The Supported vs Unsupported Ross in Pediatric Patients: Neoaortic Root and Ventricular Function.

BACKGROUND: The supported Ross is used to mitigate the neoaortic root dilation that has been described with the unsupported Ross. There is limited literature assessing the efficacy of the supported Ross in young patients. In this study, the fate of the neoaortic root was compared in the supported and unsupported Ross procedure in adolescent patients. METHODS: A retrospective review was performed of patients who underwent the Ross procedure between 1996 and 2019. An analysis was conducted of patients aged 10 to 18 years who underwent the supported and unsupported Ross operation, without a Konno enlargement, to assess for longitudinal echocardiographic changes. Given differences in follow-up time, both regression analysis and Mann-Whitney nonparametric tests were used to correct for time from discharge to most recent follow-up. RESULTS: The median follow-up time for supported and unsupported Ross patients without a Konno enlargement was 2.90 years (0.21-13.03 years) and 12.13 years (2.63-19.47 years), respectively. Unsupported Ross patients experienced a higher rate of change per year in the aortic annulus (P = .003 and P = .014) and aortic sinus (P = .002 and P = .002) diameters, respectively. There was no significant difference in the rate of change of end-diastolic left ventricular internal diameter (P = .703 and P = .92) and aortic insufficiency (P = .687 and P = .215) between the supported and unsupported Ross patients. CONCLUSIONS: Progressive dilation of the neoaortic root in unsupported Ross patients is significantly mitigated with the supported Ross with excellent stability. The supported Ross is safe and effective and may play an increasing role in the management of children with aortic disease.

The Impact of and Barriers to Cardiac Rehabilitation Following Cardiac Surgery in the Adult With Congenital Heart Disease.

INTRODUCTION: There is a paucity of literature evaluating the impact of and barriers to participation in cardiac rehabilitation (CR) in the adult congenital heart disease population. The aims of this study were to evaluate the impact of CR on physical activity and health-related quality of life, as well as to evaluate the barriers to participation in CR in a post-operative adult congenital heart disease population. METHODS: Patients ≥18 yr of age seen in the Wisconsin Adult Congenital Heart Disease Program and post-open sternotomy surgery from 2010-2015 were eligible for inclusion. Subjects were mailed a novel physical activity survey and the validated EuroQOL-5D 3L health questionnaire. A retrospective medical record review was performed to extract demographic and clinical data. RESULTS: One hundred thirty-five patients underwent open sternotomy surgery from 2010-2015. Of these, 22 were excluded because of intellectual disability, three opted out, and three survey packets were returned to the sender. A total of 54 of the remaining 107 patients returned completed surveys. Of these, 47 (87%) were referred to CR. Thirty-five patients completed the entire CR program (74%). Those who completed CR were more likely to develop a home/independent exercise program (P = .027). Barriers to completing CR included insurance coverage, psychiatric disease, and a perception that CR would not be of benefit. CONCLUSION: Completing CR was associated with developing a home/independent exercise program in post-sternotomy adult patients with congenital heart disease. Barriers to participating in and completing CR in this population could lead to an improved completion rate if modified.

Coronary artery disease screening in adults with congenital heart disease prior to cardiac surgery.

OBJECTIVE: As adults with congenital heart disease (CHD) grow older, preoperative screening for coronary artery disease (CAD) may be indicated prior to CHD surgery. Data regarding the indications for preoperative CAD screening in this population are limited. Current practice is to follow guidelines for patients with valvular heart disease; however, the risk for CAD in certain congenital heart diagnoses may be higher than the general population. This study aimed to assess the results of preoperative CAD screening in patients prior to CHD surgery. DESIGN: Retrospective study. SETTING: Single tertiary center. PATIENTS: Patients ≥35 years that had CHD surgery from 1/1/2007 to 5/1/2017. OUTCOME MEASURES: Data regarding CAD risk factors and preoperative CAD screening results were obtained. Prevalence and risk factors for CAD were analyzed, along with their relationship to perioperative outcomes. RESULTS: A total of 73 patients underwent CAD screening with either cardiac catheterization (56%) or computed tomography angiography (34%) prior to CHD surgery. Overall 16 (22%) patients were found to have CAD. Only two patients had severe coronary stenosis and underwent coronary bypass grafting at time of CHD surgery. Patients with CAD were more likely to be older and have history of hypertension, dyslipidemia, and tobacco smoking. CHD diagnosis was not significantly associated with presence of CAD. CONCLUSION: CAD is common in asymptomatic older patients referred for screening prior to CHD surgery; however, severe CAD requiring concomitant coronary intervention is uncommon. Preoperative CAD screening should be based on age and traditional CAD risk factors, rather than underlying CHD.

Cardiac Arrest Secondary to Traumatic Aortopulmonary Window During Transcatheter Pulmonary Valve Implantation in Supported Ross.

We present a patient with a supported Ross procedure and severe pulmonary homograft stenosis who developed cardiac arrest while undergoing transcatheter pulmonary valve replacement and was found to have a large iatrogenic aortopulmonary window. Cardiopulmonary resuscitation was initiated followed by covered stent placement, extracorporeal membrane oxygenation support, and ultimately emergent surgery with a good outcome. (Level of Difficulty: Advanced.).

Identifying self-reported neurocognitive deficits in the adult with congenital heart disease using a simple screening tool.

OBJECTIVE: Children with congenital heart disease (CHD) and adults with acquired heart disease are at an increased risk of neurocognitive impairment. The objective of this study was to determine the prevalence of self-reported neurocognitive impairment and its risk factors in the adult congenital heart disease (ACHD) population. DESIGN: The Wisconsin Adult Congenital Heart Disease Program recently began screening ACHD patients to identify those with significant self-perceived neurocognitive impairments. Screening consists of using a validated neuro-oncology screening instrument that has been modified for the ACHD population. Patients who answer this survey in a predetermined fashion consistent with significant self-perceived neurocognitive deficits are referred for a formal neurocognitive evaluation. Demographic and clinical information are obtained by chart review. RESULTS: Three hundred ten patients (49% males) completed the screening process. The average age was 30 years (range: 17-69 years). For the cohort, 57 (18%) patients had no prior cardiac surgeries, 85 (28%) one surgery, 77 (25%) two, and 91 (29%) at least three surgeries. Of those screened, 106 (34%) met criteria for a formal neurocognitive evaluation. Patients who were referred had undergone a greater number of prior cardiac surgeries (2.2 vs 1.7, P = .008) and were more likely to have severe complexity CHD (P = .006). Of those patients who were referred, the worst perceived functioning was in math and attention. CONCLUSION: There is a high prevalence of ACHD patients with significant self-perceived neurocognitive deficits. Simple screening questionnaires may help identify those patients at high risk and allow for timely and appropriate referral for formal neurocognitive evaluation, diagnosis, and therapy.

Reintervention following stage 1 palliation: A report from the NPC-QIC Registry.

BACKGROUND: Single ventricle heart disease with aortic arch hypoplasia has high morbidity and mortality, with the greatest risk after stage 1 palliation. Residual lesions often require catheter-based or surgical reintervention to minimize risk. We sought to describe the types, frequency, and risk factors for re-intervention between stage 1 and stage 2 palliation, utilizing the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) registry. METHODS: The NPC-QIC registry, consisting of patients discharged after stage 1 palliation, was queried. Hybrid stage 1 palliation patients were excluded from this study. The primary risk factor was shunt type and the primary outcome was re-intervention. RESULTS: Of 1156 patients, (50%) had re-intervention. There was no difference in total rate of re-intervention by shunt type (BT shunt 52% vs. RVPA shunt 48%; P = .17). Patients with a BT shunt had increased re-intervention during stage 1 hospitalization (P =.002). During the interstage period, following discharge from stage 1 palliation, patients with a BT shunt had increased aortic arch re-intervention (P < .005), while patients with an RVPA shunt had increased re-intervention on the shunt and the pulmonary arteries (P = .02). Postoperative mechanical ventilation >14 d (P < .01) was the only risk factor associated with re-intervention by multivariable analysis, regardless of shunt type. CONCLUSIONS: Re-intervention between stage I and stage 2 palliation is common. There is no difference in cumulative frequency of re-intervention between shunt types, though types and timing of re-intervention varied between shunt types. Longitudinal assessment of the NPC-QIC database is important to identify long term outcomes of patients requiring re-intervention.

The Impact of the Left Ventricle on Right Ventricular Function and Clinical Outcomes in Infants with Single-Right Ventricle Anomalies up to 14 Months of Age.

BACKGROUND: Children with single-right ventricle anomalies such as hypoplastic left heart syndrome (HLHS) have left ventricles of variable size and function. The impact of the left ventricle on the performance of the right ventricle and on survival remains unclear. The aim of this study was to identify whether left ventricular (LV) size and function influence right ventricular (RV) function and clinical outcome after staged palliation for single-right ventricle anomalies. METHODS: In the Single Ventricle Reconstruction trial, echocardiography-derived measures of LV size and function were compared with measures of RV systolic and diastolic function, tricuspid regurgitation, and outcomes (death and/or heart transplantation) at baseline (preoperatively), early after Norwood palliation, before stage 2 palliation, and at 14 months of age. RESULTS: Of the 522 subjects who met the study inclusion criteria, 381 (73%) had measurable left ventricles. The HLHS subtype of aortic atresia/mitral atresia was significantly less likely to have a measurable left ventricle (41%) compared with the other HLHS subtypes: aortic stenosis/mitral stenosis (100%), aortic atresia/mitral stenosis (96%), and those without HLHS (83%). RV end-diastolic and end-systolic volumes were significantly larger, while diastolic indices suggested better diastolic properties in those subjects with no left ventricles compared with those with measurable left ventricles. However, RV ejection fraction was not different on the basis of LV size and function after staged palliation. Moreover, there was no difference in transplantation-free survival to Norwood discharge, through the interstage period, or at 14 months of age between those subjects who had measurable left ventricles compared with those who did not. CONCLUSIONS: LV size varies by anatomic subtype in infants with single-right ventricle anomalies. Although indices of RV size and diastolic function were influenced by the presence of a left ventricle, there was no difference in RV systolic function or transplantation-free survival on the basis of LV measures.

Anatomic Repair of Congenitally Corrected Transposition of the Great Arteries: Single-Center Intermediate-Term Experience.

We present our experience for patients who have undergone an anatomic repair (AR) for congenitally corrected transposition of the great arteries (CCTGA) at the Children's Hospital of Wisconsin. A retrospective chart review of patients who underwent AR for CCTGA from 2001 to 2015 was performed. The cohort consisted of 15 patients (74% male). Median age of anatomic repair was 15 months (range 4.5-45.6 months). Four patients had a bidirectional Glenn (BDG) prior to AR. At the time of AR,-9 (60%) underwent Senning/Rastelli procedure, 4 (26.6%) had double switch operation, and 2 (13.3%) underwent only Senning with VSD closure. Median duration of follow-up was 5.5 years (0.05-14 years). Reoperations prior to discharge included BDG, revision of pulmonary venous baffle, closure of residual VSD, and pacemaker placement. Late reoperations included left ventricular outflow tract obstruction repair, conduit replacement, melody valve placement, and pacemaker implantation. At their most recent follow-up, no patient had heart failure symptoms and only 1 had severely diminished function that improved with cardiac resynchronization therapy. Moderate mitral regurgitation was noted in 15% (2/13), and severe in 7% (1/13). Moderate tricuspid regurgitation was noted in 15% (2/13). One patient, 7% (1/13), developed moderate aortic insufficiency. There was a 100% survival at the time of the most recent follow-up. Patients with CCTGA who have undergone AR have excellent functional status and mid-term survival but reinterventions are common. Longer term studies are needed to determine both the extent and spectrum of reinterventions as well as long term survival.

Center Variability in Timing of Stage 2 Palliation and Association with Interstage Mortality: A Report from the National Pediatric Cardiology Quality Improvement Collaborative.

For infants with single-ventricle lesions with aortic arch hypoplasia, the interstage period from discharge following stage 1 palliation (S1P) until stage 2 palliation (S2P) remains high risk. Significant variability among institutions exists around the timing of S2P. We sought to describe institutional variation in timing of S2P, determine the association between timing of S2P and interstage mortality, and determine the impact of earlier S2P on hospital morbidity and mortality. The National Pediatric Cardiology Quality Improvement Collaborative registry was queried. Centers were divided based on median age at S2P into early (n = 15) and late (n = 16) centers using a cutoff of 153 days. Groups were compared using Chi-squared or Wilcoxon rank-sum test. Multivariable logistic regression was used to determine risk factors for interstage mortality. The final cohort included 789 patients from 31 centers. There was intra- and inter-center variability in timing of S2P, with the median age by center ranging from 109 to 214 days. Late centers had a higher mortality (9.9 vs. 5.7 %, p = 0.03) than early centers. However, the event rate (late: 8.2 vs. early: 5.8 deaths per 10,000 interstage days) was not different by group (p = 0.26). Survival to hospital discharge and hospital length of stay following S2P were similar between groups. In conclusion, in a large multi-institution collaborative, the median age at S2P varies among centers. Although optimal timing of S2P remains unclear, centers performing early S2P did not experience worse S2P outcomes and experienced less interstage mortality.

Growth Trajectory in Children with Trisomy 21 with and without Atrioventricular Septal Defect.

OBJECTIVE: Trisomy 21 is associated with poor weight gain and atrioventricular septal defects. The impact of atrioventricular septal defects on weight gain in the setting of Trisomy 21 has not previously been described in the recent era. This study aimed to determine if such an association is present. DESIGN: Patients with Trisomy 21 with and without atrioventricular septal defects were identified. Clinical, surgical, and postoperative data were collected were for these patients and then compared between patients. Specifically, weight for age z-scores were compared at various time points in a univariate and multivariate fashion. Effect of timing of surgery in those with an atrioventricular septal defect was also studied. RESULTS: A total of 86 patients were identified, 42 with an atrioventricular septal defect. There was a difference in weight for age z-scores between patients with and without atrioventricular septal defects only at 2 months (P value .038) and 6 months (P value .003) of age. This persisted after multivariate regression which demonstrated atrioventricular septal defects as an independent risk factor. There was no difference noted in weight at 2 years of age in patients undergoing atrioventricular septal defect repair before and 150 days of life. CONCLUSION: There was a statistically significant, but not clinically relevant, difference in weight between the patients with Trisomy 21 with and without atrioventricular septal defects in our cohort. Those with atrioventricular septal defects required more nutritional intervention, such as gastrostomy tube placement. Timing of repair did not alter outcomes at midterm follow-up.

The Impact of Obesity on Postoperative Outcomes in Adults with Congenital Heart Disease Undergoing Pulmonary Valve Replacement.

OBJECTIVE: The impact of obesity on surgical morbidity in adults with congenital heart disease is currently unknown. The aim of our study was to investigate the impact of obesity on postoperative outcomes in adults with congenital heart disease undergoing reoperation for pulmonary valve replacement. METHODS: A retrospective analysis was performed assessing the influence of obesity on surgical outcomes. Obesity was defined as a body mass index ≥30 kg/m2. RESULTS: The mean body mass index of the cohort was 25.9 ± 6.9 kg/m2 . The cohort included 71 patients with 17 patients (24%) being obese. There was no postoperative mortality. Obese patients had a longer hospital length of stay (6.6 vs. 4.7 days; P < .001) and increased incidence of postoperative arrhythmias (29% vs. 5.6%; P = .003) compared with nonobese patients. Multivariable analysis performed using logistic regression with backwards elimination demonstrated obesity was independently associated with hospital length of stay >5 days (odds ratio [OR] = 5.2; 95% confidence interval [CI]: 1.5-18.2, P = .01) and with increased postoperative arrhythmias (OR = 4.2; 95% CI: 1.7-40, P < .01). CONCLUSIONS: Obesity is associated with increased morbidity in adults with congenital heart disease undergoing pulmonary valve replacement, including longer hospitalization and higher risk for postoperative arrhythmias.

Tricuspid repair at pulmonary valve replacement does not alter outcomes in tetralogy of Fallot.

BACKGROUND: Chronic pulmonary regurgitation after tetralogy of Fallot repair often leads to progressive right ventricle dilation, dysfunction, and frequently, pulmonary valve replacement. For those with significant tricuspid regurgitation at the time of pulmonary valve replacement, it is unknown whether concomitant tricuspid valve repair improves postoperative outcomes. METHODS: This is a retrospective review of patients after tetralogy of Fallot repair who underwent pulmonary valve replacement between 1999 and 2012. Preoperative and postoperative echocardiograms were assessed for tricuspid regurgitation (vena contracta) and right ventricular size and function (Tomtec software). RESULTS: Sixty-two patients underwent pulmonary valve replacement. Thirty-six (58%) had greater than or equal to moderate tricuspid regurgitation on preoperative echocardiogram. Significant predictors were not identified. Of the 36, 18 (50%) underwent concomitant tricuspid valve repair at the time of pulmonary valve replacement. After surgery, there was a significant reduction in the degree of tricuspid regurgitation (p < 0.001) and measures of right ventricular size (p < 0.05) in both cohorts. Between surgical groups, there was no statistical difference in the grade of tricuspid regurgitation (p = 0.47) or measures of right ventricular size (p > 0.4) at 6-month follow-up. CONCLUSIONS: Tricuspid regurgitation is a common finding in repaired tetralogy of Fallot, although risk factors for its development remain unclear. After pulmonary valve replacement with or without tricuspid valve repair there is significant improvement in the degree of tricuspid regurgitation and right ventricular size. Finally, 6 months after pulmonary valve replacement there were no statistical differences between those patients undergoing concomitant tricuspid valve repair and those undergoing pulmonary valve replacements alone.

Effect of feeding modality on interstage growth after stage I palliation: a report from the National Pediatric Cardiology Quality Improvement Collaborative.

OBJECTIVES: Achieving adequate growth after stage 1 palliation for children with single-ventricle heart defects often requires supplemental nutrition through enteral tubes. Significant practice variability exists between centers in the choice of feeding tube. The impact of feeding modality on the growth of patients with a single ventricle after stage 1 palliation was examined using the multiinstitutional National Pediatric Cardiology Quality Improvement Collaborative data registry. METHODS: Characteristics of patients were compared by feeding modality, defined as oral only, nasogastric tube only, oral and nasogastric tube, gastrostomy tube only, and oral and gastrostomy tube. The impact of feeding modality on change in weight for age z-score during the interstage period, from stage 1 palliation discharge to stage 2 palliation, was evaluated by multivariable linear regression, adjusting for important patient characteristics and postoperative morbidities. RESULTS: In this cohort of 465 patients, all groups demonstrated improved weight for age z-score during the interstage period with a mean increase of 0.3±0.8. In multivariable analysis, feeding modality was not associated with differences in the change in weight for age z-score during the interstage period (P=.72). Risk factors for poor growth were a diagnosis of hypoplastic left heart syndrome (P=.003), vocal cord injury (P=.007), and lower target caloric goal at discharge (P=.001). CONCLUSIONS: In this large multicenter cohort, interstage growth improved for all groups and did not differ by feeding modality. With appropriate caloric goals and interstage monitoring, adequate growth may be achieved regardless of feeding modality and therefore local comfort and complication risk should dictate feeding modality.

Improving interstage survival after Norwood operation: outcomes from 10 years of home monitoring.

OBJECTIVE: Infants who undergo Norwood stage 1 palliation (S1P) continue with high-risk circulation until stage 2 palliation (S2P). Routine care during the interstage period is associated with 10% to 20% mortality. This report illustrates the sustained reduction of interstage mortality over 10 years associated with use of home monitoring. METHODS: Daily monitoring of oxygen saturation and weight was done for all patients discharged to home after S1P. Notification of the care team occurred for oxygen saturation<75% or >90%, weight gain<20 g over 3 days, weight loss>30 g, or intake<100 cc/kg/d. Breach of these criteria marked an interstage event. Interstage outcomes are reported. Patient characteristics and perioperative variables were compared between patients with and without interstage events. RESULTS: Over 10 years, 157 patients were discharged after S1P with home monitoring. Interstage survival was 98%. Breach of home criteria occurred in 59% (93 out of 157), with violation of oxygen saturation<75% the most common event. Patient characteristics, operative data, and early postoperative morbidity did not differ between patients with and without events. CONCLUSIONS: Home monitoring after S1P is associated with excellent interstage survival. Although a breach of monitoring criteria occurred in more than half of patients, our analysis failed to identify independent predictors of interstage events. Analysis of variables predicting mortality could not be assessed due to the low frequency of death in this cohort. Failure to identify specific variables for interstage events suggests that home monitoring, as part of an interstage surveillance program, should be applied to all S1P hospital survivors.

Significant response to oral Etoposide in the treatment of an unresectable cardiac sarcoma.

Primary cardiac sarcomas are rare and carry a poor prognosis. The standard of care is complete resection. Outcomes for patients without complete resection are dismal, and the benefit of adjuvant therapy is uncertain. A 9-year-old girl presented with a large right-sided cardiac mass. After biopsy, the tumor was classified as an undifferentiated sarcoma. Resection was not feasible due to apparent invasion of the right ventricle and atrioventricular groove. Treatment with oral etoposide resulted in a 97% reduction in tumor volume and allowed for complete resection of residual tumor. She is alive with no evidence of disease 25 months from diagnosis.

Feeding dysfunction in children with single ventricle following staged palliation.

OBJECTIVE: To determine the prevalence of feeding dysfunction in children with single ventricle defects and identify associated risk factors. STUDY DESIGN: Patients aged 2-6 years with single ventricle physiology presenting for routine cardiology follow-up at the Children's Hospital of Wisconsin were prospectively identified. Parents of the patients completed 2 validated instruments for assessment of feeding dysfunction. Chart review was performed to retrospectively obtain demographic and diagnostic data. RESULTS: Instruments were completed for 56 patients; median age was 39 months. Overall, 28 (50%) patients had some form of feeding dysfunction. Compared with a normal reference population, patients with single ventricle had statistically significant differences in dysfunctional food manipulation (P < .001), mealtime aggression (P = .002), choking/gagging/vomiting (P < .001), resistance to eating (P < .001), and parental aversion to mealtime (P < .001). Weight and height for age z-scores were significantly lower in subjects with feeding dysfunction (-0.84 vs -0.33; P < .05 and -1.46 vs -0.56; P = .001, respectively). Multivariable analysis identified current gastrostomy tube use (P = .02) and a single parent household (P = .01) as risk factors for feeding dysfunction. CONCLUSION: Feeding dysfunction is common in children with single ventricle defects, occurring in 50% of our cohort. Feeding dysfunction is associated with worse growth measures. Current gastrostomy tube use and a single parent household were identified as independent risk factors for feeding dysfunction.

Renal dysfunction is common among adults after palliation for previous tetralogy of Fallot.

Long-term survival after tetralogy of Fallot (TOF) repair is excellent. However, little is published regarding late noncardiac complications. This study aimed to determine the prevalence and risk factors for renal dysfunction among adults after TOF repair. For this study, 56 adult patients with complete repair of TOF were identified, and their charts were retrospectively reviewed. An estimated glomerular filtration rate (eGFR) for each patient was calculated using the Modification of Diet in Renal Disease formula (MDRD). Using each patient's eGFR, he or she was classified into stages based on the National Kidney Foundation chronic kidney disease (CKD) staging. Clinical parameters were compared among patients with and those without renal dysfunction to identify risk factors for renal impairment. The median estimated eGFR rate for the cohort was 78 ml/min/1.73 m(2). Based on the National Kidney Foundation CKD staging system, 54 % of the patients had at least stage 2 chronic renal disease. The risk factors identified were hypertension (p < 0.01), type 2 diabetes mellitus (p < 0.05), longer follow-up evaluation (p < 0.005), older age at complete repair (p < 0.05), and use of daily diuretics (p < 0.05). After repair of TOF, renal dysfunction is common at late follow-up evaluation. The study findings show the importance of routine assessment of renal function and the need to limit or avoid future episodes of acute kidney injury in this at-risk population.

Aortic aneurysms remain a significant source of morbidity and mortality after use of Dacron(®) patch aortoplasty to repair coarctation of the aorta: results from a single center.

Aortic aneurysm formation after coarctation repair is a serious and life-threatening complication. Repairs using synthetic materials such as Dacron(®) may carry the highest risk of aneurysm formation and rupture. The authors sought to determine the prevalence of aneurysm formation in patients who previously underwent coarctation repair using Dacron(®) patch aortoplasty at their institution. Between 1977 and 1994, 63 patients underwent isolated coarctation repair using Dacron(®) patch aortoplasty. Aneurysms were defined as an aortic dimension 1.5 times that of the aorta at the level of the diaphragm as shown by angiography, computed tomography (CT) scan, or magnetic resonance imaging (MRI). Of 61 early survivors, 29 (47 %) experienced an aneurysm in the area of previous repair. Nine patients (31 %) had spontaneous rupture of the aneurysm, which caused death in seven cases. Elective or emergent aneurysm repair was performed for 20 patients without complication, and 2 patients are being monitored at this writing. The mean interval from patch placement to aneurysm repair was 15 years (range, 4-27 years). Overall freedom from the development of an aortic aneurysm was 97 % at 5 years, 90 % at 10 years, 69 % at 20 years, and 42 % at 25 years. After repair of coarctation using Dacron(®) patch aortoplasty, the risk for aneurysm formation in the area of repair and death from rupture is extremely high. Therefore, in accordance with the 2008 American Heart Association/American College of Cardiology (AHA/ACC) guidelines, all patients with repaired aortic coarctation should undergo either CT or MRI imaging at least every 5 years to assess for aortic aneurysm formation. More frequent imaging should be obtained for patients previously repaired with Dacron(®) patch aortoplasty.

Long-term outcomes of the neoaorta after arterial switch operation for transposition of the great arteries.

BACKGROUND: After the arterial switch operation (ASO) for transposition of the great arteries (TGA), the native pulmonary root and valve function in the systemic position, and the long-term risk for neoaortic root dilation and valve regurgitation is currently undefined. The aim of this study was to determine the prevalence and progression of neoaortic root dilation and neoaortic valve regurgitation in patients with TGA repaired with the ASO. METHODS: Measurements of the neoaortic annulus, neoaortic root at the level of the sinuses of Valsalva, and the degree of neoaortic regurgitation were assessed by serial transthoracic echocardiograms on 124 patients with TGA at a median follow-up of 7.2 years (range, 1 to 23 years) after the ASO at our institution. RESULTS: Neoaortic root dilation with z scores of 2.5 or greater was identified in 66%, and the root diameter z score increased at an average rate of 0.08 per year over time after ASO. Freedom from neoaortic root dilation at 1, 5, 10, and 15 years after ASO was 84%, 67%, 47%, and 32%, respectively. Risk factors for root dilation include history of double-outlet right ventricle (p = 0.003), previous pulmonary artery banding (p = 0.01), and length of follow-up (p = 0.04). Neoaortic valve regurgitation of at least moderate degree was present in 14%. Neoaortic root dilation was a significant risk factor for neoaortic valve regurgitation (p < 0.0001). No patient required reintervention on the neoaorta or neoaortic valve during follow-up. CONCLUSIONS: Progressive neoaortic root dilation is common in patients with TGA after the ASO. Continued surveillance of this population is required.