Aortic Coarctation Patients Research Articles

Relationship between obesity, cardiac hemodynamics, and heart failure in adults with coarctation of aorta.

Patients with coarctation of aorta (COA) have arterial stiffening and left ventricular (LV) diastolic dysfunction similar to patients with heart failure with preserved ejection fraction (HFpEF) and obese subjects. However, the relationship between obesity, cardiac hemodynamics, and HF in adults with COA is unknown. The purpose of this study was to compare cardiac hemodynamics and prevalence of HFpEF between COA patients with vs without obesity, and to assess the relationship between obesity and HFpEF in this population. Adults with COA who underwent right heart catheterization were divided into an obese group (body mass index, BMI > 30 kg/m2) or a non-obese group (BMI ≤ 30 kg/m2). We also selected a control group of subjects without structural heart disease and with normal invasive hemodynamics at rest (n = 36). HFpEF was defined as having clinical symptoms of HF (exertional dyspnea or fatigue), LV ejection fraction of at least 50%, and pulmonary artery wedge pressure (PAWP) greater than 15 mm Hg at rest. Of 99 COA patients, 29 (29%) had obesity. The obese COA group had higher right atrial pressure and PAWP, and worse pulmonary and systemic vascular function compared with the non-obese COA group and the control group. The overall prevalence of HFpEF in adults with COA was 32%, and the prevalence was higher in COA patients with obesity (55%) compared with those without obesity (23%). Obesity was associated with HFpEF after adjustment for demographic indices, comorbidities, and vascular function. The abnormal hemodynamics and higher prevalence of HFpEF in COA patients with obesity underscores the need for intervention to address obesity in this population.

Clinical features, haemodynamics, and outcomes of heart failure with preserved ejection fraction in coarctation of aorta.

There are similarities in the pathogenesis of cardiac remodelling and dysfunction in heart failure with preserved ejection fraction (HFpEF) and coarctation of aorta (COA). We hypothesized that clinical HFpEF would be highly prevalent in adults with COA, and that the presence of HFpEF would increase the risk of mortality in this population. The aim of this study was to define the clinical features, haemodynamics, and prognostic implications of HFpEF in COA. Consecutive adults with repaired COA that underwent right heart catheterization were identified retrospectively. HFpEF was defined as heart failure symptoms (exertional dyspnoea or fatigue), preserved left ventricular ejection fraction ≥50%, and pulmonary artery wedge pressure at rest >15 mmHg. Of 99 COA patients, 32 (32%) had HFpEF. The correlates of HFpEF were obesity (adjusted odds ratio [OR] 4.15, 95% confidence interval [CI] 1.31-13.2), atrial fibrillation (adjusted OR 3.13, 95% CI 1.00-10.7), total arterial compliance index (adjusted OR 0.12, 95% CI 0.06-0.41 per 1 ml/mmHg*m2), and pulmonary artery compliance index (adjusted OR 0.36, 95% CI 0.15-0.56 per 1 ml/mmHg*m2). Of 99 patients, 24 (24%) died and 5 (5%) underwent heart transplant. The 10-year cumulative incidence of death/transplant was higher in COA patients with HFpEF compared with patients without HFpEF (39% vs. 12%, p = 0.001). The presence of HFpEF was associated with increased risk of death/transplant (adjusted hazard ratio 1.68, 95% CI 1.16-3.11). Heart failure with preserved ejection fraction is common in adults with COA and is associated with greater risk of death/transplant, emphasizing a pressing need for interventions to prevent and treat HFpEF in COA.

Exercise-induced hypertension after aortic coarctation repair: Our experience and a systematic review.

The exact prevalence and clinical significance of excessive increase in blood pressure in response to exercise in patients with repaired coarctation of aorta (CoA) remains unknown. This study aimed to investigate the impact of different definitions of exercise-induced hypertension (EIH) on the prevalence rates in our adult patients with repaired CoA. A systematic review of the available literature was also performed. We retrospectively analyzed exercise test data from adult patients with repaired CoA followed at the national referral center for adult congenital heart disease between 1998 and 2021. The three most reported definitions of EIH in patients with repaired CoA were used for the analysis of EIH prevalence. We also performed a systematic search of the PubMed electronic bibliographic database. Full-text versions of all potentially relevant articles on EIH in CoA were reviewed for relevance. Our registry included 161 adult CoA patients. Complete exercise test results were available in 74 patients (59% male, median age 39 years [range 20-68 years]). The prevalence of EIH in our cohort varied from 24 to 41%, depending on the definition used. We identified eleven eligible articles from 184 publications. The reported prevalence rate of EIH in the studies ranged from 13% to 82%. EIH is common in patients with repaired CoA; however, the rates of EIH vary greatly depending on the definition used. A standardized and uniform EIH definition is needed to accurately assess the prevalence and clinical relevance of EIH in this population.

Abstract 11913: Prognostic Value of Right Ventricular-Pulmonary Arterial Coupling in Adults With Repaired Coarctation of Aorta

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.

Relationship between left atrial myopathy and atrial fibrillation in adults with coarctation of aorta

BackgroundAlthough patients with coarctation of aorta (COA) have clinical risk factors for atrial fibrillation (AF), there are limited data about AF prevalence, and role of left atrial (LA) indices for risk stratification in this population. We hypothesized that LA indices (LA reservoir strain and LA volume index) were associated with AF, and would identify patients at risk for AF progression. MethodsWe analyzed electrocardiograms/Holters, and echocardiograms of adult COA patients at Mayo Clinic (2000–2018). ResultsOf 776 patients, 726(94 %), 46(5.9 %) and 4(0.5 %) had no history of AF, paroxysmal AF, and persistent AF respectively; yielding AF prevalence of 6.4 %. LA reservoir strain (AUC 0.782 [0.751–0.808]) had more robust association with AF as compared to LA volume index (AUC difference −0.115, p < 0.001).Among 726 patients without prior AF, 25(3.4 %) had new-onset AF during follow-up. LA reservoir strain <25 % and LA volume index >34 ml/m2 were independent predictors of new-onset AF (HR 1.81 [1.15–3.85], and HR 1.41 [1.03–4.78], respectively). Of 46 patients with paroxysmal AF, 22(48 %) had recurrent AF, and LA reservoir strain <25 % was an independent predictor of recurrent AF (HR 1.94 [1.41–4.17]). LV pressure overload and stiffness indices were associated with progressive LA dysfunction and new-onset AF. ConclusionsCollectively, these data suggest that LA strain can potentially be used for AF risk stratification. Further studies are required to determine whether LA strain can proactively identify patients that will respond favorably to different antiarrhythmic therapies, and whether interventions to reduce LV pressure overload will improve LA function and reduce AF progression.

Incidence and risk factors for late complications after the arterial switch operation.

The purpose of this study was to evaluate our institution's 16-year arterial switch operation (ASO)experience and to determine early and late mortality and late morbidity, as well as the need for reoperation and catheter intervention, and finally, to explore risk factors for late complications and reintervention. The clinical data of 185 transposition of the great arteries (TGA)patients who received ASO treatment in our center from January 2006 to January 2022 were continuously included for retrospective study. There were 13 early deaths (7.03%), 5 late deaths (3.01%), and 6 lost to follow-up. The median follow-up time for the 166 hospitalized survivors was 88.5 (2190) months. Moderate or above new aortic valve regurgitation (NAR;in this article, NAR represents moderate or greater reflux unless otherwise specified) occurred in 19 cases (11.45%), and aortic root dilation (ARD) occurred in 28 cases (16.87%). Late right ventricular outflow tract obstruction (RVOTO) occurred in 33 cases (19.88%). There were 18 patients (10.84%) who underwent late re-intervention, and the most common indication for intervention was RVOTO, followed by recurrent aortic coarctation in patients undergoing concurrent arch repair and NAR or ARD. Receiver operating characteristicsanalysis found that NAR had the strongest predictive power for ARD, followed by RVOTO, followed by bicuspid native pulmonary valve (BPV), and aorto-pulmonary diameter mismatch (APDMM) was the weakest. Multivariate analysis showed that APDMM, previous pulmonary artery banding (PAB), and mild NAR at discharge were independent risk factors for late NAR and ARD. Low surgical weight was an independent risk factor specific to NAR, and BPV was an independent risk factor specific to ARD. Older surgical age and ARD were independent risk factors for late RVOTO. Older surgical age, operation before 2014, late RVOTO, and late ARD were independent risk factors for late intervention. No reintervention events for coronary dysfunction were found in the late stage, but one patient occurred myocardial infarction due to coronary embolism after reoperation. Early and late survival rates after ASO in TGA patients have been remarkably improved in recent decades. Increased rates of NAR, ARD, recurrent coarctation of the aorta, and RVOTO as children age are major future outcomes of concern and may imply more late reinterventions. Careful follow-up of neo-aortic valve and root function is imperative, especially in patients with APDMM, previous PAB, mild NAR at discharge, low surgical weight, and BPV structures.

Cardiac Remodeling and Disease Progression in Patients With Repaired Coarctation of Aorta and Aortic Stenosis.

Valvulo-arterial impedance (Zva) is used for assessment of left ventricular (LV) global pressure load in patients with aortic stenosis (AS) and impaired arterial compliance. Because patients with repaired coarctation of aorta (COA) have impaired arterial compliance, we hypothesized that COA patients with greater than or equal to moderate AS (AS-COA group) will have higher Zva, symptomatic progression, and cardiovascular events, as compared to non-COA patients with similar AS severity (AS group). Propensity matching (1:1) of 71 AS-COA and 71 AS patients based on age, sex, body mass index, and aortic valve mean gradient (cohort 1). Of 172 patients, 117 patients (AS-COA [n=62]; AS [n=55]) underwent aortic valve replacement, cohort 2. Cohort 1 was used to assess the relationship between preoperative Zva, cardiac remodeling, and symptomatic progression, while cohort 2 was used to assess the relationship between postoperative Zva, LV mass index regression (reduction in LV mass index after aortic valve replacement), and cardiovascular events. The AS-COA group had higher Zva (4.2±0.6 versus 3.5±0.4 mm Hg/mL·m2, P<0.001), more advanced cardiac remodeling, and higher 5-year incidence of symptomatic progression (85% versus 51%, P<0.001). Preoperative Zva was independently associated with cardiac remodeling (r=0.66, P<0.001) and symptomatic progression (hazard ratio, 1.06 [1.02-1.10], per mm Hg/mL·m2 increase in Zva). The AS-COA group had higher postoperative Zva (3.3±0.5 versus 2.4±0.4 mm Hg/mL·m2, P<0.001), less robust LV mass index regression at 1-year post-aortic valve replacement, and higher 5-year incidence of cardiovascular events. Postoperative Zva was independently associated with LV mass index regression (r=-0.46, P<0.001) and cardiovascular events (hazard ratio, 1.06 [1.02-1.10], per mm Hg/mL·m2 increase in Zva). Adults with AS-COA had higher LV global pressure load, cardiac remodeling, symptomatic progression, and cardiovascular events as compared to non-COA patients with similar severity of AS. Zva can identify patients at risk for adverse outcomes, and perhaps should be used for risk stratification with regards to timing of aortic valve replacement.

Paradoxical increase in ambulatory SBP in coarctation of aorta a compared to essential hypertension.

The relationship between office vs. ambulatory blood pressure (BP) indices are well-studied in patients with essential hypertension and based on these data, it is known that the average 24-h ambulatory BP is typically lower than office BP. However, emerging data show that office SBP underestimates arterial afterload in patients with coarctation of aorta (COA), and a minimal increase in stroke volume during low-intensity exercise results in an exaggerated rise in SBP as compared to those with essential hypertension. We hypothesized that COA patients will have higher ambulatory SBP and a higher prevalence of masked hypertension compared to patients with essential hypertension. Case-control study of 118 COA patients and 118 patients with essential hypertension matched by age, sex, BMI and office SBP. Although both groups had similar office SBP (132 ± 17 mmHg) by design, the COA group had paradoxical increases in 24-h ambulatory SBP (135 ± 14 vs. 126 ± 13; P < 0.001) and daytime ambulatory SBP (142 ± 16 vs. 130 ± 13; P < 0.001), and less nocturnal dipping (-3 ± 5 vs. -9 ± 4; P < 0.001). The COA group also had a higher prevalence of masked hypertension [36 (31%) vs 14 (12%); P < 0.001), and worse arterial function indices. These findings underscore the potential limitations of relying on office SBP for screening/monitoring of hypertension in COA and potential pitfalls in extrapolating essential hypertension guidelines recommendations to the treatment of COA. It also provides the rationale for further studies to determine if pharmacologic BP interventions guided by ambulatory BP data will improve clinical outcomes in the COA population.

Abstract 10305: Cardiac Remodeling and Disease Progression in Patients with Repaired Coarctation of Aorta and Aortic Stenosis

Introduction: Valvuloarterial impedance (Zva) is used for assessment of left ventricular (LV) global pressure load in patients with aortic stenosis (AS) and impaired arterial compliance. Since patients with coarctation of aorta (COA) have impaired arterial compliance, we hypothesized that COA patients with ≥moderate AS (AS-COA group) will have higher Zva, symptomatic progression, and cardiovascular events, as compared to non-COA patients with similar AS severity (AS group). Methods: 1:1 propensity matching of 71 AS-COA and 71 AS patients based on age, sex, body mass index, and aortic valve mean gradient. Results: The AS-COA group had higher Zva (4.2±0.6 vs 3.5±0.4 mmHg/ml*m 2 , p&lt;0.001), more advanced cardiac remodeling, and higher 5-year incidence of symptomatic progression (85% vs 51%, p&lt;0.001). Pre-operative Zva was independently associated with cardiac remodeling (r=0.66, p&lt;0.001) and symptomatic progression (HR 1.08, 1.05-1.12, per mmHg/ml*m 2 increase in Zva). Of 172 patients, 117 underwent aortic valve replacement (AVR). The AS-COA group had higher residual Zva(3.3±0.5 vs 2.4±0.4 mmHg/ml*m 2 , p&lt;0.001), less robust LV mass index (LVMI) regression at 1-year post-AVR, and higher 5-year incidence of cardiovascular events. Post-operative Zva was independently associated with LVMI regression (r=-0.46, p&lt;0.001) and cardiovascular events (HR 1.06, 1.02-1.10, per mmHg/ml*m 2 increase in Zva). Conclusions: Adults with repaired COA repair and moderate-severe AS had higher LV global pressure load, cardiac remodeling, symptomatic progression, and cardiovascular events as compared to non-COA patients with similar severity of AS. Zva can identify patients at risk for adverse outcomes, and perhaps should be used for risk stratification with regards to timing of AVR.

Abstract 10306: Right Heart Dysfunction in Adults with Coarctation of Aorta - Prevalence and Prognostic Implications

Introduction: Chronic elevation of left heart filling pressure causes pulmonary vascular remodeling, pulmonary hypertension, and right heart dysfunction. Although diastolic dysfunction is relatively common in patients with coarctation of aorta (COA), there are limited data about the prevalence and prognostic implications of pulmonary hypertension and right heart dysfunction in this population. The purpose of the study was to assess right heart function and hemodynamics in patients with COA, and to determine the relationship between right heart indices and clinical outcomes. Methods: Right heart structure, function and hemodynamics were assessed with these indices: right atrial (RA) volume, RA pressure, RA reservoir strain, right ventricular (RV) global longitudinal strain, RV end-diastolic area, RV systolic pressure, and tricuspid regurgitation severity. These indices were used to generate a composite right heart hemodynamic score (RHHS), range 0-5. We then assessed the relationship between RHHS and primary outcome (death/transplant) and secondary outcomes (heart failure hospitalization and hepatorenal function). Results: Of 821 COA patients, RA dysfunction, RV dysfunction, and pulmonary hypertension were present in 16%, 14%, and 20% of patients respectively. RHHS was independently associated with primary and secondary outcomes (p&lt;0.001). For every unit increase in RHHS, we observed 83% increase in death/transplant and 71% increase in heart failure hospitalization. Conclusions: The current study underscores an important point, which is that COA is not just a disease of the left heart but can result in right heart dysfunction, and that the onset of right heart dysfunction is associated with adverse outcomes.

Prognostic implications of left heart diastolic dysfunction in adults with coarctation of aorta

Abstract Background Left atrial dysfunction (LAD) and left ventricular diastolic dysfunction (LVDD) has been described in patients with coarctation of aorta (COA). However, it is unknown whether LA function and LV diastolic function indices can be used for prognostication in this population. The purpose of this study was to determine whether LAD and LVDD were associated with mortality in adults with COA. We hypothesized that LA and LV diastolic function indices will predict transplant-free survival in COA patients. Methods Retrospective study of COA patients (age ≥18) at Mayo Clinic (2000–2018). LVDD was determined using the 2016 guidelines for LVDD assessment, and LAD was assessed using LA reservoir strain. Results Of 721 patients, LV diastolic function could be determined in 635 (88%), while 86 (12%) were classified as indeterminate diastolic function. Of 635 patients, 414 (65%) had no LVDD, while 146 (23%), 53 (8%), and 22 (4%) had grade I/II/III LVDD respectively. The mean LA reservoir strain was −39±11%, and patients were divided into quartiles: top quartile (reference group), mild LAD, moderate LAD, and severe LAD. Of 635 patients, 49 died and 4 underwent heart transplant. On multivariate analysis, Grade III diastolic dysfunction (but not Grade I and II) was associated with mortality as compared to normal diastolic function. On the other hand, there was an incremental risk of mortality across LA strain quartiles: mild LAD (HR 1.16, 1.04–2.06), moderate LAD (HR 1.75, 1.27–3.58), and severe LAD (HR 3.49, 1.88–7.16). Of the 86 patients with indeterminate diastolic function, LAD was associated with a lower 5-year transplant-free survival as compared to normal LA function (83% vs 91%, p=0.06). Conclusions LAD (but not LVDD) was associated with incremental risk of mortality, and thus can be used for prognostication in all patients including those with indeterminate diastolic function. Although the current study did not identify a consistent relationship between LVDD severity classifications and mortality among COA patients, it does not imply that LVDD is benign. Rather it suggests that the conventional criteria for LVDD severity classifications may not be generalizable to every disease subgroup Funding Acknowledgement Type of funding sources: None.

Paradoxical increase in ambulatory systolic blood pressure in coarctation of aorta as compared to idiopathic hypertension

Abstract Background The relationship between office vs ambulatory blood pressure (BP) indices is well-studied in patients with idiopathic hypertension, and based on these data, it is known that average 24-hour ambulatory BP is typically lower than office BP. However, emerging data show that office systolic BP (SBP) underestimates arterial afterload in patients with coarctation of aorta (COA), and minimal increase in stroke volume during low intensity exercise results in exaggerated rise in SBP as compared to those with idiopathic hypertension. We hypothesized that COA patients will have higher ambulatory SBP and a higher prevalence of masked hypertension compared to patients with idiopathic hypertension. Methods Case-control study of 118 COA patients and 118 patients with idiopathic hypertension matched by age, sex, body mass index and office SBP. Results Although both groups had similar office SBP (132±17 mmHg) by design, the COA group had paradoxical increases in 24-hour ambulatory SBP (135±14 vs 126±13, p&amp;lt;0.001) and daytime ambulatory SBP (142±16 vs 130±13, p&amp;lt;0.001), and less nocturnal dipping (−3±5 vs −9±4, p&amp;lt;0.001). The COA group also had a higher prevalence of masked hypertension (36 [31%] vs 14 [12%], p&amp;lt;0.001), and worse arterial function indices. Conclusion These findings underscore the potential limitations of relying on office SBP for screening/monitoring of hypertension in COA and potential pitfalls in extrapolating idiopathic hypertension guidelines recommendations to the treatment of COA. It also provides rationale for further studies to determine if pharmacologic BP interventions guided by ambulatory BP data will improve clinical outcomes in the COA population. Funding Acknowledgement Type of funding sources: None.

Coarctation of aorta is associated with left ventricular stiffness, left atrial dysfunction and pulmonary hypertension

Brachial systolic blood pressure (BP) is the most commonly used metric for monitoring hypertension. However, recent studies suggest that brachial systolic BP underestimates left ventricle (LV) systolic load in patients with coarctation of aorta (COA). Since brachial systolic BP is used as a surrogate of arterial afterload in clinical practice, it is important to determine how well it correlates with LV remodeling and stiffness in patients with COA as compared to patients with idiopathic hypertension. This is cross-sectional study of COA patients with hypertension (COA group) and adults with idiopathic hypertension (control group). Both groups were matched 1:1 based on age, sex, BMI and systolic BP. We hypothesized that the COA group will have higher LV systolic and diastolic stiffness, and more advanced left atrial remodeling and pulmonary hypertension. We assessed LV systolic stiffness using end-systolic elastance, and diastolic stiffness using LV stiffness constant and chamber capacitance (LV-end-diastolic volume at an end-diastolic pressure of 20mm Hg) RESULTS: There were 112 patients in each group. Although both groups had similar systolic BP, the COA group had a higher end-systolic elastance (2.37 ± 0.74 vs 2.11 ± 0.54 mm Hg/mL, P= .008), higher LV stiffness constant (6.91 ± 0.81 vs 5.93 ± 0.79, P= .006) and lower LV-end-diastolic volume at an end-diastolic pressure of 20mm Hg (58 ± 9 vs 67 ± 11 mL/m2, P< .001). Additionally, the COA group had more advanced left atrial remodeling and higher pulmonary artery pressures which is corroborating evidence of high LV filling pressure. COA patients have more LV stiffness and abnormal hemodynamics compared to non-COA patients with similar systolic BP, suggesting that systolic BP may underestimate LV systolic load in this population. Further studies are required to determine whether the observed LV stiffness and dysfunction translates to more cardiovascular events during follow-up, and whether adopting a stricter systolic BP target in clinical practice or changing threshold for COA intervention will lead to less LV stiffness and better clinical outcomes.

Personalized Pre- and Post-Operative Hemodynamic Assessment of Aortic Coarctation from 3D Rotational Angiography.

Coarctation of Aorta (CoA) is a congenital disease consisting of a narrowing that obstructs the systemic blood flow. This proof-of-concept study aimed to develop a framework for automatically and robustly personalizing aortic hemodynamic computations for the assessment of pre- and post-intervention CoA patients from 3D rotational angiography (3DRA) data. We propose a framework that combines hemodynamic modelling and machine learning (ML) based techniques, and rely on 3DRA data for non-invasive pressure computation in CoA patients. The key features of our framework are a parameter estimation method for calibrating inlet and outlet boundary conditions, and regional mechanical wall properties, to ensure that the computational results match the patient-specific measurements, and an improved ML based pressure drop model capable of predicting the instantaneous pressure drop for a wide range of flow conditions and anatomical CoA variations. We evaluated the framework by investigating 6 patient datasets, under pre- and post-operative setting, and, since all calibration procedures converged successfully, the proposed approach is deemed robust. We compared the peak-to-peak and the cycle-averaged pressure drop computed using the reduced-order hemodynamic model with the catheter based measurements, before and after virtual and actual stenting. The mean absolute error for the peak-to-peak pressure drop, which is the most relevant measure for clinical decision making, was 2.98 mmHg for the pre- and 2.11 mmHg for the post-operative setting. Moreover, the proposed method is computationally efficient: the average execution time was of only [Formula: see text] minutes on a standard hardware configuration. The use of 3DRA for hemodynamic modelling could allow for a complete hemodynamic assessment, as well as virtual interventions or surgeries and predictive modeling. However, before such an approach can be used routinely, significant advancements are required for automating the workflow.

Differences in resting and ambulatory blood pressure data in coarctation of aorta vs idiopathic hypertension

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&amp;lt;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

Increased prevalence of left ventricular diastolic dysfunction in adults with coarctation of aorta

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 &amp;lt;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&amp;gt;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&amp;lt;0.001), respectively. Compared to controls, the prevalence of LV diastolic dysfunction was higher in COA patients for every age group: &amp;lt;40 years (63% vs 13%, p&amp;lt;0.001); 41–60 years (87% vs 33%, p&amp;lt;0.001); age &amp;gt;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&amp;lt;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

Potential benefits of ambulatory blood pressure monitoring in coarctation of aorta

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

Determinants of left ventricular hypertrophy and dysfunction in adults with coarctation of aorta

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&amp;lt;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 &amp;lt;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

Predictors of left ventricular reverse remodelling after coarctation of aorta intervention.

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 remodelling after COA intervention. COA severity indices were defined as Doppler COA gradient, systolic blood pressure (SBP, upper-to-lower-extremity SBP gradient, aortic isthmus ratio. LV remodelling indices were defined as LV mass index (LVMI), LV global longitudinal strain (LVGLS), e' and E/e'. LV reverse remodelling was defined as the difference between indices obtained pre-intervention and 5-year post-intervention (delta LVMI, e', E/e', LVGLS).Of the COA indices analysed 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 remodelling, and residual aortic isthmus ratio <0.7 was predictive of suboptimal LV reverse remodelling post-intervention. Considering the known prognostic implications of LV remodelling and reverse remodelling in response to pressure overload, these results support the use of aortic isthmus ratio for timing of COA intervention, and for prognostication post-intervention.

Is the burden of late hypertension and cardiovascular target organ damage in children and adolescents with coarctation of the aorta after early successful repair different to healthy controls?

Cardiovascular morbidity is high in patients with coarctation of aorta even after successful repair. This study aimed to assess the frequency of late hypertension and the relationship between ambulatory hypertension and cardiovascular target organ damage in children and adolescents after early and successful repair of coarctation of the aorta. Twenty-five children and adolescents (mean age 13.5 ± 3.43 years) with repaired coarctation of the aorta (median age at repair 4 months, arm-leg gradient <20 mmHg) and 16 healthy controls were included. Office and ambulatory blood pressure, pulse wave velocity, and left ventricular mass index were assessed. Both day- and night-time systolic blood pressure standard deviation score and left ventricular mass index were significantly higher in patients compared to controls (p ≤ 0.001 for all), whereas pulse wave velocity did not differ. The prevalence of masked hypertension, isolated nocturnal hypertension, and left ventricular hypertrophy were 40, 28, and 24%, respectively. Left ventricular mass index was higher in patients with sustained hypertension, masked hypertension, and normotension compared to controls (p < 0.05). In multivariate analysis, higher night-time systolic blood pressure standard deviation score was the only independent predictor of left ventricular mass index. The present study reveals a high prevalence of masked hypertension, isolated nocturnal hypertension, and left ventricular hypertrophy in children and adolescents with coarctation of the aorta even after early and successful repair. Ambulatory blood pressure monitoring should be considered to diagnose hypertension. All coarctation of aorta patients should be followed up lifelong and encouraged to establish a healthy lifestyle starting from childhood.