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. (C) 2023 by The Society of Thoracic Surgeons

Background: Women with congenital heart disease (CHD) have increased risk for adverse events during pregnancy and delivery. Prior studies have assessed pregnancy and delivery outcomes at tertiary referral centers (TRC). The aim of our study was to assess pregnancy outcomes in women with CHD who deliver in a non-tertiary referral center (non-TRC). Methods: Clinical demographics were collected, including anatomic complexity, physiologic state and pre-pregnancy risk assessment. Patients were stratified by delivery location, either TRC or non-TRC. Maternal and neonatal complications of pregnancy were reported. Results: Women with CHD who delivered in a TRC had a higher pre-pregnancy risk when assessed by the Zahar and CARPREG-II scores, and had more patients fall into a higher WHO classification. There was no difference in rates of maternal cardiac complications between delivery locations (11%) and neonatal complications (20%) between deliveries at TRC and non-TRC. Conclusions: There were not increased maternal cardiac or neonatal complications when delivery occurred at a non-TRC. Neonatal complications remained high regardless of delivery location. This study suggests that proper risk assessment may help identify women who are candidates for safe delivery at non-TRC in women with CHD, and that neonatal resources should be considered when planning delivery location.

In a retrospective survey study, completing a cardiac rehabilitation (CR) program was found to be associated with developing a home/independent exercise program in post-sternotomy adults with congenital heart disease. Barriers to completing CR included lack of insurance coverage, psychiatric disease, and a perception that the program would not be beneficial. 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.

Background: Intranasal dexmedetomidine is an attractive option for procedural sedation in pediatrics due to ease of administration and its relatively short half-life. This study sought to compare the safety and efficacy of intranasal dexmedetomidine to a historical cohort of pediatric patients sedated using chloral hydrate in a pediatric echo lab. Methods: Chart review was performed to compare patients sedated between September, 2017 and October, 2019 using chloral hydrate and intranasal dexmedetomidine. Vital signs, time to sedation, duration of sedation, need for second dose of medication, rate of failed sedation, and impact on vital signs were compared between groups. Subgroup analysis was performed for those with complex and cyanotic heart disease. Results: Chloral hydrate was used in 356 patients and intranasal dexmedetomidine in 376. Patient age, complexity of heart disease, and duration of sedation were similar. Rates of failed sedation were very low and similar. Average heart rate and minimum heart rate were lower for those receiving intranasal dexmedetomidine than chloral hydrate. Impact on vital signs was similar for those with complex and cyanotic heart disease. No adverse events occurred in either group. Conclusions: Sedation with intranasal dexmedetomidine is comparable to chloral hydrate in regards to safety and efficacy for children requiring echocardiography. Consistent with the mechanism of action, patients receiving intranasal dexmedetomidine have a lower heart rate without morbidity.

The objective of our study was to determine the prevalence, risk factors, and the impact of obstructive sleep apnea (OSA) in the adult with congenital heart disease (ACHD). One hundred forty-nine consecutive patients seen in our ACHD program were screened for OSA using the Berlin Questionnaire. Demographic and clinical details on subjects were collected through a chart review. Clinical variables were analyzed to determine risk factors for positive OSA screen, as well as associated outcomes. Seventy-seven (52%) of our cohort were females. The median age of the cohort was 33 years (range = 18–74) and median weight was 79 kg (range = 50–145 kg). Overall, 47 (31%) of our cohort were found to have a positive OSA screen using the Berlin questionnaire. Median age of the patients whom tested positive was 34 years. Compared to patients with a negative screen, patients with a positive OSA screen were more likely to be heavier with a median weight of 99 kg vs 71 kg (p < 0.01) and a larger BMI (31 vs 25 kg/m2, p < 0.01). Overall, 55% of patients whom screened positive were obese (defined as a BMI > 30) compared to 15% in the negative group (p < 0.02). Patients with a positive screen were more likely to have other co-morbidities including diabetes (p < 0.04), hypertension (p < 0.05), depression (p < 0.002), and were more likely to have decreased exercise capacity (p < 0.01) and a defibrillator (p < 0.007). Our data demonstrates that OSA is common in the ACHD patient and is associated with increasing weight and BMI. Patients with a positive screen are at increased risk for multiple co morbidities including diabetes, hypertension, and depression. We believe our data supports the use of screening protocols for OSA in the ACHD population in effort to identify early, treat, and potentially prevent late complications.

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.

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.)

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.

Transcatheter valve-in-valve and valve-in-ring implantation has become a common approach to treating patients with failed bioprosthetic tricuspid valves as well as failed surgical repairs of the tricuspid valve where an annuloplasty ring has been utilized. We describe a case where a Melody valve was percutaneously implanted in a native tricuspid valve with severe stenosis following surgical repair without a supporting annuloplasty ring.

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.

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.

The letter of recommendation (LoR) is a required part of applications for residency and fellowship programs. Literature describing program director (PD) perceptions of LoR features and the unwritten code behind the phrases letter writers use when describing an applicant is sparse.

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.

Pregnancy is known to induce significant hemodynamic changes. The purpose of our studywas to determine the effects of these hemodynamic changes during pregnancy on systemic ventricular function (SVF) inwomen with pre-existing cardiac disease. A retrospective review ofwomen followed in theWisconsin Adult Congenital Heart Disease programwith a concurrent pregnancy from 2006 to 2015 was conducted. The SVF was measured in a baseline echocardiogram(obtained <= 12 months prior to the estimated dated of conception (EDC)), an echocardiogramduring pregnancy, and a post-partum echocardiogram. To verify stability of the SVF in the years prior to pregnancy, SVF was also measured on a control echocardiograms (obtained > 12 months prior to EDC) and compared to the baseline echocardiogram. Systemic ventricular function was assessed in each echocardiogram by measuring global peak longitudinal strain (GPLS) and ejection fraction (EF). A total of 46 pregnancies were included. The average age at the EDC was 25.7 +/- 5 years and the median gestational age at time of delivery was 38.0 weeks. The most common cardiac anomaly was coarctation of the aorta (26.1%) followed by tetralogy of Fallot (13%). Themean GPLS for the control echocardiograms, baseline echocardiograms, pregnancy echocardiograms and post-partum echocardiograms were as follows: -18.9 +/- 2.5, -18.0 +/- 3.4%,-15.9 +/- 2.9% and-17.9 +/- 3.6%. When measuring GPLS there was a significant drop in SVF during pregnancy (0 = 0.04) with a return to baseline in the post-partum period. When SVF was measured by EF there was a strong trend toward a significant drop in SVF during pregnancy (p=0.08). Our results show there is a significant drop in systemic ventricular function during pregnancy in women with pre-existing cardiac disease when measured by GPLS, which returns to baseline in the post-partum period. (c) 2016 Elsevier Ireland Ltd. All rights reserved.

Background: Congenitally corrected transposition of the great arteries (CCTGA) is a rare cardiac malformation resulting from abnormal looping of the embryonic cardiac tube that results in atrioventricular and ventriculoarterial discordance. Surgical options include a physiologic repair (PR) or

Background: The prevalence of sexual dysfunction(SD) and its impact on quality of life (QOL) inadults with congenital heart disease (CHD) is not well known. The aims of this study were to: determine the prevalence of SD, evaluate the risk factors associated with SD, and determine the association between SD and QOL in adults with CHD.Methods: This was a cross-sectional study of adults (>= 18 years) with CHD presenting for routine follow-up at our institution. Subjects completed the CDC HRQOL-14 "Health Days Measure" to assess mental and physical health, and either the Sexual Health Inventory for Men or the Female Sexual Function Index to assess sexual function. Baseline characteristics were obtained via chart review.Results: 105 subjects were enrolled. The mean age was 31.9 +/- 11.7 years, 53 (51%) weremen, 81% were NYHA Functional Class 1, and 76% had moderate or complex CHD. The rates of SD were 28% overall in the cohort, 30% in men and 25% in women. Men with SD were more likely to be taking spironolactone (p < 0.001) and digoxin (p=0.002). Men with SD reported a greater number of days of poor mental health (p=0.004), feeling anxious, worried or tense (p=0.003), needing assistance (p=0.042), and inhibited activity of daily living (p=0.009). Women with SD were more likely to have atrial arrhythmias (p = 0.002) and to report fewer days feeling healthy and energetic (p = 0.031).Conclusions: SD is highly prevalent and associated with several indicators of worse health-related QOL in this young and well-functioning population. (C) 2016 Elsevier Ireland Ltd. All rights reserved.

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.

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.