Citations

Abstract Aims Conotruncal anomalies share common embryogenic defects of the outflow tracts and great arteries, which result in a predisposition to aortic aneurysms. The purpose of this study was to describe the prevalence and risk of progressive aortic aneurysms in adults with conotruncal anomalies. Methods and results Retrospective study of adults with conotruncal anomalies that underwent cross-sectional imaging 2003–20. Aneurysm was defined as aortic root/mid-ascending aorta >2.1 mm/m2/>1.9 mm/m2, progressive aneurysm as increase by >2 mm, and severe aneurysm as dimension >50 mm. Of 2261 patients (38 ± 12 years; male 58%), 1167 (52%) had an aortic aneurysm, and 205 (14%) had a severe aortic aneurysm. Mean annual increase in aortic root/mid-ascending aorta was 0.3 ± 0.1 mm/0.2 ± 0.1 mm. The 3-, 5-, and 7-year cumulative incidence of the progressive aortic aneurysm was 4%, 7%, and 9%, respectively. The rate of aneurysm growth decreased with age, with no significant growth after age 40 years. There was an excellent correlation between aortic indices from cross-sectional imaging and echocardiography. Of 950 females, 184 had ≥1 pregnancy, and 81 (44%) of the 184 patients had aortic aneurysm prior to pregnancy. There was no aortic dissection or progression of the aortic aneurysm during pregnancy. Overall, there was no aortic dissection during 7984 patient-years of follow-up. Conclusions Aortic aneurysm was common in patients with conotruncal anomalies. However, the risk of progressive aneurysm or dissection was low. Collectively, these data suggest a benign natural history and perhaps a less frequent need for cross-sectional imaging. Further studies are required to determine the optimal timing for surgical intervention in this population.

Background: 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, 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 coarctation of aorta and to determine the relationship between right heart indices and cardiovascular events defined as heart failure hospitalization, heart transplant, or cardiovascular death. Methods: Right heart structure, function, and hemodynamics were assessed with these indices: right atrial volume, right atrial pressure, right atrial reservoir strain, right ventricular global longitudinal strain, right ventricular end-diastolic area, right ventricular systolic pressure, and tricuspid regurgitation severity. Right heart hemodynamic score, range 0 to 5, was generated based on the correlation between the right heart indices and cardiovascular events, using half of the cohort (derivation cohort, n=411), and then tested on the validation cohort (n=410). The goodness of fit and discrimination power was compared using C statistics and risk score. Results: The median follow-up in the derivation cohort was 8.2 (4.0–11.1) years, and 59 (14%) patients had cardiovascular events during this period. Right heart hemodynamic score was independently associated with cardiovascular events (hazard ratio, 1.64 [95% CI, 1.38–2.17]) for every unit increase in right heart hemodynamic score after adjustment for clinical and echocardiographic indices (C statistic, 0.718 [95% CI, 0.682–0.746]). The right heart hemodynamic score was also independently associated with cardiovascular events in the validation cohort (C statistic, 0.711 [95% CI, 0.679–0.741]). The C statistic difference (0.007 [95% CI, 0.014–0.022]) and risk score (0.86 [95% CI, 0.54–1.17]) suggest a good model fit. Conclusions: The current study underscores the prognostic importance of right heart dysfunction in patients with coarctation of aorta and suggests that right heart indices should be used for risks stratification in this population.

Background 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. Methods Case-control study of 118 COA patients and 118 patients with essential hypertension matched by age, sex, BMI and office SBP. Results 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. Conclusion 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.

Background: We sought to investigate temporal trends in patient characteristics, waitlist, and posttransplant outcomes after heart transplantation in the United States. Methods: Using data from the United Network of Organ Sharing, we identified adults listed for heart transplantation between 1991 and 2019. Patients were divided into 4 eras based on the 3 time points in which changes were made to the patient selection/allocation policy (Era 1=January 1991–January 1999; Era 2=January 1999–July 2006; Era 3=July 2006–October 2018; and Era 4=October 2018–March 2020), and patient characteristics, waitlist, and posttransplant outcomes were evaluated for each era. Results: Between 1991 and 2019, 95 179 patients were added to the heart transplantation waitlist. Compared with Era 1, patients listed in Era 4 were older (mean age: 50 versus 52 years) and with higher risk comorbidities (eg, 10% versus 28.8% diabetes, 23.3% versus 35.6% obese). Over the study period, 22 738 patients died or were permanently delisted for deterioration on the waitlist while 61 687 were transplanted. Compared with the preceding era, there was significant decrease in death or deterioration in the last 2 eras (sub-hazard ratio, 0.67 [95% CI, 0.65–0.70] for Era 3 versus Era 2 and sub-hazard ratio, 0.65 [95% CI, 0.58–0.73] for Era 4 versus 3). Across the years, 27.1% to 40.5% of those on the waitlist were transplanted. Among those transplanted, there was increase in the rates of in-hospital stroke (2.8% in Era 1 to 3.7% in Era 4), renal failure requiring dialysis (7.2%–17.1%), and length-of-stay (14–17days), P <0.001. However, this did not negatively impact short-term survival when compared with the preceding era (1-year graft survival from Era 1 to Era 4=84.1%, 86.4%, 90.4%, and 89.7%, respectively). Conclusions: There have been significant changes in the characteristics of patients listed for heart transplantation. Although transplant volume has increased, the wide supply-demand gap persisted. The last two changes in the allocation policy achieved their primary objective of reducing waitlist mortality.

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&lt;0.001) and daytime ambulatory SBP (142±16 vs 130±13, p&lt;0.001), and less nocturnal dipping (−3±5 vs −9±4, p&lt;0.001). The COA group also had a higher prevalence of masked hypertension (36 [31%] vs 14 [12%], p&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.

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.

Abstract Background The landscape of heart transplantation (HT) has changed significantly with respect to patient selection, surgical techniques, and patient outcomes. We sought to investigate temporal trends in patient characteristics, waitlist and post-transplant outcomes after HT in the U.S. Methods We queried the national database of the United Network of Organ Sharing (UNOS) to identify adults listed for HT in the U.S. between 1991 and 2019. Patients were divided into four eras based on the three time points in which changes were made to the patient selection/allocation policy (1999, 2006, and 2018), and patient characteristics as well as waitlist and post-transplant outcomes were evaluated for each era. Results Between 1991 and 2019, a total of 95,179 patients were added to the waitlist for HT in the U.S. Compared to era 1, patients listed in era 4 were older (mean age: 52.4 years), more female (27.6%) and ethnic minorities (40%), and with higher-risk comorbidities (28.8% diabetes, 35.6% obese). Over the study period, there were 22,070 waitlist deaths and 61,687 transplants. Compared to the preceding era, there was significant decrease in waitlist mortality in the last 2 eras (e.g., sub-hazard ratio for era 4 vs era 3 =0.37, 95% CI=0.32–0.44). For each year, only 27.1% to 40.5% of those on the waitlist were transplanted. Among those who were transplanted, there was increase in the rates of in-hospital stroke (2.8% in era 1 to 3.7% in era 4), renal failure requiring dialysis (7.2% to 17.1%), and hospital length of stay (14 to 17 days), p-values&lt;0.001 for all. However, this has not negatively impacted short-term survival when compared to the preceding era (1-year graft survival = 89.7% in era 4). Based on a projection model, we predict a 47% increase in living adult heart transplant recipients to to 44,366 in 2040. Conclusion There have been significant changes in the characteristics of patients listed for HT in the U.S., including an increasing proportion of high-risk co-morbidities. Although the transplant volume has increased, the wide supply-demand gap persisted. The last 2 changes in the allocation policy in 2006 and 2018 achieved their primary objective of reducing waitlist mortality. Funding Acknowledgement Type of funding sources: None.

Abstract Background There are limited data about outcomes after repair of native coarctation of aorta (COA) in adulthood. The purpose of this study was to describe the procedural outcomes, hemodynamic improvement, regression of LV hypertrophy and cardiovascular events in adults undergoing repair of native COA. Methods The primary outcomes were procedural complications, re-interventions, and hemodynamic improvement (residual COA gradient &lt;20 mmHg) after COA repair. The secondary outcomes were improvement in the severity of hypertension, regression of left ventricular mass index (LVMI), and incidence of cardiovascular events (atrial fibrillation, heart failure hospitalization, and cardiovascular death) after COA repair. Clinical and imaging indices were assessed pre-intervention, and at 1-year (Y1) and 3-years (Y3) post-intervention. Results A total of 172 patients (age 38 [27–48]) underwent COA repair (surgical 161; transcatheter 11). There were no procedural deaths, and all patients had residual COA gradient &lt;20 mmHg. One patients that received transcatheter stent therapy required re-dilation of stent at 12 months post-intervention. There as a reduction in the prevalence of patients requiring anti-hypertensive therapy from 73% (pre-intervention) to 59% and 64% at Y1 and Y3 respectively. However, 72% and 69% of the patients still had systolic blood pressure &gt;130 mmHg (stage 1 and 2 hypertension) at Y1 and Y3 respectively. As compared to patients without hypertension at Y1, patients with stage 1 and stage 2 hypertension had less robust LVMI regression (% change in LVMI 12±5% vs 9±6% vs 5±3%, p&lt;0.001). Hypertension (HR 1.16, 1.05–1.27) and LVMI regression (HR 0.86, 0.81–0.90) were independently associated with cardiovascular events. Conclusions Persistent hypertension was common after repair of native COA in adults. Hypertension (including stage 1 hypertension) was associated with suboptimal regression of LVMI and cardiovascular events. These results are concerning, and highlight the importance of early COA diagnosis and repair, and optimal medical therapy for hypertension after COA repair. Funding Acknowledgement Type of funding sources: None.

Abstract Background Certain factors such as left ventricular (LV) geometry and loading conditions affect the validity and reliability of LV ejection fraction (LVEF) as a true measure of LV contractility. LV global longitudinal strain (LVGLS) is less sensitive to these factors, and it has superior prognostic performance in patients with acquired heart disease. The purpose of this study was to determine the clinical implications of using LVGLS (instead of LVEF) as the measure of LV systolic function in adults with Ebstein anomaly given the inherent abnormalities of LV geometry and preload in this population. Methods Retrospective cohort study of 673 adults with Ebstein anomaly (2003–2018). We hypothesized that LVGLS had a stronger correlation with heart failure indices and transplant-free survival compared to LVEF. Results Compared to LVEF, LVGLS had stronger correlations with cardiac index (r=0.46 vs r=0.21, p=0.007), glomerular filtration rate (r=0.57 vs r=0.19, p&lt;0.001), and NT-proBNP (r=−0.64 vs r=−0.41, p=0.01). Of 673 patients, 514 (76%) had normal LV systolic function (LVGLSNormal-LVEFNormal), 87 (13%) had subclinical LV dysfunction (LVGLSLow-LVEFNormal) and 66 (10%) had overt LV dysfunction (LVGLSLow-LVEFLow). Compared to the overt LV dysfunction group, the subclinical LV dysfunction group had similar 10-year transplant-free survival (64% vs 63%, p=0.6), but were less likely to be on heart failure therapy (12% vs 82%, p&lt;0.001). LVGLS (but not LVEF) was the independent predictor of transplant-free survival Conclusions LVGLS provided more robust risk stratification and prognostication than LVEF in patients with Ebstein anomaly, and patients with low LVGLS had reduced transplant-free survival regardless of LVEF. The use of LVGLS (rather than LVEF) as the measure of LV systolic function has important clinical implications with regards to initiation of medical and surgical therapies. Funding Acknowledgement Type of funding sources: None.

AbstractAimsThe prognostic implication of left atrial (LA) dysfunction and left ventricular diastolic dysfunction (LVDD) in patients with coarctation of aorta (COA) is unknown. The purpose of this study was to determine whether LA dysfunction and LVDD were associated with mortality in COA patients.Methods and resultsThis is a retrospective review of adults (age ≥18 years) with repaired COA that underwent transthoracic echocardiogram (2000–18). LVDD was determined using the 2016 guidelines for LV diastolic function assessment, and LA dysfunction was assessed using LA reservoir strain. Of 721 patients, LV diastolic function could be determined in 635 (88%); and 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 LA dysfunction, moderate LA dysfunction, and severe LA dysfunction. Grade III LVDD (but not Grades I and II) was associated with death/transplant. On the other hand, there was an incremental risk of death/transplant across LA strain quartiles: mild LA dysfunction [hazard ratio (HR) 1.16, 1.04–2.06], moderate LA dysfunction (HR 1.75, 1.27–3.58), and severe LA dysfunction (HR 3.49, 1.88–7.16). Of 86 patients with indeterminate diastolic function, there was a trend towards a lower 5-year transplant-free survival in patients with LA dysfunction vs. normal LA function (83% vs. 91%, P = 0.06).ConclusionLA dysfunction (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.