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.

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.

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.

The prevalence of sexual dysfunction and its association with quality of life in adults with congenital heart disease.

BACKGROUND: The prevalence of sexual dysfunction (SD) and its impact on quality of life (QOL) in adults 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 (≥18years) 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±11.7years, 53 (51%) were men, 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.

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.

Feeding dysfunction in single ventricle patients with feeding disorder.

PURPOSE: To determine whether caregivers of children with single ventricle heart defects identified as having feeding disorder will report more frequent feeding dysfunction, or maladaptive mealtime behavior and/or interactions, when compared with reference populations. METHODS: As part of routine evaluation, parents of children evaluated at the Feeding, Swallowing, and Nutrition Center at the Children's Hospital of Wisconsin completed previously validated questionnaires to assess feeding dysfunction and parental stress. Parental responses for single ventricle patients were compared with all other children evaluated with a feeding disorder. RESULTS: Questionnaires were completed in eight patients with single ventricle heart defects. The mean age was 36 ± 23 months, with five females (63%). Mean weight-for-age z-score was -1.4 ± 0.9. Compared with noncardiac feeding clinic children, there was more reported child resistance to eating (83 ± 15% vs. 44 ± 2%; P = .05). Single ventricle parents were more likely to report distress (50 ± 18% vs. 21 ± 2%; P = .04) and a difficult child (63 ± 17% vs. 31 ± 2%; P = .05). There was also more defensive responding among parents of single ventricle children (63 ± 17% vs. 29 ± 2%; P = .04). CONCLUSION: Single ventricle patients evaluated for disordered feeding more frequently showed resistance to eating and parental distress than noncardiac feeding clinic patients. Parents of these children underestimated the degree of feeding difficulty by defensive responding and had more parental distress. These findings suggest that feeding dysfunction can contribute to longer-term feeding and growth problems in single ventricle patients with feeding disorder.

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.

The spectrum of congenital heart disease and outcomes after surgical repair among children with Turner syndrome: a single-center review.

Turner syndrome (TS), a genetic abnormality affecting 1 in 2,500 people, is commonly associated with congenital heart disease (CHD). However, the surgical outcomes for TS patients have not been well described. This study reviewed the spectrum of CHD in TS at the authors' center. The authors report outcomes after coarctation of the aorta (CoA) repair or staged palliation of hypoplastic left heart syndrome (HLHS) and then compare the surgical outcomes with those of non-TS patients undergoing like repair. This retrospective chart review was conducted at the Children's Hospital of Wisconsin from 1999 to 2011. Of the 173 patients with TS, 77 (44.5 %) were found to have CHD. Left-sided obstructive lesions were the most common. However, the spectrum of CHD was wide and included systemic and pulmonary venous abnormalities as well as abnormalities of the coronary arteries. In the comparative analysis of CoA repair, the TS patients younger than 60 days had longer aortic cross-clamp times (24 vs. 16 min; p = 0.001) and longer hospital stays (12 vs. 6 days; p ≤ 0.0001) than the non-TS patients. At the follow-up assessment after 8.8 ± 9.1 years, 17 % of the TS patients had hypertension, but no patient had required reintervention, and no deaths had occurred. Finally, three of the four TS patients with HLHS died within the first year. The spectrum of CHD within TS is wide and not limited to bicuspid aortic valve or CoA. Additionally, patients with TS undergoing CoA repair may have a more challenging early postoperative course but experience outcomes similar to those of non-TS patients. Finally, patients who have TS combined with HLHS remain a challenging population with generally poor survival.

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.

Extracardiac complications in adults with congenital heart disease.

With the increasing number of adults living with repaired, or unrepaired, congenital heart disease, there is a growing incidence of extracardiac comorbidities. These comorbidities can affect various organ systems in complex ways, and may have a significant impact on a patient's quality of life and survival. Many of these potential complications may go undiagnosed until there is already a significant bearing on the patient's life. Therefore, it is important for physicians who care for the adult congenital patient to be mindful of these potential extracardiac complications, and actively assess for these complications in their adult congenital practice. Continued research to identify modifiable risk factors is needed so that both preventative and therapeutic management options for these extracardiac complications may be developed.