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Background: Aortic valve disease is common in adults with coarctation of aorta. However, no systematic comparative analyses have been performed of the clinical course of aortic valve disease for male vs female patients in this population. The purpose of this study was to compare cardiac remodelling, onset of symptoms, and incidence of aortic valve replacement (AVR) for male vs female patients. Methods: A retrospective study was conducted of adults with repaired coarctation of aorta and ≥ moderate aortic stenosis and/or aortic regurgitation. Cardiac remodelling (left ventricular [LV], left atrial, right ventricular [RV], and right atrial structure and function) and symptomatic and/or functional class were determined at the baseline encounter. Development of new-onset symptoms and the incidence of AVR were ascertained for the period from baseline to last encounter. Results: We identified 214 patients (121 male [57%], 93 female [43%]). Although both groups had a similar aortic valve gradient, aortic valve area indexed to body surface area, aortic regurgitation severity, and functional status at baseline, female patients had more LV concentric hypertrophy and remodelling, left atrial hypertension and dysfunction, elevated RV systolic pressure, and RV systolic dysfunction. Of 151 patients without symptoms at baseline,102 (72%) developed symptoms. Female sex was independently associated with new-onset symptoms (adjusted hazard ratio 1.14, [95% confidence interval 1.05-1.23]). Of 214 patients, 191 (89%) underwent AVR. Female sex was not associated with AVR upon multivariable analysis. However, LV concentric hypertrophy and remodelling (both of which were more common in female patients) were associated with new-onset symptoms and AVR. Conclusions: Female patients, compared to male patients, had more-advanced cardiac remodelling, and more-rapid onset of symptoms, but a similar risk of AVR. Résumé: Contexte: La valvulopathie aortique est fréquente chez les adultes atteints de coarctation de l'aorte. Cependant, aucune analyse comparative systématique de l'évolution clinique de la maladie valvulaire aortique n'a été réalisée chez les hommes par rapport aux femmes dans cette population. L'objectif de cette étude était de comparer le remodelage cardiaque, l'apparition des symptômes et l'incidence du remplacement valvulaire aortique (RVA) chez les hommes et les femmes. Méthodes: Une étude rétrospective a été menée sur des adultes ayant subi une correction de la coarctation de l'aorte et présentant une sténose aortique ≥ modérée et/ou une régurgitation aortique. Le remodelage cardiaque (structure et fonction du ventricule gauche [VG], de l'oreillette gauche, du ventricule droit [VD] et de l'oreillette droite) et la classe symptomatique et/ou fonctionnelle ont été déterminés lors de la consultation initiale. L'apparition de nouveaux symptômes et l'incidence du RVA ont été déterminées pour la période allant de la consultation initiale à la dernière consultation. Résultats: Nous avons identifié 214 patients (121 hommes [57 %], 93 femmes [43 %]). Bien que les deux groupes aient présenté une similarité de profil concernant le gradient valvulaire aortique, la surface valvulaire aortique indexée sur la surface corporelle, la sévérité de régurgitation aortique et leur état fonctionnel au départ, les patientes avaient plus d'hypertrophie concentrique et de remodelage du VG, d'hypertension et de dysfonction de l'oreillette gauche, de pression systolique élevée du VD et de dysfonction systolique du VD. Sur 151 patients sans symptômes au départ, 102 (72 %) ont développé des symptômes. Le sexe féminin a été associé de manière indépendante à l'apparition de nouveaux symptômes (rapport de risque ajusté 1,14, [1,05-1,23]). Sur 214 patients, 191 (89 %) ont subi un RVA. Le sexe féminin n'était pas associé à un RVA lors de l'analyse multivariée. Cependant, l'hypertrophie et le remodelage concentrique du VG (tous deux plus fréquents chez les femmes) étaient associés à l'apparition de nouveaux symptômes et à un RVA. Conclusions: Les femmes, comparées aux hommes, présentaient un remodelage cardiaque plus avancé et une apparition plus rapide des symptômes, mais un risque similaire de RVA.

Background The purpose of this study was to describe the correlates and outcomes in adults with unrepaired partial anomalous pulmonary venous return and intact atrial septum (PAPVR‐IAS). Methods and Results We identified adults with PAPVR‐IAS who received care at the Mayo Clinic, while those with unrepaired PAPVR‐IAS comprised the reference group. Clinical indices (New York Heart Association class, peak oxygen consumption, and NT‐proBNP [N‐terminal pro‐B‐type natriuretic peptide]) and echo‐derived right heart indices (right atrial [RA] volume, RA reservoir strain, right ventricular [RV] free wall strain, RV end‐diastolic area, and RV systolic pressure) were assessed at baseline and 3‐year and 5‐year follow‐up. There were 80 patients and 38 patients with unrepaired versus repaired PAPVR‐IAS, respectively. The clinical predictors of surgical repair were the number of anomalous veins, RA volume, and RV end‐diastolic area. The PAPVR‐IAS risk score, derived from these clinical predictors, was associated with surgical repair (adjusted odds ratio, 1.37 [95% CI, 1.24–1.65] per unit increase in risk score; area under the curve, 0.742). Among patients with unrepaired PAPVR‐IAS with 3‐year (n=73) and 5‐year follow‐up (n=36), there was no temporal change in clinical indices (New York Heart Association class, predicted peak oxygen consumption, and NT‐proBNP) and right heart indices (RA volume index, RA reservoir strain, RV end‐diastolic area index, RV free wall strain, and RV systolic pressure). Conclusions The PAPVR‐IAS risk score can be used to assess the odds of requiring surgical repair. Furthermore, there was no temporal deterioration in clinical and right heart indices during follow‐up in adults with unrepaired PAPVR‐IAS.

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. Résumé: 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.

Background There are limited data about the impact of timing of pulmonary valve replacement (PVR) on right heart reverse remodeling in patients with pulmonary regurgitation following intervention for isolated pulmonary valve stenosis (PS). This study compared differences in postprocedural right heart reverse remodeling after early versus late PVR (defined as PVR before versus after attainment of the conservative consensus criteria proposed by Bokma et al, 2018) in patients with prior intervention for PS, using patients with tetralogy of Fallot as the reference group. Method and Results Right atrial reservoir strain and right ventricular free wall strain was measured at baseline, 1 and 3 years after PVR. There were 114 patients with PS (early PVR, 87 [76%]; late PVR, 27 [24%]) and 291 patients with tetralogy of Fallot (early PVR, 197 [67%]; late PVR, 96 [33%]). The PS group had greater improvement in right atrial reservoir strain at 1 year (12%±4% versus 8%±4%; P

Background: Right atrial (RA) dysfunction and atrial arrhythmias are relatively common in adults with repaired tetralogy of Fallot. The purpose of this study was to determine whether RA function improved after surgical pulmonary valve replacement (PVR), and the association between postoperative RA reverse remodeling and late postoperative atrial arrhythmias. Method: RA reverse remodeling (ΔRA reservoir strain based speckle tracking echocardiography) was calculated as: ([postoperative RA reservoir strain – preoperative RA reservoir strain]/preoperative RA reservoir strain)x100. Optimal RA reverse remodeling was defined as ΔRA reservoir strain >15%. Results: Of 411 patients (age 36 ± 13 years), preoperative RA reservoir strain was 31 ± 13%, postoperative RA reserve remodeling was 13 ± 9%, and 171 (42%) had optimal RA reserve remodeling. Preoperative RA reservoir strain (β±SE 1.12 ± 0.09, p

Background: Aging is associated with acquired comorbidities that potentially influence the natural history and outcomes of adults with congenital heart disease (CHD). The purpose of this study was to compare the clinical characteristics, as well as the incidence and correlates of all-cause mortality between different age groups. Method: Adults with CHD were categorized into 3 age groups based on age at baseline encounter: Group 1 (age 18–40 years); Group 2 (age 41–65 years), and Group 3 (age >65 years). Results: Of 5930 patients (age 37 ± 15 years), 3009 (51%), 2422 (41%), and 499 (8%) were in Groups 1, 2 and 3, respectively. Compared to Group 1, patients in Groups 2 and 3 were less likely to have complex CHD, but more likely to have acquired comorbidities, end-organ dysfunction, ventricular systolic dysfunction, and valvular heart disease. Compared to Group 1, Groups 2 and 3 had higher incidence of all-cause mortality (7.2 versus 15.3 versus 47.8 per 1000 patient-years, respectively, p

Background: Patients with palliated pulmonary valve stenosis (PVS) have less cardiac remodeling and symptoms as compared to patients with repaired tetralogy of Fallot (TOF) presenting with similar severity of right ventricular outflow tract (RVOT) disease. What is not known is whether patients with PVS versus TOF presenting with similar severity of RVOT disease at baseline, would have similar (or different) pace of cardiac remodeling and disease progression over time. The study objective was to compare temporal changes in clinical and cardiac function indices between adults with palliated PVS and repaired TOF presenting with moderate/severe RVOT disease. Methods: Cardiac function indices (based on strain imaging) and clinical indices (N-terminal pro–B-type natriuretic peptide [NT-proBNP], model for end-stage liver disease excluding international normalized ratio [MELD-XI], peak oxygen consumption [VO2]), were assessed at baseline, 3 years, and 5 years. Temporal changes were calculated as relative changes from baseline (Δ). Cardiovascular adverse event was assessed as time-to-event outcome. Results: Compared to TOF group (n = 173), the PVS group (n = 173) had less temporal change in right atrial reservoir strain (−9±4% versus −21 ± 6%, p

Background: Although patients with coarctation of aorta (COA) have clinical risk factors for atrial fibrillation (AF), there are limited data about AF prevalence, and role of left atrial (LA) indices for risk stratification in this population. We hypothesized that LA indices (LA reservoir strain and LA volume index) were associated with AF, and would identify patients at risk for AF progression. Methods: We analyzed electrocardiograms/Holters, and echocardiograms of adult COA patients at Mayo Clinic (2000–2018). Results: Of 776 patients, 726(94 %), 46(5.9 %) and 4(0.5 %) had no history of AF, paroxysmal AF, and persistent AF respectively; yielding AF prevalence of 6.4 %. LA reservoir strain (AUC 0.782 [0.751–0.808]) had more robust association with AF as compared to LA volume index (AUC difference −0.115, p 34 ml/m2 were independent predictors of new-onset AF (HR 1.81 [1.15–3.85], and HR 1.41 [1.03–4.78], respectively). Of 46 patients with paroxysmal AF, 22(48 %) had recurrent AF, and LA reservoir strain

Background: Pulmonary atresia with intact ventricular septum is associated with significant morbidity and mortality, but there are limited data to guide risk stratification in this population. The purpose of this study was to assess the role right atrial (RA) strain indices for prognostication in this population. Methods: This is a retrospective study of adults (aged ≥18 years) with pulmonary atresia with intact ventricular septum and biventricular repair who underwent echocardiogram (2003-2019). RA reservoir strain was used as the primary metric of RA function, and RA dysfunction was defined as RA reservoir strain 11); (3) incident atrial arrhythmias/heart failure hospitalization; (4) heart transplant/cardiovascular death. Results: Of the 43 patients in the study, RA strain imaging was feasible in 95%, and RA dysfunction was present in 95%. Of the 43 patients, 67% and 49% had functional impairment and hepatorenal dysfunction, respectively; 44% developed incident atrial arrhythmia/heart failure hospitalization and 14% died during follow-up. RA reservoir strain was independently associated with all indices of clinical outcomes. Conclusion: Collectively, these data suggest that RA strain imaging was feasible in almost all patients and can be used for risk stratification in this population. There was a high prevalence of comorbidities including hepatorenal dysfunction. Further studies are needed to determine the prognostic implications of hepatorenal dysfunction (a previously unrecognized complication), and whether using RA function indices for clinical decision making will lead to improved outcomes in this population. Résumé: Contexte: L’atrésie pulmonaire à septum ventriculaire intact est associée à une morbidité et à une mortalité importantes, mais il existe peu de données pour guider la stratification des risques au sein de la population touchée. L’étude dont il est question dans notre article visait à évaluer le rôle des indices de déformation (strain) atriale droite (AD) dans la formulation d’un pronostic en présence de cette cardiopathie. Méthodologie: Nous présentons ici une étude rétrospective portant sur un groupe d’adultes (âgés de 18 ans ou plus) atteints d’atrésie pulmonaire à septum ventriculaire intact qui avaient subi une réparation biventriculaire et une échocardiographie (2003-2019). La déformation AD pendant la phase réservoir a été retenue comme mesure principale de la fonction AD, et la dysfonction AD correspondait à une déformation AD < 31 % pendant la phase réservoir. Les résultats cliniques ont été évalués à l’aide de quatre indices distincts : (1) déficience fonctionnelle (classes II à IV de la New York Heart Association); (2) dysfonction hépatorénale (modèle d’hépatopathie en phase terminale excluant un rapport international normalisé > 11); (3) épisodes d’arythmie auriculaire incidente/hospitalisation pour cause d’insuffisance cardiaque; (4) transplantation cardiaque/décès d’origine cardiovasculaire. Résultats: Au sein du groupe de 43 patients à l’étude, l’imagerie de la déformation AD était réalisable et une dysfonction AD existait dans 95 % des cas respectivement. Parmi les 43 patients, 67 % présentaient une déficience fonctionnelle et 49 %, une dysfonction hépatorénale; 44 % présentaient une arythmie auriculaire incidente ou avaient été hospitalisés pour insuffisance cardiaque et 14 % étaient décédés au cours de la période de suivi. La déformation AD pendant la phase réservoir était indépendamment associée à tous les indices des résultats cliniques. Conclusion: Globalement, ces données donnent à penser que l’imagerie de la déformation AD était réalisable chez presque tous les patients et qu’elle peut servir à la stratification des risques au sein de la population considérée. La prévalence de maladies concomitantes était élevée, notamment en ce qui concerne la dysfonction hépatorénale. D’autres études devront être menées pour cerner l’incidence pronostique de la dysfonction hépatorénale (complication non reconnue antérieurement) et pour déterminer si l’utilisation d’indices de la fonction AD lors de la prise de décisions cliniques se traduira par de meilleurs résultats chez les patients.

Aorto-ventricular tunnels are rare, congenital extracardiac connections arising from the tubular ascending aorta, above the sinotubular junction, which communicate directly with the ventricular cavity. We present long-term follow-up in a series of five adults undergoing pediatric repair and describe the cardiovascular features and outcomes. Significant aortic regurgitation, ascending aortic aneurysm and left ventricular noncompaction were found to be highly prevalent, emphasizing the need for ongoing surveillance following repair of this anomaly.

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.

Abstract 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: 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.

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: Despite the significant risk of cardiovascular mortality after tetralogy of Fallot (TOF) repair, there are limited data about left ventricular (LV) cardiomyopathy in this population, thus creating important knowledge gaps. This study aims to address some of these knowledge gaps by describing the risk and prognostic implications of LV systolic dysfunction (LVD) after TOF repair. Methods: We performed a cohort study of adult patients after TOF repair with an echocardiographic assessment of LV ejection fraction (LVEF) to determine the association between LVD and cardiovascular events, defined as sustained ventricular tachycardia, aborted sudden death, heart transplantation, or death. Prevalent and incidence LVD were defined as LVEF < 50% at baseline or new decrease in LVEF to

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.

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.

Background: Data on the features of double-chambered right ventricular (DCRV) in adults are limited. It is also unclear how invasive and Doppler-derived assessments of right ventricular (RV) obstruction in DCRV correlate. Methods: Retrospective review of 17 adults (age ≥18 years) diagnosed with DCRV who underwent right heart catheterization at Mayo Clinic, MN between 1990 and 2021. Clinical, echocardiographic, and invasive hemodynamics were abstracted from the medical charts. Results: Mean age was 44 ± 19 years and 7 (41%) patients were female. Two patients (12%) had undergone prior surgical repair of DCRV with ventricular septal defect (VSD) closure and had residual/recurrent obstruction; a VSD had been previously documented in 16 patients (94%). RV inflow to pulmonary artery systolic gradient at catheterization was 42 (19; 95) mmHg. The correlation coefficient between RV inflow to pulmonary artery systolic gradients and Doppler-derived peak gradients was 0.87 (p

Background: Total cavopulmonary connection (TCPC) is associated with a lower risk of incident atrial arrhythmias as compared to atriopulmonary Fontan, but the risk of recurrent atrial arrhythmias is unknown in this population. The purpose of this study was to determine the incidence and risk factors for recurrent atrial arrhythmias and thromboembolic complications in patients with TCPC. Methods: This is a retrospective multicenter study conducted by the Alliance for Adult Research in Congenital Cardiology (AARCC), 2000–2018. The inclusion criteria were TCPC patients (age > 15 years) with prior history of atrial arrhythmia. Results: A total of 103 patients (age 26 ± 7 years; male 58 [56%]) met inclusion criteria. The mean age at initial arrhythmia diagnosis was 13 ± 5 years, and atrial arrhythmias were classified as atrial flutter/tachycardia in 85 (83%) and atrial fibrillation in 18 (17%). The median duration of follow-up from the first episode of atrial arrhythmia was 14.9 (12.1–17.3) years, and during this period 64 (62%) patients had recurrent atrial arrhythmias (atrial flutter/tachycardia 51 [80%] and atrial fibrillation 13 [20%]) with annual incidence of 4.4%. Older age was a risk factor for arrhythmia recurrence while the use of a class III anti-arrhythmic drug was associated with a lower risk of recurrent arrhythmias. The incidence of thromboembolic complication was 0.6% per year, and the cumulative incidence was 4% and 7% at 5 and 10 years respectively from the time of first atrial arrhythmia diagnosis. There were no identifiable risk factors for thromboembolic complications in this cohort. Conclusions: Although TCPC provides superior flow dynamics and lower risk of incident atrial arrhythmias, there is a significant risk of recurrent arrhythmias among TCPC patients with a prior history of atrial arrhythmias. These patients may require more intensive arrhythmia surveillance as compared to other TCPC patients.

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

Cardiac magnetic resonance imaging derived right ventricular (RV) volumes are often necessary for optimal timing of pulmonary valve replacement in patients with tetralogy of Fallot (TOF). This practice is based on previous studies that reported preoperative RV volumetric thresholds that predicted postoperative RV remodeling. As a result, pulmonary valve replacements are being performed even in asymptomatic patients based on RV volumetric thresholds that predict complete postoperative RVOT remodeling. Hence, RV volumes are now being used as surrogate markers/endpoints for future cardiovascular outcomes. Unfortunately, there are no studies showing survival benefit for performing pulmonary valve replacement at smaller RV volumes. This review underscores some of the limitations of using RV volumes as surrogate markers for clinical outcomes, and also highlights knowledge gaps about the pathophysiologic mechanism of cardiovascular death in the TOF population. Keywords: Right ventricular volumes, Tetralogy of fallot, Cardiovascular mortality

Background: Left heart filling pressures, as measured by pulmonary artery wedge pressure (PAWP), is associated with heart failure related mortality. Because of the prognostic importance of PAWP, several echocardiographic indices have been proposed for noninvasive assessment of PAWP. However, these indices have not been validated in the congenital heart disease population. The purpose of this study was to determine the correlation between echocardiographic indices of PAWP, and the effect of high PAWP on transplant-free survival in adults with tetralogy of Fallot (TOF). Methods: Retrospective study of adult TOF patients that underwent cardiac catheterization at Mayo Clinic, 1990–2017. We selected these pre-defined set of echocardiographic indices of LV diastolic function: mitral valve early velocity (E), mitral valve early and late velocity ratio (E/A), mitral valve deceleration time (DT), mitral annular tissue Doppler early velocity (e′), and left atrial volume index (LAVI). Results: Of the echocardiographic indices analyzed among 213 patients (age 37 ± 14 years), only E velocity (β = 5.83, standard error = 1.52, p 28 ml/m2 had sensitivity of 79% and specificity of 63% (AUC 0.712), and E velocity > 1.0 m/s had sensitivity of 66% and specificity of 89% (AUC 0.692), for detecting PAWP > 15 mmHg. LAVI > 28 ml/m2 was associated with reduced 10-year transplant-free survival (49% vs 90%, p

Background: Prevalence of pacemaker-induced cardiomyopathy (PICM) in adults with congenital heart disease is unknown. Tetralogy of Fallot (TOF) is a common diagnosis in the adult congenital heart disease population, and the purpose of this study was to determine association between frequent right ventricular (RV) pacing and temporal decrease in left ventricular ejection fraction (LVEF) from pre-implantation to 2-years post-implantation (LVEFpost-pre) in TOF patients. Methods: We studied TOF patients that received RV leads only (N = 51) and a reference group of 7 patients with atrial pacing or biventricular pacing. We defined PICM as a ≥10% decrease in LVEF resulting in LVEF 40%) and LVEFpost-pre. Results: PICM occurred in 2 (4%) of 51 patients in RV pacing group. LVEFpost-pre was +3% (95% confidence interval [CI] 0% to +5%) in the reference group and −4% (95% CI −11% to +2%) in RV pacing group. No significant difference occured in LVEFpost-pre between the reference group (LVEFpost-pre +3%) vs RV pacing ≤20% (LVEFpost-pre +1%) vs RV pacing 21–40% (LVEFpost-pre −3%) vs RV pacing >40% (LVEFpost-pre −5%), p = 0.318. There was also no association between frequent RV pacing and LVEFpost-pre, R2 = 0.307, p = 0.10. Conclusion: PICM occurred in 4% of TOF patients receiving RV pacing, and there was no association between frequent RV pacing and temporal decline in LVEF. Further studies are required to determine the long-term impact of RV pacing in the TOF population, and explore optimal treatment strategies. Keywords: Tetralogy of Fallot, Right ventricular pacing, Left ventricular cardiomyopathy

Background: Noninvasive cardiac output assessment is important for prognostication in patients with heart failure. Electrical velocimetry (EV), an impedance cardiography technique, can be used for noninvasive cardiac output assessment. The purpose of this study was to determine the feasibility, reproducibility and accuracy of cardiac output assessment by EV in adults with congenital heart disease (CHD). Methods: Cross-sectional study of CHD patients that had simultaneous cardiac output assessment by Fick and EV (using Cardiotronic monitor, Osypka Medical). We divided the cohort into: Group 1 patients (n = 54) had hemodynamic assessment at rest only, while Group 2 patients (n = 7) had assessment both at rest and peak exercise. Results: EV cardiac output assessment was feasible in 100% of the patients. There was good correlation between Fick-derived and EV-derived cardiac index (r = 0.89, p

Background Right atrial pressure (RAP), a composite metric of right ventricular diastolic function, volume status, and right heart compliance, is a predictor of mortality in patients with heart failure due to acquired heart disease. Because patients with tetralogy of Fallot (TOF) might have abnormal right atrial and ventricular mechanics caused by myocardial injury and remodeling, we hypothesized that RAP would be associated with disease severity and cardiovascular adverse events in this population. Methods and Results We performed a cohort study of adults with TOF who underwent right heart catheterization at the Mayo Clinic Rochester between 1990 and 2017. The objective was to determine the association between RAP and multiple domains of disease severity in TOF (percentage of predicted peak oxygen consumption, atrial or ventricular arrhythmia, and heart failure hospitalization), as well as cardiovascular adverse events, defined as sustained ventricular tachycardia, resuscitated or aborted sudden death, heart transplantation, or death. Among 225 patients (113 male; mean age: 39±14 years), mean RAP was 10.7±5.2 mm Hg and median was 10 mm Hg (interquartile range: 7–13 mm Hg). Increasing RAP was associated with atrial or ventricular arrhythmias (odds ratio: 5.01; 95% CI, 1.22–23.49; P

Background Premature coronary artery disease (CAD) is common in patients with coarctation of aorta (COA), but there are limited data about any direct relationship (or lack thereof) between COA and CAD. We hypothesized that atherosclerotic cardiovascular disease risk factors, rather than COA diagnosis, was the primary determinant of CAD occurrence in patients with COA. Methods and Results This is a retrospective study of 654 COA patients and a control group of 876 patients with valvular pulmonic stenosis and tetralogy of Fallot to determine prevalence and independent risk factors for CAD. There was no evidence of a difference in the unadjusted CAD prevalence between the COA and control groups (7.8% versus 6.3%, P=0.247), but premature CAD was more common in COA patients (4.4% versus 1.8%, P=0.002). In the analysis of a propensity‐matched cohort of 126 COA and 126 control patients, there was no evidence of a difference in overall CAD prevalence (6.3% versus 5.6% versus P=0.742) and premature CAD prevalence (4.8% versus 3.2%, P=0.518). The multivariable risk factors for CAD were hypertension (odds ratio [OR] 2.14; 95% CI 1.36–3.38), hyperlipidemia (OR 3.33; 95% CI 2.02–5.47), diabetes mellitus (OR 1.98; 95% CI 1.31–3.61), male sex (OR 2.05; 95% CI 1.33–3.17), and older age per year (OR 1.06; 95% CI 1.04–1.07). Conclusions After adjusting for atherosclerotic cardiovascular disease risk factors, we did not find evidence of a difference in CAD risk between the patients with COA and other patients with congenital heart disease.

Background The purpose of this study was to compare the incidence of pregnancy‐related adverse outcomes (PRAO) between patients with versus without hemodynamically significant right ventricle outflow tract (RVOT). Methods and Results This was a retrospective cohort study of all pregnant patients with isolated RVOT lesions undergoing evaluation at the Mayo Clinic, 1990 to 2017. Hemodynamic significance was defined as ≥moderate pulmonary/conduit stenosis (≥3 m/s) and/or ≥moderate regurgitation. Patients with concomitant significant left heart disease were excluded. PRAO was defined as cardiovascular, obstetric, and/or neonatal complications occurring during the pregnancy through 6 weeks postpartum. A total of 224 pregnancies in 114 patients with RVOT lesions were identified; 38 pregnancies occurred in 24 patients with hemodynamically significant RVOT. Forty‐eight (21%) pregnancies ended in spontaneous abortion. Of the 173 completed pregnancies, median gestational age at delivery was 38 (35–40) weeks and median birth weight 2965 (2065–4122) g. Seven pregnancies (4%) were complicated by cardiovascular events, 14 (8%) by obstetric complications, with adverse neonatal outcomes occurring in 38 (22%). There were no maternal deaths. The incidence of spontaneous abortion and PRAO were similar in both the RVOT and hemodynamically significant RVOT groups. As an isolated condition, Tetralogy of Fallot–pulmonary atresia was associated with spontaneous abortion and neonatal complications. Conclusions The risk of cardiovascular complications was low in patients with isolated RVOT lesions, and hemodynamically significant RVOT lesions were not associated with either cardiovascular complications or PRAO. Further studies are required to explore the factors responsible for PRAO in patients with Tetralogy of Fallot–pulmonary atresia.

Background A previous study reported histologic abnormalities in the pulmonary artery (PA) of patients with tetralogy of Fallot (TOF). However, the potential effect of these anatomical findings on PA vascular function has not been studied. We hypothesized that TOF patients had abnormal PA vascular function, and that PA vascular function was associated with exercise capacity. Methods and Results This a study of adult TOF patients who had cardiac magnetic resonance imaging and echocardiogram (on the same day) at Mayo Clinic, 2002–2015. In order to test the study hypothesis, we compared PA elastance index (PAEi) between 207 TOF patients and a referent group of 8 subjects without structural heart disease. PAEi was calculated as a quotient of PA systolic pressure and cardiac magnetic resonance imaging–derived right ventricular stroke volume. Mean age was 33±13 and 36±4 years in the TOF and referent groups respectively. TOF patients had higher PAEi compared with the referent group (0.62±0.12 versus 0.48±0.08 mm Hg/mL/m2; P=0.001). There was a good correlation between PAEi and peak oxygen consumption (adjusted R2=0.73; r=0.85; P

Background Available outcomes data for anticoagulation therapy in adults with congenital heart disease (CHD) provide assessment of global risk of this therapy for CHD patients (a heterogeneous population), but the risk of complications for the different CHD diagnoses is unknown. The purpose of the study was to describe the indications for anticoagulation, and the incidence and risk factors for major bleeding complication in adults with tetralogy of Fallot. Methods and Results We queried Mayo Adult Congenital Heart Disease (MACHD) database for tetralogy of Fallot patients (aged ≥18 years) that received anticoagulation, 1990–2017. Of 130 patients (42±14 years, 75 men [58%]), warfarin and direct oral anticoagulants were used in 125 (96%) and 5 (4%), respectively because atrial arrhythmias (n=109), mechanical prosthetic valve (n=29), intracardiac thrombus (n=4), pulmonary embolism (n=6), stroke (n=3), and perioperative anticoagulation (n=44). The median hypertension, abnormal renal or liver function; stroke; bleeding history or predisposition; labile international normalized ratio; elderly (>65 years); drug or alcohol use score for the entire cohort was 1 (0–2) and 27 (21%) had hypertension, abnormal renal or liver function; stroke; bleeding history or predisposition; labile international normalized ratio; elderly (>65 years); drug or alcohol use score ≥2. There were 14 minor bleeding events (1.6% per year) and 11 major bleeding events (1.3% per year) in 8 patients during median follow‐up of 74 months (856 patient‐years). Mechanical prosthesis (hazard ratio 1.78, CI 1.29–3.77, P=0.021) and hypertension, abnormal renal or liver function; stroke; bleeding history or predisposition; labile international normalized ratio; elderly (>65 years); drug or alcohol use score ≥2 (hazard ratio 1.41, CI 1.03–3.88, P=0.046) were risk factors for major bleeding events. All‐cause mortality was higher in patients with major bleeding events (n=6, 75%) compared with patients without major bleeding events (n=25, 21%), P=0.001. Conclusions Considering the heterogeneity of the CHD population, data from the current study may be better suited for clinical decision‐making in tetralogy of Fallot patients.

Background Thoracic aortic aneurysm is common in patients with tetralogy of Fallot (TOF); the incidence of thoracic aortic dissection (TAD) is unknown, but generally considered to be uncommon. The purpose of this study was to determine incidence and risk factors for TAD in TOF patients. Methods and Results This work is a retrospective review of the National Inpatient Sample (NIS) database for cases of ascending TAD among all hospital admissions in adults with TOF, 2000–2014. Of 18 353 admissions in TOF patients, 11 (0.06%; 6 per 10 000 admissions) of these were TAD‐related admissions. For the TAD‐related admissions, mean age was 49.8±7.2 years; aortic surgical interventions were performed during 8 of the admissions, and overall in‐hospital mortality was 45% (5 of 11). Risk factors associated with TAD‐related admission were age >60 years (odds ratio, 2.41; 95% CI, 1.23–3.25; P=0.013), male sex (odds ratio, 6.91; 95% CI, 4.85–8.54; P

Background Patients with tetralogy of Fallot (TOF) remain at risk for cardiovascular events despite successful repair. Some of the current risk stratification tools require advanced imaging and invasive studies, and hence are difficult to apply to routine patient care. A recent study showed that QRS fragmentation (QRS‐f) is predictive of mortality in patients with TOF. The current study aims to validate this result by assessing whether severity of QRS‐f could predict all‐cause mortality in a different TOF population. Methods and Results The authors reviewed the Mayo Adult Congenital Heart Disease database for patients with TOF who had ECG from 1990–2017. QRS‐f was defined as notches in QRS complex in ≥2 contiguous leads on ECG, not related to bundle branch block, and classified as none, mild (≤3 leads), moderate (4 leads), or severe (≥5 leads). Of 465 patients (age 37±14 years) in the study, QRS‐f was present in 161 (35%): mild (n=43, 9%), moderate (n=77, 17%), and severe (n=41, 9%). There were 55 deaths (12%) during 13.6±8.2 years of follow‐up. Severity of QRS‐f remained an independent predictor of all‐cause mortality after adjustment for other ECG parameters, patient demographics, and atrial and ventricular arrhythmia (hazard ratio, 1.74 per class; 95% confidence interval, 1.08–2.93 [P=0.041]). Conclusions The presence of severe QRS‐f may be used as complementary data to the usual clinical indices to determine whether interventions such as invasive electrophysiology study should be performed in patients with nonsustained ventricular tachycardia or to proceed with pulmonary valve replacement in patients with severe pulmonary regurgitation with ventricular volumes below the guideline‐directed threshold for intervention.

Racial variability in certain prenatal risk factors, such as prenatal vitamin supplementation and termination of pregnancy for fetal anomaly, has altered the racial prevalence of birth defects. Analysis of a single large representative population is required to analyze current racial differences in the prevalence of birth defects in the United States. Methods: This is a population-based cross-sectional study to analyze racial differences in the prevalence of birth defects. We reviewed all live births in the 2008 Nationwide Inpatient Sample (NIS) database and determined birth prevalence of 55 selected birth defects in Caucasians. We then calculated the relative risk of these birth defects in African–Americans, Hispanics, and Asians relative to Caucasians. Result: Overall birth defect prevalence was 29.2 per 1000 in a cohort of 1,048,252 live births, of which 51% were Caucasians. Compared with Caucasians, the risk of overall birth defects was lower in African–Americans (relative risk = 0.9, confidence interval 0.8–0.9) and Hispanics (relative risk = 0.9, confidence interval 0.8–0.9). The risk of overall birth defects was similar in Caucasians and Asians. Relative to the Caucasians, African–Americans had a lower risk of cardiac, genitourinary, and craniofacial malformations but a higher risk of musculoskeletal malformations. Hispanics had a lower risk of genitourinary and gastrointestinal malformation. Asians had a higher risk of craniofacial and musculoskeletal malformations. Conclusion: This is a comprehensive description of racial differences in the risk of birth defects in the United States. Observed racial differences in the risk of birth defects may be related to genetic susceptibilities, to cultural or social differences that could modify exposures, or to the many potential combinations between susceptibilities and exposures.