Citations

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

Background: Chronic left ventricular (LV) pressure overload is common in adults with repaired coarctation of aorta (COA). However, there is no validated systematic approach for assessing cardiac remodeling and damage in this population. The purpose of this study was to evaluate the role of cardiac damage staging scheme (validated for calcific aortic stenosis) for prognostication in adults with repaired COA. Methods: Patients were classified using a modified cardiac damage scheme: stage 0 (no cardiac damage), stage 1 (LV damage), stage 2 (left atrial damage), stage 3 (pulmonary vascular/tricuspid valve damage), and stage 4 (right ventricular damage). Results: Of 795 patients (age 36±16 years), 270 (34%) had stage 0, 198 (25%) had stage 1, 225 (28%) had stage 2, 37 (5%) had stage 3, and 65 (8%) had stage 4 cardiac damage. The 795 patients were followed for 7.3 (2.8-9.6) years, and 94 (12%) died, of which 75 (9%) were cardiovascular deaths. Cardiac damage stage was independently associated with all-cause mortality (adjusted HR 1.31, 95% CI 1.22-1.40, per unit increase in stage), and cardiovascular mortality (adjusted HR 1.42, 95% CI 1.28-1.55, per unit increase in stage). The addition of the cardiac damage stage to the base model (clinical and echocardiographic variables) provided significant incremental improvement in the prediction of 5-year risk of all-cause mortality over the base model (net reclassification index 0.39, p<0.001). Hypertension was associated with cardiac damage and mortality. Conclusions: The cardiac damage scheme can be used for risk stratification, and potentially guide therapy in the COA population.

Background: Aortic regurgitation (AR) can develop in adults with repaired coarctation of aorta (COA), but there are limited data about left ventricular (LV) remodeling and clinical outcomes in this population. The purpose of the study was to compare LV remodeling (LV mass index [LVMI], LV ejection fraction [LVEF], and septal E/e’) and onset of symptoms before aortic valve replacement (AVR), and LV reverse remodeling (%-change in LVMI, LVEF and E/e’) after AVR in patients with versus without repaired COA presenting with AR. Methods:: Asymptomatic adults with repaired COA presenting with moderate/severe AR (AR-COA group) were matched 1:2 to asymptomatic adults without COA and similar severity of AR (control group). Results: Although both groups (AR-COA n=52, and control n=104) had similar age, sex, body mass index, aortic valve gradient, and AR severity, the AR-COA group had higher LVMI (124±28 versus 102±25 g/m2, p<0.001) and E/e’ (12.3±2.3 versus 9.5±2.1, p=0.02) but similar LVEF (63±9% versus 67±10%, p=0.4). COA diagnosis (adjusted HR 1.95, 95%CI 1.49-2.37, p<0.001), older age, E/e’, and LV hypertrophy wereassociated with onset of symptoms. Of 89 patients (AR-COA n=41, and control n=48) with echocardiographic data at 1-year post-AVR, the AR-COA group had less regression of LVMI (-8% [95%CI -5 to -11] versus -17% [95%CI -15 to -21], p<0.001) and E/e’ (-5% [95% CI -3 to -7] versus -16% [95% CI -13 to -19], p<0.001). Conclusions: Patients with COA and AR had a more aggressive clinical course, and perhaps may require a different threshold for surgical intervention.

Background: Left atrial (LA) reservoir strain <18% and booster strain <8% have been proposed as the optimal threshold to detect increased left ventricular (LV) filling pressure in patients with acquired heart disease. This study aimed to determine whether these LA strain cut-off points can detect increased LV filling pressure in adults with coarctation of aorta (COA). Methods: Retrospective study of adults with COA (n=126; age 36±16 years), that underwent non-simultaneous cardiac catheterization and echocardiography. Increased LV filling pressure was defined as pulmonary artery wedge pressure (PAWP) >12 mmHg or LV end-diastolic pressure (LVEDP) >16 mmHg. Results: The median PAWP was 13 (11-18) mmHg, and had a good correlation with LA reservoir strain (r=-0.69, p<0.001) and LA booster strain (r=-0.61, p<0.001). LA reservoir strain <18% had superior diagnostic power to detect PAWP >12 mmHg as compared to LA volume index >34 ml/m2, septal E/e’ >15, lateral E/e’ >13, and tricuspid regurgitation velocity >2.8 m/s (p<0.05 for all). The median LVEDP was 17 (14-20) mmHg, and LVEDP had a modest correlation with LA reservoir strain (r=-0.39, p<0.001) and LA booster strain (r=-0.33, p=0.002). LA reservoir strain <18% had superior diagnostic power to detect LVEDP >16 mmHg as compared to LA volume index >34 ml/m2, septal E/e’ >15, lateral E/e’ >13 and tricuspid regurgitation >2.8 m/s (p<0.05 for all). Conclusions: These data suggest that LA strain could be used to identify patients with increased LV filling pressure thereby improving patient selection for cardiac catheterization and interventions.

Background: The prognostic implications of exercise induced hypertension (EIH) in adults with repaired coarctation of aorta (COA) and treated hypertension are unknown. The purpose of this study was to assess the relationship between EIH and cardiovascular events, and to determine whether exercise blood pressure (BP) improved risk stratification in this population. Methods: Retrospective study of patients with COA on antihypertensive therapy that underwent exercise testing (2003-2019). BP was measured at rest in 3 different clinical settings and averaged to determine the resting BP. EIH was defined as systolic BP (SBP) >210 (males) or >190 (females) at peak exercise. Cardiovascular event was defined as atrial fibrillation, ventricular tachycardia, heart failure hospitalization, heart transplant, and cardiovascular death. Results: Of 327 patients (age 35±13 years), 116 (35%) had EIH. Although the resting BP was similar between patients with versus without EIH, the EIH group had higher pulsatile arterial load, more advanced left ventricular remodeling, and were less likely to be on angiotensin converting enzyme inhibitor/angiotensin receptor blocker (ACEI/ARB). EIH was associated with cardiovascular events (HR 1.06 [95%CI 1.02-1.08]) independent of resting SBP, and improved prognostic accuracy above resting SBP (C-statistic 0.671 [95%CI 0.645-0.694] to 0.727 [95%CI 0.709-0.750], p=0.01). ACEI/ARB was associated with a lower risk of cardiovascular events. Conclusions: EIH was associated with cardiovascular events independent of resting BP, and patients receiving ACEI/ARB had lower risk of EIH and cardiovascular events. These data suggest that exercise BP could be used to assess adequacy of antihypertensive therapy, and to guide titration of antihypertensive therapy.

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 &gt;2.1 mm/m2/&gt;1.9 mm/m2, progressive aneurysm as increase by &gt;2 mm, and severe aneurysm as dimension &gt;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.