Egbe, Alexander C.

Abstract Aim The normal (i.e. expected) haemodynamics in adults post-Fontan remain poorly delineated. Moreover, the definitions of elevated exercise pulmonary artery (PA) and PA wedge pressure (PAWP) for this population have not been described. Methods and results Seventy-two adults post-Fontan undergoing exercise catheterization were categorized into abnormal (Group I, n = 59; defined as resting mean PA ≥14 mmHg and/or PAWP ≥12 mmHg, ΔPAWP/Δsystemic flow [Qs] >2 mmHg/L/min, and/or ΔPA/Δpulmonary flow >3 mmHg/L/min) and normal (Group II, n = 13) haemodynamics. Thirty-nine patients with non-cardiac dyspnoea (NCD) were included as controls. There was no difference in exercise arterial oxygen saturation (87% [81–92] vs. 89% [85–93], p = 0.29), while exercise PA pressure (27 [23–31] vs. 16 [14.5–19.5] mmHg, p < 0.001) and PAWP were higher (21 [18–28] vs. 12 [8–14] mmHg, p < 0.001) in Group I. At peak exercise, Group I had lower heart rate (97 [81–120] vs. 133 [112.5–147.5] bpm, p < 0.001) and Qs response (67.3 [43.8–93.1] vs. 105.9 (82–118.5) % predicted, p < 0.001) than Group II. Exercise superior vena cava pressures were higher (16 [14–22.5] vs. 5.5 [3–7.3] mmHg, p < 0.001) and arterial oxygen saturation lower (89% [85–93] vs. 97% [96–98], p < 0.001) in Group II compared to NCD, while no differences in PAWP, stroke volume index, heart rate, or Qs response were seen. If defined as two standard deviations above mean values for Group II, elevated PAWP and mean PA pressure post-Fontan would correspond to 20.6 mmHg and 25.8 mmHg, respectively. Conclusion PAWP >20 mmHg and mean PA pressure >25 mmHg could be used to define elevated values during exercise in adults post-Fontan. The major discrepancy in exercise haemodynamics among Group II compared to controls appears to be the degree of systemic venous hypertension and arterial desaturation.

Introduction: The incidence and risk factors for sudden cardiac death (SCD) in patients with systemic right ventricle (sRV) and D-TGA palliated with atrial switch procedures is not well understood. Methods: This cohort was examined retrospectively for the composite end point of SCD, sustained ventricular tachycardia (VT) >30 s or appropriate ICD therapy for VT. Clinical characteristics of those with and without the end point were compared using the Kruskal-Wallis or Chi-square test as appropriate. Results: The composite end point occurred in 15 of 148 (10%) patients (63.5% male) at a mean age of 36.4 (SD 10.3) years. SCD occurred in 6 patients (n=2 successfully resuscitated), sustained VT in 6 patients and ICD therapy for VT in 3 patients who had previously received a primary prevention ICD. Patients with SCD/VT had lower sRV ejection fraction (EF) compared to patients without SCD/VT [28±11 vs 40±10%, P<0.001]. The odds ratio of SCD/VT in patients with sRVEF≤35% compared to sRVEF>35% was 13.3 (95% CI 2.8 - 64.4). Kaplan Meier analysis of time to SCD/VT stratified by sRV EF is presented in figure. QRS duration, QRS fragmentation, QTc and history of systemic AV valve surgery were not significantly associated with SCD/VT. Non-sustained ventricular tachycardia (NSVT) on Holter monitoring occurred more frequently in SCD/VT patients (80 vs 39%, p=0.003). All 11 survivors of SCD/VT received an ICD; appropriate ICD therapy occurred in 27% of these patients during follow-up. Conclusion: SCD or VT occurred in one-tenth of D-TGA patients palliated with atrial switch, predominantly in 3 rd and 4 th decade of life. Reduced sRV EF was the most important association and ICD implantation for primary prevention of SCD is a reasonable consideration in those with reduced sRV EF.

Introduction: The relative diagnostic and prognostic performance of left ventricular global longitudinal strain (LVGLS) as compared to LV ejection fraction (LVEF), and the role of LVGLS for detecting early stages of LV systolic dysfunction in adults with repaired coarctation of aorta (COA) is unknown. Objective: The purpose of this study was to address these knowledge gaps. Methods: Retrospective cohort study of adults with repaired COA that underwent transthoracic echocardiogram (2003-2020). LV systolic function was assessed using LVEF (derived from volumetric analysis) and LVGLS (derived from speckle-tracking echocardiography). Results: Of the 795 patients (age 36±14 years), mean LVEF and LVGLS were 62±11%, and 21±4%, respectively. The prevalence of LV systolic dysfunction was higher when assessed using LVGLS as compared to LVEF (20% versus 6%, p<0.001). Of 795 patients, 94 (12%) patients died, of which 75 (9%) died from cardiovascular causes. LVGLS provided more robust prognostic power in predicting all-cause mortality as compared to LVEF as evidenced by a higher C -statistic (0.743, 95%CI 0.730-0.755 versus 0.782, 95% CI 0.771-0.792, p<0.001). Furthermore, patients with normal LVEF in the setting of reduced LVGLS had a higher risk of all-cause mortality (as compared to patients with normal LVGLS and LVEF) and were at risk for temporal decline in LVEF during follow-up. Conclusions: These findings suggest that the use of LVGLS for risk stratification can help identify high-risk patients, and provide opportunities for interventions, which would, in turn, improve clinical outcomes. Further studies are required to empirically test these postulates.

Introduction: Right ventricular (RV) systolic dysfunction and pulmonary hypertension is present in 20% of adults with repaired coarctation of aorta (COA). However, the prognostic value of RV to pulmonary artery (RV-PA) coupling in this population is unknown. Objective: The purpose of this study was to assess the relationship between RV-PA coupling and clinical outcomes (heart failure hospitalization and all-cause mortality) in this population. Methods: Retrospective cohort study of adults with repaired COA and normal RV systolic function defined as RV-free wall strain ≥-24%. RV-PA coupling was assessed using tricuspid annular plane systolic excursion/ RV systolic pressure (TAPSE/RVSP) ratio. Results: Of 509 patients (median age 32 [20-45] years; men 290 [57%]), the average TAPSE and RVSP were 22±5 mm and 33±9 mmHg, respectively, and TAPSE/RVSP ratio was 0.78 (0.56-0.96) mm/mmHg. Of 509 patients, 51 (10%) died and 43 (8%) were hospitalized for heart failure. TAPSE/RVSP was associated with all-cause mortality (hazard ratio 0.82, 95% confidence interval 0.78-0.86, per 0.1 mm/mmHg), and heart failure hospitalization (hazard ratio 0.86, 95% confidence interval 0.79-0.83, per 0.1 mm/mmHg) after adjustment for anatomic lesions, cardiovascular interventions, comorbidities, and echocardiographic indices. The inclusion of TAPSE/RVSP ratio in the risk models improved the prognostic power of the models to predict all-cause mortality (C-statistics difference 0.046, p<0.001), and heart failure hospitalization (C-statistics difference 0.031, p=0.007). Conclusions: TAPSE/RVSP ratio was associated with outcomes in COA patients with normal RV systolic function, suggesting that abnormal RV-PA coupling may be present prior to the onset of overt RV systolic function in this population.

Introduction: Adults with repaired tetralogy of Fallot (TOF) have right atrial (RA) remodeling and dysfunction, and RA function can be measured using speckle-tracking echocardiography. There is limited data about the role of RA strain imaging in risk stratification in this population. Hypothesis: We hypothesized that RA reservoir strain could identify TOF patients at risk of developing atrial arrhythmia. We assessed the relationship between RA reservoir strain and atrial arrhythmias in adults with repaired TOF to test this hypothesis. Methods: Retrospective cohort study of adults with repaired TOF, and no prior history of atrial arrhythmias. Atrial arrhythmia was defined as atrial fibrillation, atrial flutter/atrial tachycardia, and categorized as new onset versus recurrent atrial arrhythmias. Results: We identified 426 patients (age 33±12 years; males 208 (49%)) that met the inclusion criteria. The mean RA reservoir, conduit, and booster strains were 34±11%, 20±9%, and 15±12%, respectively. Of 426 patients, 73 (17%) developed new-onset atrial arrhythmias (atrial flutter/tachycardia n=42; atrial fibrillation n=31); annual incidence 1.9%. RA reservoir strain was associated with new-onset atrial arrhythmias (adjusted HR 0.95, 95% CI 0.93-0.97) after multivariable adjustment. Of 73 patients with new-onset atrial arrhythmia, 41 (56%) had recurrent atrial arrhythmia (atrial flutter/tachycardia n=18; atrial fibrillation n=23); annual incidence 11.2%. Similarly, RA reservoir strain was associated with recurrent atrial arrhythmias (adjusted HR 0.92, 95% CI 0.88-0.96) after multivariable adjustment. Conclusions: RA strain indices can identify patients at risk for atrial arrhythmias, and this can, in turn, be used to guide the type/intensity of therapy in such patients.

Background Little is known about outcomes following heart failure (HF) hospitalization among adults with congenital heart disease (CHD) in the United States. We aim to compare the outcomes of HF versus non‐HF hospitalizations in adults with CHD. Methods and Results Using a national deidentified administrative claims data set, patients with adult congenital heart disease (ACHD) hospitalized with and without HF (ACHDHF+, ACHDHF−) were characterized to determine the predictors of 90‐day and 1‐year mortality and quantify the risk of mortality, major adverse cardiac and cerebrovascular events, and health resource use. Cox proportional hazard regression was used to compare ACHDHF+ versus ACHDHF− for risk of events and health resource use. Of 26 454 unique ACHD admissions between January 1, 2010 and December 31, 2020, 5826 (22%) were ACHDHF+ and 20 628 (78%) were ACHDHF−. The ACHD HF+ hospitalizations increased from 6.6% to 14.0% ( P <0.0001). Over a mean follow‐up period of 2.23 ± 2.19 years, patients with ACHDHF+ had a higher risk of mortality (hazard ratio [HR], 1.86 [95% CI, 1.67–2.07], P <0.001), major adverse cardiac and cerebrovascular events (HR, 1.73 [95% CI, 1.63–1.83], P <0.001) and health resource use including rehospitalization (HR, 1.09 [95% CI, 1.05–1.14], P <0.001) and increased postacute care service use (HR, 1.56 [95% CI, 1.32–1.85], P <0.001). Cardiology clinic visits within 30 days of hospital admission were associated with lower 90‐day and 1‐year all‐cause mortality (odds ratio [OR], 0.62 [95% CI, 0.49–0.78], P <0.001; OR, 0.69 [95% CI, 0.58–0.83], P <0.001, respectively). Conclusions HF hospitalization is associated with increased risk of mortality and morbidity with high health resource use in patients with ACHD. Recent cardiology clinic attendance appears to mitigate these risks.

BACKGROUND: There are limited data about changes in cardiac function (cardiac reverse remodeling) and heart failure indices after transcatheter tricuspid valve-in-valve replacement (TT-VIVR). The purpose of this study was to evaluate cardiac reverse remodeling and temporal changes in heart failure indices after TT-VIVR in adults with congenital heart disease. METHODS: Retrospective cohort study of adults with congenital heart disease that underwent TT-VIVR and had >6 months of follow-up (January 1, 2011, to April 30, 2023). Echocardiographic indices of cardiac remodeling and heart failure indices (New York Heart Association class, NT-proBNP (N-terminal pro-brain natriuretic peptide), glomerular filtration rate, and model for end-stage liver disease excluding international normalized ratio score) were assessed preintervention and at 1-, 3-, and 5-year postintervention. RESULTS: Of 39 patients (age 39 [32–46] years), 14 (36%) and 25 (64%) received Melody valve and Sapien valve prosthesis, respectively. At 1-year post-TT-VIVR, there was a temporal improvement in right atrial reservoir strain (17±8% versus 22±8%, P <0.001), right atrial volume (81 [59–108] versus 63 [48–82] mL/m 2 , P <0.001), right atrial pressure (12±4% versus 6±4%, P <0.001), and right ventricular global longitudinal strain (−15±7% versus −20±7%, P <0.001). Similarly, there was a temporal improvement in NT-proBNP, glomerular filtration rate, model for end-stage liver disease excluding international normalized ratio score, and New York Heart Association class. The temporal improvements in heart failure indices and valve function were maintained at 3- and 5-year post-TT-VIVR. CONCLUSIONS: Considering the significant mortality risk associated with reoperations for tricuspid valve replacement, these data suggest favorable outcomes after TT-VIVR, and support TT-VIVR as a viable alternative to surgical tricuspid valve replacement, especially in high-risk patients.

Abstract Aims Left atrial (LA) dysfunction and atrial fibrillation are also relatively common in adults with coarctation of aorta (COA), and the severity of LA dysfunction is associated with a higher risk of atrial fibrillation in this population. The purpose of this study was to determine whether LA function improved after COA repair (LA reverse remodelling), and the relationship between LA reverse remodelling and atrial fibrillation. Methods and results Retrospective cohort study of adults undergoing COA repair (2003–20). LA reservoir strain was assessed pre intervention and 12–24 months post intervention, using speckle tracking echocardiography. Incident atrial fibrillation was assessed from COA repair to last follow-up. Of 261 adults who underwent COA repair [age 37 ± 13 years; males 148 (57%)], 124 (47%) and 137 (53%) presented with native vs. recurrent COA, respectively. Of 261 patients, 231 (82%) and 48 (18%) underwent surgical and transcatheter COA repair, respectively. The LA reservoir strain increased from 32 ± 8% (pre intervention) to 39 ± 7% (post intervention), yielding a relative increase of 21 ± 5%. Older age [β ± standard error (SE) −0.16 ± 0.09 per 5 years, P = 0.02], higher systolic blood pressure (β ± SE −0.12 ± 0.04 per 5 mmHg, P = 0.005), and higher residual COA mean gradient (β ± SE −0.17 ± 0.06 per 5 mmHg, P = 0.002) post intervention were associated with less LA reverse remodelling, after adjustment for sex, hypertension diagnosis, and left ventricular indices. LA reverse remodelling (hazard ratio 0.97, 95% confidence interval 0.96–0.98 per 1% increase from pre-intervention LA function, P = 0.006) was associated with a lower risk of atrial fibrillation after adjustment for age, sex, pre-intervention LA reservoir strain, and history of atrial fibrillation. Conclusion COA repair resulted in improved LA function and decreased risk for atrial fibrillation, especially in patients without residual hypertension or significant residual COA gradient.

BACKGROUND: The outcomes of catheter ablation for atrial fibrillation in adults with congenital heart disease are not well described. METHODS: In a retrospective study of adult patients with congenital heart disease who underwent catheter ablation for atrial fibrillation between 2000 and 2020 at Mayo Clinic, procedural characteristics and outcomes were collected. The primary outcomes were atrial arrhythmia (AA) recurrence following a 3-month blanking period and repeat ablation. An arrhythmia clinical severity score was assessed pre- and post-ablation based on the duration of arrhythmia episodes, symptoms, cardioversion frequency, and antiarrhythmic drug use. RESULTS: One hundred forty-five patients (age, 57±12 years; 28% female; 63% paroxysmal atrial fibrillation) underwent 198 ablations with a median follow-up of 26 months (interquartile range, 14–69). One hundred ten, 26, and 9 patients had simple, moderate, and complex congenital heart disease, respectively. All patients underwent pulmonary vein isolation, and non–pulmonary vein targets were ablated in 79 (54%). AA recurrence at 12 months was 37% (95% CI, 29%–45%). On univariate analysis, increasing left atrial volume index was associated with higher odds of AA recurrence (odds ratio, 1.03 [1.00–1.06] per 1 mL/m 2 increment; P =0.05). Noninducibility of atrial flutter was predictive of decreased odds of AA recurrence (odds ratio, 0.43 [0.21–0.90]; P =0.03). A second ablation was performed in 43 patients after a median of 20 (interquartile range, 8–37) months. Arrhythmia clinical severity scores improved following ablation, reflecting a decrease in symptoms, cardioversions, and antiarrhythmic drugs. CONCLUSIONS: Catheter ablation of atrial fibrillation is feasible and effective in patients with adult congenital heart disease and reduces symptoms. Recurrence of AA frequently requires repeat ablation.

BACKGROUND: The prognostic role of the congenital heart disease (CHD) anatomic/physiologic classification has not been systematically studied. The purpose of this study was to determine whether CHD physiologic stage provided improvement in prognostic power (to predict all-cause mortality) beyond conventional clinical risk models. METHODS: Retrospective study of adults with CHD at the Mayo Clinic (2003–2019). The CHD physiologic stage was assessed at baseline and 36 (24–48) months, and patients were classified into stages A to D at these time points. Clinical stability (remaining in the same stage), clinical improvement (moving to less advanced stage), and clinical deterioration (moving to more advanced stage) were determined at 36 months. We defined conventional clinical risk indices as age/sex, functional class, comorbidities, cardiac procedures, hepatorenal dysfunction, and ventricular/valvular dysfunction. RESULTS: Of 5321 patients, 1649 (31%), 1968 (37%), 1224 (23%), and 480 (9%) were in stages A, B, C, and D at baseline. Of 5321 patients, 4588 (86%) also had assessments at 36 months, and of these patients, 3347 (73%), 386 (8%), and 855 (19%) had clinical stability, deterioration, and improvement, respectively. Patients with clinical improvement were more likely to have undergone cardiac procedures between both assessments. Both baseline CHD physiologic stage (hazard ratio, 1.13 [95% CI, 1.09–1.17]; P <0.001, per unit increase in stage) and change in CHD physiologic stage (hazard ratio, 1.46 [95% CI, 1.32–1.61]; P =0.007, per unit increase in stage) were associated with mortality after adjustments for conventional risk indices and provided incremental improvement in prognostic power beyond conventional clinical risk models as evidence by an increase in C statistic from 0.702 (0.681–0.724) to 0.769 (0.754–0.787). CONCLUSIONS: The CHD physiologic stage can potentially be used for risk stratification, as well as to monitor disease progression and response to therapy.