Banala, Keerthana

Background: Digital health offers a potential solution to support adults with congenital heart disease (CHD), filling the critical gap to help maintain recommended lifelong care with adult CHD specialists. However, its design and implementation require a rigorous scientific approach to make it scalable and usable for the target population. Objective: To pilot test the acceptability and feasibility of a digital health intervention to support lifelong healthcare for adults with CHD. Methods: Using a community-based participatory research and a behavioral theory-based approach, we developed and piloted a digital tool, Empower My Congenital Heart (EmpowerMyCH), within the web—and mobile-based Eureka Digital Research platform at UCSF. Intervention components within EmpowerMyCH include exhibits (expert and peer-developed learning modules on CHD-related topics such as finding specialists or navigating visits, tests, procedures, etc.), peer support, and a digital medical passport (Figure 1). Participants receive an exhibit with study measure surveys every two months, with a two-month completion window. Participant satisfaction with the digital tool is measured at six months using the Client Satisfaction Questionnaire-8 (CSQ-8), a validated survey with scores ranging from 8 to 32; higher scores indicate higher satisfaction with the digital tool. Results: Of the 519 participants recruited, 495 were eligible adults with CHD, 445 consented, and 6 withdrew. The median age was 43 (ranging from 20 to 85) years, and 30.1% were males. Survey completion rate was the highest at 88.7% during the first two months after enrollment and 53.5% at 8 months. Of the five exhibits delivered so far, over 87% of participants rated the exhibits to be helpful (Figure 2). Participants found the exhibits readable, easily accessible, motivational, useful, and educational (Figure 3). The median (25th, 75th) CSQ-8 score was 21 (18, 25) among 144 participants who completed the survey. Conclusions: EmpowerMyCH, the digital health tool, appears to be acceptable to most adults with CHD to support and educate them in navigating their healthcare. The tool and its contents are accessible, user-friendly, and responsive to the needs of the CHD community, resulting in high satisfaction rates. The feasibility rates and satisfaction scores are similar to or higher for EmpowerMyCH than those of prior digital health studies. Future hybrid intervention trials can demonstrate EmpowerMyCH‘s effectiveness.

Abstract Background Several coarctation of aorta (COA) severity indices are used for timing of COA intervention, and to define severity of residual coarctation post-intervention. However, it is unclear how many of these COA indices are required in order to recommend intervention, and what degree of residual coarctation results in suboptimal recovery of the left ventricle (LV). Our aim was to assess the correlation between different COA indices and effects of chronic LV pressure overload (LV hypertrophy, diastolic and systolic dysfunction), and to determine the effect of residual coarctation on LV reverse remodeling after COA intervention. Methods COA severity indices were defined as Doppler COA gradient, systolic blood pressure, upper-to-lower-extremity systolic blood pressure gradient, aortic isthmus ratio. LV remodeling indices were defined as LV mass index (LVMI), LV global longitudinal strain (LVGLS), e' and E/e'. LV reverse remodeling was defined as the difference between indices obtained pre-intervention and 5-years post-intervention (delta LVMI, e', E/e', LVGLS). Results Of the COA indices analyzed in 546 adult COA patients, aortic isthmus ratio had the strongest correlation with LVMI (β±standard error −28.3±14.1, p<0.001), LVGLS (1.51±0.42, p=0.005), e' (3.11±1.10, p=0.014), and E/e' (−13.4±6.67, p=0.008). Residual aortic isthmus ratio also had the strongest correlation with LV reverse remodeling, and residual aortic isthmus ratio <0.7 was predictive of suboptimal LV reverse remodeling post-intervention. Conclusion Considering the known prognostic implications of LV remodeling and reverse remodeling in response to pressure overload, these results support the use of aortic isthmus ratio for timing of COA intervention, and for prognostication post-intervention. Funding Acknowledgement Type of funding source: None

Abstract Background Left ventricular (LV) pressure overload and coronary artery disease, both of which are common in patients with coarctation of aorta (COA), are risk factors for LV diastolic dysfunction. Patients with COA may have aortic vasculopathy that can result in LV pressure overload even in the absence of hemodynamically significant COA. We therefore hypothesized that patients with COA (without hemodynamically significant COA) will have more LV diastolic dysfunction compared to controls. Methods Adult patients with COA (OA peak velocity <2 m/s) were matched 1:1 to patients without structural heart disease using propensity score method based on age, sex, body mass index, hypertension and blood pressure. The objective was to compare LV diastolic dysfunction (defined as E/e>2 standard deviations above age-specific normative values) between COA patients and controls. Results Of 204 COA and 204 control patients (age 35±12 years), COA patients had higher septal and lateral E/e' ratio (12±4 vs 9±4, p=0.009) and (10±3 vs 7±3, p<0.001), respectively. Compared to controls, the prevalence of LV diastolic dysfunction was higher in COA patients for every age group: <40 years (63% vs 13%, p<0.001); 41–60 years (87% vs 33%, p<0.001); age >60 years (82% vs 56%, p=0.076). Left ventricular mass index (LVMI) was the strongest determinant of E/e' (β=2.71 per 10 g/m2, standard error = 1.25, p<0.001). Conclusion LV diastolic dysfunction was common in COA patients, and the association with LVMI suggests that COA patients may have ongoing LV pressure overload in the absence of hemodynamically significant re-coarctation. Funding Acknowledgement Type of funding source: None

Abstract Background Transcatheter stent therapy is as effective as surgery in producing acute hemodynamic improvement in patients with coarctation of aorta (COA). However, left ventricular (LV) remodeling after transcatheter COA intervention has not been systematically investigated. The purpose of this study was to compare remodeling of LV structure and function after transcatheter stent therapy vs surgical therapy for COA. Methods LV remodeling was assessed at 1, 3 and 5-years post-intervention using: LV mass index (LVMI), LV end-diastolic dimension, LV ejection fraction, LV global longitudinal strain (LVGLS), LV e' and E/e'. Results There were 44 and 128 patients in the transcatheter and surgical groups respectively. Compared to the surgical group, the transcatheter group had less regression of LVMI (−4.6 [95% CI: −5.5 to −3.7] vs −7.3 [95% CI: −8.4 to −6.6] g/m2, p<0.001), less improvement in LVGLS (2.1 [95% CI 1.8–2.4] vs 2.9 [95% CI: 2.6–3.2]%, p=0.024) and e' (1.0 [95% CI: 0.7–1.2] vs 1.5 [95% CI: 1.3–1.7] cm/s, p=0.009) at 5 years post-intervention. Exploratory analysis showed a correlation between change in LVMI and LVGLS, and between change in LVMI and e', and this correlations were independent of the type of intervention received. Conclusions Transcatheter stent therapy was associated with less remodeling of LV structure and function during mid-term follow-up. As transcatheter stent therapy become more widely used in the adult COA population, there is a need for ongoing clinical monitoring to determine if these observed differences in LV remodeling translates to differences in clinical outcomes. Funding Acknowledgement Type of funding source: None

Abstract Background Ambulatory blood pressure monitoring (ABPM) is more sensitive than resting BP assessment in detecting hypertension in patients with coarctation of aorta (COA). However, it is not known whether ABPM provides a better measure of left ventricular (LV) pressure load and LV remodeling as compared to resting BP. LV pressure overload is the lynchpin in the pathogenesis of cardiovascular mortality in COA patients, because it leads to LV remodeling, LV dysfunction and heart failure, which sets-the-stage for cardiovascular death. Therefore identifying a sensitive clinical metric that reflects LV pressure load and LV remodeling, is an important step towards optimizing antihypertensive therapy to prevent LV dysfunction and mortality. We hypothesized that ambulatory systolic BP (SBP) has better correlation with arterial load and LV remodeling indices as compared to resting SBP. Methods Our study cohort comprised of adults with isolated COA without significant residual coarctation (Doppler peak velocity ≤2.5 m/sec), aortic or mitral valve disease. All patients underwent BP measurement at rest, followed by ABPM. Arterial load (LV pressure load) was assessed using Doppler-derived effective arterial elastance index (EAI). LV remodeling was assessed using: LV mass index (LVMI), and E/e'. We divided the cohorts into 4 categories (normotensive, masked, white-coat, and overt hypertension). Results Of the 106 patients (male 69 [65%); age 39±11 years), 98 (93%) had prior COA intervention at 6±5 years. Resting SBP was 132±17, daytime ambulatory SBP was 142±16, and nighttime ambulatory SBP was 128±12 mmHg. The mean arterial load and LV remodeling indices were: EAI 3.4±0.8 mmHg/ml·m2, LVMI 106±21 g/m2, and E/e'11±4. Compared to resting SBP, ambulatory SBP had better correlation with EAI, LVMI, and E/e'. EAI, LVMI and E/e' were significantly different between hypertension categories (Figure 1). Conclusions In this study, we demonstrated that ambulatory SBP had better correlations with arterial load and LV remodeling indices as compared to resting SBP. This suggests that ambulatory SBP was a more robust metric of LV pressure overload and remodeling, and can improve detection of vascular stiffness, LV hypertrophy and elevated LV filling pressure as compared to resting SBP. The results of the current study have important clinical implications with regards to initiation and titration of antihypertensive therapy. If ABPM is used as the gold standard for diagnosis of hypertension, the masked hypertension group (who would have been misclassified as normotensive based on resting SBP alone) will qualify for antihypertensive therapy. Additionally, since ambulatory SBP had better correlation with LV remodeling indices, perhaps titration of antihypertensive therapy using ABPM will potentially reduce the risk of LV hypertrophy and dysfunction due to hypertension. Figure 1 Funding Acknowledgement Type of funding source: None

Abstract Background Ambulatory systolic blood pressure (SBP) is more sensitive than resting-SBP in detecting hypertension in patients with coarctation of aorta (COA) and patients with idiopathic hypertension. A recent study showed that compared to patients with idiopathic hypertension with similar resting-SBP, COA patients had lower arterial compliance, and hence experienced higher SBP per unit increase in stroke volume during exercise. The purpose of this study was to compare resting vs ambulatory-SBP relationship between COA patients and non-COA patients (with and without hypertension), and to determine if ambulatory-SBP had better correlation with left ventricular (LV) remodeling as compared to resting-SBP. Methods Case-control study of 106 COA patients and 106 patients with idiopathic hypertension (ControlHTN+ group) matched by age, sex, body mass index and resting-SBP. Resting/ambulatory BP relationship in COA patients and patients with idiopathic hypertension were also compared to that of 19 patients without hypertension (ControlHTN- group). LV remodeling was assessed using LV mass index, e', and E/e'. Results Although the COA and ControlHTN+ groups had similar resting-SBP (by design), the COA group had a higher ambulatory-SBP and a higher prevalence of masked hypertension (30% vs 12%, p<0.001). There was a stronger correlation between ambulatory-SBP (as compared to resting-SBP) and LVMI (r=0.36 vs r=0.55, p=0.042), and average E/e' (r=0.42 vs r=0.59, p=0.046), in the COA group but not the ControlHTN+ group. There was no difference in the resting/ambulatory BP relationship between ControlHTN+ and ControlHTN- groups, but both groups were significantly different from the COA group. Conclusion These results highlight the unique differences in disease pathophysiology between hypertension in patients with COA and those with idiopathic hypertension, and also suggest that ambulatory-SBP may be a more accurate measure of LV pressure overload in COA patients. This underscores the potential limitations of relying on resting-SBP alone for clinical decision making. Funding Acknowledgement Type of funding source: None