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Objective: Cross-sectional imaging allows identification of rare patients with a vascular ring and circumflex aorta. The key diagnostic feature is crossing of the transverse aortic arch from right to left posterior to the trachea and superior to the carina in a patient with a right aortic arch. We evaluated our patients who received an aortic uncrossing procedure., Methods: We reviewed all patients who underwent aortic uncrossing from 2002 to 2022. All patients received preoperative computed tomography imaging and bronchoscopy., Results: Eleven patients ranging in age from 1.5 to 10 years (median 4 years) underwent aortic uncrossing. Two patients had prior left ligamentum division, and 3 patients had prior left aortic arch division. All had significant clinical symptoms. Eight patients had deep hypothermic circulatory arrest (mean 34 minutes), and 3 patients had antegrade cerebral perfusion (median, 28 minutes). Patch material was not used for aortic augmentation, and no patient underwent a posterior tracheopexy or rotational esophagoplasty. Postoperative length of stay ranged from 4 to 31 days (median, 5 days). One patient required a temporary tracheostomy for bilateral recurrent laryngeal nerve paresis, which recovered. One patient required an aortic extension graft to alleviate esophageal compression from an unusual ectatic esophageal course. All patients had relief of airway symptoms and dysphagia., Conclusions: In properly selected patients with a right aortic arch and circumflex aorta, aortic uncrossing is a safe and effective therapy to treat airway and esophageal compression. The procedure can be conducted with deep hypothermic circulatory arrest or antegrade cerebral perfusion. Careful attention to the location of the esophagus and recurrent laryngeal nerves is required., (Copyright © 2023 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Background: We report current outcomes in patients supported with the HeartMate 3 (HM3) ventricular assist device in a multicenter learning network., Methods: The Advanced Cardiac Therapies Improving Outcomes Network database was queried for HM3 implants between 12/2017 and 5/2022. Clinical characteristics, postimplant course, and adverse events were collected. Patients were stratified according to body surface area (BSA) (<1.4 m 2 , 1.4-1.8 m 2 , and >1.8 m 2 ) at device implantation., Results: During the study period, 170 patients were implanted with the HM3 at participating network centers, with median age 15.3years; 27.1% were female. Median BSA was 1.68 m 2 ; the smallest patient was 0.73 m 2 (17.7 kg). Most (71.8%) had a diagnosis of dilated cardiomyopathy. With a median support time of 102.5days, 61.2% underwent transplantation, 22.9% remained supported on device, 7.6% died, and 2.4% underwent device explantation for recovery; the remainder had transferred to another institution or transitioned to a different device type. The most common adverse events included major bleeding (20.8%) and driveline infection (12.9%); ischemic and hemorrhagic stroke were encountered in 6.5% and 1.2% of patients, respectively. Patients with BSA <1.4 m 2 had a higher incidence of infection, renal dysfunction, and ischemic stroke., Conclusions: In this updated cohort of predominantly pediatric patients supported with the HM3 ventricular assist device, outcomes are excellent with <8% mortality on device. Device-related adverse events including stroke, infection, and renal dysfunction were more commonly seen in smaller patients, highlighting opportunities for improvements in care., (Copyright © 2023 International Society for the Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

Interventricular septal hematoma is a rare and life-threatening complication of pediatric cardiac surgery. Commonly seen following ventricular septal defect repair, it has also been associated with ventricular assist device (VAD) placement. Although conservative management is usually successful, operative drainage of interventricular septal hematoma occurring in pediatric patients undergoing VAD implantation should be considered.

Background: Fetal diagnoses of vascular rings have been increasing. We compared management strategies and outcomes of infants with fetal diagnosis with those with postnatal diagnosis to inform recommendations regarding optimal management., Methods: A retrospective review was performed of vascular ring operations from January 2000 to June 2019. Standard demographic data (preoperative clinical status, timing of diagnosis, cross-sectional imaging, operative and perioperative details, and clinical outcomes) were collected. Statistical analysis was performed to compare characteristics and outcomes of fetal versus postnatal diagnosis., Results: Of 190 patients, 15% (n = 29) were diagnosed prenatally. Anatomic variants were double aortic arch (n = 66, 14 fetal diagnoses), right aortic arch, aberrant left subclavian artery (n = 94, 12 fetal diagnoses), circumflex aorta (n = 7, 1 fetal diagnosis), and pulmonary artery sling (n = 19, 2 fetal diagnoses). An increasing frequency of fetal diagnoses has been noted in the past 10 years. In 2012 1 of 9 patients (11%) had a fetal diagnosis, whereas in 2018 8 of 11 (72%) had a fetal diagnosis (P < .001). Patients with a fetal diagnosis were significantly younger at the time of surgery (13.1 months [interquartile range (IQR), 20.6] vs 24.0 months [IQR, 87.0], P = .029). There was no difference in postoperative complications or length of stay (3 days [IQR, 1] for fetal diagnoses vs 4 days [IQR, 3] for postnatal diagnoses, P = .50)., Conclusions: Fetal diagnosis leads to the potential for expectant management of vascular ring patients. This has resulted in earlier time of intervention with no increase in postoperative morbidity. This may lead to improved long-term outcomes and potentially alter the natural history for these children., (Copyright © 2022 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: There is active debate regarding the optimal method of Fontan palliation. In light of this, we reviewed our experience with the nonfenestrated extracardiac Fontan including Fontan conversion., Methods: We performed a retrospective review of all nonfenestrated extracardiac Fontan and Fontan conversion operations at our institution from December 1, 1994 to December 31, 2018. Standard demographic data were collected, including underlying anatomy, preoperative ventricular and valvular function, operative details, perioperative data, and clinical outcomes. Statistical analysis included comparison between initial extracardiac Fontan patients and Fontan conversions, as well as analysis of risk factors for adverse outcomes., Results: There were 341 patients with an overall operative mortality of 4 patients (1.2%). Of these, 193 were extracardiac nonfenestrated Fontan completion operations (57%) and 148 were Fontan conversions (43%). Length of stay was 11 days (SD, 6 days) with ventilator duration of 28 hours (SD, 26 hours). Six of the completion Fontan patients (3%) required Fontan takedown at a median time of 2.5 days. Upon multivariable analysis, risk factors associated with adverse events (mortality, Fontan takedown, and/or transplant) included increased cardiopulmonary bypass time, preoperative decreased dominant ventricular function, and length of stay. Kaplan-Meier curves demonstrated that mild or greater preoperative ventricular dysfunction decreased survival as well as freedom from adverse events for both initial extracardiac Fontan and Fontan conversion patients., Conclusions: Over the past 24 years, our strategy of nonfenestrated extracardiac Fontan has achieved low operative mortality for both initial Fontan and Fontan conversion. There is a steady attrition of Fontan patients to cardiac transplantation; the key risk factor is preoperative ventricular dysfunction., (Copyright © 2021 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Given recent reports of percutaneous closure of sinus venosus atrial septal defects, we reviewed our experience with surgical repair. Owing to the high incidence of arrhythmias with the two-patch technique, since 2001 we have used either one-patch repairs or the Warden procedure., Methods: A retrospective review was performed of pediatric patients undergoing sinus venosus atrial septal defect repair at our institution from January 1, 1990, to July 1, 2018. Standard demographic data such as echocardiographic and cross-sectional imaging along with operative details and clinical echocardiographic outcomes were collected., Results: The cohort included 144 patients with a median age of 4.3 years (interquartile range, 8.5). Inferior SVASD was present in 24 patients (17%). A single autologous untreated pericardial patch was used for 114 patients (79%), a two-patch technique for 20 patients (14%, last performed in 2000), and a Warden procedure in 10 patients (7%). Median length of stay was 4 days (interquartile range, 2). On echocardiogram follow-up, no patient had pulmonary vein stenosis. One patient who had the Warden procedure required a balloon dilation of the superior caval vein 2 years postoperatively and a stent 3 years later. Two-patch patients were substantially less likely to be in normal sinus rhythm (41%) on postoperative electrocardiograms compared with the other two techniques (81% one-patch and 89% Warden, P = .02)., Conclusions: The great majority of patients with sinus venosus atrial septal defects can be successfully repaired with a single patch of autologous pericardium. We transitioned to using either a single pericardial patch or the Warden procedure, resulting in a higher frequency of normal sinus rhythm on postoperative electrocardiograms., (Copyright © 2021 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Minimal data exist on clinical decision-making in VAD implantation in pediatrics. This study aims to identify areas of consensus/variability among pediatric VAD physicians in determining eligibility and factors that guide decision-making., Methods: An 88-item survey with clinical vignettes was sent to 132 pediatric HT cardiologists and surgeons at 37 centers. Summary statistics are presented for the variables assessed., Results: Total respondents were 65 (72% cardiologists, 28% surgeons) whose centers implanted 1-5 (34%), 6-10 (40%), or >10 (26%) VADs in the past year. Consensus varied by patients' age, diagnosis, and Pedimacs profile. Highest agreement to offer VAD (97%) was a mechanically ventilated teenager with dilated cardiomyopathy. Patients stable on inotropes were less likely offered VAD (11%-25%). SV infant with Pedimacs profile 2 had the most varied responses: 37% offered VAD; estimated survival ranged from 15% to 90%. Variables considered for VAD eligibility included mild developmental delays (100% offered VAD), moderate-severe behavioral concerns (46%), cancer in remission >2 years (100%), active malignancy with good prognosis (68%) or uncertain prognosis (36%), and BMI >35 (74%) or <15 (69%). Most respondents (91%) would consider destination therapy VADs in pediatrics, though not currently feasible at 1/3 of centers. Factors with greatest influence on decision-making included HT candidacy, families' goals of care, and risks of complications., Conclusions: Significant variation exists among pediatric VAD physicians when determining VAD eligibility and estimating survival, which can lead to differences in access to emerging technologies across institutions. Further work is needed to understand and mitigate these differences., (© 2020 Wiley Periodicals LLC.)

Although minimally invasive repair of pectus excavatum has been shown to have a low complication rate in large series, several case reports have documented life-threatening complications, including bleeding and cardiac perforation. We present a rare case of an arteriovenous malformation from the internal thoracic artery to the pulmonary artery caused by occlusion of the internal thoracic artery by the Nuss bar followed by an unidentified angiogenic process. The patient became symptomatic and required transcatheter coil embolization.

Background: The HeartMate 3 ventricular assist device (VAD) is a newer centrifugal continuous-flow VAD used for bridge-to-transplant and destination therapy in adults. However, there is limited experience regarding its use in children and adults with complex congenital heart disease (CHD)., Methods: The Advanced Cardiac Therapies Improving Outcomes Network (ACTION) is a multicenter learning network comprised of pediatric hospitals implanting VADs in children and adults with complex CHD. We examined the outcomes of patients undergoing HeartMate 3 implantation at an ACTION center between December 2017 and September 2019., Results: The HeartMate 3 was implanted in 35 patients at 9 ACTION centers, with a median age of 15.7 (8.8-47.3) years, median weight of 65.7 (19.1-114.1) kg, and median body surface area (BSA) of 1.74 (0.78-2.36) m 2 . Of the cohort, 14 patients (40%) weighed <60 kg. Diagnoses included dilated cardiomyopathy (63%), dilated cardiomyopathy in neuromuscular disease (20%), and CHD (17%). Of those with CHD, most had a Fontan circulation. With a median 78 days of follow-up, there was 1 death on device (97% survival); 20 out of 35 (57%) underwent transplantation with no post-transplantation mortality. There were no episodes of stroke or pump thrombosis., Conclusions: Use of the HeartMate 3 in ACTION centers was associated with a low incidence of mortality and adverse events. Patients as small as 19 kg (BSA 0.78 m 2 ) were successfully implanted and supported, indicating that this device may be appropriate for older children and small adults., (Copyright © 2020 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

Objectives: The time course for hemodynamic normalization after pediatric heart transplantation has not been well characterized. We hypothesized that patients with a single ventricle would normalize later than those with dilated cardiomyopathy. Establishing the expected course based on the underlying pathophysiology will allow identification of patients who are outliers, requiring further investigation., Methods: We performed a retrospective review of patients with dilated cardiomyopathy, Glenn, and Fontan who underwent heart transplantation from January 2007 to December 31, 2017, and had 6-month and 1-year catheterization data. Hemodynamic data were examined for sustained normalization of pressures. Myocardial biopsies were reviewed for clinically significant rejection within the first year., Results: Ninety-four patients comprised the cohort (47 dilated cardiomyopathy, 18 Glenn, 29 Fontan) with a median age of 6.8 (12) years. Patients with dilated cardiomyopathy were more likely to normalize hemodynamics by 6 months (85% vs 28% Fontan, 44% Glenn, P < .05), and 96% of patients with dilated cardiomyopathy had normalized hemodynamics by 1 year (vs 62% Fontan, 78% Glenn, P < .001). The pulmonary capillary wedge pressure at 6 months was higher in patients who underwent the Fontan and Glenn (median 12.8 [8.8] mm Hg and 11.2 [5.9] mm Hg, respectively) compared with patients with dilated cardiomyopathy (7.0 [3.3] mm Hg, P < .001). Patients with dilated cardiomyopathy demonstrated normalized hemodynamics earlier (121 ± 72 days) than patients who underwent the Fontan (329 ± 62 days) and Glenn (233 ± 11 days, P < .001). Eighteen patients (19%) experienced significant rejection, which was not increased in patients with delayed hemodynamic normalization. The 6-month pulmonary capillary wedge pressure was associated with delayed normalization (hazard ratio, 1.36; 95% confidence interval, 1.16-1.60; P < .001)., Conclusions: Patients with a single ventricle demonstrated delayed hemodynamic normalization compared with dilated cardiomyopathy heart transplant recipients, without affecting survival or need for retransplantation., (Copyright © 2019 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Background: The purpose of this study was to assess the outcomes of slide tracheoplasty and tracheal resection in pediatric patients and analyze the data for predictors of outcomes., Methods: A retrospective review of tracheal surgery from January 1, 1993 to May 1, 2018 was performed. Demographic data, operative details, perioperative data, and clinical outcomes were collected. The study investigators' management strategy has evolved over time, with less rigid bronchoscopy, more reliance on postoperative computed tomographic imaging, and the use of inhaled Ciprodex (combination of ciprofloxacin and dexamethasone) since 2007., Results: The study included 41 patients, with a median age of 4.1 months and a median weight of 4.2 kg. There were 6 neonates and 24 infants. Slide tracheoplasty was performed in 27 patients (66%), and resection with end-to-end anastomosis was performed in 14 (34%). Eleven patients (27%) had a pulmonary artery sling. Simultaneous intracardiac repairs requiring cross-clamp and cardioplegia were performed in 9 patients (22%). Lung agenesis (n = 6) or severe hypoplasia (n = 2) was present in 8 patients (20%). Complications included tracheostomy in 5 patients (12%) and in-hospital death in 3 patients (7%). There were no cases of mediastinitis. Inhaled Ciprodex was used to decrease granulation tissue at suture lines. Median intubation time was 7 days, and median length of stay was 25.0 days. There was no difference in outcomes when comparing intracardiac repairs with the remaining patients, lung agenesis or hypoplasia vs the remaining cohort, or neonates vs infants., Conclusions: Slide tracheoplasty and tracheal resection are effective operative strategies for infants and children with tracheal stenosis, including patients with lung agenesis/hypoplasia. Simultaneous repair of intracardiac anomalies and pulmonary artery sling is recommended., (Copyright © 2020 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: The 15-mm mechanical valve was approved by the US Food and Drug Administration in March 2018. We review our experience in infants with this valve in the mitral position (MV), focusing on outcomes and timing to repeat MV replacement (MVR)., Methods: Between 2006 and 2017 7 patients underwent eight MVRs (one repeat) with a 15-mm mechanical valve. Retrospective chart review was performed to examine short- and long-term outcomes., Results: There were no operative deaths. Mean follow-up was 5.8 ± 4.8 years (range, 0.72 to 11.1). Six patients underwent an MV operation 53 ± 39 days (range, 9 to 118) before MVR with the 15-mm valve. All patients were on mechanical ventilatory support at the time of operation. Mean age, body weight, and body surface area at time of 15-mm MVR were 0.5 ± 0.3 years (range, 0.2 to 0.9), 5.6 ± 0.8 kg (range, 4.8 to 6.6), and 0.29 ± 0.03 m 2 (range, 0.27 to 0.32), respectively. Two patients required pacemaker implantation for atrioventricular block, both after their second MVR. Two patients are well at 16 and 24 months. Four patients underwent repeat MVR because of somatic growth and patient-prosthesis mismatch. Mean time to repeat MVR was 23 months (range, 6 to 40). There were two late deaths, one at 10 months unrelated to the valve in a child with a chromosomal abnormality. The other child had a congenital diaphragmatic hernia, early valve thrombosis, and died of multiple complications after a fourth MVR., Conclusions: The 15-mm mechanical valve was useful in treating MV disease in infants 2 to 12 months of age. This newly approved smallest available mechanical valve has a predicted mean time to replacement of 23 months in the mitral position., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Vascular rings with a Kommerell diverticulum (KD) most commonly occur in patients with a right aortic arch. We report on a less commonly seen subset of vascular ring patients-those with a double aortic arch and a KD., Methods: Between 2002 and 2017, 66 patients underwent an operation for a double aortic arch. Ten of those patients also had excision of a KD. We performed a retrospective medical record review of these patients to characterize their demographics and outcomes., Results: All 10 patients (7 male, 3 female) had a double aortic arch that was right dominant and also had a KD. The patients were a mean age of 4.9 ± 4.3 years (range, 6 months to 29 years), and median age was 4 years. All patients had preoperative computed tomographic angiography or magnetic resonance imaging and mean compression of the distal trachea of 63% ± 12% (range, 40% to 80%). The distal left arch was atretic in all patients. All patients underwent division of their left aortic arch, division of the ligamentum, and resection of the KD. The left subclavian artery was transferred to the left carotid artery in 2 patients. The mean size of the diverticulum was 9 × 10 mm. There were no major postoperative complications or readmissions. The postoperative length of stay was 3.1 ± 0.8 days. Five of the patients reported no related persisting symptoms. The remaining 5 patients reported substantial symptomatic relief with only minor respiratory symptoms., Conclusions: Vascular ring patients with a double aortic arch can also have a KD. In addition to dividing the smaller aortic arch and the ligamentum, we recommend excision of the KD., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Optimal surgical approach for repair of coarctation of the aorta (CoA) remains controversial. This study aimed to evaluate reintervention rates and its predictors by using a strategy of resection with extended end-to-end anastomosis (REEEA) through left thoracotomy., Methods: A retrospective analysis was performed for all patients who underwent isolated CoA repair or simultaneous repair of CoA and ventricular septal defect repair by REEEA between January 2000 and December 2015 at Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois. Patients with complex congenital heart disease were excluded. Transverse arch hypoplasia was defined as echocardiographic z-score lower than -2 or by documentation in medical or operative reports. Reintervention was defined as the need for balloon angioplasty or reoperation. Hypertension was defined as antihypertensive medication use or blood pressure greater than or equal to the 95th percentile., Results: A total of 251 patients with median age at repair of 14.6 days met inclusion criteria. Repair was by left thoracotomy in 226 (90%). Follow-up data were available for 186 of 251 patients, with median follow-up time of 5.4 years (range, 0.2 to 15.3 years); 169 (91%) of these patients underwent thoracotomy. There were no early deaths or early reoperations. A proximal transverse arch z-score lower than -4.1 or a distal transverse arch z-score of less than -2.8 was predictive of repair through sternotomy. Only 4 (2%) patients required reintervention (2 patients had balloon angioplasties, 2 had reoperations). Transverse arch hypoplasia was a risk factor for reintervention (p = 0.048), but surgical approach was not (p = 0.35). Late hypertension was identified in only 33 of 186 (18%) patients., Conclusions: Repair of CoA, even with associated transverse arch hypoplasia, by REEEA through left thoracotomy has a low mortality, low reintervention rate, and low incidence of late hypertension., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Left mainstem bronchial compression by a midline descending thoracic aorta is a rare anatomic variant. Translocation of the descending thoracic aorta to the ascending aorta has recently been described to treat this condition. We performed an aortic translocation and right pulmonary artery reimplantation in a 4-month-old infant with severe pulmonary hypertension secondary to right pulmonary artery stenosis and left bronchial compression by a midline descending thoracic aorta. The procedure was successful in ameliorating the patient's left mainstem bronchial compression and pulmonary hypertension. Descending aortic translocation should be considered when the left bronchus is compressed causing respiratory symptoms.

Background: After pediatric heart operations, we sought to determine the incidence of unplanned cardiac reinterventions during the same hospitalization, assess risk factors for these reinterventions, and explore associations between reinterventions and outcomes. We hypothesized that younger patients undergoing more complex operations would be at greater risk for unplanned cardiac reinterventions and that operative mortality and postoperative length of stay (PLOS) would be greater in patients who undergo reintervention than in those who do not., Methods: Patients aged 18 years or younger in The Society of Thoracic Surgeons Congenital Heart Surgery Database (January 2010 to June 2015) were included. We used multivariable regression to evaluate risk factors for unplanned cardiac reintervention (operation or therapeutic catheterization) and associations of reintervention with operative mortality and PLOS., Results: Of 84,404 patients (117 centers), 21% were neonates and 36% infants. An unplanned cardiac reintervention was performed in 5.4% of patients, including 11.8% of neonates, 5.2% of infants, and 2.8% of children. Independent risk factors for unplanned reintervention included presence of noncardiac anomalies/genetic syndromes, nonwhite race, younger age, lower weight among neonates and infants, prior cardiothoracic operations, preoperative mechanical ventilation, other Society of Thoracic Surgeons preoperative risk factors, and higher Society of Thoracic Surgeons-European Association for Cardiothoracic Surgery Mortality Category (adjusted p < 0.001 for all). Unplanned reintervention was a risk factor for operative mortality (adjusted odds ratio, 5.3; 95% confidence interval, 4.8 to 5.8; p < 0.001) and longer PLOS (adjusted relative risk, 2.3; 95% confidence interval, 2.2 to 2.4; p < 0.001)., Conclusions: Unplanned cardiac reinterventions are not rare, particularly in neonates, and are independently associated with operative mortality and increased PLOS. Patients at greater risk may be identified preoperatively, presenting opportunities for quality improvement., (Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Controversy remains regarding the optimal surgical approach for children with supravalvular aortic stenosis (SVAS)., Methods: Since 1997 we have used Brom three-patch aortoplasty for patients with SVAS. We prefer computed tomography (CT) imaging for preoperative evaluation rather than cardiac catheterization as it avoids the well-known morbidity of general anesthesia. The purpose of this study was to present our intermediate-term results of this strategy., Results: Twenty consecutive patients with SVAS were treated with Brom aortoplasty. Mean age was 3.7 ± 5.9 years (median, 1.5 years). Twelve patients had Williams syndrome. Ten patients had preoperative advanced medical imaging (seven CT, three magnetic resonance imaging) and did not have cardiac catheterization. Mean times for cardiopulmonary bypass and cross-clamp were 172 ± 29 minutes and 110 ± 21 minutes, respectively. Ten patients had simultaneous pulmonary artery stenosis patching. Median length of stay was seven days. There was no operative or late mortality. Mean follow-up was 6 ± 5 years. There were no reoperations on the aortic root. Fifteen patients had mild or less aortic insufficiency (AI) and two had moderate AI. One patient who had infant balloon dilation of the aortic valve and postoperative subacute bacterial endocarditis had moderate-to-severe AI and aortic stenosis (AS). One patient had moderate residual SVAS; all others had no AS. No patients had late coronary insufficiency., Conclusion: Brom aortoplasty promotes restoration of normal aortic root geometry and relief of coronary ostial stenosis, which is important in preventing myocardial ischemia. Computed tomography imaging is our preferred diagnostic modality. Intermediate-term outcomes are excellent with no recurrent SVAS, coronary events, or reoperations on the aortic valve.

Background: We describe the implementation of and outcomes associated with an acuity adaptable care model for pediatric patients undergoing cardiac surgery., Methods: Consecutive patients undergoing an index cardiac operation between July 2007 and June 2015 were included. From July 2007 through June 2010, a conventional model existed in which patients moved among units and care teams based on age, severity of illness, and operative status (conventional group). A transitional period existed between July 2010 and June 8, 2012 (transitional group). From June 9, 2012, through June 2015, an acuity adaptable model was used in which patients remained in the cardiac care unit and received care from the same clinical team throughout their hospitalization (acuity adaptable group)., Results: Included were 2,363 patients: 925 in the conventional group, 520 in the transitional group, and 918 in the acuity adaptable group. In relation to the conventional group, the adjusted odds of operative mortality in the acuity adaptable group was 0.55 (95% confidence interval: 0.26-1.18; P = .12). The failure to rescue rate (ie, number of deaths in patients with any complication divided by the number of total patients with any complication) decreased (conventional group, 8.7%; acuity adaptable group, 4.2%; P = .04). In relation to the conventional group, postoperative hospital length of stay tended to be shorter in the acuity adaptable group ( P = .07)., Conclusions: The implementation of an acuity adaptable care model was feasible in our pediatric cardiac program. The favorable associations identified between the new model and outcomes are promising but warrant confirmation in a larger, multicenter study.

Background: Anomalous aortic origin of a coronary artery (AAOCA) is a known cause of sudden death. Our hypothesis was that longer intramural length and smaller ostial diameter correlate with preoperative symptoms. If true, this would assist in the decision for surgical indications. We also assessed the accuracy of preoperative imaging to predict intramural length., Methods: Retrospective analysis of patients who underwent AAOCA unroofing from 2006 to 2014. Patients had preoperative computed tomography angiography (CTA) or magnetic resonance imaging (MRI). Intramural length was measured. Intramural lengths and ostial diameters were also measured intraoperatively (operating room [OR]). Symptoms were noted. Intramural lengths and ostial diameters were compared between patients with and without preoperative symptoms. The accuracy of intramural length measured by CTA/MRI versus the length measured in the OR was assessed using a Bland-Altman analysis., Results: Sixty-six patients underwent surgical repair of AAOCA. Fifty-two (79%) patients were symptomatic and 14 (21%) were asymptomatic. Mean age was 12.4 ± 4.0 years. There was no mortality. There was strong agreement between intramural length measured by CTA/MRI and measured in the OR. There was no significant difference in AAOCA intramural length in the symptomatic (8.6 ± 3.5 mm) and asymptomatic (8.9 ± 2.8 mm, P = .77) patients, which were measured both by CTA/MRI and intraoperatively (symptomatic 7.3 ± 2.5 mm, asymptomatic 6.9 ± 2.8 mm; P = .62). There was also no significant difference in AAOCA ostial diameters between groups (symptomatic = 1.9 ± 0.5 mm, asymptomatic = 1.6 ± 0.5 mm; P = .09)., Conclusion: Preoperative CTA/MRI was very accurate in predicting the length of surgical unroofing. There was no demonstrable correlation between preoperative symptoms and intramural AAOCA length or AAOCA ostial diameter.

Background: Computed tomographic angiography (CTA) and echocardiography (echo) are used preoperatively in coarctation of the aorta to define arch hypoplasia and great vessel branching. We sought to determine differences in quantitative measurements, as well as surgical utility, between modalities., Methods: Infants (less than six months) with both CTA and echo prior to coarctation repair from 2004 to 2013 were included. Measurements were compared and correlated with surgical approach. Three surgeons reviewed de-identified images to predict approach and characterize utility. Computed tomographic angiography radiation dose was calculated., Results: Thirty-three patients were included. No differences existed in arch measurements between echo and CTA ( z-score: -2.59 vs -2.43; P = .47). No differences between modalities were seen for thoracotomy ( z-score: -2.48 [echo] vs -2.31 [CTA]; P = .48) or sternotomy ( z-score: -3.13 [echo] vs -3.08 [CTA]; P = .84). Computed tomographic angiography delineated great vessel branching pattern in two patients with equivocal echo findings ( P = .60). Surgeons rated CTA as far more useful than echo in understanding arch hypoplasia and great vessel branching in cases where CTA was done to resolve anatomical questions that remain after echo evaluation. Two of three surgeons were more likely to choose the surgical approach taken based on CTA (surgeon A, P = .02; surgeon B, P = .01). Radiation dose averaged 2.5 (1.6) mSv and trended down from 2.9 mSv (1.8 mSv; n = 20) to 1.6 mSv (0.5 mSv; n = 7) ( P = .06) with new technology., Conclusion: Although CTA and echo measurements of the aorta do not differ, CTA better delineates branching and surgeons strongly prefer it for three-dimensional arch anatomy. We recommend CTA for patients with anomalous arch branching patterns, diffuse or complex hypoplasia, or unusual arch morphology not fully elucidated by echo.

We describe the diagnosis and surgical repair of a five-month-old infant with a congenital discontinuous right pulmonary artery. Initial echocardiogram failed to show the right pulmonary artery and revealed systemic left pulmonary artery pressure based on the tricuspid regurgitation jet. Computed tomographic angiography confirmed the diagnosis of discontinuous right pulmonary artery. The right pulmonary artery appeared essentially normal in size, and there were no significant aortopulmonary collateral arteries. Using cardiopulmonary bypass and aortic transection, we created an anastomosis between the right and the main pulmonary arteries augmented anteriorly by a pericardial patch. Postoperative lung perfusion scan demonstrated balanced pulmonary blood flow to the lungs. Pulmonary hypertension resolved over three weeks in the postoperative period, an expected outcome in this age-group.

A continuous flow extracorporeal ventricular assist device (VAD) was modified to support functionally univentricular infants and children awaiting heart transplantation. A centrifugal VAD, designed to flow from 1.5 to 8 L/min, was used as a bridge-to-transplant in four patients with functionally univentricular circulation. A variable restrictive recirculation shunt permitted lower flow ranges in small patients. In hypoxic patients, an oxygenator was incorporated into the circuit. From 2012 to 2015, the modified VAD was placed in four patients with Glenn physiology. Age ranged from 0.97 to 6.98 years (median = 2.2 yrs). Body surface area ranged from 0.41 to 0.84 m 2 (median = 0.54 m 2 ). One patient was on extracorporeal membrane oxygenation prior to VAD. A recirculation shunt was used in three patients. Three patients required temporary use of an oxygenator for 4, 10, and 27 days. Median time on the VAD was 32.3 days (range = 23-43 days). A decrease in the cavopulmonary pressure was noted in all patients, as was a fall in the B-type natriuretic peptide. Three patients survived transplant and were discharged at 28-82 days post-transplantation. One patient died after 35 days of support. Two patients experienced major bleeding events. Two patients experienced cerebrovascular accidents, one major and one minor. The centrifugal VAD successfully supported palliated functionally univentricular patients awaiting heart transplantation. The modified recirculation shunt facilitated the successful support of patients in whom optimal flows were substantially lower than that recommended by the manufacturer. The continuous-flow VAD effectively decompressed the cavopulmonary system. The design allowed placement of an in-line oxygenator in hypoxic patients. Further investigation is required to decrease the thromboembolic events, and associated morbidity, in patients supported with this device., (© 2017 International Center for Artificial Organs and Transplantation and Wiley Periodicals, Inc.)

Objectives: This review summarizes the current understanding of the pathophysiology and perioperative management of patent ductus arteriosus, atrial septal defect, ventricular septal defect, and atrioventricular septal defect., Data Source: MEDLINE and PubMed., Conclusions: The four congenital cardiac lesions that are the subject of this review, patent ductus arteriosus, atrial septal defect, ventricular septal defect, and atrioventricular septal defect, are the most commonly found defects causing a left-to-right shunt. These defects frequently warrant transcatheter or surgical intervention. Although the perioperative care is relatively straightforward for many of these patients, there are a number of management strategies and complications associated with each intervention. The treatment outcomes for all of these lesions are very good in the current era.

Background: We evaluated the outcomes of patients undergoing surgical repair of partial atrioventricular septal defect (AVSD) and analyzed the effect of age on outcome., Methods: In this single-center retrospective study, we included all children who underwent repair of partial AVSD between 1990 and 2014. We divided the patients into 4 age quartiles (first quartile: 0-0.75 years, n = 22; second quartile: 0.75-1.5 years, n = 21; third quartile: 1.5-3.75 years, n = 22; and fourth quartile: >3.75 years, n = 21). These quartiles were evaluated for their association with the time-to-event outcomes of survival, freedom from left atrioventricular valve regurgitation (LAVVR), and freedom from reoperation using log-rank analysis., Results: During the study period, 86 patients underwent partial AVSD repair at a median age of 1.5 years. There were no operative deaths and 2 late deaths (unknown cause and trauma). There were 13 reoperations. The most common cause of reoperation was left ventricular outflow tract obstruction (LVOTO) (5 patients [first quartile, 2 cases; second quartile, 1 case; third quartile, 2 cases, and fourth quartile, 0 cases]). LAVV reoperation for insufficiency or stenosis was performed in 4 patients (first quartile, 1 case; second quartile, 1 case; third quartile, 1 case; and fourth quartile, 1 case). Two patients underwent pacemaker placement (second quartile, 1 case and fourth quartile, 1 case). There were no statistically significant differences in the most common complications-LVOTO, LAVVR, and AV heart block-between the 4 age quartiles. Median follow-up was 7.1 years (interquartile range [IQR], 0.8-11.4 years). On echocardiography, 72 patients (84%) had less than or equal to mild LAVVR, 8 (9%) patients had mild to moderate LAVVR, 5 (6%) patients had moderate LAVVR, and 1 (1%) patient had severe LAVVR. Age at repair had no significant association with degree of late AV valve insufficiency., Conclusions: Results of partial AVSD repair at a median age of 1.5 years are excellent. Operating at this age is not associated with increased mortality, reoperation, or LAVVR., (Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: A continuous-flow "adult" ventricular assist device (VAD) was modified to support infants and children waiting for heart transplantation., Methods: A centrifugal VAD, designed to flow at 1.5 to 8 L/min, was used as a bridge to transplantation in pediatric patients. In smaller children and infants, a modified recirculation shunt permitted lower flow ranges. In hypoxic patients, an oxygenator was spliced into the circuit., Results: From 2010 to 2015, the VAD was placed in 13 consecutive patients. Age ranged from 0.9 to 16 years (median, 7 years). Body surface area (BSA) ranged from 0.4 to 2.1 m(2) (median, 0.8 m(2)). Ten patients had a BSA less than 1.0 m(2). Four patients were receiving extracorporeal membrane oxygenation (ECMO) before VAD. Three patients had single-ventricle physiology. Five patients had a recirculation shunt and 3 underwent insertion of an oxygenator. Median time on the VAD was 20 days (range, 2-140 days). In patients with a recirculation shunt, mean patient flow was 1.5 L/min (mean flow/BSA, 2.7 L/min/m(2)), with mean total VAD flow of 3.4 L/min. Twelve patients underwent transplantation, and 1 patient underwent VAD explantation. All patients survived and were discharged at a median of 26 days (range, 17-83 days) after transplantation. Three patients experienced major bleeding events. There were 2 cerebrovascular accidents. VAD mortality dropped from 33% (3 of 9) during 2007 to 2010 to 0% (0 of 13) between 2011 and 2015 (p = 0.05). Wait-list mortality dropped from 10% (5 of 52) to 4% (4 of 91) for these periods (p = 0.29)., Conclusions: The centrifugal VAD successfully supported pediatric patients awaiting heart transplantation. The modified recirculation shunt facilitated the successful support of patients in whom optimal flows were substantially lower than those recommended by the manufacturer. The design allows placement of an in-line oxygenator. Compared with pulsatile devices, use of this VAD was associated with a trend toward decreased mortality associated with VAD use., (Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Palliative procedures performed before the Fontan procedure may require ligation of the subclavian arteries, thereby affecting flow to the vertebral arteries. In adults with functionally univentricular heart disease, the implications of altered brainstem vascular anatomy for perioperative management of failing Fontan circulation are not known., Methods: We identified abnormal posterior circulation anatomy in an adult patient with failing Fontan circulation who experienced a brainstem stroke after Fontan conversion. We then changed our clinical practice to include detailed preoperative neurologic evaluation of adults with univentricular heart disease and failing Fontan circulation. Here, we report the clinical and neuroimaging findings in 5 consecutive patients before and after this change in practice., Results: Five patients ages 28 to 42 years had Fontan procedures performed in childhood, and underwent either Fontan conversion or cardiac transplantation. Patient 1 experienced an episode of decreased cerebral perfusion pressure on postoperative day 3, and experienced an ischemic brainstem stroke causing transient locked-in syndrome. A change in practice was made, and patients 2, 3, and 4 were evaluated preoperatively by the neurocritical care service. These patients then had higher target blood pressures perioperatively and no neurologic injury. Patient 5 was evaluated for symptoms consistent with subclavian steal. Neuroimaging in 3 patients was abnormal, with atrophic vertebral arteries, an occluded vertebral artery, and retrograde perfusion of a vertebral artery., Conclusions: In adults with failing Fontan circulation there is a potential for neurologic complications as a result of venous congestion with elevated central venous pressures, and aberrant posterior circulation. The patient's history and brain imaging may be used to identify at-risk patients and to tailor perioperative management during Fontan conversion or heart transplantation to mitigate the risk for brainstem ischemia., (Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

The term vascular ring refers to congenital vascular anomalies of the aortic arch system that compress the esophagus and trachea, causing symptoms related to those two structures. The most common vascular rings are double aortic arch and right aortic arch with left ligamentum. Pulmonary artery sling is rare and these patients need to be carefully evaluated for frequently associated tracheal stenosis. Another cause of tracheal compression occurring only in infants is the innominate artery compression syndrome. In the current era, the diagnosis of a vascular ring is best established by CT imaging that can accurately delineate the anatomy of the vascular ring and associated tracheal pathology. For patients with a right aortic arch there recently has been an increased recognition of a structure called a Kommerell diverticulum which may require resection and transfer of the left subclavian artery to the left carotid artery. A very rare vascular ring is the circumflex aorta that is now treated with the aortic uncrossing operation. Patients with vascular rings should all have an echocardiogram because of the incidence of associated congenital heart disease. We also recommend bronchoscopy to assess for additional tracheal pathology and provide an assessment of the degree of tracheomalacia and bronchomalacia. The outcomes of surgical intervention are excellent and most patients have complete resolution of symptoms over a period of time., (Copyright © 2016 Elsevier Inc. All rights reserved.)

We describe successful surgical resection of an intrapericardial rhabdomyosarcoma, which was accomplished with the aid of cardiopulmonary bypass, in a three-year-old girl. The patient completed adjuvant chemoradiotherapy and has no evidence of disease at one-year follow-up., (© The Author(s) 2015.)

Background: Since 1989 all patients with anomalous origin of the left coronary artery from the pulmonary artery at our institution have been treated with aortic implantation. The purpose of this review was to assess the late outcomes of these patients, especially regarding left ventricular (LV) function and mitral valve insufficiency., Methods: Between 1989 and 2014, 36 patients had aortic implantation of anomalous origin of the left coronary artery from the pulmonary artery. Mean age at surgery was 2.5 ± 5.1 years (median, 0.5 years). Operative strategy included antegrade cold-blood cardioplegia, main pulmonary artery transection, aortic implantation with a large button of pulmonary artery, pulmonary reconstruction with fresh autologous pericardium, and prolonged postoperative inotropic and ventilator support. Mitral regurgitation and LV dysfunction were graded as 0 to 4 (0 = none, 1 = trivial, 1.5 = trivial-mild, 2 = mild, 2.5 = mild-moderate, 3 = moderate, 3.5 = moderate-severe, and 4 = severe)., Results: Mean mitral regurgitation grade preoperatively was 2.95 ± 0.95. Mean LV dysfunction grade was 3.14 ± 1.27. Mean cross-clamp and cardiopulmonary bypass times were 49.1 ± 18 minutes (median, 48.5 minutes) and 147.5 ± 45 minutes (median, 139 minutes), respectively. There was no operative or late mortality. Four patients had delayed sternal closure. Mean duration of ventilator support was 11 ± 6.6 days (median, 9 days). Two patients required 3 and 6 days of postoperative extracorporeal mechanical circulatory support. Mean length of stay was 25 ± 18 days (median, 19 days). No patient has required reoperation for supravalvar pulmonary stenosis, coronary stenosis, or mitral valve repair or replacement. Late echocardiographic follow-up shows a mean mitral regurgitation grade of 1.67 ± 1.05 and a mean LV dysfunction grade of 0.23 ± 0.68., Conclusions: Aortic implantation is our procedure of choice for patients with anomalous origin of the left coronary artery from the pulmonary artery. No patient required mitral valve repair or transplant. There was marked improvement of mitral regurgitation grade, return to essentially normal LV function, and no mortality during a 25-year period., (Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: When the modified single-patch technique for atrioventricular septal defect (AVSD) repair was introduced by Dr Benson Wilcox, there was concern that these patients might be at risk for late subaortic stenosis and left ventricular outflow tract obstruction (LVOTO). This review evaluated our modified single-patch population for LVOTO in the postoperative period., Methods: Between January 2000 and 2013, 77 infants underwent AVSD repair with a modified single-patch technique. Median age was 4.2 months, and median weight was 5 kg. Eight patients had a prior repair of coarctation of the aorta via left thoracotomy in the newborn period., Results: The median hospital stay was 10 days. No patient required a pacemaker. The mean and median follow-up times were 4.6 and 3.7 years, respectively. Only 2 patients (2.5%) required reoperation for LVOTO; both had prior repair of coarctation of the aorta (2 of 8 vs 0 of 69, p = 0.01). A discrete fibrous subaortic membrane developed in the first patient that required resection at 3 and 7 years after repair. The other patient had LVOTO from accessory chordae of the left atrioventricular valve and required mitral valve replacement 5 months after repair. One early death occurred at 4 months postoperatively due to liver failure related to hyperalimentation., Conclusions: At intermediate term follow-up, LVOTO does not appear to be a significant postoperative issue after modified single-patch repair of AVSD. Coarctation of the aorta was the most significant predictor of late LVOTO after repair of AVSD with the modified single-patch technique., (Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Purpose: The development of pediatric ventricular assist device (VAD) circuits with lower flow ranges for infants and small children is ongoing. We present our results with modifying a readily available adult VAD to support the pediatric population., Description: The TandemHeart VAD (CardiacAssist, Pittsburgh, PA) circuit was modified to include a variable restrictive recirculation shunt to permit lower flow ranges in small pediatric patients., Evaluation: Initial benchtop flow rates and pressures were studied. Hemolysis trials were performed using whole bovine blood to compare plasma-free hemoglobin levels between modified and unmodified VAD circuits. The modified VAD was surgically implanted in 7 piglets (6 to 14 kg) and which supported them for 4 hours. Levels of hemolysis did not increase and full hemodynamic support was achieved. The modified TandemHeart VAD with a recirculation shunt was subsequently implanted in 2 pediatric patients who were bridged to transplant successfully., Conclusions: Because of its simplicity, availability, low prime volume, greater patient flow range, and lower cost, the modified TandemHeart VAD with a recirculation shunt should be considered as an alternative to extracorporeal membrane oxygenation and other pulsatile VADs in children., (Copyright © 2014 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: The morphology of ventricular septal defects (VSDs) that are doubly committed and juxtaarterial places the patient at risk for aortic valvar prolapse and aortic valvar insufficiency (AI). Surgical repair of this type of defect often involves placing sutures through the base of one or more of the leaflets of the pulmonary valve, raising concern for late pulmonary valvar insufficiency (PI). The purpose of this review was to analyze the postoperative follow-up relating to potential late complications with the aortic and pulmonary valves., Methods: Between 1980 and 2012, 106 patients with doubly committed juxtaarterial VSD underwent intracardiac repair. Median age at repair was 1.1 years. Preoperative evaluation showed 69 patients (65%) had aortic valvar prolapse and 51 (48%) had AI. Operative approach was through the pulmonary trunk in 88 (83%) of the patients. In 81 patients (76%), sutures securing the VSD patch had been placed through the base of the pulmonary valvar leaflets., Results: Operative survival was 100%. Follow-up ranges from 6 months to 17 years, with a mean of 4.9 years. No patient had heart block or residual shunting. Of the 70 patients with long-term contemporary echocardiographic follow-up, 66 (94%) had trivial or no AI and 4 (6%) had mild AI. Of these patients, 49 (70%) had trivial or no PI, and 21 (30%) had mild PI. In 1 patient having aortic valvoplasty at the time of VSD closure, the aortic valve was replaced 7 months later. No other patient had worrisome progression of their AI or PI., Conclusions: The incidence of aortic valvar prolapse and AI in the setting of doubly committed juxtaarterial VSD is quite high. The optimal surgical approach is through the pulmonary trunk. Sutures placed through the base of the pulmonary valvar leaflets do not predispose to clinically significant late pulmonary valvar insufficiency. Timely surgical closure of this type of defect prevents progression of AI., (Copyright © 2014 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

A communication between the right pulmonary artery (RPA) and the left atrium is a rare congenital anomaly that presents with cyanosis and heart failure. We describe the surgical repair of an RPA to left atrial fistula using cardiopulmonary bypass in a neonate. Advanced imaging (computed tomography scan) guided the surgical approach. Although previous reports have associated a patent ductus arteriosus with high neonatal mortality, in our case, the ductus arteriosus was actually important for maintaining enough total pulmonary blood flow.

The majority of patients having surgical intervention for a vascular ring have resolution of their symptoms. However, 5% to 10% of these patients develop recurrent symptoms related either to airway or esophageal compression and may require reoperation. In our series of 300 patients with vascular rings, we performed a reoperation on 26 patients, not all of whom were originally operated on at our institution. The four primary indications for reoperation were Kommerell diverticulum (n = 18), circumflex aorta (n = 2), residual scarring (n = 2), and tracheobronchomalacia requiring aortopexy (n = 4). All patients undergoing reoperation have had preoperative evaluation with bronchoscopy and computed tomographic scanning (CT) with 3-dimensional reconstruction. Patients with dysphagia have had a barium esophagram and esophagoscopy. Patients with a Kommerell diverticulum have undergone resection of the diverticulum and transfer of the left subclavian artery to the left carotid artery. The aortic uncrossing procedure has been used in patients with a circumflex aorta. Aortopexy has been used to treat anterior compression of the trachea by the aorta. Results of these reinterventions have been successful in nearly all cases. Lessons learned from these reoperations can be applied to prevent the need for reoperation by properly selecting the correct initial operation. A dedicated team caring for these children consisting of medical imaging, otolaryngology, cardiovascular-thoracic surgery, and critical care is imperative., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Background: Patients with failing Fontan circulation are at high risk for complications after heart transplantation (HTx) because of multiple prior operations, elevated panel reactive antibody, hepatic dysfunction, coagulopathy, protein-losing enteropathy (PLE), and poor nutrition. The purpose of this review was to evaluate the outcome of HTx for these patients, including those who are status post-Fontan conversion., Methods: Of 206 heart transplants at Ann & Robert H. Lurie Children's Hospital of Chicago from 1990 to 2012, 22 patients had a failing Fontan. Median age at HTx was 12.2 years, median interval from initial Fontan to HTx was 7.1 years. Potential preoperative risk factors included PLE (n = 15), mechanical ventilation (n = 8), prior Fontan conversion (n = 7), renal failure (n = 3), and plastic bronchitis (n = 2) Median number of prior operations was 3. Donor branch pulmonary arteries were used in 17 patients., Results: There were 5 early deaths (23%), due to graft failure (1), pulmonary hypertension (1), and infection (3). There were 3 late deaths (13%) at 1, 5, and 8 years. Two of 3 patients with preoperative renal failure died. Survivors who had preoperative PLE (n = 11) and preoperative plastic bronchitis (n = 2) experienced complete resolution of these pathological conditions after heart transplantation. Median length of stay was 30 days. Five of 7 Fontan conversion patients survived, and 6 of 8 preoperative ventilator-dependent patients survived. One-, 5-, and 10-year survival was 77%, 66%, and 45%, respectively., Conclusions: The operative mortality of HTx for patients with a failing Fontan is high. Using the donor branch pulmonary arteries greatly facilitated the transplant. Because infection caused the majority of early deaths, lower intensity initial immunosuppression may be warranted. Transplantation was successful in treating PLE in all survivors. Prior Fontan conversion was not a risk factor. Preoperative mechanical ventilation was not a risk factor. Preoperative renal failure may be a relative contraindication. Earlier referral of failing Fontan patients may improve results., (Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)