Functional Capacity Assessment in Adults After Fontan Palliation: A Cardiopulmonary Exercise Test-Invasive Exercise Hemodynamics Correlation Study.

Although cardiopulmonary exercise testing (CPET) parameters have known prognostic value in adults after Fontan palliation, there are limited data correlating treadmill CPET with invasive exercise hemodynamics. Furthermore, the invasive hemodynamic underpinnings of exercise limitations have not been thoroughly investigated. This is retrospective analysis of 55 adults (≥18 years) after Fontan palliation who underwent treadmill CPET before invasive exercise hemodynamic testing by way of supine cycle protocol between November 2018 and April 2023. The median age was 32.2 (24.1 to 37.2) years. The peak heart rate (HR) was 139.7 ± 28.1 beats per minute and the peak oxygen consumption (VO2) was 19.1 ± 5.7 ml/kg/min (47.4 ± 13.5% predicted). VO2/HR was directly related to exercise stroke volume index (r = 0.50, p = 0.0002), whereas no association was seen with exercise arterio-mixed venous O2 content difference (r = 0.14, p = 0.32). Peak HR was inversely related to exercise pulmonary artery (PA) pressures (r = -0 61, p <0.0001) and PA wedge pressures (PAWP) (r = -0.61, p <0.0001). Moreover, %predicted VO2 was inversely related to exercise PA pressures (r = -0.50, p <0.0001) and PAWP (r = -0.55, p <0.0001). Peak VO2 ≤19.1 ml/kg/min had a sensitivity of 81% and a specificity of 76% (area under the curve = 0.82) for predicting a ΔPAWP/ΔQs ratio >2 mm Hg/L/min and/or a ΔPA:ΔQp >3 mm Hg/L/min, whereas a predicted peak VO2 ≤48% had a sensitivity of 74% and a specificity of 81% (area under the curve = 0.79) for the same parameters. In summary, lower peak HR and peak VO2 were associated with higher exercise PAWP and PA pressure. Peak VO2 ≤48% predicted provided the optimal cutoff for predicting increased indexed exercise PAWP or PA pressures; therefore, low peak VO2 should alert clinicians of abnormal underlying hemodynamics.

Fontan hemodynamics in adults with obesity compared to overweight and normal body mass index: a retrospective invasive exercise study.

AIMS: The effects of obesity on Fontan hemodynamics are poorly understood. Accordingly, we assessed its impact on exercise invasive hemodynamics and exercise capacity. METHODS: Seventy-seven adults post-Fontan undergoing exercise cardiac catheterization (supine cycle protocol) were retrospectively identified using an institutional database and categorized according to the presence of obesity (body mass index [BMI] >30 kg/m2) and overweight/normal BMI (BMI≤30 kg/m2). RESULTS: There were 18 individuals with obesity (BMI 36.4±3 kg/m2) and 59 (BMI 24.1±3.6 kg/m2) with overweight/normal BMI. Peak oxygen consumption (VO2) on noninvasive cardiopulmonary exercise testing was lower in patients with obesity (15.6±3.5 vs 19.6±5.8 ml/kg/min, p=0.04). At rest, systemic flow (Qs) (7.0 [4.8; 8.3] vs 4.8 [3.9; 5.8] l/min, p=0.001), pulmonary artery (PA) pressure (16.3±3.5 vs 13.1±3.5 mmHg, p=0.002), and PA wedge pressure (PAWP) (11.7±4.4 vs 8.9±3.1 mmHg, p=0.01) were higher, while arterial O2 saturation was lower (89.5% [86.5; 92.3] vs 93% [90; 95]) in obesity compared to overweight/normal BMI. Similarly, patients with obesity had higher exercise PA pressure (29.7±6.5 vs 24.7±6.8 mmHg, p=0.01) and PAWP (23.0±6.5 vs 19.8±7.3 mmHg, p=0.047), but lower arterial O2 saturation (82.4±7.0% vs 89% [85; 92], p=0.003). CONCLUSION: Adults post-Fontan with obesity have worse aerobic capacity, increased Qs, higher filling pressures, and decreased arterial O2 saturation compared to those with overweight/normal BMI, both at rest and during exercise, mirroring the findings observed in the obesity phenotype of heart failure with preserved ejection fraction. Whether treating obesity and its cardiometabolic sequelae in Fontan patients will improve hemodynamics and outcomes requires further study.

Seventy-seven adults post-Fontan undergoing exercise cardiac catheterization (supine cycle protocol) at Mayo Clinic, MN were categorized according to according to the presence of obesity (body mass index [BMI] >30 kg/m2) and overweight/normal BMI (BMI≤30 kg/m2). Adults post-Fontan with obesity have worse aerobic capacity, increased cardiac output, higher filling pressures, decreased arterial O2 saturation, and lower systemic vascular resistance compared to individuals with overweight or normal BMI, both at rest and during exercise.These findings mirror the observations in the obesity phenotype of heart failure with preserved ejection fraction. Whether treating obesity and its cardiometabolic sequelae in Fontan patients will improve hemodynamics and outcomes requires further study.

High-Gradient Aortic Stenosis With Valve Area >1.0 cm2: The "Forgotten" Discordant Hemodynamic Phenotype.

BACKGROUND: Clinical features and outcomes in severe aortic stenosis (AS) have been described according to the hemodynamic phenotypes. OBJECTIVES: The aim of this study was to investigate the clinical features and prognosis of patients with high-gradient (HG) AS with aortic valve area (AVA) >1.0 cm2. METHODS: A total of 3,209 patients were identified according to AVA (cm2), peak velocity (m/s), systolic mean pressure gradient (MG) (mm Hg): HG-AVA >1 = >1.0, ≥4, and ≥40, HG-AVA ≤1 = ≤1.0, ≥4, and ≥40; LG-AVA ≤1 (low-gradient) = ≤1.0, <4, and <40; moderate AS = 1.0 1 accounted for 230 individuals (7.2%). Compared with others, patients with HG-AVA >1 were younger (70.2 ± 12.0 years), more frequently male (85.7%), had fewer comorbidities, larger body surface area and stroke volume (115 ± 19.3 mL), and had higher prevalence of bicuspid valve (39.6%). After a follow-up of 944 days (Q1-Q3: 27-2,212 days), 1,523 deaths occurred. Compared with the HG-AVA >1 group, all-cause mortality was higher in HG-AVA ≤1 (HR: 1.4; 95% CI: 1.1-1.7), LG-AVA ≤1 (HR: 2.8; 95% CI: 2.2-3.6), and moderate AS (HR: 1.4; 95% CI: 1.1-1.7). These differences were no longer significant after adjustment for age, comorbidities, bicuspid valve, and cardiac function. In the HG-AVA >1 group, patients with aortic valve replacement had better survival outcomes than those without aortic valve replacement (P < 0.001) after balancing the 2 groups. CONCLUSIONS: The underlying relative high-flow status is responsible for HG in patients with HG-AVA >1. This profile has better prognosis than others, being related to underlying younger age and better general and cardiac conditions, but aortic valve replacement may still benefit these patients.

Multimodal Imaging in Mycobacterium Chimaera Cardiovascular Infections: The Mayo Clinic Experience.

Objective: To review the salient features of multimodality cardiovascular imaging in patients with disseminated Mycobacterium chimaera (MC) infections after exposure to contaminated heater-cooler units during cardiopulmonary bypass. Patients and Methods: Twelve patients with confirmed MC infection were retrospectively identified after a review from January 1, 2010, to April 30, 2021. The electronic medical records were examined with a focus on transthoracic echocardiography, transesophageal echocardiography, cardiac computed tomography (CT), cardiac magnetic resonance imaging, and positron emission tomography-CT. Results: Three (27.3%) patients had diagnostic findings of endocarditis on transthoracic echocardiography, with most patients having nonspecific abnormalities including elevated prosthetic valve gradients or prosthetic leaflet thickening. Transesophageal echocardiography identified 4 (36.7%) patients with vegetations and 3 (27.3%) with aortic root abscess or pseudoaneurysm, with more common findings such as mild aortic root or prosthetic leaflet thickening. Six (50%) patients underwent cardiac CT imaging, which found aortic root pseudoaneurysms or abscesses, prosthetic ring dehiscence, and leaflet thickening. Three (25%) patients underwent cardiac magnetic resonance imaging demonstrating prosthetic valve vegetations, leaflet thickening, and abnormal myocardial delayed enhancement in a noncoronary distribution, suggesting myocarditis. Ten (83%) patients underwent positron emission tomography-CT, 4 (40%) had an abnormal fluorodeoxyglucose uptake around the cardiac prosthetic material, and 7 (70%) had a fluorodeoxyglucose uptake in other organs, suggesting concomitant multiorgan involvement. Conclusion: Multimodality cardiovascular imaging is central to the management of patients with disseminated MC and can help establish a preliminary diagnosis while awaiting confirmatory microbiological data, potentially reducing the time to diagnosis. Imaging findings are subtle and atypical, not always meeting classically modified Duke's criteria for infectious endocarditis. Clinicians should have a high index of suspicion for the disease and a low threshold for repeat imaging when initial testing is equivocal.

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

Objectives: 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. Methods: 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. Results: 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. Conclusions: The abnormal hemodynamics and higher prevalence of HFpEF in COA patients with obesity underscores the need for intervention to address obesity in this population.

PA-VSD Without MAPCA(s): Review of Long-Term Outcomes and Reinterventions.

BACKGROUND: Pulmonary atresia with ventricular septal defect without major aortopulmonary collateral arteries (MAPCAs) is an uncommon form of congenital heart disease. As more patients with congenital heart disease live to adulthood, the objective of this article was to review the long-term results of this specific population. METHODS: A review of the PubMed database was performed using pertinent key words (pulmonary atresia, tetralogy of Fallot, conduit, right ventricle-pulmonary artery) concentrating on studies from 1990-present and published in English. Most studies of pulmonary atresia-ventricular septal defect patients included those with and without MAPCAs. Analysis included examination of the entire cohort, consideration of the proportion of patients with MAPCAs, and any subgroup analysis of the patients without MAPCAs. RESULTS: Survival is approximately 80% at ten years and is improved with complete repair and larger pulmonary arteries. Some studies have found genetic syndromes and extracardiac anomalies to impact survival, while others have not. Incomplete repair has been shown to be associated with worse survival. Independent of initial management strategy, patients with pulmonary atresia and ventricular septal defects without MAPCAs require repeat intervention on the right ventricular outflow tract. Hypoplastic pulmonary arteries have been shown to be a risk factor for reintervention, and decreased conduit durability has been shown with younger age at implantation of conduit. CONCLUSIONS: Long-term outcomes have improved for patients with pulmonary atresia-ventricular septal defect without MAPCAs, with complete repair and adequate pulmonary arteries favorable for survival. Long-term outcomes include reinterventions, both catheter-based and surgical, predominantly on the right ventricular outflow tract.

Cardiac remodelling during pregnancy in women with congenital heart disease and systemic left ventricle.

AIMS: Women with congenital heart disease (CHD) are at risk of pregnancy-related adverse outcomes (PRAO). The purpose of this study was to assess temporal changes in cardiac structure and function (cardiac remodelling) during pregnancy, and the association with PRAO in women with CHD. METHODS AND RESULTS: Retrospective study of pregnant women with CHD and serial echocardiograms (2003-2021). Cardiac structure and function were assessed at pre-specified time points: prepregnancy, early pregnancy, late pregnancy, and postnatal period. PRAO was defined as the composite of maternal cardiovascular, obstetric, and neonatal complications. The study comprised 81 women with CHD (age, 29 ± 5 years). Compared to the baseline echocardiogram, there was a relative increase in right ventricular systolic pressure (RVSP) (relative change 13 ± 5%, P < 0.001, in early pregnancy; and 18 ± 5%, P < 0.001, in late pregnancy). There was a relative decrease in right ventricle free wall strain (RVFWS) (relative change -11 ± 3%, P < 0.001, in late pregnancy; and -11 ± 4%, P = 0.003, in postnatal period), and a relative decrease in RVFWS/RVSP (relative change, -10 ± 5%, P = 0.02 in early pregnancy, -26 ± 7%, P < 0.001, in late pregnancy, and -14 ± 5%, P < 0.001, in postnatal period). Baseline right ventricular to pulmonary arterial (RV-PA) coupling, and temporal change in RV-PA coupling were associated with PRAO, after adjustment for maternal age and severity of cardiovascular disease. CONCLUSION: Women with CHD had a temporal decrease in RV systolic function and RV-PA coupling, and these changes were associated with PRAO. Further studies are required to delineate the aetiology of deterioration in RV-PA coupling during pregnancy, and the long-term implications of right heart dysfunction observed in the postnatal period.

Haemodynamic contributors to pulmonary hypertension across the spectrum of adult congenital heart disease.

AIMS: Adult congenital heart disease (ACHD) includes multiple disease states that predispose to pulmonary hypertension (PH). Haemodynamically, PH depends on abnormalities in three components: pulmonary blood flow (Qp), pulmonary vascular resistance (PVR) and pulmonary venous pressure (PVP). We sought to evaluate the prevalence and prognostic impact of individual haemodynamic abnormalities in ACHD. METHODS AND RESULTS: Retrospective study of ACHD patients undergoing cardiac catheterization at Mayo Clinic between 1999 and 2022 who were followed for the combined endpoint of death/heart transplantation. Among 1005 patients, 37% had mean pulmonary artery pressure (mPAP) ≥25 mmHg with more systemic ventricular disease, cyanotic disease and shunt lesions, highest N-terminal pro-B-type natriuretic peptide and worse right heart remodelling/dysfunction. Among those with biventricular circulation, elevated PVP, PVR and mPAP were associated with prognosis, but not increased Qp >8 L/min. However, risk of death/transplant increased for PVR only at ≥3 Wood units (hazard ratio [HR] 3.00, 95% confidence interval [CI] 2.17-4.15; p < 0.0001) and for mPAP only at ≥25 mmHg (HR 3.15, 95% CI 2.17-4.58; p < 0.0001), not at current recommended lower cutpoints. Combined abnormalities in PVP and PVR were associated with worst outcome (HR 5.20, 95% CI 3.55-7.63; p < 0.0001) with intermediate risk with either abnormality (HR 2.11, 95% CI 1.46-3.04; p < 0.0001). Findings were consistent across type of biventricular ACHD. Only with the Fontan (univentricular) circulation was a lower mPAP threshold (20 mmHg) associated with adverse outcomes. CONCLUSIONS: Elevation of mPAP ≥25 mmHg in ACHD with a biventricular circulation is prognostically important regardless of disease phenotype, but milder PH of 21-25 mmHg is not associated with adverse outcome unless associated with Fontan circulation. Elevation in PVP >15 mmHg and PVR ≥3 Wood units were each individually associated with mortality with combined abnormalities associated with greatest risk. Categorizing PH in ACHD by haemodynamic mechanism (PVR, PVP or Qp) allows meaningful prognostication, and may allow more unified study of targeted therapies across heterogeneous disease states in ACHD.

Outcomes of Esophageal Varices in Adults With Fontan Palliation and Liver Cirrhosis.

Background: The purpose of this study was to define the risk and outcomes of esophageal varices in adults with Fontan palliation and liver cirrhosis undergoing esophagogastroduodenoscopy (EGD). Method: The results of EGD, abdominal ultrasound, and liver biopsy, as well as clinic notes from the hepatologist, were reviewed to determine the diagnosis of cirrhosis and esophageal varices. The incidence of acute gastrointestinal bleeding complication was assessed among patients with esophageal varices using the time of EGD as the baseline. Results: Of 149 patients with Fontan palliation and liver cirrhosis, the prevalence of esophageal varices at baseline EGD was 34% (51 of 149). Of 98 patients without esophageal varices at baseline EGD, 27 (27%) underwent subsequent EGD, of whom 11 showed a new diagnosis of esophageal varices. The incidence of a new diagnosis of esophageal varices was 9% per year. Of 62 patients with esophageal varices, 9 (15%) had acute gastrointestinal bleeding complications during 45 (37-62) months of follow-up, yielding an incidence of 5% per year. Of the 9 patients, 8 underwent EGD and variceal banding during the hospitalization for bleeding and 1 patient died of septicaemia. Of the 8 patients who survived to hospital discharge, 2 patients were readmitted for esophageal bleeding within 12 months from the index hospitalization. Higher hepatic vein wedge pressure and hepatic vein pressure gradient were associated with esophageal varices and bleeding complications. Conclusions: In this selected sample of adults with Fontan palliation and liver cirrhosis, esophageal varices were relatively common, and patients with esophageal varices had risk of bleeding complications.

Contexte: Cette étude visait à déterminer le risque de varices œsophagiennes et leur issue clinique chez les adultes ayant fait l'objet d'une intervention de Fontan et présentant une cirrhose hépatique chez qui une œsophagogastroduodénoscopie (OGD) a été réalisée. Méthodologie: Les résultats de l'OGD, de l'échographie abdominale et de la biopsie du foie, ainsi que les notes cliniques de l'hépatologue ont été consultés pour établir les diagnostics de cirrhose et de varices œsophagiennes. L'incidence des complications hémorragiques gastro-intestinales aiguës a été évaluée chez les patients présentant des varices œsophagiennes en utilisant l'OGD initiale comme référence de départ. Résultats: Chez les 149 patients ayant fait l'objet d'une intervention de Fontan et présentant une cirrhose hépatique, la prévalence des varices œsophagiennes lors de l'OGD initiale était de 34 % (51/149). Parmi les 98 patients sans varices œsophagiennes lors de l'OGD initiale, 27 (27 %) ont subi une OGD ultérieure, et 11 d'entre eux ont alors reçu un diagnostic de varices œsophagiennes. Le taux d'incidence des nouveaux diagnostics de varices œsophagiennes était de 9 % par année. Sur les 62 patients présentant des varices œsophagiennes, 9 (15 %) ont subi des complications hémorragiques gastro-intestinales aiguës au cours d'une période de suivi de 45 (37 à 62) mois, ce qui correspond à un taux d'incidence de 5 % par année. Huit des 9 patients ont subi une OGD et une ligature des varices par bande élastique durant leur hospitalisation en raison des complications hémorragiques, et un patient est décédé des suites d'une septicémie. Deux des 8 patients en vie au moment du congé de l'hôpital ont été réhospitalisés pour une hémorragie de l'œsophage dans les 12 mois suivant la première hospitalisation. Une pression d'occlusion plus élevée de la veine hépatique ainsi qu'un plus grand gradient de pression de la veine hépatique ont été associés à la survenue de varices œsophagiennes et de complications hémorragiques. Conclusions: Dans cet échantillon d'adultes ayant fait l'objet d'une intervention de Fontan et présentant une cirrhose hépatique, la fréquence des varices œsophagiennes était relativement élevée, et les patients présentant des varices œsophagiennes étaient exposés à un risque de complications hémorragiques.

Adult Congenital Heart Disease Transplantation: Does Univentricle Physiology Impact Early Mortality?

BACKGROUND: With patients with congenital heart disease increasingly living into adulthood, there is a growing population of patients with adult congenital heart disease (ACHD) who have heart failure. Limited data exist on evaluating heart transplantation in this population. METHODS: A retrospective review was performed of patients with ACHD who underwent heart transplantation from November 1990 to January 2023. Kaplan-Meier, cumulative incidence accounting for competing risk of death, and subgroup analyses comparing those patients with biventricular (BiV) and univentricular (UniV) physiology were performed. Data are presented as median (interquartile range [IQR]) or counts (%). RESULTS: A total of 77 patients with a median age of 36 years (IQR, 27-45 years) were identified, including 57 (74%) BiV and 20 (26%) UniV patients. Preoperatively, UniV patients were more likely to have cirrhosis (9 of 20 [45.0%] vs 4 of 57 [7.0%]; P < .001) and protein losing enteropathy (4 of 20 [20.0%] vs 1 of 57 [1.8%]; P = .015). Multiorgan transplantation was performed in 23 patients (30%) and more frequently in UniV patients (10 [50%] vs 13 [23%]; P = .04). Operative mortality was 6.5%, 2 of 20 (10%) among UniV patients and 2 of 57 (4%) among BiV patients (P = .276). Median clinical follow-up was 6.0 years (IQR, 1.4-13.1 years). Survival tended to be lower among UniV patients compared with BiV patients, particularly within the first year (P = .09), but it was similar for survivors beyond 1 year. At 5 years, the incidence of rejection was 28% (IQR, 17%-38%) and that of coronary allograft vasculopathy was 16% (IQR, 7%-24%). CONCLUSIONS: Underlying liver disease and the need for heart-liver transplantation were significantly higher among UniV patients. Survival tended to be lower among UniV patients, particularly within the first year, but it was similar for survivors beyond 1 year.

Outcomes following multivalve reoperation in adults with congenital heart disease: A 30-year, single-center study.

OBJECTIVE: As patients with congenital heart disease increasingly live into adulthood, reoperative surgery is frequently required. Although half of these are valve-related procedures, little is known regarding early and late outcomes, and factors associated with adverse outcomes. METHODS: From 1993 to 2022, a total of 1960 adult patients with congenital heart disease underwent repeat median sternotomy at our institution. Of these, 502 patients (26%) underwent intervention on 2 or more valves and constituted the study cohort. RESULTS: The median age was 39 (27-51) years, and 275 patients (55%) were female. A second sternotomy was performed in 265 patients (53%), a third sternotomy was performed in 135 patients (27%), a fourth sternotomy was performed in 75 patients (15%), and a fifth or more sternotomy was performed in 27 patients (5%). Interventions were performed on 2 valves in 436 patients (87%), 3 valves in 62 patients (12%), and 4 valves in 4 patients (1%). The most common combinations were pulmonary and tricuspid in 241 patients (48%), followed by mitral and tricuspid in 85 patients (17%), aortic and pulmonary in 42 patients (8%), and aortic and mitral in 41 patients (8%). Early mortality was 4.2% overall and 2.7% for elective operations. Nonelective operations and congenital heart disease of major complexity were independently associated with early mortality. Median follow-up was 14 years. One, 5-, and 10-year survivals were 93.6%, 89.3%, and 79.5%, respectively. Factors independently associated with overall mortality were age, ventricular dysfunction, coronary artery disease, renal failure, double valve replacement, nonelective operations, and bypass time. CONCLUSIONS: Multiple valve interventions are common and confer low early mortality in the elective setting. Referral before ventricular dysfunction and in an elective setting optimizes outcomes.

Prognostic value of aortic valve calcification in non-severe aortic stenosis with preserved ejection fraction.

AIMS: Aortic valve calcification (AVC) is prognostic in patients with aortic stenosis (AS). We assessed the AVC prognostic value in non-severe AS patients. METHODS AND RESULTS: We conducted a retrospective study of 395 patients with non-severe AS, LVEF ≥ 50%. The Agatston method was used for CT AVC assessment. The log-rank test determined the best AVC cut-offs for survival under medical surveillance: 1185  arbitrary unit (AU) in men and 850 AU in women, lower than the established cut-offs for severe AS (2064 AU in men and 1274 AU in women). Patients were divided into 3 AVC groups based on these cut-offs: low (<1185 AU in men and <850 AU in women), sub-severe (1185-2064 AU in men and 850-1274 AU in women), and severe (>2064 AU in men and >1274 AU in women). Of 395 patients (mean age 73 ± 12 years, 60.5% men, aortic valve area 1.23 ± 0.30 cm2, mean pressure gradient 28 ± 8 mmHg), 218 underwent aortic valve intervention (AVI) and 158 deaths occurred during follow-up, 82 before AVI. Median survival time under medical surveillance was 2.1 (0.7-4.9) years. Compared with the low AVC group, both sub-severe and severe AVC groups had higher risk for all-cause death under medical surveillance after comprehensive adjustment including echocardiographic AS severity and coronary artery calcium score (all P ≤ 0.006); while mortality risk was similar between sub-severe and severe AVC groups (all P ≥ 0.2). This mortality risk pattern persisted in the overall survival analysis after adjustment for AVI. AVI was protective of all-cause death in the sub-severe and severe AVC (all P ≤ 0.01), but not in the low AVC groups. CONCLUSION: Sub-severe AVC is a robust risk stratification parameter in patients with non-severe AS and may inform AVI timing.

Sex Differences in Outcomes of Adults With Isolated Coarctation of the Aorta.

Background: Data are limited about the effect (or lack thereof) of sex on clinical outcomes in adults with coarctation of the aorta (COA). The purpose of this study was to compare atherosclerotic cardiovascular disease (ASCVD) risk profile, blood pressure (BP) data, echocardiographic indices, and mortality between men and women with COA. Methods: Retrospective study of adults with COA, and no associated left-sided obstructive lesions, who received care at Mayo Clinic (2003-2022). ASCVD risk profile was assessed as the prevalence of hypertension, hyperlipidemia, type 2 diabetes, obesity, smoking history, and coronary artery disease. A 24-hour BP monitor was used to assess daytime and nighttime BP and calculate nocturnal dipping. Results: Of 621 patients with isolated COA, 375 (60%) were men, and 246 (40%) were women. Women had similar ASCVD risk profile and daytime BP as men. However, women had less nocturnal dipping (7 ± 5 mm Hg vs 16 ± 7 mm Hg, P < 0.001), higher pulmonary artery mean pressure (23 mm Hg [interquartile range: 16-31] vs 20 mm Hg [interquartile range: 15-28], P = 0.04), and higher pulmonary vascular resistance index (3.41 ± 1.14 WU · m2 vs 3.02 ± 0.76 WU · m2, P = 0.006). Female sex was associated with all-cause mortality (adjusted hazard ratio 1.26, 95% confidence interval 1.04-1.94) and cardiovascular mortality (adjusted hazard ratio 1.38, 95% confidence interval 1.09-2.18). Conclusions: Women had a higher risk of both cardiovascular mortality and all-cause mortality compared to the risks in men. This difference may be related to the higher-than-expected ASCVD risk factors, abnormal nocturnal blood pressure, and pulmonary hypertension observed in women in this cohort. Further studies are required to identify optimal measures to address these risk factors.

Contexte: Il existe peu de données sur l'issue clinique en fonction du sexe chez les adultes présentant une coarctation de l'aorte (CoA). Le but de cette étude consistait donc à comparer le profil de risque de maladie cardiovasculaire athéroscléreuse (MCVAS), les données relatives à la pression artérielle (PA), les indices échocardiographiques et le taux de mortalité chez des hommes et des femmes présentant une CoA. Méthodologie: Il s'agissait d'une étude rétrospective réalisée chez des adultes présentant une CoA en l'absence de lésions obstructives gauches, soignés à la clinique Mayo entre 2003 et 2022. Le profil de risque de MCVAS a été évalué en fonction de la prévalence de l'hypertension, de l'hyperlipidémie, du diabète de type 2, de l'obésité, des antécédents tabagiques et de la coronaropathie. Une surveillance sur 24 heures a été utilisée pour évaluer la PA diurne et nocturne, en plus de calculer la chute nocturne de la PA. Résultats: Parmi les 621 patients présentant une CoA isolée, 375 (60 %) étaient des hommes et 246 (40 %) étaient des femmes. Les femmes présentaient une PA diurne et un profil de risque de MCVAS semblables aux hommes. Elles présentaient néanmoins une chute nocturne de la PA moins prononcée (7 ± 5 mmHg vs 16 ± 7 mmHg, p < 0,001), une pression artérielle pulmonaire moyenne plus haute (23 mmHg [max.-min. : 16-31] vs 20 mmHg [max.-min. : 15-28], p = 0,04) et un indice de résistance vasculaire pulmonaire plus élevé (3,41 ± 1,14 UW · m2 vs 3,02 ± 0,76 UW · m2, p = 0,006). Le sexe féminin a été associé à un plus fort taux de mortalité toutes causes confondues (rapport de risques ajusté : 1,26; intervalle de confiance à 95 % : 1,04-1,94) et de mortalité cardiovasculaire (rapport de risques ajusté : 1,38; intervalle de confiance à 95 % : 1,09-2,18). Conclusions: Les femmes sont exposées à un risque de mortalité cardiovasculaire et de mortalité toutes causes confondues plus élevé que les hommes. Cette différence pourrait être attribuable au rôle plus important que prévu joué par les facteurs de risque de MCVAS ainsi qu'à la pression artérielle nocturne anormale et à l'hypertension pulmonaire chez les femmes de cette cohorte. D'autres études sont nécessaires pour savoir quels seraient les paramètres optimaux qui permettraient d'évaluer ces facteurs de risque.

Atrial and Ventricular Arrhythmia in Adults With Shone Complex: A Single-Center Cohort Study.

Background: Multilevel obstruction in left ventricular inflow and outflow predisposes to arrhythmias in Shone's complex (SC). Objectives: The purpose of this study was to study the prevalence and outcomes (heart failure [HF] hospitalization, cardiac transplant, death) of cardiac arrhythmias in adults with SC. Methods: Adults with SC (defined as ≥2 lesions out of supramitral ring, parachute mitral valve, subvalvular/valvular aortic stenosis (AS), and aortic coarctation) seen at Mayo Clinic between January 1999 and March 2020 were identified and evaluated for the presence of sustained atrial fibrillation, atrial flutter, and ventricular arrhythmias (VA). Kaplan-Meier survival analysis was used to calculate the occurrence of these arrhythmias. Results: Seventy-three patients with SC (mean age at first visit 33 ± 13 years) were identified. Most common anomalies were valvular AS (88%), coarctation (85%), parachute mitral valve (44%), subvalvular AS (44%), and supramitral ring (25%). Atrial arrhythmias were diagnosed in 24 patients (33%) at a mean age of 34.6 ± 12.7 years. Patients with atrial fibrillation and atrial flutter had higher number of surgeries, left atrial size, right ventricular systolic pressure, and HF hospitalizations. A rhythm control approach was used in majority of patients (75% on antiarrhythmic drugs and 50% underwent catheter ablation). Sustained VA occurred in 6 of 73 patients of whom 4 had an ejection fraction <40%. Death and cardiac transplantation occurred in 11 and 3 patients, respectively, during a median follow-up of 7.3 ± 6.0 years. Conclusions: In adults with SC, atrial arrhythmias occurred in one-third of patients, were associated with more HF hospitalizations, and frequently required rhythm control. Prevalence of sustained VA was 8% and implantable cardioverter-defibrillator implantation should be considered in those with reduced ejection fraction.