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Objectives: The role of timing of coronary artery bypass grafting after acute myocardial infarction on early and late outcomes remains uncertain. Methods: We reviewed 1631 consecutive adult patients who underwent isolated coronary artery bypass grafting with information on timing of acute myocardial infarction. Early and late mortality were compared between patients receiving coronary artery bypass grafting within 24 hours after acute myocardial infarction, between 1 and 7 days after acute myocardial infarction, and more than 7 days after acute myocardial infarction. Sensitivity analyses were performed in subgroups of patients with ST-segment elevation myocardial infarction or non–ST-segment elevation myocardial infarction, and other high-risk groups. Results: A total of 124 patients (5.7%) underwent coronary artery bypass grafting within 24 hours, 972 patients (51.2%) received coronary artery bypass grafting between 1 and 7 days after acute myocardial infarction, and 535 patients (43.2%) underwent coronary artery bypass grafting more than 7 days after acute myocardial infarction. Overall operative mortality was 2.7% with comparable adjusted early mortality among 3 groups. Over a median follow-up of 13.5 years (interquartile range, 8.9-17.1), compared with patients receiving coronary artery bypass grafting between 1 and 7 days after acute myocardial infarction, those receiving coronary artery bypass grafting at 7 days had greater adjusted risk for late overall mortality (hazard ratio, 1.39, 95% CI, 1.16-1.67; P 7 days had a higher risk of late mortality [hazard ratio, 1.38, 95% CI, 1.14-1.67, P

Background: Patients with hypertrophic cardiomyopathy (HCM) are at increased risk of developing cardiac arrhythmias and have a high prevalence of cardiac implantable electronic device (CIED) use. Tricuspid regurgitation (TR) is a potential complication of device leads and can be severe enough to prompt surgical intervention. Methods: We identified 21 consecutive patients who underwent tricuspid valve (TV) surgery for device lead-induced TR late following septal myectomy (SM) for obstructive HCM. The primary endpoint was long-term all-cause mortality. Results: The median patient age was 63 years (range, 55-71 years), 19 patients (91%) had New York Heart Association class III or IV limitation, and all patients were receiving diuretics for right heart failure. The median interval between device implantation and TV surgery was 4 years (range, 1.5-8.5 years). Eight patients (38%) underwent pacemaker implantation due to complete heart block following SM. Preoperatively, TR was severe in 81% of the patients. The primary mechanism of lead-induced TR was leaflet impingement without adherence (n = 15; 75%). Nine patients (43%) underwent TV replacement, and 12 patients (57%) underwent repair. Only 1 patient died early postoperatively. Patients with lead-induced TR had markedly reduced long-term survival compared to the overall population of patients undergoing SM; 5-year survival was 58%, compared to 96% for the contemporary SM group. Conclusions: Late lead-induced TR is a potential complication of CIEDs in patients with HCM who have undergone SM. Although TV repair and replacement can be done with acceptable early mortality, late patient survival is poor.

Papillary fibroelastomas (PFEs) are small, slowly growing benign cardiac tumors with clinically significant risk of embolization. Surgical excision is the definitive treatment of symptomatic PFE and is conventionally performed through a median sternotomy. In this study, we report a series of 12 patients, who underwent robotic-assisted PFE removal at the Mayo Clinic. PFE involved the mitral valve, left atrium, and tricuspid valve. No major complications occurred after the procedure, and most patients were discharged 4 days after the surgery. On follow-up, 1 patient demonstrated pericarditis.

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.

Objective: To assess risks and benefits of cardiac intervention in adults with Down syndrome (DS). Patients and Methods: A retrospective review was conducted using data from a study we published in 2010. Patients aged 18 years or older with DS who underwent cardiac operation or percutaneous intervention from February 2009 through April 2022 (new cohort) were compared with patients in the previous study (January 1969 through November 2007; remote cohort) at Mayo Clinic. Results: In total, 81 adults (43 men; 38 women) with DS underwent 89 cardiac interventions (84 surgical; 5 percutaneous) at a mean age of 33 years. Twenty-six patients presented with complete atrioventricular canal defect (17%) or tetralogy of Fallot (15%). The most common adult procedures were valve interventions: mitral (31%), tricuspid (15%), and pulmonary (12%). Of pulmonary valve interventions in the new cohort, 33% were performed percutaneously. The postoperative mortality rate was low (1% total). The mean time between last operation and death was 16 years. Conclusion: Adults with DS can undergo cardiac operation and percutaneous intervention with low morbidity and mortality risk and good long-term survival.

Abstract Hypertrophic cardiomyopathy (HCM) is a genetically heterogenous condition with about half of cases remaining genetically elusive or non-genetic in origin. HCM patients with a positive genetic test (HCMSarc) present earlier and with more severe disease than those with a negative genetic test (HCMNeg). We hypothesized these differences may be due to and/or reflect proteomic and phosphoproteomic differences between the two groups. TMT-labeled mass spectrometry was performed on 15 HCMSarc, 8 HCMNeg, and 7 control samples. There were 243 proteins differentially expressed and 257 proteins differentially phosphorylated between HCMSarc and HCMNeg. About 90% of pathways altered between genotypes were in disease-related pathways and HCMSarc showed enhanced proteomic and phosphoproteomic alterations in these pathways. Thus, we show HCMSarc has enhanced proteomic and phosphoproteomic dysregulation observed which may contribute to the more severe disease phenotype.

Objective: To investigate the in-hospital outcomes of acute ischemic stroke in patients with hypertrophic cardiomyopathy (HCM). Patients and Methods: Using weighted discharge data from the National Inpatient Sample, we identified 5804 nonelective hospitalizations for ischemic stroke in adult patients with HCM between 2011 and 2017. For comparison, 58,179 hospitalizations for ischemic stroke in adult patients without HCM were selected as controls using the simple random sampling method. Results: Compared with the patients without HCM, those with HCM had a higher prevalence of hyperlipidemia (62.4% vs 57.5%, respectively, P

Background: Sleep-disordered breathing (SDB) is a risk factor for heart failure symptoms in patients with hypertrophic cardiomyopathy (HCM). However, the relationship between preoperative SDB and postoperative functional status after septal myectomy (SM) is unknown. In this study, we investigated the association of preoperative SDB with late self-reported health status among patients undergoing SM for obstructive HCM. Methods: Prior to SM, an overnight pulse oximetry test was used to measure oxygen desaturation index (ODI), which indicates the average number of desaturation episodes with at least 4% of drops in oxygen level from baseline per hour of sleep. Patients reported postoperative function through a questionnaire-based survey completed 3-5 years following SM. Results: We analyzed 264 patients who underwent transaortic SM from November 2005 through April 2016. On predischarge transthoracic echocardiography post-procedure, no significant difference was present in the extent of mitral valve regurgitation (P = 0.524), left ventricular outflow tract gradient (P = 0.405), or septal thickness (P = 0.744) related to ODI. At 3 to 5 years after their operation, 236 patients (89%) reported good or excellent health. Approximately 18% (n = 48) and 8% (n = 22) of the cohort continued to experience exertional dyspnea and chest tightness, respectively, on walking 2 blocks or less. A greater ODI was not an independent predictor of worse health status or residual symptoms. Conclusions: Relief of left ventricular outflow tract obstruction in patients with obstructive HCM improves symptoms, with 89% of patients reporting good or excellent health. Preoperative SDB is not significantly associated with late functional status after septal myectomy. Résumé: Contexte: Les troubles respiratoires du sommeil (TRS) sont un facteur de risque d’insuffisance cardiaque chez les patients atteints de cardiomyopathie hypertrophique (CMH). Toutefois, la relation entre la présence préopératoire d’un TRS et le statut fonctionnel postopératoire après une myectomie septale (MS) est inconnue. Dans notre étude, nous avons examiné l’association entre les TRS préopératoires et le statut de santé à long terme, tel qu’autodéclaré par les patients ayant subi une MS dans le traitement de la CMH. Méthodologie: Un test nocturne d’oxymétrie de pouls, réalisé avant la MS, a été utilisé pour déterminer l’indice de désaturation en oxygène (IDO), qui indique le nombre moyen d’épisodes de désaturation avec une baisse d’oxygène d’au moins 4 % sous le niveau de base, par heure de sommeil. Les patients ont rapporté leur fonction postopératoire au moyen d’un sondage sous forme de questionnaire, rempli de 3 à 5 ans après la MS. Résultats: Les données de 264 patients ayant subi une MS transaortique entre novembre 2005 et avril 2016 ont été analysées. À l’échocardiographie transthoracique postopératoire réalisée avant le congé hospitalier, aucune différence significative liée à l’IDO n’a été notée quant à l'insuffisance de la valve mitrale (p = 0,524), au gradient de la voie d’éjection du ventricule gauche (p = 0,405), ou à l’épaisseur du septum (p = 0,744) des patients. Trois à cinq ans après l’intervention chirurgicale, 236 patients (89 %) ont rapporté être en bonne ou en excellente santé. Environ 18 % (n = 48) et 8 % (n = 22) des patients de la cohorte présentaient encore une dyspnée à l’effort et une oppression thoracique, respectivement, après une marche de deux coins de rue ou moins. Un IDO plus élevé ne constituait pas un facteur prédictif indépendant pour un état de santé plus défavorable ou pour la présence de symptômes résiduels. Conclusions: Le traitement d’une obstruction du sang par le ventricule gauche chez les patients présentant une CMH obstructive permet d’améliorer leurs symptômes, et 89 % des patients rapportent par la suite être en bonne ou en excellente santé. Les TRS préopératoires ne sont pas associés de manière significative au statut fonctionnel postopératoire après une myectomie septale.

A 17-year-old male elite athlete presented for evaluation after an abnormal pre-competitive college screening electrocardiogram. Subsequent evaluation revealed the presence of hypertrophic cardiomyopathy. He remained asymptomatic throughout four years of follow-up. Through shared decision making, he continued to play competitively and is now a professional athlete. (Level of Difficulty: Advanced.)

Congenital heart diseases, including single ventricle circulations, are clinically challenging due to chronic pressure overload and the inability of the myocardium to compensate for lifelong physiological demands. To determine the clinical relevance of autologous umbilical cord blood–derived mononuclear cells (UCB-MNCs) as a therapy to augment cardiac adaptation following surgical management of congenital heart disease, a validated model system of right ventricular pressure overload due to pulmonary artery banding (PAB) in juvenile pigs has been employed. PAB in a juvenile porcine model and intramyocardial delivery of UCB-MNCs was evaluated in three distinct 12-week studies utilizing serial cardiac imaging and end-of-study pathology evaluations. PAB reproducibly induced pressure overload leading to chronic right ventricular remodeling including significant myocardial fibrosis and elevation of heart failure biomarkers. High-dose UCB-MNCs (3 million/kg) delivered into the right ventricular myocardium did not cause any detectable safety issues in the context of arrhythmias or abnormal cardiac physiology. In addition, this high-dose treatment compared with placebo controls demonstrated that UCB-MNCs promoted a significant increase in Ki-67-positive cardiomyocytes coupled with an increase in the number of CD31+ endothelium. Furthermore, the incorporation of BrdU-labeled cells within the myocardium confirmed the biological potency of the high-dose UCB-MNC treatment. Finally, the cell-based treatment augmented the physiological adaptation compared with controls with a trend toward increased right ventricular mass within the 12 weeks of the follow-up period. Despite these adaptations, functional changes as measured by echocardiography and magnetic resonance imaging did not demonstrate differences between cohorts in this surgical model system. Therefore, this randomized, double-blinded, placebo-controlled pre-clinical trial establishes the safety of UCB-MNCs delivered via intramyocardial injections in a dysfunctional right ventricle and validates the induction of cardiac proliferation and angiogenesis as transient paracrine mechanisms that may be important to optimize long-term outcomes for surgically repaired congenital heart diseases.

Background This study evaluated the role of supplementing Society of Thoracic Surgeons (STS) risk models for surgical aortic valve replacement with machine learning (ML). Methods and Results Adults undergoing isolated surgical aortic valve replacement in the STS National Database between 2007 and 2017 were included. ML models for operative mortality and major morbidity were previously developed using extreme gradient boosting. Concordance and discordance in predicted risk between ML and STS models were defined using equal‐size tertile‐based thresholds of risk. Calibration metrics and discriminatory capability were compared between concordant and discordant patients. A total of 243 142 patients were included. Nearly all calibration metrics were improved in cases of concordance. Similarly, concordance indices improved substantially in cases of concordance for all models with the exception of deep sternal wound infection. The greatest improvements in concordant versus discordant cases were in renal failure: ML model (concordance index, 0.660 [95% CI, 0.632–0.687] discordant versus 0.808 [95% CI, 0.794–0.822] concordant) and STS model (concordance index, 0.573 [95% CI, 0.549–0.576] discordant versus 0.797 [95% CI, 0.782–0.811] concordant) (each P

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.

Myocardial dysfunction after Fontan palliation for univentricular congenital heart disease is a challenging clinical problem. The medical treatment has a limited impact, with cardiac transplant being the ultimate management step. Cell-based therapies are evolving as a new treatment for heart failure. Phase 1 clinical trials using regenerative therapeutic strategies in congenital heart disease are ongoing. We report the first case of autologous bone marrow–derived mononuclear cell administration for ventricular dysfunction, 23 years after Fontan operation in a patient with hypoplastic left heart syndrome. The cells were delivered into the coronary circulation by cardiac catheterization. Ventricular size decreased and several parameters reflecting ventricular function improved, with maximum change noted 3 months after cell delivery. Such regenerative therapeutic options may help in delaying and preventing cardiac transplant.

The purpose of this case is to describe the complex perioperative management of a 30-year-old woman with congenital heart disease and multiple resternotomies presenting with pulmonary homograft dysfunction and evaluation for percutaneous pulmonary valve replacement. Transvenous, transcatheter Melody valve placement caused left main coronary artery occlusion and cardiogenic shock. An Impella ventricular assist device (VAD) provided rescue therapy during operating room transport for valve removal and pulmonary homograft replacement. ECMO support was required following surgery. Several days later during an attempted ECMO wean, her hemodynamics deteriorated abruptly. Transesophageal and epicardial echocardiography identified pulmonary graft obstruction, requiring homograft revision due to large thrombosis. This case illustrates a role for Impella VAD as bridge to definitive procedure after left coronary occlusion and describes management of complex perioperative ECMO support challenges.