Citations

BACKGROUND: The purpose of this study was to assess the feasibility and prognostic value of cardiac output (CO) reserve assessment using exercise echocardiography in Fontan patients. We hypothesized that adults with Fontan palliation had lower CO reserve compared with controls, and impaired CO reserve was associated with greater congestion (NT-proBNP [N-terminal pro-B-type natriuretic peptide]) and cardiovascular events (death/transplant/heart failure hospitalization) in Fontan patients. METHODS: Thirty-seven Fontan patients and 61 controls underwent exercise echocardiography using a supine cycle ergometer. Doppler-derived CO and oxygen consumption (VO 2 ) were assessed at rest and every stage of exercise. CO reserve was calculated as ∆CO/Watt (∆CO/W) and ∆CO/∆VO 2 . RESULTS: Assessment of CO reserve was feasible in 95% of the Fontan group with modest reproducibility. Although both groups had similar CO at rest, the Fontan group had lower CO reserve with exercise as evidenced by lower ∆CO/W ratio (46±17 versus 57±19 mL/W; P <0.001) and lower ∆CO/∆VO 2 ratio (4.48±1.02 versus 5.37±2.18 mL/mL; P =0.03). There was a correlation between ∆CO/W ratio and log NT-proBNP (r=0.65; P <0.001), and between ∆CO/∆VO 2 ratio and log NT-proBNP (r=0.53; P =0.009). Impaired CO reserve was associated with congestion and cardiovascular events and provided improved prognostication (higher area under the curve and C statistics) above conventional echocardiographic indices and treadmill peak VO 2 . CONCLUSIONS: Patients with Fontan palliation had lower CO reserve, and impaired CO reserve was associated with congestion and cardiovascular events. Further studies are required to determine whether cardiac interventions can improve CO reserve and whether changes in CO reserve can be used as a surrogate end point for therapeutic response.

Abstract Aims Congenital heart disease (CHD) presents lifelong challenges that require effective self-management skills, particularly for youth transitioning to adulthood. This systematic review evaluates the impact of self-management interventions, grounded in self-efficacy, on both health and patient-reported outcomes in youths with CHD. Methods and results Adhering to Preferred Reporting Items for Systematic Review and Meta-Analysis guidelines, we conducted a systematic review and registered the protocol in PROSPERO. We included interventional studies targeting youth aged 15–24 years with CHD that employed self-management interventions based on self-efficacy. Comprehensive searches were performed in Ovid MEDLINE, Ovid EMBASE, CINAHL, the Cochrane Central Register of Controlled Trials, the Cochrane Database of Systematic Reviews, and Scopus from January 1973 to March 2024, with an update to include studies published until December 2024. Two reviewers independently screened titles, abstracts, and full texts, resolving disagreements by consensus. Data extraction followed Template for Intervention Description and Replication guidelines, and risk of bias was assessed using the Cochrane Risk of Bias in Non-Randomized Studies of Interventions and Risk of Bias 2 tools. Out of 54 potential studies, 4 met the eligibility criteria, representing 150 patients aged 16–19 years. The studies reported improvements in condition-related knowledge, self-management skills, and social support, although data on clinical health outcomes were limited. A narrative synthesis of the selected interventions was performed. Conclusion Self-management interventions using self-efficacy frameworks show promise for improving patient-reported outcomes among youth with CHD. However, there is a scarcity of data on their impact on health outcomes. Further research is needed to develop and implement interventions tailored to this population, potentially improving both self-management and clinical outcomes. Registration PROSPERO: CRD42024499008

Abstract Aims Systemic ventricular (SV) strain imaging improves early detection of systolic dysfunction in patients with congenital heart disease and biventricular circulation. However, its role for risk stratification in the Fontan population has not been systematically studied. We hypothesized that among Fontan patients with normal SV ejection fraction (Echo_EF) at baseline, those with reduced SV longitudinal strain (Echo_LS) would have a higher risk of progressive SV systolic dysfunction, greater neurohormonal activation, and cardiovascular events (heart failure hospitalization/death/transplant) during follow-up. Methods and results We studied adults with Fontan palliation and normal SV systolic function (Echo_EF &gt;50%) at baseline who underwent follow-up echocardiogram &gt;36 months after the baseline echocardiogram. Echo_LS was assessed from the apical 4-chamber window using speckle tracking echocardiography. Patients were divided into reduced vs. normal Echo_LS groups, using −18% as the cut-off point. Echo_LS, Echo_EF, and NT-proBNP were assessed at baseline and follow-up encounters. Of 272 patients (age, 28 ± 8 years), 98 (36%) and 174 (64%) had reduced vs. normal Echo_LS. The reduced_Echo_LS group had progressive SV systolic dysfunction [worse temporal decline in Echo_EF (relative_Δ_Echo_EF −12.8% vs. −2.1%, P &lt; 0.001)], and higher prevalence of new-onset SV systolic dysfunction [30% (29/98) vs. 8% (13/174), P &lt; 0.001]. The reduced Echo_LS group also had greater neurohormonal activation [relative Δ_ NT-proBNP 36 (95%CI, 27–43) % vs. 17 (95%CI, 11–23) %, P &lt; 0.001], and higher risk of cardiovascular events (adjusted hazard ratio 1.84, P = 0.009). Conclusion Echo_LS improves risk stratification in Fontan patients with normal Echo_EF and may provide an opportunity for early interventions to improve outcomes.

Background Atrial dysfunction is a risk factor for atrial arrhythmia and can be detected by atrial strain imaging in patients with biventricular circulation. However, the role of atrial strain imaging for risk stratification of atrial arrhythmia in patients with Fontan palliation has not been studied, despite the high prevalence and prognostic implications of atrial arrhythmia in this population. We hypothesized that pulmonary venous atrial strain was associated with new‐onset and recurrent atrial arrhythmia in Fontan physiology. Method and Results This is a retrospective study of adults with Fontan palliation and no prior history of atrial arrhythmia who underwent atrial strain imaging by speckle tracking echocardiography (2003–2023). New‐onset and recurrent atrial arrhythmias were ascertained from baseline encounter to last follow‐up. Of 404 patients, atrial reservoir strain assessment was feasible in 329 patients (81%), with modest intra‐ and interobserver agreement. The median atrial reservoir strain was 18% (interquartile range, 13%–24%), and atrial dysfunction (atrial reservoir strain <28%) was present in 294 (89%). Atrial reservoir strain <15% was associated with new‐onset atrial arrhythmia (adjusted hazard ratio [HR], 1.59 [95% CI, 1.09–2.34]; P =0.009), and recurrent atrial arrhythmia (adjusted HR, 2.01 [95% CI, 1.06–3.93]; P =0.003). Rhythm control therapy was associated with lower risk of recurrence compared with rate control therapy (adjusted HR, 0.92 [95% CI, 0.86–0.98]; P =0.003). Conclusions Atrial dysfunction was common and was associated with new‐onset and recurrent atrial arrhythmias. The higher prevalence of atrial arrhythmias in patients with reduced atrial strain and the apparent protective effect of rhythm control therapy suggest that rhythm control therapy may be the preferred treatment in patients with reduced atrial strain.

BACKGROUND: Bioprosthetic valve dysfunction and reoperations/reinterventions are common after aortic valve replacement (AVR) with bioprosthetic valves, leading to cycles of left ventricular (LV) pressure overload and unloading. The purpose of this study was to compare postoperative changes in LV structure and function and their relationship to clinical outcomes in young patients who underwent AVR with bioprosthetic valves (Bio_AVR group) versus mechanical prosthetic valves (Mech_AVR group). METHODS: Retrospective study of adults with congenital heart disease who underwent AVR at Mayo Clinic (2003–2023). The Bio_AVR group was matched 1:2 to the Mech_AVR group. LV indices (LV mass index, relative wall thickness, LV global longitudinal strain, averaged e′, and averaged E/e′) and clinical indices (NT-proBNP [N-terminal pro-B-type natriuretic peptide] and New York Heart Association class) were assessed preoperatively, and at 1, 5, and 10 years post-AVR. RESULTS: We studied 151 and 302 patients in the Bio_AVR and Mech_AVR groups, respectively (age 38±16 years, 58% males). Both groups had similar LV and clinical indices at baseline and 1 year post-AVR. However, the Bio_AVR group had higher LV mass index, relative wall thickness, and averaged E/e′, and lower averaged e′ and LV global longitudinal strain at 5 and 10 years post-AVR. Of 51 patients from the Bio_AVR group who underwent a second AVR, there was less robust improvement in LV indices and clinical indices after the second AVR compared with the first AVR. CONCLUSIONS: AVR with a bioprosthetic valve was associated with progressive LV hypertrophy and dysfunction, and worsening clinical status compared with mechanical prosthetic valves. This provides further evidence against the use of bioprosthetic AVR in young patients.

BACKGROUND: Exercise-induced hypertension (EIH) is common in adults with coarctation of the aorta (COA), but there are limited data about hemodynamics and outcomes in such patients. The purpose of this study was to assess changes in arterial load during exercise in patients with COA with versus without EIH, and the relationship to clinical outcomes. METHODS: We compared Doppler-derived arterial load indices (effective arterial elastance index, total arterial compliance index, systemic vascular resistance index), and clinical indices of disease severity (pulmonary congestion, aerobic capacity, and cardiovascular biomarkers) between adults with repaired COA and healthy controls. EIH was defined as systolic blood pressure (BP) at peak exercise >210 mm Hg in men or >190 mm Hg in women. RESULTS: In this prospective cohort study, we assessed patients with COA (n=41, age 43±14 years, 26 [63%] men) and healthy controls (n=41). Although both groups had similar resting systolic BP, the COA group had higher Doppler-derived arterial load indices at rest, as well as a greater rise in systolic BP and Doppler-derived arterial load indices at each stage of exercise, leading to a higher prevalence of EIH in the COA group (37% versus 10%; P =0.004). Compared with patients with COA without EIH (n=26, 63%), those with EIH had higher arterial load at rest and during exercise, as well as worse cardiac dysfunction, pulmonary congestion, and biomarkers of cardiovascular remodeling, despite no significant differences in resting systolic BP. CONCLUSIONS: BP assessment during exercise can improve risk stratification and identify patients who may benefit from intensification of medical therapy.

Abstract Aims Pulmonary regurgitation (PR) after reparative intervention for congenital heart disease has been studied extensively. However, the burden, distribution of causes, and outcome of PR in adults is unknown. The study aimed to evaluate the prevalence, types, and outcomes of moderate/severe PR in adults in the community setting. Methods and results A total of 398 adult residents of Olmsted County who had clinically indicated echocardiography 2004–2023 at Mayo Clinic, Rochester, and had moderate or severe PR were identified retrospectively. The median age was 77 years, 48% were females, and 61 (51%) had severe PR. The age- and sex-adjusted US prevalence was 0.11% (vs. 0.67% for ≥moderate tricuspid regurgitation). Moderate/severe PR was due to pulmonary hypertension in 77%, congenital/iatrogenic in 11%, primary pulmonary valve disease in 2% (88% due to carcinoid), and idiopathic isolated in 10%. In contrast, severe PR was due to congenital/iatrogenic disease in 52%, pulmonary hypertension in 39%, primary PR in 5%, and isolated idiopathic in 3%. The all-cause mortality rate per 100 person-year was 73 in primary (mostly carcinoid) PR, 16 in pulmonary hypertension–related PR (not different vs. propensity-matched patients without PR), and 6 in isolated idiopathic PR (not different vs. matched patients without PR). Conclusion Moderate or severe PR had a lower prevalence vs. TR. The most frequent cause was pulmonary hypertension for ≥moderate PR and congenital/iatrogenic for severe PR. Mortality in patients with acquired ≥moderate PR appeared to be related to the underlying cause, with no excess mortality compared with matched patients without PR. Whether specifically severe PR confers excess mortality requires future investigation.

BACKGROUND: Patients with Fontan palliation have reduced aerobic capacity because of impaired cardiac, pulmonary, and skeletal muscle function. However, the assessment of aerobic capacity in this population still relies on comparisons with people without cardiovascular disease rather than comparison with the expected aerobic capacity of other Fontan patients. The purpose of this study was to determine the expected aerobic capacity of adults with Fontan palliation. METHODS: Adults with Fontan palliation who underwent a cardiopulmonary exercise test at Mayo Clinic (2003–2023) were stratified into quartiles based on the predicted peak oxygen consumption (VO 2 ). We assessed the correlates of predicted peak VO 2 and the relationship between predicted peak VO 2 quartiles and cardiovascular outcomes (death/transplant). RESULTS: Of 323 patients (age, 29±9 years; 177 [55%] men), the median peak VO 2 was 19.1 (15.2–23.9) mL/kg per minute, and this corresponds to a predicted peak VO 2 of 51% (range, 19–88; interquartile range, 41–62). After multivariable adjustments, the correlates of predicted peak VO 2 were body mass index (β±SE, −2.61±0.95; 2.61% decrease in predicted peak VO 2 per 5 kg/m 2 increase in body mass index; P =0.009), systemic saturation (β±SE, 3.65±0.85; 3.65% increase in predicted peak VO 2 per 5% increase in oxygen saturation; P <0.001), and Fontan pressure (β±SE, −1.24±0.22; 1.24% decrease in predicted peak VO 2 per 1 mm Hg increase in Fontan pressures; P <0.001). There was a 47% increase in the risk for death/transplant from a higher predicted peak VO 2 quartile to the next lower quartile (adjusted hazard ratio, 1.47 [95% CI, 1.09–2.05]; P =0.01). CONCLUSIONS: The results of the current study would help calibrate the interpretation of exercise test data in adults with Fontan palliation and improve risk stratification in this population. It also underscores the need to maintain normal Fontan hemodynamics and body weight, which are important determinants of aerobic capacity.