Submaximal Exercise Response is Associated with Future Hypertension in Patients with Coarctation of the Aorta.

Hypertension (HTN) is common in patients with a history of coarctation of the aorta (CoA) and remains underrecognized and undertreated. Studies in the non-coarctation otherwise healthy adult population have correlated an exaggerated blood pressure response during mild to moderate exercise with subsequent diagnosis of HTN. The goal of this study was to determine if blood pressure response to submaximal exercise in normotensive CoA patients correlated with development of HTN.Retrospective chart review was performed in individuals ≥ 13 years old with CoA and no diagnosis of HTN at time of cardiopulmonary exercise testing (CPET). Systolic blood pressure (SBP) during CPET at rest, submax 1 (stage 1 Bruce or minute 2 bicycle ramp), submax 2 (stage 2 Bruce or minute 4 bicycle ramp), and peak were recorded. The primary composite outcome was HTN diagnosis or initiation of anti-hypertensive medications at follow up.There were 177 patients (53% female, median age 18.5 years), of whom 38 patients (21%) met composite outcome during a median follow up of 46 months. Men were more likely to develop hypertension. Age at repair and age at CPET were not significant covariates. At each stage of CPET, SBP was significantly higher in those who met the composite outcome. Submax 2 SBP ≥ 145 mmHg was 75% sensitive, 71% specific in males and 67% sensitive, 76% specific in females for development of composite outcome.Our study shows an exaggerated SBP response to submaximal exercise may portend an increased risk of developing hypertension during short- to mid-term follow up.

Pilot Project: Heart Chargers-A Successful Model for a Home-Based Physical Activity Program Utilizing Telemedicine for Fontan Patients.

Fontan patients have decreased exercise capacity which further declines throughout adolescence. A positive exercise capacity trajectory in children predicts better adult Fontan outcomes. Hospital-based physical activity programs improve exercise capacity and attenuate the age-expected decline in Fontan patients. The purpose of this project was to investigate the feasibility and safety of a partially reimbursable 12-month, home-based, individualized physical activity program (Heart Chargers) for Fontan patients utilizing telemedicine. The Heart Chargers team included a cardiologist, nurse coordinator, and exercise physiologists. Eligible participants with a Fontan completed a baseline cardiopulmonary exercise test (CPET) and consented to participate in Heart Chargers, a 12-month home-based exercise prescription. The individualized exercise prescription focused on skeletal and respiratory muscle strength training and aerobic activities. Participants received a Garmin© device to monitor adherence. Telephone check-ins ranged from weekly to monthly as participants gained independence. Pre- and post-program CPET and informal surveys of physical activity self-efficacy were completed. Nine participants have completed the Heart Chargers program. There was no pre-post difference in maximal or submaximal oxygen consumption (VO2), peak heart rate, or oxygen saturation. There was a significant pre-post increase in systolic blood pressure (p-value 0.004) and minute ventilation (p-value 0.012) at peak exercise. Per subjective report, exercise-related self-efficacy increased after program completion. There were no adverse events. At present, 7 participants remain actively enrolled in the program. Heart Chargers, a novel, home-based, partially reimbursable, 12-month individualized exercise program using telemedicine was successfully implemented in Fontan patients with no adverse events. The lack of decline in exercise capacity for participants is encouraging.

Impact of a paediatric cardiac rehabilitation programme upon patient quality of life.

INTRODUCTION: Cardiac rehabilitation programmes for paediatric patients with congenital heart disease (CHD) have been shown to promote emotional and physical health without any associated adverse events. While prior studies have demonstrated the effectiveness of these types of interventions, there has been limited research into how the inclusion of psychological interventions as part of the programme impacts parent-reported and patient-reported quality of life. MATERIALS AND METHODS: Patients between the ages of 7 and 24 years with CHD completed a cardiac rehabilitation programme that followed a flexible structure of four in person-visits with various multidisciplinary team members, including paediatric psychologists. Changes in scores from the earliest to the latest session were assessed regarding exercise capacity, patient functioning (social, emotional, school, psychosocial), patient general and cardiac-related quality of life, patient self-concept, and patient behavioural/emotional problems. RESULTS: From their baseline to final session, patients exhibited significant improvement in exercise capacity (p = 0.00009). Parents reported improvement in the patient's emotional functioning, social functioning, school functioning, psychosocial functioning, cognitive functioning, communication, and overall quality of life. While patients did not report improvement in these above areas, they did report perceived improvement in certain aspects of cardiac-related quality of life and self-concept. DISCUSSION: This paediatric cardiac rehabilitation programme, which included regular consultations with paediatric psychologists, was associated with divergent perceptions by parents and patients on improvement related to quality of life and other aspects of functioning despite improvement in exercise capacity. Further investigation is recommended to identify underlying factors associated with the differing perceptions of parents and patients.

Important Considerations in Pediatric Heart Failure.

PURPOSE OF REVIEW: The goal of this paper is to provide an overview of contemporary knowledge specific to the causes, management, and outcome of heart failure in children. RECENT FINDINGS: While recently there have been subtle improvements in heart failure outcomes in children, these improvements lag significantly behind that of adults. There is a growing body of literature suggesting that pediatric heart failure is a unique disease process with age- and disease-specific myocardial adaptations. In addition, the heterogenous etiologies of heart failure in children contribute to differential response to therapies and challenge the ability to obtain meaningful results from prospective clinical trials. Consideration of novel clinical trial designs with achievable but clinically relevant endpoints and focused study of the mechanisms underlying pediatric heart failure secondary to cardiomyopathies and structural heart disease are essential if we hope to advance care and identify targeted and effective therapies.

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.

Considerations for Gender-Affirming Hormonal and Surgical Care Among Transgender and Gender Diverse Adolescents and Adults With Congenital Heart Disease.

BACKGROUND: Transgender and gender diverse (TGD) individuals and long-term survivors with adult congenital heart disease (ACHD) are both growing populations with specialized needs. No studies assess temporal trends or evaluate the care of TGD individuals with ACHD. METHODS AND RESULTS: Meetings between congenital cardiology and gender-affirming care specialists identified unique considerations in TGD individuals with ACHD. A retrospective chart review was then performed to describe patient factors and outpatient trends in those with an ACHD diagnosis undergoing gender-affirming hormonal or surgical care (GAHT/S) at 1 adult and 1 pediatric tertiary care center. Thirty-three TGD individuals with ACHD were identified, 21 with a history of GAHT/S. Fourteen (66%) had moderate or complex ACHD, 8 (38%) identified as transgender male, 9 (43%) transgender female, and 4 (19%) other gender identities. Three had undergone gender-affirming surgery. There were zero occurrences of the composite end point of unplanned hospitalization or thrombotic event over 71.1 person-years of gender-affirming care. Median age at first gender-affirming appointment was 16.8 years [interquartile range 14.8-21.5]. The most common treatment modification was changing estradiol administration from oral to transdermal to reduce thrombotic risk (n=3). An increasing trend was observed from zero TGD patients with ACHD attending a gender diversity appointment in 2012 to 14 patients in 2022. CONCLUSIONS: There is a growing population of TGD patients with ACHD and unique medical and psychosocial needs. Future studies must fully evaluate the reassuring safety profile observed in this small cohort. We share 10 actionable care considerations for providers with a goal of overseeing a safe and fulfilling gender transition across all TGD patients with ACHD.

The burden of psychological trauma and post-traumatic stress disorder among adults with congenital heart disease: PTSD in ACHD.

Psychological trauma, symptoms of post-traumatic stress disorder (PTSD), and mental health conditions are common in adult congenital heart disease (ACHD). There is a gap in research examining PTSD in ACHD using the current Diagnostic and Statistical Manual of Mental Disorders, fifth edition (DSM-5) criteria in assessing patient characteristics and experiences with trauma-focused treatment. Surveys were offered to outpatients over a 6-month enrollment period to be completed by way of a QR code on their personal smart phone. Patient-reported items include a detailed medical and psychosocial history, the Oslo social support scale, adverse childhood experiences survey, and the PTSD checklist for DSM-5. Of 158 patients (77% moderate or complex heart disease) who provided complete data, a provisional diagnosis of PTSD was found in 48 patients (30%) using a PTSD checklist for DSM-5 cut-off score of ≥31. A positive PTSD screen was associated with younger age, nonwhite race, presence of heart failure, lower New York Heart Association functional class, lower linear quality of life score, lower Oslo social support scale score, an insecure caregiver relation, period of unemployment, emergency department visits, medication nonadherence, and coexisting mental health disorders. Complexity of heart disease and number of surgical and/or catheter-based interventions were not associated with PTSD, although having undergone no cardiac surgeries until adulthood (aged ≥18 years) was associated with a lower prevalence of PTSD. Those who screened positive for PTSD were more likely to report multiple traumatic events, including noncardiac traumatic events. Only 14 of 48 patients (29%) reported a known diagnosis of PTSD, although 44 patients (92%) reported having ever seen a mental health provider. A total of 18 patients (38%) reported currently having a mental health provider. A total of 30 patients (62%) had heard of at least 1 evidence-based trauma-informed therapy, and 14 (29%) had tried at least 1. In conclusion, using the DSM-5 criteria, we observed a high prevalence of potential PTSD in ACHD associated with several novel cardiac and psychosocial patient factors. Future longitudinal studies will be necessary to establish causality. Few patients with ACHD have been formally diagnosed with PTSD or have experience with evidence-based trauma-informed therapies.

Sexual Health and Well-Being in Adults With Congenital Heart Disease: A International Society of Adult Congenital Heart Disease Statement.

As health care outcomes improve the priority for those living with adult congenital heart disease have changed to a more holistic focus on quality of life and well-being. Although health care has embraced this, there are still areas where there is a deficit in advice, allyship, and advocacy. One of these deficits is in the area of sexual health and well-being. A healthy sexual life has a myriad of physical and psychosocial benefits. However, individuals with adult congenital heart disease may have significant barriers to achieving well-being in this aspect of their lives. These barriers and their potential solutions are outlined in this paper.

Clinical Outcomes of Adult Fontan-Associated Liver Disease and Combined Heart-Liver Transplantation.

BACKGROUND: The impact of Fontan-associated liver disease (FALD) on post-transplant mortality and indications for combined heart-liver transplant (CHLT) in adult Fontan patients remains unknown. OBJECTIVES: The purpose of this study was to assess the impact of FALD on post-transplant outcomes and compare HT vs CHLT in adult Fontan patients. METHODS: We performed a retrospective-cohort study of adult Fontan patients who underwent HT or CHLT across 15 centers. Inclusion criteria were as follows: 1) Fontan; 2) HT/CHLT referral; and 3) age ≥16 years at referral. Pretransplant FALD score was calculated using the following: 1) cirrhosis; 2) varices; 3) splenomegaly; or 4) ≥2 paracenteses. RESULTS: A total of 131 patients (91 HT and 40 CHLT) were included. CHLT recipients were more likely to be older (P = 0.016), have a lower hemoglobin (P = 0.025), require ≥2 diuretic agents pretransplant (P = 0.051), or be transplanted in more recent decades (P = 0.001). Postmatching, CHLT demonstrated a trend toward improved survival at 1 year (93% vs 74%; P = 0.097) and improved survival at 5 years (86% vs 52%; P = 0.041) compared with HT alone. In patients with a FALD score ≥2, CHLT was associated with improved survival (1 year: 85% vs 62%; P = 0.044; 5 years: 77% vs 42%; P = 0.019). In a model with transplant decade and FALD score, CHLT was associated with improved survival (HR: 0.33; P = 0.044) and increasing FALD score was associated with worse survival (FALD score: 2 [HR: 14.6; P = 0.015], 3 [HR: 22.2; P = 0.007], and 4 [HR: 27.8; P = 0.011]). CONCLUSIONS: Higher FALD scores were associated with post-transplant mortality. Although prospective confirmation of our findings is necessary, compared with HT alone, CHLT recipients were older with higher FALD scores, but had similar survival overall and superior survival in patients with a FALD score ≥2.

Morbidity and Mortality in Adult Fontan Patients After Heart or Combined Heart-Liver Transplantation.

BACKGROUND: An increasing number of adult Fontan patients require heart transplantation (HT) or combined heart-liver transplant (CHLT); however, data regarding outcomes and optimal referral time remain limited. OBJECTIVES: The purpose of this study was to define survivorship post-HT/CHLT and predictors of post-transplant mortality, including timing of referral, in the adult Fontan population. METHODS: A retrospective cohort study of adult Fontan patients who underwent HT or CHLT across 15 centers in the United States and Canada was performed. Inclusion criteria included the following: 1) Fontan; 2) HT/CHLT referral; and 3) age ≥16 years at the time of referral. Date of "failing" Fontan was defined as the earliest of the following: worsening fluid retention, new ascites, refractory arrhythmia, "failing Fontan" diagnosis by treating cardiologist, or admission for heart failure. RESULTS: A total of 131 patients underwent transplant, including 40 CHLT, from 1995 to 2021 with a median post-transplant follow-up time of 1.6 years (Q1 0.35 years, Q3 4.3 years). Survival was 79% at 1 year and 66% at 5 years. Survival differed by decade of transplantation and was 87% at 1 year and 76% at 5 years after 2010. Time from Fontan failure to evaluation (HR/year: 1.23 [95% CI: 1.11-1.36]; P < 0.001) and markers of failure, including NYHA functional class IV (HR: 2.29 [95% CI: 1.10-5.28]; P = 0.050), lower extremity varicosities (HR: 3.92 [95% CI: 1.68-9.14]; P = 0.002), and venovenous collaterals (HR: 2.70 [95% CI: 1.17-6.20]; P = 0.019), were associated with decreased post-transplant survival at 1 year in a bivariate model that included transplant decade. CONCLUSIONS: In our multicenter cohort, post-transplant survival improved over time. Late referral after Fontan failure and markers of failing Fontan physiology, including worse functional status, lower extremity varicosities, and venovenous collaterals, were associated with post-transplant mortality.

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.

Outcomes in Adult Congenital Heart Disease: Neurocognitive Issues and Transition of Care.

There is a growing population of patients living with congenital heart disease (CHD), now with more adults living with CHD than children. Adults with CHD have unique health care needs, requiring a thoughtful approach to cardiac, neurocognitive, mental, and physical health issues. They have increased risk of anxiety, depression, pragmatic language impairment, limited social cognition, worse educational attainment and unemployment, and delayed progression into independent adulthood. As a result, it is important to establish an individualized approach to obtain successful transition and transfer of care from the pediatric to adult health care world in this patient population.

Porcine Small Intestinal Submucosa May Be a Suitable Material for Norwood Arch Reconstruction.

BACKGROUND: Norwood palliation typically requires patch augmentation of the ascending aorta and aortic arch. Patients having undergone Norwood palliation are at risk of recurrent arch obstruction, the risk of which may be affected by the type of patch material used at the time of Norwood palliation. We sought to determine the freedom from neoaortic arch reintervention and overall survival in patients who underwent Norwood palliation utilizing porcine small intestinal submucosa (PSIS) as the patch material. METHODS: Retrospective chart review was performed to identify patients who underwent a Norwood operation utilizing PSIS material at our institution. Cardiac diagnosis, age at surgery, shunt type, need for reintervention, and outcome (survival, transplant, and death) were evaluated. RESULTS: Forty-four patients had PSIS material utilized for arch reconstruction at the time of Norwood palliation. There were only five neoaortic arch reinterventions in 4 patients (11.4%). An additional 10 reinterventions, unrelated to the PSIS patch, were performed, including five shunt revisions and five branch pulmonary artery interventions. There were 3 deaths, and 5 patients underwent transplantation. Median follow-up was 387.5 days (range, 4 to 1,513). CONCLUSIONS: Freedom from neoaortic arch reintervention and survival after Norwood palliation with PSIS patch material is promising. The PSIS appears noninferior and may be an appropriate tissue choice for Norwood palliation. Studies with longer follow-up are needed to determine the rate of neoaortic reintervention over time.

Can a Home-based Cardiac Physical Activity Program Improve the Physical Function Quality of Life in Children with Fontan Circulation?

OBJECTIVE: Patients after Fontan operation for complex congenital heart disease (CHD) have decreased exercise capacity and report reduced health-related quality of life (HRQOL). Studies suggest hospital-based cardiac physical activity programs can improve HRQOL and exercise capacity in patients with CHD; however, these programs have variable adherence rates. The impact of a home-based cardiac physical activity program in Fontan survivors is unclear. This pilot study evaluated the safety, feasibility, and benefits of an innovative home-based physical activity program on HRQOL in Fontan patients. METHODS: A total of 14 children, 8-12 years, with Fontan circulation enrolled in a 12-week moderate/high intensity home-based cardiac physical activity program, which included a home exercise routine and 3 formalized in-person exercise sessions at 0, 6, and 12 weeks. Subjects and parents completed validated questionnaires to assess HRQOL. The Shuttle Test Run was used to measure exercise capacity. A Fitbit Flex Activity Monitor was used to assess adherence to the home activity program. RESULTS: Of the 14 patients, 57% were male and 36% had a dominant left ventricle. Overall, 93% completed the program. There were no adverse events. Parents reported significant improvement in their child's overall HRQOL (P < .01), physical function (P < .01), school function (P = .01), and psychosocial function (P < .01). Patients reported no improvement in HRQOL. Exercise capacity, measured by total shuttles and exercise time in the Shuttle Test Run and calculated VO2 max, improved progressively from baseline to the 6 and 12 week follow up sessions. Monthly Fitbit data suggested adherence to the program. CONCLUSION: This 12-week home-based cardiac physical activity program is safe and feasible in preteen Fontan patients. Parent proxy-reported HRQOL and objective measures of exercise capacity significantly improved. A 6-month follow up session is scheduled to assess sustainability. A larger study is needed to determine the applicability and reproducibility of these findings in other age groups and forms of complex CHD.

The Externally Supported Ross Operation: Early Outcomes and Intermediate Follow-Up.

BACKGROUND: The externally supported Ross (supported Ross), consisting of a Dacron (DuPont, Wilmington, DE) graft to support the neoaortic root, has been used in adolescent and adult patients to prevent neoaortic dilatation. Outcomes after the supported Ross technique were compared with the Ross procedure using the standard aortic root replacement technique (standard Ross). METHODS: This was a retrospective analysis of 36 adolescent and young adult patients who underwent the Ross procedure between 1992 and 2013. The outcomes of supported Ross procedures in 26 patients were compared with the Ross procedure in 10 patients. End points included survival, neoaortic root dilatation, development of neoaortic regurgitation, and the need for reintervention. RESULTS: The median age at operation was 14 years (range, 11 to 31 years), and indications for the operation were mixed lesions (47%), followed by aortic regurgitation (42%) and stenosis (11%). There were no early deaths. The mean follow-up was 2.2 years (range, 1 to 11 years). At the 1-year (p = 0.01) and 3-year (p < 0.05) follow-up, patients in the supported Ross cohort had a smaller neoaortic root z-score. Neither cohort had a large number of patients with significant neoaortic regurgitation, with 1 patient in the supported cohort compared with 3 patients in the standard cohort. Overall, 4 patients (40%) in the standard Ross cohort had required reintervention, including 3 directed at the neoaortic root. One patient in the supported Ross cohort required early reintervention for revision of the right coronary artery. CONCLUSIONS: At intermediate follow-up, patients who underwent the supported Ross technique were less likely to have neoaortic root dilatation compared with patients who underwent a standard Ross procedure. Further studies are needed to evaluate the long-term durability of this technique, particularly in regards to the development of significant aortic regurgitation, the rate of reintervention, and application to younger and smaller patients.