Prevalence of respiratory failure and use of mechanical ventilation in heart failure patients undergoing left atrial appendage occlusion device implantation.

BACKGROUND: We sought to evaluate respiratory complications in heart failure patients undergoing left atrial appendage occlusion (LAAO) for stroke prevention in atrial fibrillation. METHODS: Adult admissions (>18 years) undergoing LAAO during 2016-2020 were identified from the National Inpatient Sample. Heart failure (HF) was stratified into systolic (SHF) and diastolic heart failure (DHF) and were compared to those without HF. Outcomes of interested included acute respiratory failure, use of non-invasive and invasive mechanical ventilation, and in-hospital mortality. RESULTS: Of 74,440 admissions for atrial fibrillation undergoing LAAO, SHF and DHF were noted in 8335 (11.2%) and 10,925 (14.7%), respectively. The SHF cohort was predominantly male (78%) whereas DHF cohort were female (53%). Compared to those without HF, presence of SHF (2.3% vs. 0.6%; adjusted odds ratio [OR] 1.61 [95% confidence interval {CI} 1.10-2.36]; p = 0.01) and DHF (2.8% vs. 0.6%; adjusted OR 2.20 [95% CI 1.58-3.06]; p < 0.001) were associated with higher rates of acute respiratory failure. SHF (1.7% vs. 0.6%; adjusted OR 1.70 [95% CI 1.07-2.71]; p = 0.02) group but not DHF (1.2% vs. 0.6%; adjusted OR 1.21 [95% CI 0.78-1.89]; p = 0.39) was associated with higher rates of non-invasive ventilation, whereas the DHF group (0.9% vs. 0.2%; adjusted OR 1.91 [95% CI 1.08-3.34]; p = 0.02) but not SHF (0.8% vs. 0.2%; adjusted OR 1.54 [95% CI 0.83-2.84]; p = 0.17) was associated with higher rates of invasive mechanical ventilation use. In-hospital mortality was comparable between cohorts. CONCLUSION: Compared to those without HF, atrial fibrillation admissions with HF undergoing LAAO had higher rates of acute respiratory failure and mechanical ventilation rates without differences in in-hospital mortality.

Prognostic Performance of Right Ventricular Global Longitudinal Strain Measurements in Patients With Ebstein Anomaly.

BACKGROUND: There are limited data on the prognostic role of right ventricular global longitudinal strain (RVGLS) in patients with Ebstein anomaly. OBJECTIVES: This study sought to assess the relationship between RVGLS and mortality and to compare prognostic performance of RVGLS with conventional echocardiographic indices of right ventricular (RV) systolic function. METHODS: This study identified adults with Ebstein anomaly with echocardiographic assessment of RV systolic function (RVGLS, RV fractional area change [RVFAC], RV tissue Doppler systolic velocity [RV s'], and tricuspid annular plane systolic excursion [TAPSE]) from 2003 to 2020. For ease of presentation, RVGLS was modeled as absolute values (ie, without the negative sign). RESULTS: Of 620 patients (median age 37 years; men 261 [42%]), the mean absolute RVGLS, RVFAC, RV s', and TAPSE were 18% ± 5%, 32% ± 9%, 14 ± 6 cm/s, and 22 ± 8 mm, respectively. There were correlations between absolute RVGLS and RVFAC (r = 0.71; P < 0.001), between absolute RVGLS and RV s' (r = 0.41; P = 0.03), and between absolute RVGLS and TAPSE (r = 0.44; P = 0.002). Of 620 patients, 47 (8%) died during follow-up, and 34 of these deaths were cardiovascular. Absolute RVGLS was independently associated with all-cause mortality (adjusted HR: 0.94; 95% CI: 0.92-0.96 per unit increase) and cardiovascular mortality (adjusted HR: 0.92; 95% CI: 0.90-0.94 per unit increase). Absolute RVGLS had superior prognostic power (ie, ability to predict mortality) as compared with RVFAC, RV s', or TAPSE. CONCLUSIONS: These data support the use of RVGLS for risk stratification in Ebstein anomaly, and further studies are required to assess how interventions may affect different patients according to risk stratification.

Clinical benefits of angiotensin receptor-Neprilysin inhibitor in adults with congenital heart disease.

BACKGROUND: There are limited data about the clinical benefits of angiotensin receptor-neprilysin inhibitor (ARNI) in adults with congenital heart disease (CHD). The purpose of the study was to assess the clinical benefits (chamber function and heart failure indices) of ARNI in adults with CHD. METHOD: In this retrospective cohort study, we compared the temporal change in chamber function and heart failure indices between 35 patients that received ARNI for >6 months, and a propensity matched control group (n = 70) of patients that received angiotensin converting enzyme inhibitor or angiotensin-II receptor blocker (ACEI/ARB) within the same period. RESULTS: Of the 35 patients in the ARNI group, 21 (60%) had systemic left ventricle (LV) while 14 (40%) had systemic right ventricle (RV). Compared to the ACEI/ARB group, the ARNI group had greater relative improvement in LV global longitudinal strain (GLS) (28% versus 11% increase from baseline, p < 0.001) and RV-GLS (11% versus 4% increase from baseline, p < 0.001), and greater relative improvement in New York Heart Association functional class (-14 versus -2% change from baseline, p = 0.006) and N-terminal pro-brain natriuretic peptide levels (-29% versus -13% change from baseline, p < 0.001). These results were consistent across different systemic ventricular morphologies. CONCLUSIONS: ARNI was associated with improvement in biventricular systolic function, functional status, and neurohormonal activation, suggesting prognostic benefit. These results provide a foundation for a randomized clinical trial to empirically test the prognostic benefits of ARNI in adults with CHD, as the next step towards evidence-based recommendations for heart failure management in this population.

Temporal Changes in Clinical Characteristics and Outcomes of Adults With Congenital Heart Disease.

BACKGROUND: The purpose of this study was to assess differences in the clinical characteristics (defined by congenital heart disease [CHD] anatomic and physiologic classification scheme) of adults with CHD across different eras, and how these differences influence outcomes (heart failure hospitalization and all-cause mortality). METHOD: Patients were divided into depending on year of baseline encounter: cohort #1 (1991-2000, n = 1,984 [27%]), cohort #2 (2001-2010, n = 2,448 [34%]), and cohort #3 (2011-2020, n = 2,847 [39%]). Patients were classified into 3 anatomic groups (simple, moderate, and complex CHD) and 4 physiologic stages (stage A-D). RESULTS: There was a temporal increase in the proportion of patients in physiologic stage C (17% vs 21% vs 24%, P < .001), and stage D (7% vs 8% vs 10%, P = .09), with a corresponding decrease in physiologic stage A (39% vs 35% vs 28%, P < .001). No temporal change in anatomic groups. There was a temporal decrease in the incidence of all-cause mortality (12.7 vs 10.6 vs 9.5 per 1,000 patient-years, P < .001). However, there was a temporal increase in the incidence of heart failure hospitalization (6.8 vs 8.4 vs 11.2 per 1,000 patient-years, P < .001). CHD physiologic stage (but not anatomic groups) was associated with heart failure hospitalization and all-cause mortality. CONCLUSIONS: There is a need for better strategies to identify and treat heart failure, and to modify the risk factors associated with heart failure and all-cause mortality.

Left ventricular adaptation to aortic regurgitation in adults with repaired coarctation of aorta.

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, and LV reverse remodeling (%-change in LVMI, LVEF and E/e') after aortic valve replacement 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 were associated with onset of symptoms. Of 89 patients (AR-COA n = 41, and control n = 48) with echocardiographic data at 1-year post- aortic valve replacement, 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.

Right Ventricular Systolic Dysfunction in Adults With Anatomic Repair of d-Transposition of Great Arteries.

The purpose of this study was to assess the prevalence of right ventricular (RV) systolic dysfunction in adults with anatomic repair of dextro-transposition of great arteries (d-TGAs), and to determine its relation to clinical outcomes across multiple domains (functional status, peak oxygen consumption, N-terminal pro-brain natriuretic peptide, and heart failure hospitalization). Adults with anatomic repair for d-TGA and with echocardiographic images for strain analysis were divided into 2 groups: (1) d-TGA status after an arterial switch operation (d-TGA-ASO group) and (2) d-TGA status after a Rastelli operation (d-TGA-Rastelli group). RV systolic function was assessed using RV global longitudinal strain (RVGLS), and RV systolic dysfunction was defined as RVGLS >-18%. We identified 151 patients (median age 21 years [19 to 28]; d-TGA-ASO group 89 [59%], and d-TGA-Rastelli group 62 [41%]). The mean RVGLS was -22 ± 4%, and 47 patients (31%) had RV systolic dysfunction. The d-TGA-Rastelli group had lower (less negative) RVGLS than that of the d-TGA-ASO group (-19 ± 3% vs -25 ± 3%, p <0.001) and higher prevalence of RV systolic dysfunction (48% vs 19%, p <0.001). RVGLS (absolute value) was associated with peak oxygen consumption (r = 0.58, p <0.001; adjusted R2 = 0.28), log-N-terminal pro-brain natriuretic peptide (r = -0.41, p = 0.004; adjusted R2 = 0.21), New York Heart Association class III/IV (odds ratio 2.29, 1.56 to 3.19, p = 0.01), and heart failure hospitalization (hazard ratio 0.93, 0.88 to 0.98, p = 0.009). RV systolic dysfunction was common in adults with d-TGA and anatomic repair and was associated with clinical outcomes. Longitudinal studies are required to determine the risk factors for progressive RV systolic dysfunction and to identify strategies for preventing RV systolic dysfunction in this population.

Risk of pulmonary artery dissection in adults with congenital heart disease.

BACKGROUND: There are limited data about the risk of pulmonary artery (PA) dissection in adults with congenital heart disease (CHD), and the purpose of this study was to estimate the incidence of PA dissection in this population. METHODS: Retrospective cohort study of adults with CHD that underwent cross-sectional imaging (2003-2020). PA aneurysm was defined as main or branch PA diameter > 40 mm or > 30 mm respectively, and severe PA aneurysm was defined as main or branch PA diameter > 50 mm. RESULTS: Of 1, 673 patients (41 ± 10 years; male 58%), 493 (24%), 286 (19%), and 306 (20%) had aneurysms of the main, right, and left PA respectively, while 66 (4%) had severe PA aneurysm. During a median follow-up of 8.2 (interquartile range 3.7-10.3) years, there was one PA dissection in a patient with Eisenmenger syndrome, thus the incidence of PA dissection was 14 per 100,000 patient-years. Of 779 females, 163 had one or more pregnancies during follow-up, and 41 (25%) of these patients had known PA aneurysm at the time of conception. There was no PA dissection during pregnancy. Of the 163 patients, 91 (56%) had cross-sectional imaging before and after pregnancy, there was no significant difference in PA dimension before versus after pregnancy (main PA 35 ± 5 versus 36 ± 4 mm, p = 0.6; right PA 21 ± 3 versus 33 ± 4 mm, p = 0.1; and left PA 23 ± 4 versus 22 ± 4 mm, p = 0.4). CONCLUSIONS: The risk of PA dissection was extremely low even in patients with severe PA aneurysm, or in patients with PA aneurysm that became pregnant. Collectively, these data suggest a benign natural history for patients without severe pulmonary hypertension and encourage and a conservative approach in managing patients with PA aneurysm.

Outcome of New-Onset Postoperative Atrial Fibrillation After Cardiac Surgery in Adults With Congenital Heart Disease.

BACKGROUND: Postoperative atrial fibrillation (POAF) is one of the most common complications after cardiac surgery. POAF is associated with a longer hospital stay, higher healthcare resource utilization, and higher risk of morbidity and mortality. As a result, the American and European guidelines recommend the use of beta-blockers and amiodarone for the prevention of POAF, and in turn, avoid the complications associated with POAF. OBJECTIVES: The purpose of this study was to determine the incidence, risk factors, and prognostic implications of new-onset POAF after cardiac surgery in adults with congenital heart disease (CHD). METHODS: A retrospective study was conducted among adults with CHD who underwent cardiac surgery (2003-2019). POAF and late-onset atrial fibrillation (AF) were defined as AF occurring within and after 30 days postoperatively, respectively. RESULTS: Of 1,598 patients (mean age 39 ± 13 years, 51% men), 335 (21%) developed POAF. Risk factors associated with POAF were older age, hypertension, left atrial (LA) reservoir strain and right atrial (RA) dysfunction, and nonsystemic atrioventricular valve regurgitation. Of 1,291 patients (81%) with follow-up ≥12 months, the annual incidence of late-onset AF was 1.5% and was higher in patients with POAF compared with those without POAF (5.9% vs 0.4%; P < 0.001). Risk factors associated with late-onset AF were POAF, older age, severe CHD, and LA and RA dysfunction. Of the 1,291 patients, 63 (5%) died during follow-up, and the risk factors associated with all-cause mortality were older age, severe CHD, hypertension, left ventricular systolic dysfunction, and LA and RA dysfunction. POAF was not associated with all-cause mortality. CONCLUSIONS: POAF was common in adults with CHD and was associated with late-onset AF but not all-cause mortality. Atrial dysfunction was independently associated with POAF, late-onset AF, and all-cause mortality. These risk factors can be used to identify patients at risk for POAF and provide a foundation for prospective studies assessing the efficacy of prophylactic therapies in this population.

Estimation of Left Ventricular Filling Pressure Using Left Atrial Strain in Coarctation of Aorta.

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. The purpose of this study was to determine whether these LA strain cut-off points can detect increased LV filling pressure in adults with coarctation of aorta (COA). METHODS: This retrospective study included adults with COA (n = 126; age, 36 ± 16 years) who underwent non-simultaneous cardiac catheterization and echocardiography. Increased LV filling pressure was defined as pulmonary artery wedge pressure (PAWP) >12 mm Hg or LV end-diastolic pressure (LVEDP) >16 mm Hg. RESULTS: The median PAWP was 13 mm Hg (interquartile range [IQR], 11-18) and PAWP had a good correlation with LA reservoir strain (r = -0.69; P<.001) and LA booster strain (r = -0.61; P<.001). LA reservoir strain <18% had superior diagnostic power to detect PAWP >12 mm Hg as compared with LA volume index >34 mL/m², septal E/e' >15, lateral E/e' >13, and tricuspid regurgitation velocity >2.8 m/s (P<.05 for all). The median LVEDP was 17 mm Hg (IQR, 14-20) and LVEDP had a modest correlation with LA reservoir strain (r = -0.39; P<.001) and LA booster strain (r = -0.33; P<.01). LA reservoir strain <18% had superior diagnostic power to detect LVEDP >16 mm Hg as compared with LA volume index >34 mL/m², septal E/e' >15, lateral E/e' >13, and tricuspid regurgitation velocity >2.8 m/s (P<.05 for all). CONCLUSIONS: These data suggest that LA strain could potentially be used to identify patients with increased LV filling pressure, thereby improving patient selection for cardiac catheterization and interventions.

Determinants and Prognostic Implications of Hepatorenal Dysfunction in Adults With Congenital Heart Disease.

BACKGROUND: There are limited data on the prognostic role of hepatorenal function indices in ambulatory patients with congenital heart disease (CHD). The purpose of this study was to determine the prevalence, risk factors, and prognostic implications of hepatorenal dysfunction, as measured by Model for End-Stage Liver Disease Excluding International Normalised Ratio (MELD-XI) score, in adults with CHD. METHODS: In this retrospective study of CHD patients with comprehensive metabolic panels (2003-2019), mild/moderate and severe hepatorenal dysfunction was defined as MELD-XI 11-15 and > 15, respectively. RESULTS: Of 4977 patients, 1376 (28%) had hepatorenal dysfunction (mild/moderate: n = 935 [19%]; severe: n = 441 [9%]). Hepatorenal dysfunction was most common in Fontan/unrepaired single ventricle (46%) and right heart disease (31%). Baseline MELD-XI was associated with all-cause mortality (HR 1.27, CI 1.21-1.33; P < 0.001) after adjustment for age, sex, and congenital heart lesion. In 3864 patients with serial MELD-XI data, there was a temporal increase in MELD-XI, and this was associated with an increased risk of mortality (HR 1.24, CI 1.15-1.36, per unit increase in MELD-XI; P = 0.004), independently from the baseline MELD-XI score. In the subset of 1856 patients that underwent surgical/transcatheter interventions, there was a postoperative reduction in MELD-XI, and this was associated with a lower risk of mortality (HR 0.94, CI 0.90-0.98, per unit decrease in MELD-XI; P = 0.008), independently from the baseline MELD-XI score. CONCLUSIONS: Hepatorenal dysfunction was common in adults with CHD. Both baseline MELD-XI score and temporal changes in MELD-XI were associated with clinical outcomes, and therefore could be used to monitor therapeutic response to interventions and for deterioration in clinical status.

Improvement in hepatic and renal function following isolated heart transplant in adults with congenital heart disease.

BACKGROUND: There are limited data about postoperative changes in hepatic and renal function in adults with congenital heart disease (CHD) undergoing isolated heart transplant. The purpose of this study was to assess postoperative changes in hepatic and renal function in this population. METHODS: Retrospective cohort study of adults with CHD undergoing isolated heart transplant at Mayo Clinic (2003-2019). Global hepatic function was assessed using the model for end-stage liver disease excluding international normalized ratio [MELD-XI]) score; hepatic fibrosis was assessed using the fibrosis-4 (FIB-4) score and aspartate/platelet ratio index (APRI); and renal function was assessed using estimated glomerular filtration rate (GFR). All indices were measured preoperatively and postoperatively (at 6 months, 1 year, 2 years). RESULTS: Of 40 patients (age 41 ± 8 years) in the study, 35 had complete preoperative and postoperative data. There was a temporal improvement in hepatic and renal indices from preop (MELD-XI 14 ± 5, APRI 0.60 ± 0.23, FIB-4 1.44 ± 0.38, GFR 59 [44-83]) to 6 months postop (MELD-XI 12 ± 6, APRI 0.49 ± 0.17, FIB-4 1.29 ± 0.33, GFR 68 [54-96]) and 1-year postop (MELD-XI 9 ± 3, APRI 0.41 ± 0.16, FIB-4 1.12 ± 0.29, GFR 82 [69-108]), p < 0.05 for all comparisons. CONCLUSIONS: CHD patients undergoing isolated heart transplant had significant improvement in hepatic and renal function. These data suggests that selected CHD patients may do well with isolated heart transplant despite reduced hepatic and renal function, and hepatic fibrosis preoperatively. More rigorous prospective studies are required to determine the relative outcomes of isolated versus combined heart-liver transplant in this population.

Prevalence and risk of progressive aortic aneurysm and dissection in adults with conotruncal anomalies.

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.