Case Report: Neo-ostium creation with saphenous vein in a patient with anomalous origin of the right coronary artery.

Anomalous origin of the right coronary artery from the left sinus of Valsalva is a rare congenital anomaly. Most patients may be asymptomatic, but some may experience major cardiac events such as syncope, arrhythmias, and sudden cardiac death. We present a 16-year-old patient, who had several syncopes, with anomalous origin of the right coronary artery from the left coronary sinus, with an intramural and interarterial course between the pulmonary artery and the aorta. We describe a new surgical procedure of neo-ostium creation with a saphenous vein.

The effect of extracorporeal membrane oxygenation on neurodevelopmental outcomes in children after repair of congenital heart disease: A pilot study from Turkey.

Background: Extracorporeal membrane oxygenation (ECMO) is widely used after congenital heart surgery. The purpose of this study is to analyze the neurodevelopmental (ND) outcomes in patients who receivedECMO support after congenital cardiac surgery. Methods: Between January 2014 and January 2021, 111 patients (5.8%) receivedECMO support after congenital heart operations, and 29 (26,1%) of these patients were discharged. Fifteen patients who met the inclusion criteria were included. A propensity score matching (PSM) analysis model was established using eight variables (age, weight, sex, Modified Aristotle Comprehensive Complexityscores, seizures, cardiopulmonary bypass duration, number of operations, and repair method) with 1:1 matching. According to the PSM model, 15 patients who underwent congenital heart operations were selected as the non-ECMO group. The Ages & Stages Questionnaire Third Edition (ASQ-3) was used for ND screening;it includes communication, physical skills (gross and fine motor), problem-solving, and personal-social skills domains. Results: There were no statistically significant differences between the patients' preoperative and postoperative characteristics. All patients were followed up for a median of 29 months (9-56 months). The ASQ-3 results revealed that communication, fine motor, and personal-social skills assessments were not statistically different between the groups. Gross motor skills (40 vs. 60), problem-solving skills (40 vs. 50), and overall scores (200 vs. 250) were better in the non-ECMO patients (P = 0.01, P = 0.03, and P = 0.03, respectively). Nine patients (%60) in the ECMO group and 3 patients (%20) in the non-ECMO group were with neurodevelopmental delay (P = 0,03). Conclusion: ND delay may occur in congenital heart surgery patients who receivedECMO support. We recommend ND screening in all patients with congenital heart disease, especially those who receivedECMO support.

Outcomes of Extracorporeal Membrane Oxygenation in Patients After Repair of Congenital Heart Defects.

Extracorporeal membrane oxygenation (ECMO) is widely used after congenital heart surgery. The purpose of this study is to analyze the factors influencing mortality and morbidity in patients who require ECMO support after congenital cardiac surgery. All 109 patients (5.8% of total cases) who underwent ECMO support after congenital heart surgery between January 2014 and 2021 were included in this single-center study. The mean age was 10.13 ± 20.55 months, and the mean weight was 6.41 ± 6.79 kg. 87 (79.8%) of the patients were under 1 year of age. A total of 54 patients (49.5%) were weaned successfully from ECMO support, and 27 of them (24.8%) were discharged. The childhood age group had the best outcomes. Seventy-seven percent of the children were weaned successfully, and 50% were discharged. 69 patients (63.3%) had biventricular physiology; weaning and survival outcomes were better than single ventricle patients (P-value 0.002 and < 0.001, respectively). Low cardiac output (n = 49; 44.9%) as an ECMO indication had better outcomes than extracorporeal cardiopulmonary resuscitation (n = 31; 28.4%) (P = 0.05). Most of the patients had ≥ 4 Modified Aristotle Comprehensive Complexity (MACC) levels, and higher MACC levels were associated with a higher mortality rate. The most common procedure was the Norwood operation (16.5%), with the worst outcome (5.5% survival). Bleeding and renal complications were the most common complications affecting outcomes. Results were more satisfactory in patients with biventricular repair, childhood, and lower MACC levels. Early initiation of ECMO in borderline patients without experiencing cardiac arrest or multiorgan failure may improve outcomes.

Analysis of outcomes in patients with abnormal laterality undergoing congenital heart surgery.

OBJECTIVE: Laterality anomalies are almost always associated with severe cardiac anomalies. Demographic properties, type of the procedures, associated anomalies, and early and mid-term prognosis of four types of laterality anomalies were analysed. METHODS: A total of 64 consecutive patients with laterality anomalies were enrolled between July 2014 and July 2020. We grouped the patients as situs solitus dextrocardia (SSD) (n = 12; 18.7%); situs inversus (SI) (n = 16; 25%); right atrial isomerism (RAI) (n = 29; 45.3%); and left atrial isomerism (LAI) (n = 7; 10.9%). TAPVC was only present in the RAI group (31%). Incidence of mitral or tricuspid atresia was higher in the SSD group (25%). All the patients were followed up with a mean of 19.06 ± 17.6 (0.1-72) months. RESULTS: Early postoperative mortality was 17 patients, among 107 procedures (15.8%). Twelve patients were in the neonatal period. All ten patients survived after isolated ductal stenting. Fourteen of the deaths were in the RAI group (48.3%). The 3-year survival rates were 85% in LAI, 78.7% in SI, 55.8% in SSD, and 38% in RAI groups. According to the multivariable Cox regression model, mechanical ventilation, kidney injury, RAI, and complex surgery in the neonatal period were independent risk factors for early mortality. CONCLUSION: Laterality anomalies are one of the most challenging patients who commonly had univentricular physiology. The most prevalent anomaly was RAI, and RAI had the worst outcome and survival. Ductal stent is an acceptable first intervention during the neonatal period in suitable patients. Complex procedures may carry a high risk of death in the neonatal period.

Management of Interrupted Aortic Arch With Associated Anomalies: A Single-Center Experience.

OBJECTIVES: Interrupted aortic arch (IAA) includes a broad spectrum of associated anomalies. In this study, we present our surgical management and patient-specific decisions regarding IAA anomalies with early- and mid-term outcomes. METHODS: The medical records of 25 patients undergoing IAA repair between 2014 and 2019 were retrospectively reviewed. Sixteen patients had type B (64%) interruptions, 7 had type A (28%) interruptions, and 2 had type C (8%) interruptions. Fourteen patients had an isolated ventricular septal defect, and 3 of them had associated left ventricular outflow tract obstruction. Other associated anomalies were functional single ventricle (n = 5), Taussig-Bing anomaly (n = 3), aortopulmonary window (n = 1), multiple ventricular septal defects (n = 1), and truncus arteriosus with dextrocardia (n = 1). The initial operation age was 17.2 ± 14 (range: 1 - 60) days. RESULTS: Single-stage total repair was performed for 15 patients. Six patients underwent aortic arch repair and pulmonary artery banding. Four patients with left ventricular outflow tract obstruction or who were premature underwent the hybrid procedure. The aortic arch repair was performed in 16 cases (64%) by the anterior patch augmentation technique, in 3 cases (12%) by the reverse left subclavian artery flap technique, and in 3 cases (12%) by direct end-to-end anastomosis. Postoperative early mortality occurred in 4 (16%) patients, and sternal closure was delayed in 13 (52%) patients. Three patients who underwent a hybrid procedure due to left ventricular outflow tract obstruction underwent biventricular repair 8 to 13 months later. Eight patients (38%) required reintervention due to arch restenosis during the follow-up period. The mean follow-up was 37.1 ± 21.7 months. CONCLUSION: Planning surgical treatment according to the characteristics of the patients and accompanying anomalies may improve the results.

Toward Routine Minimally Invasive Ventricular Septal Defect Closure Via Right Lateral Minithoracotomy.

Background: Improving the surgical results and recent advancement of transcatheter techniques for closure of ventricular septal defect (VSD) increased the demand for minimally invasive approaches. In this study, we analyzed the results of the patients who underwent VSD closure with right lateral minithoracotomy (RLMT). Methods: Between September 2014 and February 2021, 24 patients underwent minimally invasive VSD closure with RLMT. The median age of the patients was 16 months (range, 4-84 months). Fifteen patients (62.5%) were female. The median weight of the patients was 9.75 kg (range, 4.6-30 kg). The types of VSD were perimembranous in 19 patients, subaortic in three patients, inlet in one patient, and subpulmonic in one patient. Five patients had low-lying pulmonary stenosis in addition to VSD. Results: No perioperative death or major complication occurred during follow-up. All defects were repaired through RLMT. The median cardiopulmonary bypass time was 81 min (range, 44-163 min), and the aortic cross-clamp time was 65 min (range, 33-131 min). The median hospital stay was 6 days (range, 5-21 days). One patient had minimal (2 mm) residual left-to-right shunt. All families were satisfied with the cosmetic results during the follow-up. Conclusions: The RLMT method is a safe and effective alternative to standard median sternotomy for VSD closure and can be performed with favorable cosmetic and clinical results.

Outcomes of home mechanical ventilation with tracheostomy after congenital heart surgery.

OBJECTIVE: After congenital heart surgery, some patients may need long-term mechanical ventilation because of chronic respiratory failure. In this study, we analysed outcomes of the patients who need tracheostomy and home mechanical ventilation. METHODS: Amongst 1343 patients who underwent congenital heart surgery between January, 2014 and June, 2018, 45 needed tracheostomy and HMV. The median age of these patients was 6.4 months (12 days-6.5 years). Nineteen patients underwent palliation while 26 patients underwent total repair. Post-operative diaphragm plication was performed in five patients (11%). Median duration of mechanical ventilation before tracheostomy was 32 days (8-154 days). The patients were followed up with their home ventilators in ward and at home. Mean follow-up time was 36.24 ± 11.61 months. RESULTS: The median duration of ICU stay after tracheostomy was 27 days (range 2-93 days). Follow-up time in ward was median 30 days (2-156 days). A total of 12 patients (26.6%) were separated from the ventilator and underwent decannulation during hospital stay. Thirty-two patients (71.1%) were discharged home with home ventilator support. Of them, 15 patients (46.9%) were separated from the respiratory support in median of 6 weeks (1 week-11 months) and decannulations were performed. Total mortality was 31.1%. in which four patients are still HMV dependent. There was no significant difference for decannulation between total repair and palliation patients. CONCLUSION: HMV via tracheostomy is a useful option for the treatment of children who are dependent on long-term ventilation after congenital heart surgery although there are potential risks.

Successful Repair of Infantile Scimitar Syndrome with Contralateral Pulmonary Vein Stenosis in an Infant.

Infantile scimitar syndrome (SS) is a rare congenital heart disease and has high mortality. Guidelines have not been established, but surgery is indicated in symptomatic patients. Despite the various surgical approaches, outcomes continue to be disappointing. We present our surgical experience with an infantile SS patient who had stenotic pulmonary veins contralateral to the hypoplastic lung with complicated anatomy. There are few cases with this complex pathology in the literature. Moreover, our patient was the first transplant-free survivor with this complexity in the literature.

Successful repair of infantile scimitar syndrome with contralateral pulmonary vein stenosis in an infant

Abstract Infantile scimitar syndrome (SS) is a rare congenital heart disease and has high mortality. Guidelines have not been established, but surgery is indicated in symptomatic patients. Despite the various surgical approaches, outcomes continue to be disappointing. We present our surgical experience with an infantile SS patient who had stenotic pulmonary veins contralateral to the hypoplastic lung with complicated anatomy. There are few cases with this complex pathology in the literature. Moreover, our patient was the first transplant-free survivor with this complexity in the literature.

Evaluation of neonatal acute kidney injury after critical congenital heart disease surgery.

BACKGROUND: Acute kidney injury (AKI) is a common complication of congenital heart diseases (CHDs) after cardiac surgery. This study aimed to define the frequency and critical course, risk factors and short-term outcomes of AKI in postoperative CHD neonates. METHODS: Postoperatively followed term CHD newborn infants were enrolled in the study. Infants with congenital anomalies of the urinary tract and other major congenital anomalies were excluded. Neonatal modified KDIGO criteria were used to assess AKI. RESULTS: A total of 199 postoperatively followed newborn infants were included in the study. Acute kidney injury was detected in 71 (35.6%) patients. Of these patients, 24 (33.8%) were in stage 1, 14 (19.7%) in stage 2, and 33 (46.5%) in stage 3. Acute kidney injury occurred within the first week (median 1 day [IQR 1-2 days]) of cardiac surgery in 93% of the patients. The duration of invasive respiratory support and extracorporeal membrane oxygenation (ECMO) and mortality were significantly higher in stage 3 patients. Higher vasoactive-inotropic score (OR, 1.02; 95% CI, 1.0-1.04; p = 0.008) and receiving ECMO (OR, 7.9; 95% CI, 2.6-24.4; p = 0.001) were associated with risk for the development of AKI. The mortality rate was 52.1% in the AKI (+) patients, and having AKI (OR 7.1; 95% CI, 3.5-14.18) was significantly associated with mortality. CONCLUSION: Acute kidney injury, a common early complication after critical neonatal CHD cardiac surgery, is associated with increased morbidity and mortality. Stage 3 AKI is associated with significantly higher mortality rates.

Management of aortic arch hypoplasia in neonates and infants.

OBJECTIVES: Surgical management of aortic arch hypoplasia (AAH) with associated intracardiac anomalies is a challenge in newborns. We reviewed the characteristics and outcomes of neonates and infants who underwent pulmonary artery banding concomitant to arch repair and single-stage total repair at our institution. METHODS: Medical records of 60 patients undergoing aortic arch reconstruction for AAH from 2014 to 2019 were retrospectively reviewed. Twenty-five patients were female (41.6%), and the age of the patients ranged from 4 to 120 days (median, 19.5 days). The patients were divided into two groups: Group 1 (23 patients) underwent pulmonary artery banding concomitant to arch repair, and Group 2 (37 patients) underwent single-stage total repair in addition to arch repair. All arch repair procedures consisted of an extended (to the midportion of the ascending aorta) patch aortoplasty. RESULTS: Postoperative early mortality occurred in 12 patients, eight in Group 1 (34.8%) and four in Group 2 (10.8%). There was an early survival advantage in Group 2 (p = .019). Recoarctation occurred in 13 cases (21.6%), and 11 (18.3%) of them required reintervention (balloon angioplasty: 7, reoperation: 4). On univariate analysis, risk factors associated with death were pulmonary artery banding (hazard ratio [HR], 0.44; confidence interval [CI], 0.09-2; p = .019), prematurity (HR, 4.67; CI, 1.34-16.18; p = <.001), preoperative mechanical ventilation support requirement (HR, 0.048; CI, 0.52-6.39; p = .048), and functional single ventricle (HR, 0.43; CI, 0.1-1.86; p = .006). The mean duration of follow-up was 21.9 ± 15.1 months, and there was no late death in either group. CONCLUSION: Single-stage repair of AAH with intracardiac pathologies has better results than palliation, according to survival rates and postoperative results. The use of the patch augmentation technique in AAH is valid and associated with an acceptable incidence of recurrent arch obstruction.

Comparison of different pulmonary valve reconstruction techniques during transannular repair of tetralogy of fallot.

BACKGROUND: Transannular patch, which results in pulmonary insufficiency (PI), is usually required during repair of tetralogy of fallot (TOF). In this study, we compared three types of pulmonary valve reconstruction techniques during transannular repair of TOF. METHODS: Between February 2014 and January 2018, 50 patients with TOF underwent primary repair with transannular patch. These patients were divided into three groups. In Group 1, (n = 15), a single gluteraldehyde-treated autologous pericardial patch (standard method) was reconstructed as monocusp. In Group 2, (n = 16) Nunn's bileaflet pulmonary valve reconstruction technique was used with pericardial patch. In Group 3, (n = 19), Nunn's bileaflet technique was performed with expanded polytetrafluoroethylene membrane. The outcomes of the patients and early and midterm competency of the pulmonary valves were analyzed. RESULTS: These techniques were significantly effective in early postoperative period. Freedom from moderate to severe PI were 73.3%; 100% and 89.4%, respectively. Mortality, duration of intensive care unit and hospital stay were similar between the groups. The mean follow-up period was 17.5 ± 13.0 (3-57) months. Freedom from moderate to severe PI decreased to 40%; 81.2% and 73.7%, respectively at the end of the follow-up period. Presence of moderate to severe PI was significantly higher in Group 1 (p = .018 between Groups 1 and 2, p = .048 between Groups 1 and 3). CONCLUSION: All three pulmonary valve reconstruction techniques provided competent pulmonary valves. Nunn's bileaflet technique had better outcome at midterm. It has a potential to delay right ventricular dysfunction at long-term.

Collateral dependent cerebral and coronary circulation in a newborn.

Association of interrupted aortic arch with aortic atresia as a variant of hypoplastic left heart syndrome (HLHS) is extremely rare and could not be compatible with life without a reliable source for cerebral and coronary blood flow. The patient with a large collateral artery between the descending aorta and the right subclavian artery is presented.

Early and midterm results of ductal stent implantation in neonates with ductal-dependent pulmonary circulation: a single-centre experience.

OBJECTIVE: We aimed to determine the early and midterm outcomes of ductal stenting in neonates with ductal-dependent pulmonary blood flow. METHODS: Between January, 2014 and July, 2018, 102 patients who underwent 115 cardiac catheterisation procedures for ductal stent implantation in our department were retrospectively reviewed. The age of the neonates ranged from 3 to 30 days (median: 11 days) and their weights ranged from 1.8 to 5.8 kg (mean, 2.8 ± 0.53 kg). Fifty-two patients had functional single ventricle and 50 had biventricular physiology. Thirty-one patients' weights were <2,500 g (30.3%). The patent ductus arteriosus was vertical in 60 patients (58.8%). The mean ductal length was 12.4 ± 4.1 mm (range, 7.8-23 mm), and the mean narrowest ductal diameter was 2.1 ± 0.7 mm (range, 1.2-3.4 mm). RESULTS: The technical success rate was 85.2%. Procedure-related mortality occurred in three patients (2.9%). After the procedure, the aortic oxygen saturation increased from a mean of 73.1 ± 6.2% to a mean of 90.4 ± 4.3% (p < 0.001), and the ductus diameter increased from a mean of 2.1 ± 0.7 mm to a mean of 4.2 ± 0.9 mm (p < 0.001). Either transcatheter or surgical reinterventions were required in 35 patients (34.3%) during the follow-up period after a median of 101 days (2-356 days). Thirty-three patients (32.3%) were bridged to surgical repair after a median of 288 days (163-650 days). The median duration of palliation with ductal stents was 210 days (range, 2-525 days). CONCLUSION: Ductus arteriosus stenting may be a reasonable and effective alternative to surgery for the initial palliation procedure in neonates with ductus-dependent pulmonary flow.

Endophlebectomy of the common femoral vein and endovascular iliac vein recanalization for chronic iliofemoral venous occlusion.

BACKGROUND: Chronic post-thrombotic occlusion of the iliofemoral veins causes significant morbidity, which can be alleviated if venous drainage is restored. We report our technique of surgical endophlebectomy and patchplasty of the common femoral vein (CFV) in conjunction with iliac vein stenting to restore venous flow from the infrainguinal venous system to the vena cava. METHODS: There were 157 patients who underwent CFV endophlebectomy combined with iliocaval recanalization. Questionnaires were completed both preoperatively and postoperatively to allow comparison. These included the Clinical, Etiology, Anatomy, and Pathophysiology clinical classification; the Venous Clinical Severity Score; the Villalta scale; the Venous Insufficiency Epidemiological and Economic Study on Quality of Life/Symptoms; and the 36-Item Short Form Health Survey quality of life questionnaire. RESULTS: Mean follow-up duration was 14.4 ± 2.9 months (range, 10-29 months). The mean preoperative Venous Clinical Severity Score was 15.3 ± 2.2, and this fell to 6.1 ± 1.8 after treatment (P < .001). The mean preoperative Villalta score dropped from 12.7 ± 2.6 to 6.3 ± 1.4 (P < .001). The quality of life and symptom severity scores were improved after 3 months by 17.2 points for quality of life (P < .001) and 20.5 points for symptom severity (P < .001). Primary patency was 81% (124/153) and secondary patency was 89.5% (137/153) at 12 months. Wound complications related to groin incision and lymphatic fistulas were observed in 22.8% (35/153) and 28.7% (44/153), respectively. CONCLUSIONS: The hybrid operation of CFV endophlebectomy in conjunction with iliac vein recanalization should be considered a safe and effective treatment option in patients with severe post-thrombotic syndrome and iliofemoral veno-occlusive disease.

Utility of Cervical Cannulation During Difficult Resternotomy in Congenital Heart Surgery.

BACKGROUND: Adhesions due to previous surgeries and some anatomical difficulties may make resternotomy dangerous in children. Femoral vessels are usually small and may not be suitable for cannulation. The aim of this report is to describe our experience with cervical cannulation during risky resternotomy in children. METHODS: Between January 2014 and January 2018, cervical cannulation was performed in eight pediatric patients during sternal reentry. Their ages were between 3 months and 17 years (mean: 5.4 years). Three patients underwent stage III extracardiac Fontan operation with the diagnosis of hypoplastic left heart syndrome. Three patients had supravalvular aortic and/or pulmonary stenosis after previous arterial switch operation. One patient had proximal aortic arch stenosis and subvalvular aortic stenosis after interrupted aortic arch repair. The last patient had aortic root pseudoaneurysm and aortic insufficiency due to endocarditis. Through a separate cervical incision, a polytetrafluoroethylene graft was anastomosed to the common carotid artery and the arterial cannula was inserted into the graft. Antegrade selective cerebral perfusion (ASCP) was used in two patients. RESULTS: During resternotomies, no major injury or bleeding occurred. Three-month-old patient who had previous interrupted aortic arch repair died despite extracorporeal membrane oxygenation support due to sepsis and multi-organ failure. Median intensive care unit stay and hospital stay were 3 days (1-40 days) and 17 days (7-60 days), respectively. Mean follow-up was 17.9 ± 15.8 months. All patients were in good clinical condition. CONCLUSIONS: Cervical cannulation may be a useful and safe technique during high-risk resternotomy in children. This technique may also simplify the performance of ASCP if necessary.

Staged Biventricular Repair After Hybrid Procedure in High-Risk Neonates and Infants.

BACKGROUND: Single-stage biventricular repair remains a challenging and difficult decision in high-risk newborns and early infants with the presence of left ventricular outflow tract obstruction (LVOTO) or borderline hypoplasia of the left ventricle (LV). METHODS: Six high-risk patients underwent the initial hybrid procedure (bilateral pulmonary banding + ductal stenting) for staged biventricular repair. Their median age was 17 days (range: 7-55 days). The diagnosis was interrupted aortic arch (IAA), ventricular septal defect (VSD), and LVOTO (n = 3); IAA and VSD (n = 1); and aortic annular hypoplasia, aortic arch hypoplasia, VSD, and LVOTO (n = 1). The last patient had borderline LV with large atrial septal defect (ASD) and aortic arch hypoplasia. The patient with borderline LV had also ASD closure with small fenestration. RESULTS: One patient died of sepsis after the hybrid procedure. Other patients underwent biventricular repair 8 to 13 months later. Three patients had conventional repair with conal septum resection. The other patient with IAA, in whom LVOTO was considered nonresectable, underwent Yasui operation. The last patient with borderline LV had enough development of left heart structures during follow-up and underwent aortic arch repair. One patient who had conal septum resection died after biventricular repair. One patient needed a tracheostomy; four patients were discharged uneventfully and their clinical conditions were good on postoperative year 1. CONCLUSION: Staged biventricular repair with the initial hybrid procedure may be a feasible and safe alternative in high-risk neonates and early infants. Hybrid intervention may provide the development of cardiac structures in time and a better evaluation for the possibility of biventricular repair in borderline patients.

Incidence and treatment of pulmonary vein stenosis after repair of total anomalous pulmonary venous connection.

BACKGROUND: In this study, we aimed to investigate the incidence and treatment of pulmonary vein stenosis after repair of total anomalous pulmonary venous connection. METHODS: Between December 2010 and December 2016, a total of 40 patients (25 males, 15 females; mean age 45±41 days; range, 2 days to 6 months) who underwent total anomalous pulmonary venous connection repair were retrospectively analyzed. RESULTS: Eighteen (45%) of the patients were supracardiac, seven (17.5%) were cardiac, eight (20%) were infracardiac, and seven (17.5%) were mixed-type anomaly. Twelve patients (30%) had obstruction of the pulmonary venous pathways. Hospital mortality was seen in three patients (7.5%). All of non-survivors received emergent surgery and the causes of death were sepsis and multiple organ failure. A total of 23 complications were seen in 20 patients (50%) mainly delayed sternal closure (n=11; 27.5%) and prolonged mechanical ventilation (n=6; 15%). The mean follow-up was 12.2±9.6 (range, 1 to 36) months in all patients, except one. Pulmonary vein stenosis developed in three patients (8.3%) after repair. All of them underwent sutureless repair. CONCLUSION: Total anomalous pulmonary venous connection can be repaired with low mortality and morbidity rates and favorable mid-term outcomes. Close follow-up is necessary due to the risk of development of pulmonary vein stenosis. Sutureless repair may provide relief in such cases.

Outcomes of hybrid and Norwood Stage I procedures for the treatment of hypoplastic left heart syndrome and its variants.

BACKGROUND: In this study, we present the outcomes of hybrid and Norwood Stage I procedures for the treatment of hypoplastic left heart syndrome and its variants. METHODS: In this study, a total of 97 pediatric patients who were operated due to hypoplastic left heart syndrome and its variants between March 2011 and October 2018 were retrospectively analyzed. Thirty-two of the patients (28 males, 4 females; median age 5 days; range, 1 to 25 days) underwent Norwood Stage I operation (Group N), while the remaining 65 patients (44 males, 21 females; median age 6 days; range, 1 to 55 days) underwent a hybrid procedure (Group H). Both treatment strategies were compared. RESULTS: The median body weight in Group H was significantly lower and the number of patients with a low birth weight (<2,500 g) was significantly higher than Group N (p=0.002 and 0.004, respectively). The postoperative early mortality rate was similar between the groups. Univariate and multivariate analyses revealed that the need for preoperative mechanical ventilation was a significant factor for mortality (p=0.004 and 0.003, respectively). Syndromic appearance was also a significant factor the multivariate analysis (p=0.03). There was a statistically significant difference between the groups in terms of the inter-stage mortality rates (p=0.0045). Second-stage procedure was performed in 32 patients. The early mortality rate after the Glenn operation was 7.6%. Six patients died after comprehensive Stage II operation. Five patients underwent biventricular repair and 8 patients had third-stage fenestrated extracardiac Fontan operation (Group N, n=7 and Group H, n=1). The Kaplan-Meier survival curve demonstrated that Group N had a higher survival rate at both one and five years than Group H, although the difference was not statistically significant (p=0.15). Subgroup analysis showed that the Norwood procedure with Sano modification had the highest survival rate with 40% at five years. CONCLUSION: Our study results show that patients undergoing the Norwood procedure have a more uneventful course of inter-stage period and Stage II and III, despite drawbacks early after Stage I procedure. Based on our experiences, we recommend performing the hybrid intervention in patients with a poor clinical condition and a body weight of <2,500 g.