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Patients with pulmonary atresia and intact ventricular septum (PA-IVS) require intervention early in life, and most survive to a definitive procedure of either Fontan circulation or right ventricle to pulmonary artery (RV-PA) repair. It remains unknown how surgical strategy impacts hemodynamics and comorbidities in adults. Retrospective analysis of adults (age ≥18 years) with PA-IVS undergoing hemodynamic catheterization at Mayo Clinic, MN between January 2000 through January 2020 was performed. Fourteen patients in the RV-PA group (71% biventricular, 29% 1.5 ventricle repair) and 19 post-Fontan patients [9 lateral tunnel (48%), 6 atriopulmonary (32%), and 4 extracardiac (21%)] were identified. Median age was 29 (21, 34) years. There were no differences in demographics and laboratory data (including MELD-XI) between groups. All patients assessed for liver disease had evidence of hepatic congestion or cirrhosis (14 in the Fontan group and 4 in the RV-PA group). Invasive hemodynamics were comparable between groups with the Fontan and RV-PA groups having similar systemic venous pressure (15.7±4.4 vs. 14.3±6.2, p=0.44) and cardiac output (2.2±0.6 vs. 2.0±0.4 L/min/m2, p=0.23). There was no difference in transplant-free survival (p=0.92; 5-year transplant-free survival RV-PA 84%, Fontan 80%). Hemodynamic derangements, namely elevated systemic venous pressure and low cardiac output, are prevalent in patients with PA-IVS undergoing cardiac catheterization regardless of surgical strategy.

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

Fontan palliation represents one of the most remarkable surgical advances in the management of individuals born with functionally univentricular physiology. The operation secures adult survival for all but a few with unfavourable anatomy and/or physiology. Inherent to the physiology is passive transpulmonary blood flow, which produces a vulnerability to adequate filling of the systemic ventricle at rest and during exertion. Similarly, the upstream effects of passive flow in the lungs are venous congestion and venous hypertension, especially marked during physical activity. The pulmonary vascular bed has emerged as a defining character on the stage of Fontan circulatory behaviour and clinical outcomes. Its pharmacologic regulation and anatomic rehabilitation therefore seem important strategic therapeutic targets. This review seeks to delineate the important aspects of pulmonary artery development and maturation in functionally univentricular physiology patients, pulmonary artery biology, pulmonary vascular reserve with exercise, and pulmonary artery morphologic and pharmacologic rehabilitation.

Background The professional guidelines assume that the myocardial volume in systole (MVs) is equal to that in diastole (MVd), despite some limited evidence that points to the contrary. The aim of this manuscript is to determine whether this is true in healthy myocardium using gold standard cardiac MRI, as well as transthoracic echocardiography (TTE). The secondary aim is to determine whether there are similar MV changes in patients with heart failure with reduced ejection fraction (HFrEF). Method A prospectively derived cohort at Mayo Clinic of 115 adult subjects (mean age 42.8 years, 58% female) with no cardiac risk factors was identified. Cardiac MRI was obtained on all 115 patients, 51 of whom also consented to a TTE. MRI from a retrospectively derived cohort of 50 HFrEF patients was also collected. MVs and MVd was calculated using standard approaches with inclusion of the papillary muscles. Results In the healthy population, MRI demonstrated MVs/MVd = 0.87 (SD 0.04) and TTE demonstrated MVs/MVd = 0.79 (SD 0.07), suggesting compressibility (p Conclusion Contrary to currently accepted standards, healthy myocardium is compressible but HFrEF myocardium is incompressible. The ratio MVs/MVd merits further study in an expanded normal cohort and in disease states.

In patients with normal left ventricular ejection fraction, it may be difficult to distinguish between the normal and diseased heart. Novel assessments of ventricular function, such as extracellular volume imaging, myocardial perfusion imaging and myocardial contraction fraction are emerging to better assess disease burden in these cases. This study endeavored to determine whether the ratio of myocardial volume in systole to myocardial volume in diastole (MVs/MVd), differs between normal hearts and those with disease states characterized by normal ejection fraction.Consecutive patients from 2008 to 2018 with hypertrophic cardiomyopathy (HCM), cardiac amyloidosis, and heart failure with preserved ejection fraction (HFpEF) who underwent cardiac magnetic resonance imaging (MRI) were selected for inclusion, along with a sex- and age-matched cohort of normal volunteers who also underwent cardiac MRI. Manual tracings were performed on each MRI to calculate MVs/MVd, which was then compared across subgroups.Included were 50 patients with HCM, 50 patients with cardiac amyloidosis, 26 patients with HFpEF, and 30 normal subjects. Age was 54.1 years (SD 16.7); mean MVs/MVd was 0.88 (SD 0.04) in the normal subgroup, 1.03 (SD 0.06) in HCM patients, 1.03 (SD 0.06) in cardiac amyloidosis patients, and 0.97 (SD 0.02) in HFpEF patients, with all pathology subgroups different from the normal subgroup (p .0001 for each). The ratio of MVs/MVd discriminated diseased from normal with c statistic 0.989 (p .001).This study suggests that a novel and easily-captured metric of ventricular function, MVs/MVd, can differentiate normal ventricular function from multiple cardiomyopathies with normal ejection fractions.

Pulmonary vascular disease, pulmonary endothelial dysfunction, liver fibrosis, renal disease, and exercise intolerance are common in adults with Fontan physiology. Although the pathophysiologic mechanisms linking these phenomena have been studied, certain aspects are not well understood.This study hypothesized that impaired pulmonary vascular reserve (VR) plays a central role linking these abnormalities, and that patients with abnormal pulmonary VR with exercise, compared with patients with normal VR, would display poorer pulmonary endothelial function, greater liver stiffness, more renal dysfunction, and poorer exercise capacity.Symptomatic adults with the Fontan palliation (n = 29) underwent invasive cardiopulmonary exercise testing, echocardiography, and assessment of microvascular function. Abnormal pulmonary VR was defined by the slope of increase in pulmonary pressure relative to cardiac output with exercise3 mm Hg/l/min. Pulmonary endothelial function was assessed using reactive hyperemia index. End-organ function was assessed using magnetic resonance elastography-derived liver stiffness, glomerular filtration rate, N-terminal pro-B-type natriuretic peptide, and peak oxygen consumption (VoCompared with individuals with normal VR (n = 8), those with abnormal VR (n = 21) displayed higher central and pulmonary venous pressures, and more severely impaired cardiac output and stroke volume responses to exertion, but similar pulmonary vascular resistance at rest. Patients with abnormal VR displayed more severely impaired reactive hyperemia index, increased liver stiffness, lower glomerular filtration rate, higher N-terminal pro-B-type natriuretic peptide, and lower peak VoPulmonary vascular limitations in Fontan physiology are related to pulmonary endothelial and end-organ dysfunction, suggesting a mechanistic link between these commonly observed findings, and these abnormalities are more apparent during exercise testing, with little relationship at rest.

Background: Hepatic venous pressure gradient (HVPG) is measure of portal pressure and a prognostic tool in patients with viral and alcoholic cirrhosis; its utility is unknown in patients with Fontan-associated liver disease (FALD). Limited data suggest that patients with FALD have normal HVPG. On the basis of the available data, we hypothesized that there would be no association between HVPG, liver disease severity, and transplant-free survival in FALD. Methods: A retrospective study of Fontan patients who had liver biopsy and HVPG assessment at Mayo Clinic was performed. HVPG was calculated as wedged HVP minus free HVP; liver disease severity was measured by histologic assessment of fibrosis and standard clinical liver disease risk scores. Results: Of 56 patients (aged 28 ± 7 years), the mean Fontan pressure was 16 ± 4 and the mean HVPG was 1.4 ± 0.3 mm Hg (range, 0-3). Perisinusoidal fibrosis and periportal fibrosis were present in 56 (100%) and 54 (94%) patients, respectively; 18 (32%) met criteria for cirrhosis. There was no correlation between HVPG and degree of hepatic fibrosis. Similarly, there was no correlation between HVPG and any clinical liver disease risk score. Six (11%) patients died and 2 (4%) underwent heart transplantation during follow-up; HVPG was not associated with transplant-free survival. Conclusions: HVPG is not elevated in FALD even in the setting of cirrhosis and does not correlate with liver disease severity or clinical outcomes. These results suggest the limited diagnostic and prognostic role of HVPG in the management of FALD and highlight the potential pitfalls of using HVPG in this population. Résumé: Contexte: Le gradient de pression veineuse hépatique (GPVH) est une mesure de la pression portale et un paramètre permettant d’établir un pronostic chez les patients atteints de cirrhose virale ou alcoolique; on ne connaît toutefois pas son utilité en cas d’hépatopathie associée à une intervention de Fontan (FALD, pour Fontan-associated liver disease). Des données limitées semblent indiquer que les patients atteints d’une FALD ont un GPVH normal. En nous fondant sur les données disponibles, nous avons formulé l’hypothèse qu’il n’y a pas de lien entre le GPVH, la gravité de l’hépatopathie et la survie sans transplantation chez les patients atteints d’une FALD. Méthodologie: Nous avons étudié rétrospectivement les dossiers de patients de la clinique Mayo ayant subi une intervention de Fontan ainsi qu’une biopsie hépatique et une évaluation du GPVH. Le GPVH a été obtenu en soustrayant la pression veineuse hépatique libre de la pression veineuse hépatique bloquée, tandis que la gravité de l’hépatopathie a été mesurée à partir d’une évaluation histologique de la fibrose et de scores de risque cliniques d’hépatopathie couramment utilisés. Résultats: Chez les 56 patients de l’étude (âge moyen : 28 ± 7 ans), la pression dans le circuit de Fontan était de 16 ± 4 en moyenne et le GPVH, de 1,4 ± 0,3 mmHg (plage : 0 à 3) en moyenne. Une fibrose périsinusoïdale et une fibrose périportale étaient présentes chez 56 (100 %) et 54 (94 %) patients, respectivement; 18 (32 %) des patients répondaient aux critères diagnostiques d’une cirrhose. Il n’y avait pas de corrélation entre le GPVH et le degré de fibrose hépatique, ni entre le GPVH et aucun des scores de risque cliniques d’hépatopathie. Six (11 %) patients sont décédés et 2 (4 %) patients ont subi une transplantation cardiaque durant le suivi; aucun lien n’a été établi entre le GPVH et la survie sans transplantation. Conclusions: Le GPVH n’est pas élevé chez les patients présentant une FALD même en cas de cirrhose, et il n’est pas corrélé avec la gravité de l’hépatopathie ni avec les résultats cliniques. Ces résultats semblent indiquer que l’utilité diagnostique et pronostique du GPVH dans la prise en charge de la FALD est limitée, et fait ressortir les écueils auxquels on pourrait se heurter en utilisant ce paramètre dans cette population de patients.

Patients with Shone complex (SC) have multiple left-sided obstructive lesions and thus are at risk for left ventricular (LV) remodeling, LV diastolic dysfunction and pulmonary hypertension. Yet, to date, there has been no description of hemodynamics in adults with SC. Retrospective chart review of 25 patients with SC who underwent cardiac catheterization at Mayo Clinic, MN between 2002 and 2019 was performed. SC was defined as multiple left-sided obstructive lesions in the presence of an anatomically abnormal mitral valve. Median age was 32 years (22.5, 42) and 15 patients (60%) were female. The majority of patients (84%) had history of coarctation of the aorta, 10 (40%) had subaortic stenosis, 11 (44%) had prior aortic valve replacement, and 10 (40%) had prior mitral valve replacement. Structural disease at the time of catheterization which warranted intervention within the next year was present in 13 patients (52%). The mean LV end-diastolic pressure was 21.3 ± 9.0 mm Hg (>15 mm Hg in 71%), pulmonary artery peak systolic pressure was 55.4 ± 13.4 mm Hg, and the pulmonary artery mean pressure was 37.0 ± 9.4 mm Hg (>20 mm Hg in 96%). During a mean follow-up of 8.3 ± 4.4 years, there were 7 deaths (28%) and 3 additional patients (12%) underwent cardiac transplantation. In conclusion, adults with SC who underwent catheterization showed significant left-sided heart and pulmonary vascular remodeling. Elevated LV end-diastolic pressure and pulmonary artery pressures were highly prevalent. There were high mortality and cardiac transplant rates in our cohort.

BACKGROUND: Transcatheter stent therapy is as effective as surgery in producing acute hemodynamic improvement in patients with coarctation of aorta (COA). However, left ventricular (LV) remodeling after transcatheter COA intervention has not been systematically investigated. The purpose of this retrospective cohort study was to compare remodeling of LV structure and function after transcatheter stent therapy vs surgical therapy for COA. METHODS: LV remodeling was assessed at 1, 3 and 5-years post-intervention using: LV mass index (LVMI), LV end-diastolic dimension, LV ejection fraction, LV global longitudinal strain (LVGLS), LV e’ and E/e’. RESULTS: There were 44 and 128 patients in the transcatheter and surgical groups respectively. Compared to the surgical group, the transcatheter group had less regression of LVMI (−4.6[95%CI −5.5 - −3.7] vs −7.3[95%CI −8.4 - −6.6] g/m(2), p

Aims Right atrial pressure (RAP) provides a composite measure of right ventricular diastolic dysfunction, right atrial compliance, and volume status, and these three variables are typically abnormal in adults with repaired tetralogy of Fallot (TOF). RAP is a well‐established prognostic metric in patients with pulmonary hypertension, and recent data suggest that RAP is associated with clinical outcomes in TOF. The purpose of this study was to determine the role of inferior vena cava (IVC) haemodynamics (size and collapsibility) for the assessment of RAP and its potential application for risk stratification and prognostication in the TOF population. Methods and results Adult TOF patients with echocardiographic assessment of IVC haemodynamics were divided into patients with (derivation cohort, n = 256) and without (validation cohort, n = 492) cardiac catheterization data. We assessed the correlation between IVC haemodynamics, RAP, and disease severity indices [arrhythmias, peak oxygen consumption (VO2), and heart failure hospitalization] in derivation cohort and compared it with the correlations in the validation cohort. IVC haemodynamics correlated with RAP (r = 0.52, P

Background: Inferior vena cava (IVC) size and collapsibility provide a noninvasive estimate of right heart filling pressures, an important determinant of right heart hemodynamic performance that is not measured by cardiac magnetic resonance imaging (CMRI). We hypothesized that compared with CMRI risk model alone, a combined CMRI-IVC risk model will have better correlation with disease severity and peak oxygen consumption in patients with tetralogy of Fallot (TOF). Methods: We performed a retrospective review of patients with TOF with moderate/severe pulmonary regurgitation who underwent CMRI and echocardiography. A CMRI risk model was constructed using right ventricular (RV) end-diastolic volume index, RV end-systolic volume index, RV ejection fraction, and left ventricular ejection fraction. We added IVC hemodynamic classification to the CMRI indices to create CMRI-IVC risk model, and IVC hemodynamics were modeled as a categorical variable: normal vs mild/moderately abnormal (dilated IVC or reduced collapsibility) vs severely abnormal IVC hemodynamics (dilated IVC and reduced collapsibility). We defined disease severity as atrial arrhythmias, ventricular arrhythmias, and heart failure hospitalization. Results: Of 207 patients, 131 (63%), 72 (35%), and 4 (2%) had normal, mild/moderately abnormal, and severely abnormal IVC hemodynamics, respectively. Compared with the CMRI risk model, the CMRI-IVC risk model had a better correlation with disease severity (area under the curve, 0.62; 95% confidence interval, 0.51-0.74 vs area under the curve 0.84, 95% confidence interval, 0.78-0.91, P = 0.006) and peak oxygen consumption (r = 0.35, P = 0.042 vs r = 0.43, P = 0.031, Meng test P = 0.026). Conclusions: The combined CMRI-IVC risk model had a better correlation with disease severity compared with CMRI indices alone and can potentially improve risk stratification in the population with TOF. Résumé: Contexte: Le diamètre et la collapsibilité de la veine cave inférieure (VCI) permettent d’estimer de façon non invasive les pressions de remplissage du cœur droit, un déterminant important de la capacité hémodynamique cardiaque droite que ne mesure pas l’imagerie par résonance magnétique cardiaque (IRMC). Notre hypothèse était que, comparativement au modèle de risque IRMC seul, un modèle de risque combiné IRMC-VCI présenterait une meilleure corrélation avec la gravité de la maladie et la consommation maximale d’oxygène chez les patients atteints de la tétralogie de Fallot (TF). Méthodologie: Nous avons effectué une étude rétrospective de cas de TF avec régurgitation pulmonaire modérée ou sévère où les patients ont subi un examen d’IRMC et une échocardiographie. Nous avons créé un modèle de risque IRMC intégrant l’indice du volume télédiastolique ventriculaire droit, l’indice du volume télésystolique ventriculaire droit, la fraction d’éjection ventriculaire droite et la fraction d’éjection ventriculaire gauche. Nous avons ajouté une classification hémodynamique de la VCI aux indices d’IRMC pour créer le modèle de risque IRMC-VCI, et les caractéristiques hémodynamiques de la VCI ont été modélisées en tant que variable nominale : état normal vs anomalie légère ou modérée (VCI dilatée ou collapsibilité réduite) vs anomalie sévère des caractéristiques hémodynamiques de la VCI (VCI dilatée et collapsibilité réduite). Nous avons défini la gravité de la maladie en distinguant les arythmies auriculaires, les arythmies ventriculaires et l’insuffisance cardiaque entraînant une hospitalisation. Résultats: Au sein d’un groupe de 207 patients, les caractéristiques hémodynamiques de la VCI présentaient un état normal, une anomalie légère ou modérée et une anomalie sévère dans 131 cas (63 %), 72 cas (35 %) et 4 cas (2 %), respectivement. Comparativement au modèle de risque IRMC, le modèle de risque IRMC-VCI a présenté une meilleure corrélation avec la gravité de la maladie (aire sous la courbe = 0,62 et intervalle de confiance à 95 % = 0,51-0,74 vs aire sous la courbe = 0,84 et intervalle de confiance à 95 % = 0,78-0,91, p = 0,006) et avec la consommation maximale d’oxygène (r = 0,35, p = 0,042 vs r = 0,43, p = 0,031, p = 0,026 pour le test de Meng). Conclusions: Le modèle de risque combiné IRMC-VCI a présenté une meilleure corrélation avec la gravité de la maladie comparativement aux indices d’IRMC seuls. Il pourrait améliorer la stratification du risque au sein de la population atteinte de la TF.

Background Cardiovascular disease is the leading cause of death in patients with Turner syndrome (TS), and cardiovascular surgery is frequently required for management of these patients. TS is associated with medical comorbidities than can complicate the care of this patient population. Aim To describe the cardiovascular surgical outcomes of patients with TS. Methods A retrospective case series was compiled of 51 consecutive TS patients who had at least one cardiovascular surgery at Mayo Clinic Rochester from 1977-2017. The baseline clinical data of these patients were reviewed including demographics, medical comorbidities, congenital heart disease history, and medications. Echocardiographic reports were analyzed in detail. Operative reports and surgical hospital courses were reviewed. Long-term mortality was determined using medical records and the Social Security Death Index. Survival analysis was performed with the Kaplan Meier method. Results The cohort comprised 51 TS patients, average age at the time of surgery at Mayo Clinic was 28 (8-41) years, and 23 (45%) patients were under the age of 18. At the time of first Mayo Clinic surgery, 18 (35%) patients had previously undergone cardiac surgery at another institution. The most common procedures were repair of aortic coarctation in 14 (28%) patients, aortic valve replacement in 6 (12%) patients, and composite aortic root/ascending aorta replacement in 7 (14%) patients, with 7 patients undergoing repair of more than one lesion. Aortic dissection required operative intervention in 5 patients. After initial Mayo Clinic surgery, subsequent operations were required in 6 (13%) patients. Average hospital length of stay was 6 ± 2 d. There were 4 (8%) early surgical deaths. Freedom from death was 97% and 89% at 10 and 20 years, and the freedom from reoperation was 93% and 81% at 10 and 20 years. Conclusion Cardiovascular surgery is associated with 8% early mortality given the medical complexity of TS patients. Those who survive to dismissal have good survival. Later cardiovascular reoperations are not rare.

Pulmonary valve replacement (PVR) is associated with improvement in symptoms and right ventricular remodeling in patients with tetralogy of Fallot (TOF). There are limited population-based data about outcomes after PVR. We therefore hypothesized a temporal increase in annual volume of PVR and decrease in in-hospital mortality after PVR in the United States.We reviewed the National Inpatient Sample (NIS) for PVR performed in adults (18 years) with TOF, 2000-2014. The primary outcome was trends in admissions for PVR, in-hospital mortality after PVR, and age at time of PVR. In order to assess trends, we divided the study period into tertiles: early era (2000-2004), mid era (2005-2009) and late era (2010-2014).There were 18,353 admissions in adults with TOF diagnosis, of which PVR was performed in 1230 (6.7%), and 90 (7.3%) were transcatheter PVRs. The median age at PVR was 34 years and in-hospital mortality was 1.5%. Comparisons by study era showed temporal increase in the proportion of admissions for PVR (3.7% vs 6.3% vs 9.6%, p 0.001), decrease in in-hospital mortality (4.1% vs 1.2% vs 0.8%, p = 0.002), and a decrease in age at the time of PVR (35.8 vs 33.8 vs 31.0 years, p 0.001).The proportion of admissions for PVR increased while in-hospital mortality and age at time of PVR decreased over time. A younger age at the time of PVR highlights important concerns and knowledge gaps about the cumulative lifetime risk of reinterventions and prosthetic valve endocarditis. Further studies are required to address these knowledge gaps.

Background:The risk of endocarditis varies with CHD complexity and the presence of prosthetic valves. The purpose of the study was therefore to describe incidence and outcomes of prosthetic valve endocarditis in adults with repair tetralogy of Fallot.Methods:Retrospective review of adult tetralogy of Fallot patients who underwent prosthetic valve implantation, 1990–2017. We defined prosthetic valve endocarditis-related complications as prosthetic valve dysfunction, perivalvular extension of infection such abscess/aneurysm/fistula, heart block, pulmonary/systemic embolic events, recurrent endocarditis, and death due to sepsis.Results:A total of 338 patients (age: 37 ± 15 years) received 352 prosthetic valves (pulmonary [n = 308, 88%], tricuspid [n = 13, 4%], mitral [n = 9, 3%], and aortic position [n = 22, 6%]). The annual incidence of prosthetic valve endocarditis was 0.4%. There were 12 prosthetic valve endocarditis-related complications in six patients, and these complications were prosthetic valve dysfunction (n = 4), systemic/pulmonary embolic events (n = 2), heart block (n = 1), aortic root abscess (n = 1), recurrent endocarditis (n = 2), and death due to sepsis (n = 1). Three (50%) patients required surgery at 2 days, 6 weeks, and 23 weeks from the time of prosthetic valve endocarditis diagnosis. Altogether three of the six (50%) patients died, and one of these deaths was due to sepsis.Conclusions:The incidence, complication rate, and outcomes of prosthetic valve endocarditis in tetralogy of Fallot patients underscore some of the risks of having a prosthetic valve. It is important to educate the patients on the need for early presentation if they develop systemic symptoms, have a high index of suspicion for prosthetic valve endocarditis, and adopt a multi-disciplinary care approach in this high-risk population.

Patients with tetralogy of Fallot (TOF) have abnormal right ventricular (RV) afterload because of residual or recurrent outflow tract obstruction, often with abnormal pulmonary artery (PA) vascular function. The purpose of this study was to determine if RV afterload was independently associated with death and/or heart transplant in patients with TOF. This is a retrospective study of TOF patients that underwent cardiac catheterization for clinical indications at Mayo clinic between 1990 and 2015. Invasively measured RV systolic pressure (RVSP) was used to define RV afterload. To explore clinical utility for echocardiographic estimates of invasive data, correlations between invasive and Doppler-derived indices of RV afterload were examined. Among 266 patients with TOF (age 35 ± 14 years, TOF-pulmonary atresia 117 [44%]), RVSP was 72 ± 28 mm Hg, PA systolic pressure 45 ± 19 mm Hg, mean PA pressure 27 ± 10 mm Hg, pulmonary vascular resistance 4.2 ± 3.1 WU, and PA wedge pressure 14 ± 5 mm Hg. Over a mean follow up of 12.9 years, there were 35 deaths and 4 heart transplants. Invasively measured RVSP (hazard ratio 1.25, 95% confidence interval 1.12 to 1.37; p0.001) and TOF-pulmonary atresia (hazard ratio 1.18, 95% confidence interval 1.08 to 1.41; p = 0.023) were independent risk factors for death and/or transplant. Doppler-derived RVSP was well-correlated with invasive RVSP (r = 0.92, p0.001), and was also independently associated with the combined end point. RVSP, a composite measure of RV afterload, is independently prognostic in patients with TOF, and can be reliably assessed using Doppler echocardiography. Further study is required to test whether interventions to reduce RVSP can improve outcomes in patients with TOF.

Background Atrial arrhythmia is a late complication after tetralogy of Fallot (TOF) repair, but arrhythmia outcomes data are limited. Objectives The purpose of the study was to describe atrial arrhythmia presentations, outcomes of antiarrhythmic therapy, and impact of arrhythmia on transplant-free survival. Methods We reviewed the MACHD (Mayo Adult Congenital Heart Disease) Registry and identified 113 patients (age 49 ± 13 years) with documented arrhythmia, and 302 patients without history of arrhythmia, 1990-2017. We classified arrhythmias into atrial fibrillation and atrial flutter/tachycardia based on the rhythm on the first abnormal electrocardiogram. Results At the time of first documented arrhythmia, 58(51%) had atrial fibrillation while 55(49%) had atrial flutter/tachycardia. Of the 113 patients, 14(12%) received rhythm control with class I/III antiarrhythmic drugs (AAD), 79(70%) had direct current cardioversion, 9(8%) received rate control with class II/IV AAD, and 11(10%) received only anticoagulation. Successful cardioversion occurred in 100(89%) patients, and arrhythmia recurrence rate was 16 per 100 patient-years. The multivariate risk factors for death and/or heart transplant were atrial fibrillation (HR 1.94, CI 1.10-3.15, P = .031) and older age (HR 1.63, CI 1.12-2.43, P = .019) per 5 year increment. Conclusions Atrial fibrillation, but not atrial flutter, was associated with reduced survival in our repaired TOF cohort. Further studies are required to determine if more aggressive antiarrhythmic therapy will improve survival in patients with atrial fibrillation.

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 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.

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 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 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 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.

Background Patients with Ebstein anomaly have impaired aerobic capacity, but the relationship between aerobic capacity and clinical outcomes are unknown. The purpose of this study was to determine the prognostic role of aerobic capacity and the hemodynamic mechanism underlying temporal deterioration in aerobic capacity in this population. Methods Retrospective cohort study of adults with Ebstein anomaly that underwent exercise test (2000–2018). Primary objective was to determine the relationship between aerobic capacity (%-predicted peak oxygen consumption, VO2) and cardiovascular adverse events (death, heart transplant, aborted sudden death, and heart failure hospitalization). Secondary objective was to delineate the relationship between temporal change in hemodynamic indices and subsequent decline in peak VO2. Results We studied 248 patients (peak VO2 22.4 ​± ​7.1 ​ml/kg/min; 64 ​± ​21 %-predicted), and these events occurred during follow-up (death n ​= ​12, transplant n ​= ​1, aborted sudden death n ​= ​7, and heart failure hospitalization n ​= ​11). Peak VO2 was an independent predictor of cardiovascular adverse events (hazard ratio 1.32; 95% confidence interval 1.18–1.59, p ​= ​0.002 per 5%-point decrease). Of 101 patients with ≥2 exercise tests, %-predicted peak VO2 decreased by 2.3%-points/year, and rate of decline correlated with change in right atrial (RA) strain and left heart hemodynamics. Hemodynamic deterioration started with RA dysfunction, followed by left heart hemodynamic impairment, and then decline in peak VO2. Conclusions Peak VO2 is a predictor of cardiovascular adverse events in Ebstein anomaly. Temporal decline in peak VO2 was preceded by RA dysfunction and left heart hemodynamic impairment suggesting that impaired aerobic capacity represents a late stage in disease pathogenesis.

The purpose of the study was to determine which out-of-office BP measurement modality has the best correlation with central aortic systolic BP, arterial stiffness (a determinant of central aortic pressure) and LV mass index (LVMI) which represents LV adaptation to elevated central aortic pressures. In this prospective study, we demonstrated that 24h ambulatory systolic BP has better correlation with central aortic systolic BP (arterial load) and LVMI as compared to home systolic BP. 24h ambulatory systolic BP also correlated with arterial stiffness (determinant of arterial load), although this correlation was comparable to that observed with home systolic BP. Collectively, these results suggest that ambulatory systolic BP provides the best approximation of arterial load, which is the underpinning hemodynamic stress leading to LV remodeling, end-organ dysfunction, and cardiovascular mortality in patients with COA. These findings provide a foundation for further studies to determine whether titrating antihypertensive therapy based on ambulatory BP indices will slow the progression of LV remodeling and end-organ dysfunction due to hypertension, and in turn, reduce cardiovascular death in this population.

The purpose of this study was to describe procedural outcomes, hemodynamic improvement, regression of left ventricular (LV) mass hypertrophy, and cardiovascular. The primary outcomes were procedural complications, reinterventions, and hemodynamic improvement after coarctation of aorta (COA) repair. The secondary outcomes were improvement in the severity of hypertension, regression of LV mass index, and incidence of cardiovascular events (atrial fibrillation, ventricular tachycardia, heart failure hospitalization, and cardiovascular death) after COA repair. Secondary outcomes were assessed only in patients with isolated COA who had clinical and imaging follow-up at 1 year and 3 years postintervention. Of 172 patients that underwent COA repair (surgical 161; transcatheter 11), there were no procedural deaths, and all patients had residual COA gradient P =0.4 at baseline, 1 year and 3 years). Postintervention hypertension (both stage 1 and 2) were independent risk factors for suboptimal left ventricular mass index regression and cardiovascular events. Persistent hypertension was common after repair of native COA in adults and was associated with suboptimal left ventricular mass index regression and cardiovascular events. These results suggest that optimal blood pressure control with medical therapy after COA repair may result in improved clinical outcomes.

Aims Increases in extravascular lung water (EVLW) during exercise contribute to symptoms, morbidity, and mortality in patients with heart failure and preserved ejection fraction (HFpEF), but the mechanisms leading to pulmonary congestion during exercise are not well-understood. Methods and results Compensated, ambulatory patients with HFpEF (n = 61) underwent invasive haemodynamic exercise testing using high-fidelity micromanometers with simultaneous lung ultrasound, echocardiography, and expired gas analysis at rest and during submaximal exercise. The presence or absence of EVLW was determined by lung ultrasound to evaluate for sonographic B-line artefacts. An increase in EVLW during exercise was observed in 33 patients (HFpEFLW+, 54%), while 28 (46%) did not develop EVLW (HFpEFLW−). Resting left ventricular function was similar in the groups, but right ventricular (RV) dysfunction was two-fold more common in HFpEFLW+ (64 vs. 31%), with lower RV systolic velocity and RV fractional area change. As compared to HFpEFLW−, the HFpEFLW+ group displayed higher pulmonary capillary wedge pressure (PCWP), higher pulmonary artery (PA) pressures, worse RV-PA coupling, and higher right atrial (RA) pressures during exercise, with increased haemoconcentration indicating greater loss of water from the vascular space. The development of lung congestion during exercise was significantly associated with elevations in PCWP and RA pressure as well as impairments in RV-PA coupling (area under the curve values 0.76–0.84). Conclusion Over half of stable outpatients with HFpEF develop increases in interstitial lung water, even during submaximal exercise. The acute development of lung congestion is correlated with increases in pulmonary capillary hydrostatic pressure that favours fluid filtration, and systemic venous hypertension due to altered RV-PA coupling, which may interfere with fluid clearance. Clinical trial registration NCT02885636.

One of the goals of lifelong care in adults with tetralogy of Fallot (TOF) is early identification and treatment of patients at high risk for adverse events. Clinical risk stratification tools are critical for achieving this goal. We reviewed the Mayo Adult Congenital Heart Disease database and identified 465 TOF patients (age 37 ± 14 years, men 223 [48%]) seen at Mayo Clinic Rochester between 1990 and 2017. The aim was to determine the risk factors for death and/or heart transplant through a comprehensive analysis of 8 groups of variables (demographics, co-morbidities, medications, heart rhythm, echocardiography, cardiac magnetic resonance imaging, cardiac catheterization, and cardiopulmonary exercise test data) using univariable and multivariable Cox proportional hazard models. The end point of death and/or transplant occurred in 57 (12%) patients during a follow-up of 13.6 ± 8.2 years, yielding an event rate of 0.9% per year. Independent risk factors were age42 years, atrial fibrillation, ≥moderate QRS fragmentation, left ventricular ejection fraction50%, right ventricular end-diastolic pressure16 mm Hg, and left ventricle end-diastolic pressure16 mm Hg. There is nearly a twofold increase in the risk of death and/or transplant per unit increase in number of risk factors (hazard ratio 1.92, 95% confidence interval 1.62 to 2.27, p0.001). In conclusion, the current study provides risk stratification indices based on a comprehensive risk model of all clinical variables in an unselected TOF population. Further studies are required to determine whether interventions targeted at modifying these risk factors will alter the annual event rate.

Background:There are limited outcome data in adults with tetralogy of Fallot and pulmonary atresia. The purpose of this study was to describe re-operations and all-cause mortality in adults with tetralogy of Fallot and pulmonary atresia.Methods:Retrospective review of adults with repaired tetralogy of Fallot and pulmonary atresia who received care at the Mayo Adult Congenital Heart Disease Clinic, 1990–2016. All-cause mortality was calculated as events per 100 patient-years from the time of first presentation to the Adult Congenital Heart Disease Clinic.Results:Of the 221 patients, the age at initial tetralogy of Fallot repair was 6 (5–13) years, and the age at first presentation to the clinic was 27 – 8 years. All patients had at least one right ventricular to pulmonary artery conduit re-operation. There were 31 deaths (14%) at mean age of 41 – 14 years. The causes of death were end-stage heart failure (n = 17), sudden cardiac death (n=9), post-operative death after cardiac surgery (n = 2), sepsis with multi-system organ failure (n = 2), and unknown (n = 1). All-cause mortality rate was 1.7 per 100 patient-years. The risk factors for all-cause mortality were older age (>12 years) at the time of repair (hazard ratio 1.41, 95 confidence interval 1.06–2.02, p = 0.033), non-sustained ventricular tachycardia (hazard ratio 1.36, 95 confidence interval 1.17–2.47, p = 0.015), and left ventricular ejection fraction Conclusion:Based on a review of 221 adults with repaired tetralogy of Fallot and pulmonary atresia, all patients had re-operations and all-cause mortality rate was 1.7 events per 100 patient-years. The current study provides important outcomes data for risk stratification in adults with tetralogy of Fallot and pulmonary atresia.

Lesion complexity and prosthetic valves are known risk factors for infective endocarditis in patients with congenital heart disease. Tetralogy of Fallot (TOF) is the most common complex/cyanotic congenital heart disease and often requires prosthetic valve implantation. Population-based risk of endocarditis in TOF patients is unknown.We reviewed the National Inpatient Sample (NIS) and identified admissions in TOF patients (18 years), 2000 to 2014. The primary outcome was to describe incidence of endocarditis-related admissions. To assess trends, we divided the study period into tertiles: early (2000 to 2004), mid (2005 to 2009) and late (2010 to 2014) eras. The secondary outcome was to compare in-hospital mortality, complications, and health care resource utilization between admissions with and without endocarditis.There were 393 (2.1%) endocarditis-related admissions among 18,353 admissions, and the incidence of endocarditis-related admissions increased over time: 1.9% (early era) vs 2.2% (mid-era) vs 2.4% (late era), P0.001. Overall in-hospital mortality was 6%. In addition to previously described risk factors for endocarditis, such as previous pacemaker/defibrillator or prosthetic valve implantation, we observed an association between endocarditis-related admissions and male gender, black race, and lower socioeconomic class. In comparison with admissions without endocarditis, the endocarditis-related admissions had higher in-hospital mortality, complications, and health care resource utilization measured by length of stay, inflation-adjusted hospitalization cost, and type of hospital discharge.Incidence of endocarditis-related admissions increased over time and was associated with higher mortality, complications, and health care resource utilization. Further studies are required to investigate the observed temporal increase in incidence of endocarditis and explore new strategies to improve outcomes.

Objectives This study hypothesized that atrial fibrillation was associated with heart failure (HF) hospitalization, and that patients who received rhythm control therapy had a lower incidence of HF hospitalization and mortality. Background Atrial fibrillation is a known risk factor for HF hospitalization and mortality in patients with acquired heart disease. Although atrial arrhythmias are common in adults with tetralogy of Fallot (TOF), data about prevalence and outcomes of therapy for atrial fibrillation are very limited. Methods The MACHD (Mayo Adult Congenital Heart Disease) database was queried for adults with repaired TOF and documented atrial fibrillation from 1990 to 2017. Primary endpoint was HF hospitalization defined as admission for volume overload (pulmonary congestion and/or peripheral edema) requiring intravenous diuretics. Secondary endpoint was the effect of rhythm control therapy on HF hospitalization and all-cause mortality. Patients were divided into rhythm control and rate control groups based on the therapy initiated at the time of arrhythmia diagnosis. Results Of 415 patients, 27 (7%) had 42 HF hospitalizations. Of these 415 patients, 88 (21%) had atrial fibrillation at age 49 ± 13 years. Atrial fibrillation was an independent risk factor for HF hospitalization (adjusted hazard ratio: 2.67; 95% confidence interval: 1.04 to 7.34; p = 0.045). The 88 patients were divided into the rhythm control group (n = 61, 69%) and the rate control group (n = 27, 31%). The rate control group had higher unadjusted annual incidence of HF hospitalization (13% vs. 3%; p = 0.001) and all-cause mortality (11% vs. 4%; p = 0.002). Conclusions Atrial fibrillation was a risk factor for HF hospitalization and mortality in TOF patients, and rhythm control therapy was protective against these adverse events.

Background There are limited data about outcomes of coronary artery disease (CAD) in adults with repaired tetralogy of Fallot (TOF). The purpose of this study was to describe the prevalence and treatment of CAD in adults with TOF, and the impact of CAD on long-term survival. Methods Retrospective review of MACHD database for adults with repaired TOF who underwent aortic root/selective coronary angiogram, 1990-2017. Patients were categorized into three groups: (1) No CAD defined as normal coronary angiogram; (2) Mild CAD defined as ≤50% stenosis in all vessels; and, (3) Significant CAD defined as >50% stenosis in any vessel. Results We identified 105 (23%) of 465 TOF patients that had angiograms; mean age 47 ± 12 years. The prevalence of mild CAD and significant CAD was 19% (20 patients) and 15% (16 patients), respectively. Of these 16 patient with significant CAD, 9 (56%), 3 (19%), and 4 (24%) patients received guideline directed medical therapy, percutaneous coronary intervention, and coronary artery bypass grafting, respectively. Significant CAD was an independent risk factor for mortality (HR: 2.03, 95% CI 1.64-4.22, P = .022) after adjustment for differences in age, and prevalence of atrial fibrillation and renal dysfunction. Conclusions Based on a review of a selected cohort of 105 TOF patients, the prevalence of mild CAD and significant CAD was 19% and 15%, respectively. Significant CAD was an independent risk factor for mortality. There is need for more research to determine optimal noninvasive diagnostic strategies and optimal patient selections and methods for revascularization.

Left atrial (LA) function is impaired in heart failure with preserved ejection fraction (HFpEF) and predicts disease severity and risk of adverse outcome. We sought to evaluate whether LA strain could enhance diagnosis of HFpEF.Consecutive patients with unexplained exertional dyspnoea and image quality suitable for measuring LA strain underwent invasive cardiopulmonary exercise testing to ascertain the presence of HFpEF or non-cardiac causes of dyspnoea (NCD). LA reservoir strain was measured in all patients, while LA conduit and booster strain were measured in patients with sinus rhythm. LA strain was measurable in 363 of 378 examinations (96.5%; HFpEF: 238, NCD: 125). LA reservoir strain (29 ± 16% vs. 40 ± 13%, P 0.0001) and conduit strain (18 ± 10% vs. 22 ± 10%, P = 0.0001) were significantly impaired in HFpEF compared to NCD. Of all echocardiographic indices, LA reservoir strain best discriminated HFpEF from NCD [area under the curve (AUC) 0.719, P 0.0001], outperforming E/e' (AUC difference +0.117, P 0.0001), LA enlargement (+0.090, P = 0.001), tricuspid regurgitation velocity 2.8 m/s (+0.082, P = 0.0085), left ventricular hypertrophy (+0.0159, P 0.0001) and left ventricular global longitudinal strain (+0.0198, P 0.0001). Indexing LA reservoir strain to estimated LA pressure (E/e') as a surrogate for LA compliance further improved diagnostic performance (AUC 0.772, P 0.0001; +0.053 compared to LA reservoir strain alone, P = 0.003).Left atrial reservoir strain may provide enhanced diagnostic accuracy beyond conventional echocardiographic measures to discriminate HFpEF from NCD. Further study is required to verify the diagnostic utility of LA strain.

To review our experience with balloon testing prior to atrial septal defect (ASD) closure in adults with left ventricular (LV) diastolic dysfunction.ASD closure in patients with LV diastolic dysfunction may precipitate LV failure. Temporary ASD occlusion has been used in this scenario but data are limited.Retrospective review of 27 patients age ≥ 50 years undergoing temporary ASD balloon occlusion between 2000 and 2018 for suspected LV diastolic dysfunction or elevated LV end-diastolic pressure (LVEDP).Median age was 72 years (IQR 66.7; 75.2). Atrial fibrillation was seen in 58% of patients, hypertension in 58%, and coronary artery disease in 26%; 52% were females. Median ASD size was 13 mm (10; 18) and Qp/Qs 1.8 (1.6; 2.2). Median LVEDP was 14 mmHg (12; 22); pulmonary artery wedge pressure (PAWP) 12 mmHg (9; 16.5) and left atrial pressure (LAP) 13.5 mmHg (8; 16.3). After a median of 5 min (3; 10) of balloon occlusion, patients with baseline LVEDP ≥15 mmHg had more significant increases in LVEDP (9 [6; 12] vs. 2 mmHg [0.5; 5]; p = 0.03) and LAP/PAWP (10.5 [8.3; 16.3] vs. 1.5 mmHg [-1.5; 3]; p = 0.0003) than those with baseline LVEDP15 mmHg. None of those with a baseline LVEDP15 mmHg had a LAP/PAWP15 mmHg during balloon testing compared to 92% of patients with a baseline LVEDP ≥15 mmHg.LVEDP might be used to predict LAP post-ASD closure. Comorbidities typically associated with LV diastolic dysfunction are common in these patients and should be considered in their management.

Background Arrhythmias, conduction abnormalities, and intracardiac thrombus are common in patients with cardiac amyloidosis (CA). Outcomes of direct-current cardioversion (DCCV) for atrial arrhythmias in patients with CA are unknown. Objectives This study sought to examine DCCV procedural outcomes in patients with CA. Methods Patients with CA scheduled for DCCV for atrial arrhythmias from January 2000 through December 2012 were identified and matched 2:1 with control patients by age, sex, type of atrial arrhythmia, and date of DCCV. Results CA patients (n = 58, mean age 69 ± 9 years, 81% male) were included. CA patients had a significantly higher cardioversion cancellation rate (28% vs. 7%; p Conclusions Patients with CA undergoing DCCV had a significantly high cancellation rate mainly due to a high incidence of intracardiac thrombus even among patients who received adequate anticoagulation. Although the success rate of restoring sinus rhythm was high, tachyarrhythmias and bradyarrhythmias complicating DCCV were significantly more frequent in CA patients compared with control patients.

Postcardiac surgery cardiogenic shock (PCCS) is seen in 2% to 6% of patients who undergo cardiac surgery. There are limited large-scale data on the use of mechanical circulatory support (MCS) in these patients. This study sought to evaluate the in-hospital mortality, trends, and resource utilization for PCCS admissions with and without MCS. A retrospective cohort of PCCS between 2005 and 2014 with and without the use of temporary MCS was identified from the National Inpatient Sample. Admissions for permanent MCS and heart transplant were excluded. Propensity-matching for baseline characteristics was performed. The primary outcome was in-hospital mortality and secondary outcomes included trends in use, hospital costs and lengths of stay. In the period between 2005 and 2014, there were 132,485 admissions with PCCS, with 51.3% requiring MCS. The intra-aortic balloon pump was the predominant device used with a steady increase in other devices. MCS use for more frequent in younger patients, males and those with higher co-morbidity. There was a decrease in MCS use across all demographic categories and hospital characteristics over time. Older age, female sex, previous cardiovascular morbidity and MCS use were independently predictive of higher in-hospital mortality. In 6,830 propensity-matched pairs, PCCS admissions that required MCS use, had higher in-hospital mortality (odds ratio 2.4; p

Rationale: Pulmonary vascular resistance fails to decrease appropriately during exercise in patients with heart failure with preserved ejection fraction (HFpEF). Interventions that enhance pulmonary vasodilation might be beneficial in this cohort but could also worsen left atrial hypertension, exacerbating lung congestion. Intravenous β-agonists reduce pulmonary vascular resistance but are not suitable for chronic use. Objective: We hypothesized that the inhaled β-adrenergic agonist albuterol would improve pulmonary vasodilation during exercise in patients with HFpEF, without increasing left heart filling pressures. Methods and Results: We performed a randomized, double-blind, placebo-controlled trial testing the effects of inhaled albuterol on resting and exercise hemodynamics in subjects with HFpEF using high-fidelity micromanometer catheters and expired gas analysis. The primary end point was pulmonary vascular resistance during exercise. Subjects with HFpEF (n=30) underwent resting and exercise hemodynamic assessment and were then randomized 1:1 to inhaled, nebulized albuterol or placebo. Rest and exercise hemodynamic testing was then repeated. Albuterol improved the primary end point of exercise pulmonary vascular resistance as compared with placebo (−0.6±0.5 versus +0.1±0.7 WU; P =0.003). Albuterol enhanced cardiac output reserve and right ventricular pulmonary artery coupling, reduced right atrial and pulmonary artery pressures, improved pulmonary artery compliance, and enhanced left ventricular transmural distending pressure (all P Conclusions: Albuterol improves pulmonary vascular reserve in patients with HFpEF without worsening left heart congestion. Further study is warranted to evaluate the chronic efficacy of β-agonists in HFpEF and other forms of pulmonary hypertension. Clinical Trial Registration: URL: http://www.clinicaltrials.gov . Unique identifier: NCT02885636.

BACKGROUND High levels of vasoactive inotrope support (VIS) after congenital heart surgery are predictive of morbidity in pediatric patients. We sought to discern if this relationship applies to adults with congenital heart disease (ACHD). METHODS We retrospectively studied adult patients (≥18 years old) admitted to the intensive care unit after cardiac surgery for congenital heart disease from 2002 to 2013 at Mayo Clinic. Vasoactive medication dose values within 96 hours of admission were examined to determine the relationship between VIS score and poor outcome of early mortality, early morbidity, or complication related morbidity. RESULTS Overall, 1040 ACHD patients had cardiac surgery during the study time frame; 243 (23.4%) met study inclusion criteria. Sixty-two patients (25%), experienced composite poor outcome [including eight deaths within 90 days of hospital discharge (3%)]. Thirty-eight patients (15%) endured complication related early morbidity. The maximum VIS (maxVIS) score area under the curve was 0.92 (95% CI: 0.86-0.98) for in-hospital mortality; and 0.82 (95% CI: 0.76-0.89) for combined poor clinical outcome. On univariate analysis, maxVIS score ≥3 was predictive of composite adverse outcome (OR: 14.2, 95% CI: 7.2-28.2; P

BACKGROUND: Elevation in central venous pressure (CVP) plays a fundamental pathophysiologic role in Fontan circulation. Because there is no sub-pulmonary ventricle in this system, CVP also provides the driving force for pulmonary blood flow. We hypothesized that this would make Fontan patients more susceptible to even low-level elevation in pulmonary vascular resistance index (PVRI), resulting in greater systemic venous congestion and adverse outcomes. METHODS: Adult Fontan patients and controls without congenital heart disease undergoing clinical evaluation that included cardiac catheterization and echocardiography were examined retrospectively. Outcomes including all-cause mortality and the development of Fontan associated diseases (FAD, defined as protein losing enteropathy, cirrhosis, heart failure hospitalization, arrhythmia, or thromboembolism) were assessed from longitudinal assessment. RESULTS: As compared to controls (n=82), Fontan patients (n=164) were younger (36 vs 45 years, p

BACKGROUND: Brachial systolic blood pressure (BP) is the most commonly used metric for monitoring hypertension. However, recent studies suggest that brachial systolic BP underestimates 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. METHODS: 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 (LA) remodeling and pulmonary hypertension. We assessed LV systolic stiffness using end-systolic elastance (Ees), and diastolic stiffness using LV stiffness constant (β) and chamber capacitance (LV-end-diastolic volume at an end-diastolic pressure of 20mmHg, LVEDVI(20)) RESULTS: There were 112 patients in each group. Although both groups had similar systolic BP, the COA group had a higher Ees (2.37±0.74 vs 2.11±0.54 mmHg/ml, p=0.008), higher β (6.91±0.81 vs 5.93±0.79, p=0.006) and lower LVEDVI(20) (58±9 vs 67±11 ml/m(2), p

Background: Left ventricular global longitudinal strain (LVGLS) can detect early phases of LV systolic dysfunction, but its application has not been studied in Ebstein anomaly. We hypothesized that LVGLS can detect early phases of LV systolic dysfunction and that patients with occult LV systolic dysfunction will have worse hemodynamics, end-organ dysfunction, and suboptimal postoperative LV reverse remodeling after tricuspid valve surgery in comparison to patients with normal LV systolic function. Methods: In this retrospective cohort study, 371 Ebstein patients that underwent tricuspid valve surgery were divided into 3 groups: normal LV systolic function (normal LVGLS and LV ejection fraction; n=244, 77%), occult LV systolic dysfunction (abnormal LVGLS with normal LV ejection fraction; n=44, 14%), and overt LV systolic dysfunction (abnormal LVGLS and LV ejection fraction; n=27, 9%). Results: Compared with the normal LV function group, the occult group had smaller LV volume and cardiac output (2.1±0.4 versus 2.9±0.6 L/min per m 2 , P 2 , P =0.01), and suboptimal postoperative LV reverse remodeling. Although both the occult and overt groups had a similar degree of end-organ dysfunction (glomerular filtration rate, 78±14 versus 82±16 mL/min per 1.73 m 2 , P =0.3), the occult group was less likely to be on heart failure therapy (48% versus 96%, P Conclusions: Abnormal LVGLS was associated with suboptimal postoperative LV reverse remodeling. These data suggest that LVGLS can potentially be used for risk stratification and provides a foundation for further studies to determine whether optimal heart failure therapy or tricuspid valve intervention can improve outcomes for LV systolic dysfunction in patients with Ebstein anomaly.

ObjectiveBioprosthetic valve thrombosis (BPVT) is increasingly recognised as a major cause of prosthetic dysfunction in the first years postimplantation. How early abnormal gradients can be detected prior to diagnosis and how fast they normalise with anticoagulant therapy is unknown. We set forth to (1) evaluate patterns of increase in gradients prior to BPVT diagnosis and (2) characterise time-course of response to anticoagulation.MethodsPatients treated with warfarin for BPVT (1999–2019) with clinically significant reduction of mean gradients (≥25%) were identified retrospectively. Recovery was defined as gradient decrease ≥50%, to postimplantation or to normal-range gradients per position, model and size. Time-to-BPVT (implantation—BPVT diagnosis), potential diagnostic delay (first abnormal gradient by position, model and size—BPVT diagnosis) and time-to-recovery (BPVT diagnosis—complete resolution) were recorded.Results77 patients were identified; 32 (42%) aortic (23 surgical—12 porcine, 11 pericardial; 9 transcatheter); 24 (31%) mitral; 21 (27%) tricuspid. Median time-to-BPVT was 24, 21 and 10 months, respectively. Potential diagnostic delay was median 21 months for aortic, 4 months for mitral, but 0 for tricuspid. Recovery was significantly faster in mitral than aortic (median 2.5 vs 4.8 months, p=0.038) and tricuspid (median 5.9 months, p=0.025) positions. Porcine aortic valves responded faster than pericardial aortic valves (median 2.9 vs 20.3 months, p=0.004).ConclusionGradients start to increase months before the clinical BPVT diagnosis. Recovery is faster in mitral and surgical aortic porcine valves; a longer warfarin trial seems indicated in tricuspid and surgical aortic pericardial valves.

Background: Warfarin is known to be effective in the treatment of bioprosthetic valve thrombosis (BPVT). However, the time-course of response to anticoagulation has not been fully characterized. Hypothesis: We hypothesized that response to warfarin can differ depending on valve position. Methods: We retrospectively identified 45 patients with BPVT diagnosis between 2002 and 2019 that involved either the mitral (MV) or the aortic valve (AV) prostheses, all with eventual echocardiographic resolution with warfarin. BPVT involved AV in 26 (57.8%) patients and MV in 19 (42.2%). Mean gradients (MGs) were tracked starting from BPVT diagnosis until recovery, which was defined as MGs decreasing to initial post-implantation MGs, to 50% below MGs at BPVT diagnosis, or to < 20 mmHg in AV and < 5 mmHg in MV. Kaplan Meier analysis was used to compare recovery time between MV and AV. Duration is reported relative to the time of BPVT diagnosis. Results: Recovery was faster in MV than in AV patients treated with warfarin, with median time of 3.8 months in the AV and 1.8 months in the MV group, p < 0.001 (Figure 1). At 1 month follow up, 15.4% recovered in AV group and 21.1% in MV, p = 0.605. At 3 months, 38.5% recovered in the AV and 78.9% in the MV, p = 0.009. At 6 months, 57.7% of AV group recovered while 100% recovered in the MV group, p = 0.001. The longest time for recovery was 30 months in 1 AV patient. Conclusion: Recovery using warfarin tended to be faster in BPVT involving the MV compared with the AV. In our study, all patients with MV BPVT recovered by 6 months, while this only happened in half the patients with AV BPVT. This suggests that a longer duration of warfarin trial may be needed in the case of AV disease. This might also suggest that the pathogenesis may differ between the two valves.

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

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 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

Background Transcatheter stent therapy is as effective as surgery in producing acute hemodynamic improvement in patients with coarctation of aorta (COA). However, left ventricular (LV) remodeling after transcatheter COA intervention has not been systematically investigated. The purpose of this study was to compare remodeling of LV structure and function after transcatheter stent therapy vs surgical therapy for COA. Methods LV remodeling was assessed at 1, 3 and 5-years post-intervention using: LV mass index (LVMI), LV end-diastolic dimension, LV ejection fraction, LV global longitudinal strain (LVGLS), LV e' and E/e'. Results There were 44 and 128 patients in the transcatheter and surgical groups respectively. Compared to the surgical group, the transcatheter group had less regression of LVMI (−4.6 [95% CI: −5.5 to −3.7] vs −7.3 [95% CI: −8.4 to −6.6] g/m2, p Conclusions Transcatheter stent therapy was associated with less remodeling of LV structure and function during mid-term follow-up. As transcatheter stent therapy become more widely used in the adult COA population, there is a need for ongoing clinical monitoring to determine if these observed differences in LV remodeling translates to differences in clinical outcomes. Funding Acknowledgement Type of funding source: None

Background: Patients with coarctation of aorta (COA) have arterial stiffening, and this is associated with impaired cardiac reserve and hypertensive systolic blood pressure (SBP) response during exercise. However, whether patients with COA have concomitant left ventricular (LV) stiffening and the potential impact of combined ventricular-arterial stiffening on exercise hemodynamics are unknown. Methods: We studied 174 patients with repaired COA (aged 39 ± 11 years and male 103 [59%]) and 174 matched controls. Our study hypotheses are: (1) patients with COA have higher ventricular-arterial stiffness (end-systolic elastance [Ees] and arterial elastance [Ea]) as compared with controls; (2) ventricular-arterial stiffness was associated with LV stroke volume augmentation (ΔLVSV) and SBP augmentation (ΔSBP) during exercise among patients with COA. Results: Despite similar systolic SBP, patients with COA had higher Ea (1.8 ± 0.4 vs 1.4 ± 0.4 mm Hg/mL, P < 0.001), higher Ees (2.41 ± 0.65 vs 2.17 ± 0.40 mm Hg/mL, P < 0.001), but similar Ea/Ees (0.87 ± 0.29 vs 0.83 ± 0.33, P = 0.2). ΔLVSV was 6.1 ± 1.4 mL/beat. Combined ventricular-arterial stiffness had a stronger correlation with ΔLVSV as compared with Ea alone (r = −0.53 vs r = −0.41, P = 0.006) and as compared with Ees alone (r = −0.53 vs r = −0.46, P = 0.02). ΔSBP was 48 ± 21 mm Hg. Combined ventricular-arterial stiffness had a stronger correlation with ΔSBP as compared with Ea alone (r = 0.57 vs r = 0.43, P < 0.001) and as compared with Ees alone (r = 0.57 vs r = −0.39, P < 0.001). Conclusion: Patients with COA had combined ventricular-arterial stiffening, and this was associated with impaired cardiac reserve and hypertensive SBP response during exercise. These findings provide foundation for further studies to determine whether drugs that reduce both ventricular and arterial stiffness will improve exercise capacity and hemodynamics in this unique population. Résumé: Contexte: La coarctation de l’aorte (CA) s’accompagne d’une rigidification des artères, qui est à son tour associée à une altération de la réserve de force du cœur et à une augmentation de la pression artérielle systolique (PAS) à l’effort. On ne sait toutefois pas si la CA entraîne aussi une rigidification du ventricule gauche, et on ne connaît pas les répercussions possibles d’une rigidification ventriculaire et artérielle sur les paramètres hémodynamiques à l’effort. Méthodologie: Nous avons étudié 174 patients présentant une CA corrigée (âge : 39 ± 11 ans; hommes : 103 [59 %]) et 174 témoins appariés. Nos hypothèses étaient les suivantes : 1) la rigidité ventriculaire et artérielle (élastance télésystolique [ETS] et élastance artérielle [EA]) est plus importante chez les sujets présentant une CA que chez les sujets témoins; 2) la rigidité ventriculaire et artérielle est associée à une augmentation du volume d’éjection ventriculaire gauche (ΔVEVG) et à une augmentation de la pression artérielle systolique (ΔPAS) à l’effort chez les patients présentant une CA. Résultats: Malgré une PAS comparable, les patients présentant une CA avaient une EA plus élevée que les sujets témoins (1,8 ± 0,4 vs 1,4 ± 0,4 mmHg/ml, p < 0,001) et une ETS également plus élevée (2,41 ± 0,65 vs 2,17 ± 0,40 mmHg/ml, p < 0,001), mais un rapport EA/ETS similaire (0,87 ± 0,29 vs 0,83 ± 0,33, p = 0,2). La ΔVEVG était de 6,1 ± 1,4 ml/battement. La rigidité ventriculaire et artérielle mixte était plus fortement corrélée avec une ΔVEVG, comparativement à l’EA seule (r = -0,53 vs r = -0,41, p = 0,006) et à l’ETS seule (r = -0,53 vs r = -0,46, p = 0,02). La ΔPAS était de 48 ± 21 mmHg. La rigidité ventriculaire et artérielle mixte était plus fortement corrélée avec la ΔPAS, comparativement à l’EA seule (r = 0,57 vs r = 0,43, p < 0,001) et à l’ETS seule (r = 0,57 vs r = -0,39, p < 0,001). Conclusion: La CA s’accompagnait d’une rigidification ventriculaire et artérielle, elle-même associée à une altération de la réserve de force du cœur et à une augmentation de la PAS à l’effort. Ces résultats pourront servir de fondements à des études complémentaires visant à déterminer si un traitement qui réduit la rigidité ventriculaire et artérielle pourrait améliorer la capacité à l’effort et les paramètres hémodynamiques dans cette population particulière.

BackgroundThere is an increasing evidence that COVID-19 could be complicated by coagulopathy which may lead to death; especially in severe cases. Hence, this study aimed to build concrete evidence regarding the incidence and mortality of pulmonary embolism (PE) in patients with COVID-19.MethodsWe performed a systematic search for trusted databases/search engines including PubMed, Scopus, Cochrane library and Web of Science. After screening, the relevant data were extracted and the incidences and mortality rates from the different included studies were pooled for meta-analysis.ResultsTwenty studies were finally included in our study consisting of 1896 patients. The results of the meta-analysis for the all included studies showed that the incidence of PE in patients with COVID-19 was 17.6% with the 95% confidence interval (CI) of 12.7 to 22.5%. There was significant heterogeneity (I2□=□91.17%). Additionally, the results of meta-analysis including 8 studies showed that the mortality in patients with both PE and COVID-19 was 43.1% with the 95% confidence interval (CI) of 19 to 67.1%. There was significant heterogeneity (I2□=□86.96%).ConclusionPE was highly frequent in patients with COVID-19. The mortality in patients with both COVID-19 and PE was remarkable representing almost half of the patients. Appropriate prophylaxis and management are vital for better outcomes.

Background The improved life expectancy observed in patients living with human immunodeficiency virus (HIV) infection has made age‐related cardiovascular complications, including arrhythmias, a growing health concern. Hypothesis We describe the temporal trends in frequency of various arrhythmias and assess impact of arrhythmias on hospitalized HIV patients using the Nationwide Inpatient Sample (NIS). Methods Data on HIV‐related hospitalizations from 2005 to 2014 were obtained from the NIS database using International Classification of Diseases, 9th Revision (ICD‐9) codes. Data was further subclassified into hospitalizations with associated arrhythmias and those without. Baseline demographics and comorbidities were determined. Outcomes including in‐hospital mortality, cost of care, and length of stay were extracted. SAS 9.4 (SAS Institute Inc., Cary, NC) was utilized for analysis. A multivariable analysis was performed to identify predictors of arrhythmias among hospitalized HIV patients. Results Among 2 370 751 HIV‐related hospitalizations identified, the overall frequency of any arrhythmia was 3.01%. Atrial fibrillation (AF) was the most frequent arrhythmia (2110 per 100 000). The overall frequency of arrhythmias increased over time by 108%, primarily due to a 132% increase in AF. Arrhythmias are more frequent among older males, lowest income quartile, and nonelective admissions. Patients with arrhythmias had a higher in‐hospital mortality rate (9.6%). In‐hospital mortality among patients with arrhythmias decreased over time by 43.8%. The cost of care and length of stay associated with arrhythmia‐related hospitalizations were mostly unchanged. Conclusions Arrhythmias are associated with significant morbidity and mortality in hospitalized HIV patients. AF is the most frequent arrhythmia in hospitalized HIV patients.

Hepatorenal dysfunction is a risk factor for mortality in patients with chronic tricuspid regurgitation due to acquired heart disease. Ebstein anomaly is the most common cause of primary tricuspid regurgitation in adults with congenital heart disease, but the prevalence and prognostic implications of hepatorenal dysfunction are unknown in this population.The purpose of this study was to determine the risk factors and prognostic implications of hepatorenal dysfunction, as measured primarily by the use of model for end-stage liver disease excluding international normalized ratio (MELD-XI score), as well as looking at other associated factors.This was a retrospective study of adults with Ebstein anomaly who received care at Mayo Clinic from 2003 to 2018.Of 692 patients, the median MELD-XI score was 10.2 (interquartile range: 9.4 to 13.3); 53 (8%) died and 3 (0.4%) underwent heart transplant. MELD-XI was an independent predictor of death/transplant (hazard ratio: 1.32; 95% confidence interval: 1.11 to 2.06; p 0.001). In the subset of patients with serial MELD-XI scores (n = 416), temporal change in MELD-XI score (ΔMELD-XI) was also a predictor of death/transplant. In the subset of patients who underwent tricuspid valve surgery (n = 344), a post-operative improvement in MELD-XI score (ΔMELD-XI) was associated with improved long-term survival. Impaired right atrial (RA) reservoir strain and elevated estimated RA pressure were associated with worse baseline MELD-XI and ΔMELD-XI scores.Hepatorenal dysfunction is a predictor of mortality in Ebstein anomaly, and RA dysfunction and hypertension are hemodynamic biomarkers that can identify patients at risk for deterioration in hepatorenal function and mortality. These data highlight the prognostic importance of noncardiac organ-system dysfunction, and provide complementary clinical risk stratification metrics for management of these patients.

Background Fontan-associated liver disease (FALD) is universal in patients with a Fontan circulation. Hepatocellular carcinoma (HCC) is one of its severe expressions, and, though rare, frequently fatal. The purpose of this study was to describe the clinical presentation, risk factors, and outcomes of HCC in patients with a Fontan circulation. Methods A multicenter case series of Fontan patients with a diagnosis of HCC formed the basis of this study. The case series was extended by published cases and case reports. Clinical presentation, tumor characteristics, laboratory and hemodynamic findings as well as treatment types and outcomes, were described. Results Fifty-four Fontan patients (50% female) with a diagnosis of HCC were included. Mean age at HCC diagnosis was 30 ± 9.4 years and mean duration from Fontan surgery to HCC diagnosis was 21.6 ± 7.4 years. Median HCC size at the time of diagnosis was 4 cm with a range of 1 to 22 cm. The tumor was located in the right hepatic lobe in 65% of the patients. Fifty-one percent had liver cirrhosis at the time of HCC diagnosis. Fifty percent of the patients had no symptoms related to HCC and alpha-fetoprotein was normal in 26% of the cases. Twenty-six patients (48%) died during a median follow-up duration of 10.6 (range 1–50) months. Conclusions HCC in Fontan patients occurs at a young age with a 1-year survival rate of only 50%. Meticulous liver surveillance is crucial to detect small tumors in the early stage.

Background: Left ventricular (LV) pressure overload and coronary artery disease are common in patients with coarctation of aorta (COA), and they 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 mild COA (without hemodynamically significant COA) will have more LV diastolic dysfunction compared to controls. Methods: Adult patients with mild COA (Doppler peak velocity 2 standard deviations above age-specific normative values) between adults with repaired COA and controls. Results: Of 204 COA and 204 control patients (age 35 ± 12 years), patients with COA had higher septal and lateral E/e′ ratio (12 ± 4 vs 9 ± 4, p = 0.009) and (10 ± 3 vs 7 ± 3, p 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

ObjectiveTo assess the prevalence of elevated systemic right ventricular (sRV) end-diastolic pressure and pulmonary arterial hypertension in adults with transposition of the great arteries (TGA) who have undergone atrial switch operation.MethodsForty-two adults (aged ≥18 years) with complete TGA and atrial switch palliation undergoing cardiac catheterisation between 2004 and 2018 at Mayo Clinic, MN, were identified. Clinical, echocardiographic and invasive haemodynamic data were abstracted from the medical charts and procedure logs.ResultsMean age was 37.6±7.9 years; 28 were male (67%). The Mustard operation was performed in 91% of individuals. Mean estimated sRV ejection fraction by echocardiography was 33.3%±10.9% and ≥moderate tricuspid (systemic atrioventricular valve) regurgitation was present in 15 patients (36%). Mean sRV end-diastolic pressure was 13.2±5.4 mm Hg. An sRV end-diastolic pressure >15 mm Hg was present in 35% of individuals whereas a pulmonary artery wedge pressure (PAWP) >15 mm Hg was seen in 59%. Mean pulmonary artery pressure ≥25 mm Hg was seen in 47.5% of patients with PAWP being >15 mm Hg in all but one patient.ConclusionIn adults after atrial switch, elevated sRV end-diastolic pressure was present in only one-third of patients whereas increased PAWP was seen in almost 60%. These findings are most likely related to a combination of decreased pulmonary atrial (functional left atrium) compliance and, in a subset of patients, pulmonary venous baffle obstruction. Elevation in pulmonary pressures was highly prevalent with concomitant elevation in PAWP being present in essentially all patients.

Background: Patients with vascular stiffening may display increased arterial afterload that is out of proportion to systolic blood pressure (SBP). Since vascular and endothelial dysfunction develop in patients with coarctation of aorta (COA), we hypothesized that for any SBP, patients with mild COA (COA peak velocity Methods: We studied 204 COA patients (age 35±12 y) and 204 matched controls. Doppler-derived arterial afterload was assessed using effective arterial elastance index and total arterial compliance index. Results: Despite similar SBP, the mild COA group displayed higher arterial afterload as evidenced by a higher elastance index (3.3±0.9 versus 2.9±0.7 mm Hg/mL·m 2 ; P 2 ; P 2 ; P r =0.43, P Conclusions: COA patients had higher arterial afterload compared with controls with similar SBP. In comparison to SBP, Doppler-derived arterial load indices correlate more strongly with LV hypertrophy. These data suggest that SBP may underestimate LV afterload in this population. This has important clinical implications since titration of antihypertensive therapy is currently based on SBP.

ObjectivesWomen with Turner syndrome (TS) are frequently counselled against pregnancy due to lack of data and unclear aortic dissection risk. However, with advances in fertility therapy, more women with TS are contemplating pregnancy. This study compared rates of adverse cardiovascular (CV) outcomes among: (1) pregnant and non-pregnant women with TS and (2) pregnant women with TS with/without structural heart disease.MethodsRetrospective analysis of pregnant and age-matched non-pregnant controls with TS (2005–2017) across 10 CV centres was done. Data were collected at initial evaluation in pregnancy and outcomes were assessed to 6 months postpartum. Adverse CV events were defined as CV death, aortic dissection/rupture and/or aortic intervention. Non-pregnant age-matched controls were followed over the same time period.ResultsSixty-eight pregnancies were included (60 women, mean age 33 years, 48% primigravid, 49% fertility therapy, 80% structurally normal heart, 25% XO karyotype). Based on American Society of Reproductive Medicine criteria, 10 pregnancies occurred in women stratified to high-risk category. There were no CV events in the pregnant women or in the non-pregnant women with TS. Obstetric events complicated 12 (18%) pregnancies with 9 (13%) attributed to hypertensive disorder of pregnancy. Fetal events included small for gestational age neonates (18%), preterm delivery (15%) and fetal death (3%).ConclusionsThis study helps to refine the approach to pregnancy in women with TS. Among women with TS without structural heart disease, pregnancy does not impose an increased risk of CV outcomes. Among women with TS with structural heart disease, the risk of pregnancy is not as prohibitive as previously described but does require ongoing evaluation.