National Variation in Congenital Heart Surgery Outcomes.

BACKGROUND: Optimal strategies to improve national congenital heart surgery outcomes and reduce variability across hospitals remain unclear. Many policy and quality improvement efforts have focused primarily on higher-risk patients and mortality alone. Improving our understanding of both morbidity and mortality and current variation across the spectrum of complexity would better inform future efforts. METHODS: Hospitals participating in the Society of Thoracic Surgeons Congenital Heart Surgery Database (2014-2017) were included. Case mix-adjusted operative mortality, major complications, and postoperative length of stay were evaluated using Bayesian models. Hospital variation was quantified by the interdecile ratio (IDR, upper versus lower 10%) and 95% credible intervals (CrIs). Stratified analyses were performed by risk group (Society of Thoracic Surgeons-European Association for Cardiothoracic Surgery [STAT] category) and simulations evaluated the potential impact of reductions in variation. RESULTS: A total of 102 hospitals (n=84 407) were included, representing ≈85% of US congenital heart programs. STAT category 1 to 3 (lower risk) operations comprised 74% of cases. All outcomes varied significantly across hospitals: adjusted mortality by 3-fold (upper versus lower decile 5.0% versus 1.6%, IDR 3.1 [95% CrI 2.5-3.7]), mean length of stay by 1.8-fold (19.2 versus 10.5 days, IDR 1.8 [95% CrI 1.8-1.9]), and major complications by >3-fold (23.5% versus 7.0%, IDR 3.4 [95% CrI 3.0-3.8]). The degree of variation was similar or greater for low- versus high-risk cases across outcomes, eg, ≈3-fold mortality variation across hospitals for STAT 1 to 3 (IDR 3.0 [95% CrI 2.1-4.2]) and STAT 4 or 5 (IDR 3.1 [95% CrI 2.4-3.9]) cases. High-volume hospitals had less variability across outcomes and risk categories. Simulations suggested potential reductions in deaths (n=282), major complications (n=1539), and length of stay (101 183 days) over the 4-year study period if all hospitals were to perform at the current median or better, with 37% to 60% of the improvement related to the STAT 1 to 3 (lower risk) group across outcomes. CONCLUSIONS: We demonstrate significant hospital variation in morbidity and mortality after congenital heart surgery. Contrary to traditional thinking, a substantial portion of potential improvements that could be realized on a national scale were related to variability among lower-risk cases. These findings suggest modifications to our current approaches to optimize care and outcomes in this population are needed.

Variability in clinical decision-making for ventricular assist device implantation in pediatrics.

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.

Vascular Rings With Tracheoesophageal Compression: Management Considerations.

Our series of vascular ring patients treated at Ann & Robert H. Lurie Children's Hospital of Chicago has now exceeded 450 patients (see Central Picture). The recommendations from this review are based on our experience with those patients. Since 1990, we have used preoperative cross-sectional imaging in all vascular ring patients. This has led us to refine our operative strategies and carefully tailor the operation to the individual's unique anatomy. In particular for right aortic arch patients, we now address the Kommerell diverticulum in most cases and carefully consider Kommerell diverticulum resection with left subclavian artery transfer. In double aortic arch patients, we also look for a Kommerell diverticulum and resect the diverticulum if present. This will decrease the number of patients presenting for a reoperation after initial vascular ring surgery. Since 1947, we have operated on 217 patients with a right aortic arch. The median age is 1.5 years. Since 2006, 47 out of 111 patients have had Kommerell diverticulum resection and left subclavian artery transfer to the left carotid artery. Mean length of stay is 4.5 days. In the same time period, 187 patients have been treated for a double aortic arch. The median age is 1 year. Twelve of these patients also had resection of a Kommerell diverticulum. The mean length of stay in this group was 3 days. Advanced preoperative cross-sectional imaging leads to precise operative techniques tailored to the unique anatomy of each vascular ring patient.

BK polyomavirus infection in pediatric heart transplant recipients: a prospective study.

BKV infection and nephropathy complicate pediatric HTx, but the incidence and time course of the disease are unknown. We assessed the incidence of BKV infection and its association with kidney dysfunction in pediatric HTx recipients. A single center prospective study compared pediatric (<18 years) HTx recipients, with and without BKV infection, who received an allograft between September 2013 and December 2014. Screening of urine for BKV was performed prior to transplant, and at week 1, and at months 3, 6, 9, 12, and 15 months post-transplantation. Serum for BKV DNA was assayed if BK viruria was present. Statistics included Fisher's exact test and Student's t test. Twelve patients were enrolled. Two patients were removed per parent request. Two (20%) had BK viruria and one (10%) had BK viremia. No patients developed BKVN. BK viruria was present within 2 months following transplantation. There were no identifiable risk factors for BKV infection and no statistically significant difference in renal function between the groups; however, there was a trend toward worsening renal function in those with BKV infection. BKV infection can occur early following heart transplantation. Screening for BK viruria should be considered in HTx recipients.

Successful Bridge-to-Transplant of Functionally Univentricular Patients With a Modified Continuous-Flow Ventricular Assist Device.

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 m2 (median = 0.54 m2 ). 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.

Rescuing the Late Failing Fontan: Focus on Surgical Treatment of Dysrhythmias.

Patients with a failing Fontan frequently have dysrhythmias. This review will focus on the treatment of those dysrhythmias based on the 140 patients who have had a Fontan conversion with arrhythmia surgery at Ann & Robert H. Lurie Children's Hospital of Chicago (Chicago, IL). Important technical points to be emphasized are the following:• Atrial fibrillation is extremely common in patients with a failing Fontan and strong consideration should be given to a Cox-maze III during any Fontan conversion• Sinus node dysfunction is common post-conversion and all patients should receive a dual-chamber epicardial pacemaker• Careful preoperative imaging with either computed tomographic or magnetic resonance imaging is needed to predict re-entry problems at the time of repeat sternotomy• If the patient has protein-losing enteropathy, ascites, or a right or indeterminate ventricular morphology, strong consideration should be given to heart transplantation rather than a Fontan conversion and arrhythmia surgery. Freedom from cardiac death or transplant for patients undergoing Fontan conversion with arrhythmia surgery is 84% at 10 years. The effects of atrial arrhythmia operations are durable in most patients. There is increasing evidence that earlier referral of patients with an atriopulmonary Fontan for Fontan conversion will improve long-term results. Fontan conversion should be performed at centers with the institutional expertise to care for these complex patients. Note that this is an eroding patient population. The time to act may be now.

Risk Factors for Cardiac Arrest or Mechanical Circulatory Support in Children with Fulminant Myocarditis.

In children with fulminant myocarditis (FM), we sought to describe presenting characteristics and clinical outcomes, and identify risk factors for cardiac arrest and mechanical circulatory support (MCS). A retrospective review of patients with FM admitted at our institution between January 1, 2004, and June 31, 2015, was performed. We compared characteristics and outcomes of FM patients who received cardiopulmonary resuscitation (CPR) and/or were placed on MCS (CPR/MCS group) to those who did not develop these outcomes (Control group). There were 28 patients who met criteria for FM. Median age was 1.2 years (1 day-17 years). Recovery of myocardial function occurred in 13 patients (46%); 6 (21%) had chronic ventricular dysfunction, 6 (21%) underwent heart transplantation, and 3 (11%) died prior to hospital discharge (including one death following heart transplant). Of the 28 FM patients, 13 (46%) developed cardiac arrest (n = 11) and/or received MCS (n = 8). When compared to controls, patients in the CPR/MCS group had a higher peak b-type natriuretic peptide (BNP) levels (p = 0.03) and peak inotropic scores (p = 0.02). No significant differences were found between groups in demographics; chest radiograph, electrocardiogram, or echocardiogram findings; or initial laboratory values including BNP, troponin, C-reactive protein, lactate, and creatinine (p > 0.05 for all). Children with FM are at high risk of cardiovascular collapse leading to the use of CPR or MCS. Aside from peak BNP levels and inotropic scores, the most presenting characteristics were not helpful for predicting these outcomes. FM patients should ideally receive care in centers that provide emergent MCS.

Understanding the spectrum of sinus venosus interatrial communications.

BACKGROUND: It is still thought by some that a common wall is to be found in the normal heart between the attachments of the caval and pulmonary veins, with absence of this wall underscoring the presence of sinus venosus defects. Recent findings using episcopic microscopy in developing mice have shown the deficiencies of this notion. Understanding that the superior rim of the oval fossa is a fold, rather than a true septum, which can be distorted in the presence of partially anomalous pulmonary venous drainage, has provided an alternative explanation for the morphogenesis of sinus venosus defects. METHODS: We reviewed our experience with patients suspected of having a sinus venosus defect from August, 2011, through October, 2015, analysing the findings in light of the current hypotheses used to explain the development of the defects, along with correlations made by inspection of autopsy specimens. RESULTS: We evaluated findings from 16 patients, with a mean age of 7.7 years, ranging from 2.7 to 15 years. Of the group, 13 were ultimately diagnosed with a superior sinus venosus defect, two with an inferior defect, and one with isolated anomalous pulmonary venous connection in the absence of an interatrial communication. Initially, two patients were thought to have oval fossa defects, one from each subtype, but were correctly diagnosed following cardiac magnetic resonance interrogation. Anomalous pulmonary venous connections were present in all cases. CONCLUSION: Appreciation of the changes occurring during normal cardiac development helps in understanding the anatomical substrate underscoring the spectrum of sinus venosus defects. The lesions are veno-venous connections due to partially anomalous pulmonary venous connections, producing interatrial communications outside the confines of the interatrial septum.

Transcranial Doppler Identification of Neurologic Injury during Pediatric Extracorporeal Membrane Oxygenation Therapy.

BACKGROUND: We used transcranial Doppler to examine changes in cerebral blood flow velocity in children treated with extracorporeal membrane oxygenation. We examined the association between those changes and radiologic, electroencephalographic, and clinical evidence of neurologic injury. METHODS: This was a retrospective review and prospective observational study of patients 18 years old and younger at a single university children's hospital. Transcranial Doppler studies were obtained every other day during the first 7 days of extracorporeal membrane oxygenation, and 1 additional study following decannulation, in conjunction with serial neurologic examinations, brain imaging, and 6- to 12-month follow-up. RESULTS: The study included 27 patients, the majority (26) receiving veno-arterial extracorporeal membrane oxygenation. Transcranial Doppler velocities during extracorporeal membrane oxygenation were significantly lower than published values for age-matched healthy and critically ill children across different cerebral arteries. Neonates younger than 10 days had higher velocities than expected. Blood flow velocity increased after extracorporeal membrane oxygenation decannulation and was comparable with age-matched critically ill children. There was no significant association between velocity measurements of individual arteries and acute neurologic injury as defined by either abnormal neurologic examination, seizures during admission, or poor pediatric cerebral performance category. However, case analysis identified several patients with regional and global increases in velocities that corresponded to neurologic injury including stroke and seizures. CONCLUSIONS: Cerebral blood flow velocities during extracorporeal membrane oxygenation deviate from age-specific normal values in all major cerebral vessels and across different age groups. Global or regional elevations and asymmetries in flow velocity may suggest impending neurologic injury.

New-onset diabetes mellitus after heart transplantation in children - Incidence and risk factors.

Diabetes mellitus is a recognized complication of SOT in adults and is associated with decreased graft and patient survival. Little is known about NOD in pediatric HT recipients. We aimed to characterize the incidence and describe risk factors for development of NOD after HT in children. Children who developed diabetes after HT were identified from the OPTN database. Demographic and clinical data before and after transplant were compared between patients with and without NOD. A total of 2056 children were included, 56% were male, 54% were Caucasian, and 62% had cardiomyopathy prior to HT. NOD developed in 219 children (11%) after HT. The incidence of NOD was 2.4, 9.0, and 10.4% at one, five, and 10 yr after HT, respectively. Obesity (HR: 4.32), dialysis prior to transplant (HR: 2.38), African American race (HR: 1.86), transplant before year 2000 (HR: 1.82), female gender (HR: 1.68), and older age at transplant (HR: 1.28) were independent predictors of NOD. The major modifiable risk factor for NOD is obesity, imparting the maximum hazard. Improved surveillance for diabetes in high-risk patients and specific prevention and intervention strategies are imperative in this population.

Shunt Lesions Part I: Patent Ductus Arteriosus, Atrial Septal Defect, Ventricular Septal Defect, and Atrioventricular Septal Defect.

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.

Clarifying the anatomy of hearts with concordant ventriculo-arterial connections but abnormally related arterial trunks.

Hearts in which the arterial trunks arise from the morphologically appropriate ventricles, but in a parallel manner, rather than the usual spiralling arrangement, have long fascinated anatomists. These rare entities, for quite some time, were considered embryological impossibilities, but ongoing experience has shown that they can be found in various segmental combinations. Problems still exist about how best to describe them, as the different variants are often described with esoteric terms, such as anatomically corrected malposition or isolated ventricular inversion. In this review, based on our combined clinical and morphological experience, we demonstrate that the essential feature of all hearts described in this manner is a parallel arrangement of the arterial trunks as they exit from the ventricular mass. We show that the relationship of the arterial roots needs to be described in terms of the underlying ventricular topology, rather than according to the arrangement of the atrial chambers. We then discuss the importance of determining atrial arrangement on the basis of the morphology of the appendages, following the precepts as set out in the so-called "morphological method" and distinguished according to the extent of the pectinate muscles relative to the atrioventricular junctions as opposed to basing diagnosis on the venoatrial connections. We show that, when approached in this manner, the various combinations can be readily diagnosed in the clinical setting and described in straightforward way.

Gestational age at birth and outcomes after neonatal cardiac surgery: an analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database.

BACKGROUND: Gestational age at birth is a potentially important modifiable risk factor in neonates with congenital heart disease. We evaluated the relationship between gestational age and outcomes in a multicenter cohort of neonates undergoing cardiac surgery, focusing on those born at early term (ie, 37-38 weeks' gestation). METHODS AND RESULTS: Neonates in the Society of Thoracic Surgeons Congenital Heart Surgery Database who underwent cardiac surgery between 2010 and 2011 were included. Multivariable logistic regression was used to evaluate the association of gestational age at birth with in-hospital mortality, postoperative length of stay, and complications, adjusting for other important patient characteristics. Of 4784 included neonates (92 hospitals), 48% were born before 39 weeks' gestation, including 31% at 37 to 38 weeks. Compared with a 39.5-week gestational age reference level, birth at 37 weeks' gestational age was associated with higher in-hospital mortality, with an adjusted odds ratio (95% confidence interval) of 1.34 (1.05-1.71; P=0.02). Complication rates were higher and postoperative length of stay was significantly prolonged for those born at 37 and 38 weeks' gestation (adjusted P<0.01 for all). Late-preterm births (34-36 weeks' gestation) also had greater mortality and postoperative length of stay (adjusted P≤0.003 for all). CONCLUSIONS: Birth during the early term period of 37 to 38 weeks' gestation is associated with worse outcomes after neonatal cardiac surgery. These data challenge the commonly held perception that delivery at any time during term gestation is equally safe and appropriate and question the related practice of elective delivery of fetuses with complex congenital heart disease at early term.

Extracorporeal Membrane Oxygenation in Pediatric Trisomy 21: 30 Years of Experience from the Extracorporeal Life Support Organization Registry.

OBJECTIVES: To describe the use of extracorporeal membrane oxygenation (ECMO) in patients with trisomy 21 (T21), to identify risk factors for hospital mortality, and to compare outcomes with those of patients without T21. STUDY DESIGN: Children under age 18 years registered in the Extracorporeal Life Support Organization Registry were included. Comparisons between patients with T21 and patients without T21 were performed using the χ(2) or Wilcoxon rank-sum test and multivariable logistic regression. RESULTS: The study cohort included 623 patients with T21 and 46 239 patients without T21. The prevalence of T21 was 13.5/1000 patients receiving ECMO. ECMO utilization in patients with T21 increased over time, with 60% of cases occurring in the last decade. There was no significant difference in survival between patients without T21 and those with T21 (63% vs 57%; P = .23). In patients with T21, independent risk factors for mortality before cannulation were a cardiac indication for ECMO support and milrinone use (P ≤ .001 for both). Multivariable risk factors for mortality on ECMO included hemorrhagic, neurologic, renal, and pulmonary complications (P < .04 for all). CONCLUSION: The use of ECMO in patients with T21 has increased over time. Patients with a cardiac indication for ECMO have higher mortality compared with those supported for respiratory indications. Despite differences in indications for ECMO, patients with T21 have similar hospital survival as those without T21; thus, by itself, a diagnosis of T21 should not be considered a risk factor for in-hospital mortality when contemplating ECMO cannulation.

Role for immune monitoring to tailor induction prophylaxis in pediatric heart recipients.

UNLABELLED: rATG is used for HTx induction but is costly and associated with infection and PTLD. HYPOTHESIS: Tailoring rATG induction with CD3 monitoring results in less infection, reduced costs, and similar rejection. Retrospective review of HTx recipients receiving rATG induction. Control cases received "usual" rATG dosing (1.5 mg/kg/day typically × 5 days). Starting in October 2009, absolute CD3 monitoring (target <25 cells/mm(3) ) guided rATG dosing (study cases). Outcomes included first-year incidence of infection/rejection, direct costs of therapy, and incidence of PTLD/death. Study cases (n = 32) received fewer doses of rATG (median 4 vs. 5, p < 0.001) and less total rATG (median 3.2 vs. 7.4 mg/kg, p < 0.001) compared with controls (n = 17). There was no difference in incidence of infection, rejection, or patient survival during the first year post-HTx. There was one early death in both groups and one late case of PTLD in the control group. Drug savings were significant (median drug cost per patient $2718 vs. $4756, p < 0.001). CD3-tailored rATG induction in HTx recipients is associated with reduced drug costs and similar rates of rejection/infection. Longer follow-up will determine whether extended benefits are associated with this induction monitoring strategy.

Reoperation after vascular ring repair.

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.

Atrial Septal Defect Closure: Not Always Straightforward.

A nine-year old boy presented following a chest x-ray for COVID-19 infection that showed cardiomegaly. Transthoracic echocardiogram revealed a large atrial septal defect, dilated right heart, and normal function. Surgical repair was performed eight weeks after his COVID diagnosis. After weaning from cardiopulmonary bypass, pulmonary artery pressures were 2/3 systemic and ectopy was seen, requiring inotropes, nitric oxide, lidocaine, and amiodarone. He was discharged on postoperative day (POD) 5. On POD 6, he presented with acute right foot ischemia. Computed tomography showed a large aortic thrombus, requiring emergent thrombectomy. Coagulopathy workup was negative. Cardiac magnetic resonance imaging (CMR) and catheterization showed reduced biventricular function and diastolic dysfunction. Diuretics and ß blockers were started, with gradual improvement in left ventricular systolic function.

The aortic uncrossing procedure.

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.