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.

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.

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.

Outcomes of arterial switch operation for Taussig-Bing anomaly versus transposition of great arteries and ventricular septal defect.

BACKGROUND: This study aims to present the outcomes of arterial switch operation for Taussig-Bing anomaly versus transposition of the great arteries and ventricular septal defect. METHODS: Between November 2010 and December 2016, a total of 100 consecutive arterial switch operations in 42 pediatric patients (25 males, 17 females; median age 17 days; range, 2 to 210 days) were performed in two centers. Among them, 42 patients had associated ventricular septal defect and were diagnosed with Taussig-Bing anomaly (n=15) or transposition of the great arteries and ventricular septal defect (n=27). Aortic arch anomalies were present in six patients (40%) with Taussig-Bing anomaly and two patients (7.4%) with transposition of the great arteries and ventricular septal defect (p=0.016). Coronary anomaly was observed in five (33.3%) and six (22.2%) patients, respectively. All patient had a large defect (multiple n=4), except for eight patients who had moderate defect in the transposition of the great arteries group. RESULTS: Early mortality was 13.3% in the Taussig-Bing anomaly group and 7.4% in the other group. Extracorporeal membrane oxygenation support was needed in three patients. Delayed sternal closure was used in most of the patients (92.9%). The median length of intensive care unit and hospital stays were similar between the groups. The median follow-up was 16 (range, 1 to 50) months. Two patients including one planned (debanding and multiple defect closure) and one unplanned (residual defect closure) were reoperated. Three patients needed aortic balloon angioplasty due to recoarctation and one patient underwent pulmonary balloon angioplasty. Overall reintervention rate was 18.4%. CONCLUSION: Although the incidence of aortic arch and coronary anomalies were higher in patients with Taussig-Bing anomaly, early and mid-term results were similar. Based on these results, primary arterial switch operation seems to be successfully performed for both pathologies.

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.

Outcomes of truncus arteriosus repair with bovine jugular vein conduit.

BACKGROUND: In this study, we aimed to evaluate the outcomes of truncus arteriosus repair in patients undergoing Rastelli type truncus arteriosus. METHODS: A total of 13 patients (7 males, 6 females; median age 37 days; range, 16 to 60 days) underwent repair of truncus arteriosus using Contegra conduits between January 2011 and March 2 017. P reoperative d iagnosis w as t runcus a rteriosus type 1 (Edwards-Collett) in eight, type 2 in three, type 3 in one, and type 4 in one patient. Contegra conduits used for operations were 14 mm (n=5), 12 mm (n=7), and 16 mm (n=1). RESULTS: Early death was seen in two patients (15.4%). The median intensive care and hospital stays were 10 (range, 6 to 14) and 20 (range, 14 to 41) days, respectively. The median follow-up was 36 (range, 2 to 66) months. In four patients (31%), the conduit sizes severely increased during follow-up and reached 23 mm in two patients and 20 mm in one patient, and 18 mm in the other patient. Three patients had moderate distal conduit stenosis. Moderate pulmonary insufficiency was detected in four patients and severe pulmonary insufficiency in one patient. Two patients had moderate truncal valve insufficiency and one patient had moderate residual ventricular septal defect. None of the patients needed reoperation. CONCLUSION: Contegra conduit is a good alternative for repair of truncus arteriosus in infants; however close follow-up is necessary, as distal conduit stenosis and conduit dilatation may develop.

The impact of pulmonary valve-sparing techniques on postoperative early and midterm results in tetralogy of Fallot repair.

BACKGROUND: In this study, we analyzed the impact of pulmonary valve-sparing techniques on early and midterm postoperative results of tetralogy of Fallot repair. METHODS: A total of 64 patients diagnosed with tetralogy of Fallot, who underwent total correction operation by the same surgeon between November 2010 and September 2015 were included in this retrospective study. Mean age of the patients was 20.0±14.2 months (5.5-96 months). Forty patients (62.5%) were male. Thirty two of the patients (50%) were under one year of age. Pulmonary valve-sparing techniques were performed in 29 patients (Group 2), while transannular patch was applied in the remaining 35 patients (Group 1). Pericardial monocusp valve was constructed in 15 patients in Group 1. In Group 2, pulmonary valve-sparing techniques were transatrial repair in nine patients; transatrial-transpulmonary in eight, infundibular patch in eight, and infundibular-pulmonary patch in four patients. RESULTS: There was no early postoperative mortality in Group 2. Five patients (14.2%) in Group 1 died in the early postoperative period (p=0.058). Pulmonary monocusp insertion was performed in 2 (13.3%) of these patients (p=1). The causes of mortality were sudden cardiac arrest (n=2), multiorgan failure (n=1), low cardiac output (n=1), and neurological complications (n=1). Five patients in Group 1 required extracorporeal membrane oxygenation support (ECMO). Three of them were separated from ECMO and two of the patients that were separated were discharged uneventfully. Total postoperative morbidity rate was significantly higher in Group 1 (51.4% vs. 6.8%) (p=0.0001). Morbidity rate was significantly lower in patients with pulmonary monocusp insertion than patients in the same group without a monocusp (p=0.0176). Forty nine (83%) of the patients were followed up for a median of 6.5 (1-24) months. While free pulmonary regurgitation was detected in all non-monocusp patients in Group 1, pulmonary regurgitation was absent or mild in Group 2. Twelve (80%) of the patients in Group 1 who had monocusp insertion were followed up. Only two of these patients had free pulmonary regurgitation (16.6%). The rest of them had mild (n=6) or mildmoderate pulmonary regurgitation (n=4). CONCLUSION: Mortality and morbidity rates are lower when pulmonary valvesparing techniques are used in repair of tetralogy of Fallot. Monocusp pulmonary valve insertion may improve results in patients who require transannular patch repair. It is suggested that every effort should be made to achieve a competently working pulmonary valve during repair.

Extracorporeal cardiopulmonary resuscitation for refractory cardiac arrest in children after cardiac surgery.

OBJECTIVE: Extracorporeal membrane oxygenation (ECMO) is used to provide cardiorespiratory support during cardiopulmonary resuscitation (extracorporeal cardiopulmonary resuscitation; ECPR) unresponsive to conventional methods. In this study, the results of ECPR in a cardiac arrest setting after cardiac surgery in children were analyzed. METHODS: In this retrospective cohort study, between November 2010 and June 2014, 613 congenital heart operations were performed by the same surgical team. Medical records of all the patients who experienced cardiac arrest and ECPR in an early postoperative period (n=25; 4%) were analyzed. Their ages were between 2 days and 4.5 years (median: 3 months). Sixteen patients had palliative procedures. In 88% of the patients, cardiac arrest episodes occurred in the first 24 h after operation. Mechanical support was provided by cardiopulmonary bypass only (n=10) or by ECMO (n=15) during CPR. RESULTS: The CPR duration until commencing mechanical support was <20 min in two patients, 20-40 min in 11 patients, and >40 min in 12 patients. Eleven patients (44%) were weaned successfully from ECMO and survived more than 7 days. Five of them (20%) could be discharged. The CPR duration before ECMO (p=0.01) and biventricular physiology (p=0.022) was the key factor affecting survival. The follow-up duration was a mean of 15±11.9 months. While four patients were observed to have normal neuromotor development, one patient died of cerebral bleeding 6 months after discharge. CONCLUSION: Postoperative cardiac arrest usually occurs in the first 24 h after operation. ECPR provides a second chance for survival in children who have had cardiac arrest. Shortening the duration of CPR before ECMO might increase survival rates.

Early Degeneration of Extracellular Matrix Used for Aortic Reconstruction During the Norwood Operation.

The use of prosthetic patch material is often required during the surgical reconstruction of complex congenital cardiac malformations. Decellularized porcine small intestinal submucosa, a type of extracellular matrix, was recently introduced as a patch for cardiac and vascular tissue repair. Extracellular matrix was used for aortic reconstruction during the Norwood procedure in 8 consecutive neonates with hypoplastic left heart syndrome. Rapid degeneration or aneurysm formation developed in 3 of these patients who came to second-stage surgery. Extracellular matrix should be used cautiously for aortic reconstruction in infants until additional reports from other centers provide confirmation of its safety.

Right Atrial Flap Repair for Left Superior Vena Cava Draining into Left Atrium.

There are different surgical methods for the repair of persistent left superior vena cava that connects directly to the left atrium. We describe an extracardiac surgical technique that includes direct anastomosis of persistent left superior vena cava to the right atrium with right atrial flap and autologous pericardium. We have performed this procedure in four cases and there is no obstruction at postoperative control studies. Right atrial flap repair is a feasible extracardiac technique that offers growth potential.