight of the patients was 7.2 kg (range, 1.8-80 kg). Among the patients, 53% were males and 47.5% were younger than one year of age. Of totally 1,950 operations, mortality was observed in 149 (7.6%) and morbidity was observed in 541 (27.7%). Areas under the receiver operating characteristic curve for mortality were 0.803, 0.795, 0.729, and 0.712 for the Society of Thoracic Surgeons and European Association for Cardiothoracic Surgery mortality categories, Aristotle Comprehensive Complexity, Risk Adjustment in Congenital Heart Surgery, and Aristotle Basic Complexity scores, respectively. Areas under the receiver operating characteristic curve for morbidity were 0.732, 0.731, 0.730, and 0.685 for the Society of Thoracic Surgeons and European Association for Cardiothoracic Surgery mortality categories, Risk Adjustment in Congenital Heart Surgery, Aristotle Comprehensive Complexity, and Aristotle Basic Complexity scores, respectively. CONCLUSION: Society of Thoracic Surgeons and European Association for Cardiothoracic Surgery mortality categories, Risk Adjustment in Congenital Heart Surgery, Aristotle Basic Complexity, and Aristotle Comprehensive Complexity score systems were effective in predicting the morbidities and mortalities of patients who underwent congenital heart surgery and evaluating the performance of the surgical centers. Society of Thoracic Surgeons and European Association for Cardiothoracic Surgery mortality categories were on the forefront due to high feasibility and performance. Aristotle Basic Complexity score system had the lowest performance. Combinations of systems will provide the most benefit during evaluation of results.

The effect of intravenous iloprost on pulmonary artery hypertension after paediatric congenital heart surgery.

OBJECTIVES: To investigate the effects of intravenous iloprost on pulmonary artery hypertension (PAH) in infants undergoing congenital heart surgery. METHODS: In this prospective, randomized study, the study group (n = 15) received a continuous infusion of iloprost (2.0 ng/kg/min) that was delivered immediately after weaning from cardiopulmonary bypass and continued for 72 h postoperatively. Patients in the control group (n = 12) were managed conventionally. The groups were compared in terms of postoperative data, including systolic and mean pulmonary artery (PA) pressures, PA/systemic pressure ratio, lactate level, PAH crisis, ventilation time, reintubation and lengths of intensive care unit (ICU) and hospital stay. Transthoracic echocardiography was used to assess PA pressures at 1 day, 7 days and 30 days after surgery. RESULTS: No mortality occurred. PAH crisis occurred in 2 (16.6%) patients in the control group and 4 (26.7%) patients in the study group (P = 0.53). Postoperative PA pressures and PA/systemic pressure ratios were similar between the groups (P > 0.05). The durations of ICU (P = 0.40) and hospital (P = 0.98) stays were similar between the groups. Echocardiographic studies demonstrated a significant decrease in postoperative PA pressures in the control (P = 0.001) and study (P = 0.0001) groups. However, no significant change was observed between the groups (P > 0.05). The Tukey multiple comparison test showed a significant decrease in PA pressures at each follow-up in both groups (P < 0.05). CONCLUSIONS: Intravenous iloprost demonstrated no additional benefit over the conventional management of infants with PAH after repair of intracardiac defects. Clinicians may prefer other alternative agents in infants with a high risk of PAH crisis.

Extracorporeal cardiopulmonary resuscitation for breath-holding spells followed by cardiac arrest due to left main coronary artery stenosis.

Non-syndromic congenital supravalvular aortic stenosis (SVAS) leads to ventricular hypertrophy and increased oxygen consumption, and when combined with other factors reduces coronary blood flow, potentially resulting in myocardial ischemia and sudden cardiac death. While the anatomic obstruction of coronary circulation is as common in non-syndromic SVAS as in Williams syndrome, it often remains unacknowledged. Extracorporeal membrane oxygenation (ECMO) is an elective procedure that can be used to support patients with cardiac arrest during diagnosis as a way to reduce cardiopulmonary load in preparation for surgery or further treatment. In this report, we describe the rare case of an infant with severe SVAS and mild valvular pulmonary and left main coronary artery stenosis, as well as breath-holding spells. After multiple cardiac arrests, the infant underwent diagnostic catheter angiography on ECMO and had the pathology surgically corrected.