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Background Conduction abnormalities including bundle branch block are recognized sequelae following septal myectomy in patients with hypertrophic cardiomyopathy, but their incidence and effect on long-term mortality have not been well studied. Objectives This study was conducted to determine the impact of conduction abnormalities on mortality in patients with obstructive hypertrophic cardiomyopathy following myectomy. Methods The outcomes of 2,482 patients with obstructive hypertrophic cardiomyopathy who underwent transaortic septal myectomy from 1961 to 2016 were analyzed. Pre-operative and early post-operative electrocardiograms were reviewed to determine conduction status. The study endpoint was all-cause mortality. Results Mean age at operation was 54.2 ± 14.6 years, and 1,370 patients (55.2%) were men. Pre-operatively, 2,159 patients (87.0%) had normal conduction, of whom 38.8% developed left bundle branch block, 1.1% developed right bundle branch block (RBBB), and 0.6% had complete heart block (CHB) after myectomy. Among 112 patients with baseline RBBB, 34.8% developed CHB post-operatively. Overall, only 2.3% of patients developed CHB. Over a median follow-up period of 8.6 years, overall mortality differed among post-operative conduction groups after adjustment for age, sex, and concomitant procedures (p = 0.015). Specifically, mortality in those with paced rhythm after myectomy was significantly increased relative to those with normal conduction (hazard ratio: 1.57; 95% confidence interval: 1.15 to 2.14; p = 0.005), although no significant difference was observed in left bundle branch block and RBBB (vs. normal) groups. Conclusions Left bundle branch block is a common sequela after septal myectomy but does not influence post-operative mortality. Myectomy-related CHB is rare in patients with baseline normal conduction, while pre-existing RBBB greatly increases the risk for CHB. Persistent paced rhythm after operation is an independent predictor of mortality.

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.

Objective:To evaluate the health status and quality of life of young patients who had cone reconstruction for Ebstein anomaly.Methods:We reviewed all patients who had cone reconstruction from 2007 to 2016 at our institution. Prospective surveys were mailed to all eligible patients. Quality of life was assessed using the PedsQL 4.0 Generic Core Scales, including four domains: physical, emotional, social, and school functioning.Results:Of 116 eligible patients, 72 (62%) responded. About 96% reported their health as excellent or good, and 52% were symptom-free. Only 37% of patients were taking any medications, the most common of which was aspirin (30%). Only 19% had been hospitalised for cardiac reasons following cone reconstruction. The average self-reported quality of life was 85.3/100, whereas the average parent proxy-reported quality of life was 81.8/100. There was no difference by self or parent proxy-report in quality of life between cone reconstruction patients and healthy children; however, quality of life was significantly better compared with children with other chronic health conditions. By self-report and parent proxy-report, 15.1 and 16.7% of patients were deemed “at risk” for reduced quality of life, respectively. Socially, 63/64 (98%) patients over 5 years old were either full-time students or working full-time.Conclusion:Children with Ebstein anomaly following cone reconstruction have excellent quality of life comparable with healthy peers and significantly better than other children with chronic health conditions. Families of children with Ebstein anomaly can expect excellent quality of life, long-term health status, and social functioning following cone reconstruction.

ObjectiveThis study aimed to identify characteristics, spectrum of tricuspid regurgitation (TR) severity and treatment patterns in patients considered for intervention of severe TR at a tertiary centre. The population being considered for TR intervention is currently not well defined and the role of transcatheter interventions is unclear.MethodsThe study involved 87 patients with severe TR considered for intervention from 1 March 2016 to 12 November 2018 at Mayo Clinic. Patients receiving medications alone were compared with those receiving intervention to identify patterns in demographics, clinical/echocardiographic associations and survival.ResultsMean age was 80±9 (56% female), 93% had atrial fibrillation and 64% had chronic kidney disease ≥3 a. Follow-up was 331±276 days; 95% were symptomatic with 6 min walk distance of 270±110 m. Loop diuretics were used in 93%; aldosterone antagonists in 35%. Mean tricuspid annular plane systolic excursion was 15.6±3.8 mm, effective regurgitant orifice area (EROA) 82±32 mm2 and stroke volume index 39±11 mL/m2; 48% had at least moderate right ventricular (RV) dysfunction, and 75% did not undergo intervention. Patients receiving intervention showed trends towards larger EROA (93±33 vs 75±31 mm2), better right ventricular function and more severe symptoms. Overall group 30-day and 1-year survival were 100% and 76%, respectively.ConclusionsPatients with severe TR considered for intervention are commonly elderly with atrial fibrillation, advanced TR and RV dysfunction; 75% were treated with medications alone and not offered intervention. Patients with greater EROA, better RV function and more severe symptoms were more likely to receive intervention. These findings have implications for future trial design.

[Extract] The 2018 ACHD guideline is a full revision of the “2008 ACC/AHA Guidelines for the Management of Adults with Congenital Heart Disease” (S1.4-1), which was the first U.S. guideline to be published on the topic. This revision uses the 2008 ACHD guideline as a framework and incorporates new data and growing ACHD expertise to develop recommendations. Congenital heart disease (CHD) encompasses a range of structural cardiac abnormalities present before birth attributable to abnormal fetal cardiac development but does not include inherited disorders that may have cardiac manifestations such as Marfan syndrome or hypertrophic cardiomyopathy. Also not included are anatomic variants such as patent foramen ovale. Valvular heart disease (VHD) may be congenital, so management overlaps with the “2014 AHA/ACC Guidelines for the Management of Patients With Valvular Heart Disease” (S1.4-2), particularly for bicuspid aortic valve (BAV) disease. Where overlap exists, this document focuses on the diagnosis and treatment of congenital valve disease when it differs from acquired valve disease, whether because of anatomic differences, presence of concomitant lesions, or differences to consider given the relatively young age of patients with ACHD. This guideline is not intended to apply to children (

The appropriate implementation of new technology, root cause analysis of "imperfect" outcomes, and the continuous reappraisal of postgraduate training are needed to improve the care of tomorrow's patients. Healthcare delivery remains one of the most expensive sectors in the United States, and the application of new and expensive technology that is necessary for the advancement of this complex specialty must be aligned with providing the best care for our patients. There are a several pathways to innovation: One is partnering with industry and the other is the investigational laboratory. Innovation and the funding thereof come from both the public and private sector. Most new trials that are likely to impact cardiothoracic surgery are industry-sponsored trials to meet the requirements necessary for regulatory approval. Cost considerations are paramount when considering integration of innovative technology and treatments into a clinical cardiothoracic surgical practice. The value of any new innovation is determined by the quality divided by the cost, and lean initiatives maximize this equation. The importance and implications of conflict of interest have been a concern for physicians, particularly when new technology or procedures are incorporated into clinical practice, and full disclosures by medical professionals and others involved are essential. Our societies and associations provide a platform for presentation and peer-reviewed discussion of new procedures, innovations, and trials and provide a venue for the sharing of knowledge on the highest quality patient care through education and research.

Background Surgical mitral valve repair is the conventional treatment for severe degenerative mitral regurgitation (MR). MitraClip therapy has emerged as a viable option in high-risk surgical patients. We sought to compare conventional surgery to MitraClip therapy in patients with severe degenerative mitral valve prolapse (MVP) and previous cardiac interventions. Methods From January 2012 to May 2016, 131 patients with previous cardiac surgery and subsequent intervention for degenerative MVP were included in this analysis: 75 (57.3%) underwent surgical repair and 56 (42.7%) underwent MitraClip placement. Follow-up was available in all early survivors at median of 11 (interquartile range, 0 to 32) months for surgery and 11 (interquartile range, 3 to 21) months for MitraClip patients. Results MitraClip patients were older (75.7 ± 8.6 years of age versus 68.6 ± 13.1 of age; p Conclusions In patients with previous cardiac interventions and severe degenerative MVP, a repeat conventional surgery is safe and durable. Percutaneous MitraClip repair is effective but associated with higher risk of residual MR, and should only be considered in selected patients. Careful patient selection using a heart team approach is recommended.

Background The Hospital Consumer Assessment of Healthcare Providers and Systems (HCAHPS) is a publicly reported survey of patient experience with in-hospital care. We reviewed institutional HCAHPS survey data to assess our patients’ experiences after cardiac surgery and to identify targets for practice improvement. Methods We reviewed data from patients undergoing the most common cardiac operations, with dismissal from October 1, 2012 to September 30, 2015. We used top-box methodology to combine survey results into nine domains, including the global (composite) hospital rating, dichotomized as high versus low. Multivariable logistic regression analysis was used to evaluate the independent associations of variables with low global ratings. Key driver analysis using domain means and Spearman correlation between the global rating and the eight other domains identified targets for quality improvement. Results Among 1,315 surveyed patients a low global hospital rating was independently associated with low perceived overall health (fair or poor vs excellent; odds ratio [OR], 5.4; p = 0.001), younger age (18 to 59 vs ≥70 years; OR, 1.6; p = 0.048), prolonged length of stay (OR, 1.6; p = 0.02), and robotic mitral valve (MV) repair (robotic vs open repair; OR, 2.4; p = 0.045). Patients undergoing transcatheter aortic valve replacement (TAVR) reported global ratings similar to that of patients with open aortic valve operations (OR, 0.9; p = 0.64). Key drivers of patient experience were care transitions and communication regarding medications. Conclusions The primary targets of improvement for our institution are care transitions and communications regarding medications. The less-invasive procedures (robotic MV repair, TAVR) were not independently associated with higher patient-experience scores.

Unicuspid aortic valve (UAV) is a rare malformation that is often difficult to distinguish from a bicuspid aortic valve (BAV) with commissural fusion by echocardiography or intraoperative surgical inspection. This study assessed the accuracy of intraoperative surgical inspection and two-dimensional echocardiography in diagnosing UAV compared to a gold standard of pathological diagnosis. The Mayo Clinic echocardiographic database, tissue registry database and electronic medical record were searched for all patients assigned a diagnosis of UAV by any technique. Transthoracic (TTE), transesophageal (TEE) echocardiographic, and surgical diagnoses were compared to pathological diagnosis as the standard. A clinical diagnosis of UAV was applied to 380 patients by 1 or more method and in 196 (52%) a pathologic evaluation was available to compare to the clinical description given by TTE, TEE, or surgical inspection. Of these 196 patients, only 58 (30%) had a pathological diagnosis of UAV; the majority were found to be BAVs by pathologic evaluation (n = 132, 67%). For diagnosing UAV, the sensitivity and specificity were 15% and 87% for TTE, 28%, and 82% for TEE, and 52% and 51% for surgical inspection, respectively. Valves with bicuspid morphology and extensive commissural fusion were frequently misclassified as UAV by all methods. In conclusion, intraoperative surgical inspection and echocardiography have limitations for diagnosing UAV due to difficulties in accurately assigning a correct morphological diagnosis, which suggests that the current understanding of the natural history of UAV may be inaccurate.

Background Mitral valve regurgitation (MR) mediated by systolic anterior motion (SAM) in obstructive hypertrophic cardiomyopathy (HCM) is traditionally characterized by a posteriorly directed jet on Doppler echocardiography. Many believe that MR in the absence of a posteriorly directed jet signals the presence of intrinsic mitral valve (MV) disease. Methods A total of 709 adult patients with obstructive HCM who underwent septal myectomy were evaluated; 330 of these patients had >2 + MR preoperatively and constituted the study group. SAM-mediated MR was defined as MR that was eliminated or substantially reduced by myectomy for relief of left ventricular outflow tract obstruction with no need for MV intervention. Results On preoperative transthoracic echocardiography, 168 of 258 patients with SAM-mediated MR and nine of 28 patients with intrinsic MV disease had isolated posterior jets, corresponding to sensitivity and specificity of 65.1% and 67.9% for identifying SAM-mediated MR; the positive predictive value was 94.9% and the negative predictive value was 17.4%. On prebypass transesophageal echocardiography, 169 of 284 patients with SAM-mediated MR and five of 28 patients with intrinsic MV disease had isolated posterior jets, corresponding to sensitivity and specificity of 59.5% and 82.1%; the positive predictive value and negative predictive value were 97.1% and 16.7%. Conclusion A posteriorly directed jet of MR in obstructive HCM correlates highly with SAM as the underlying pathophysiologic mechanism, but because of the low negative predictive value, clinicians should be cautious in using the jet direction of MR on preoperative transthoracic echocardiography to guide the decision for concomitant MV surgery during septal myectomy for HCM.

Background Mitral annular calcification (MAC) is associated with worse outcomes after mitral valve replacement (MVR). With limited data available on long-term outcomes, we reviewed our experience of MVR in presence of MAC. Methods A retrospective review of 1,710 consecutive patients who underwent MVR between January 2000 and December 2015 was performed. Patients with isolated primary MVR (n = 496) were included, whereas patients with concomitant cardiac surgery (n = 1,068), previous MVR (n = 110), and mitral valve (MV) endocarditis (n = 36) were excluded. MV calcification was classified as MAC present in anterior/posterior annulus and vertically at the level of leaflets/subvalvular apparatus. A conservative approach towards annular debridement was followed. Results Our sample's mean age was 64.4 ± 14.1 years, and included 279 (56%) women. MV calcification was observed in 169 (34%) patients with MAC in 115 (23%). Older age, higher ejection fraction, peripheral vascular disease, diabetes, dialysis, and previous aortic valve surgery were associated with increased prevalence of MAC. Patients with MV calcification had higher stroke rate (p = 0.040), patients with anterior leaflet and commissural calcification had higher pacemaker implantation (p = 0.010, p = 0.001, respectively), and patients with circumferential MAC had higher postoperative dialysis (p = 0.006). Operative mortality was not significantly different (p = 0.466) between MAC (n = 1, 1%) and non-MAC (n = 9, 2%) patients. MAC was associated with late mortality (unadjusted hazard ratio, 1.62; 95% confidence interval, 1.20 to 2.18), though on multivariable analysis age, diabetes, dialysis, hypertension, previous aortic valve surgery, previous coronary artery bypass grafting, and MVR with a bioprosthetic valve were found to be independent risk factors for mortality whereas MAC was not. Conclusions A conservative approach to treat MAC achieves satisfactory results. Patients with MAC have significant comorbidities contributing to a worse survival, though MAC in itself is not a risk factor for mortality.

Repair of Ebstein anomaly has evolved over the last decade, and timing of repair remains variable. There have been no studies of exercise or functional capacity in patients who have had tricuspid valve surgery for Ebstein anomaly in adulthood. We aimed to compare exercise capacity before and after tricuspid valve repair or replacement for Ebstein anomaly in adults at Mayo Clinic. We performed a retrospective chart review of all patients with Ebstein anomaly who underwent tricuspid valve surgery at Mayo Clinic between June 2007 and January 2015. We compared pre- and postoperative echocardiograms, exercise tests, and clinic visits. Tricuspid valve surgery was done for 322 patients, and 32 patients met criteria of native tricuspid valve repair or replacement at age 18 or older and had maximal pre- and postoperative exercise tests. Nineteen patients had valve repair, and 13 had valve replacement. Surgery for Ebstein anomaly resulted in significant reduction in tricuspid regurgitation and right ventricular size. There was a significant improvement in NYHA functional class after surgery; however, there was no significant improvement in functional aerobic capacity (FAC), metabolic equivalents (METs), exercise time, or [Formula: see text] after surgery. Patients who had an atrial shunt closed during surgery had improved minimum blood oxygen saturations during exercise, though no improvement in exercise capacity. In our cohort, patients who had tricuspid valve repair or replacement for Ebstein anomaly reported an improvement in functional capacity; however, this did not reflect improvement in measured exercise capacity, despite excellent surgical results by echocardiography.

There is a paucity of cardiac surgery outcomes data for patients with Noonan syndrome (NS). Our objective was to evaluate early results in these patients. Between January 1999 and December 2015, 29 patients (18 males, 62%) with NS underwent cardiac surgery at our institution. Mean age was 23 ± 17.9 years; 12 (41%) were under 18 years of age. Fourteen patients (48%) had prior sternotomies. The primary operations for the main diagnosis were pulmonary valve/conduit replacement/repair (n = 14, 48%), septal myectomy for obstructive hypertrophic cardiomyopathy (n = 7, 24%), aortic valve replacement/repair (n = 4, 14%), atrial septal defect (ASD) repair (n = 2, 7%), and cardiac transplantation (n = 2, 7%). Concomitant procedures were performed in 24 patients (83%), most commonly right ventricular outflow tract reconstruction (n = 13, 45%), mitral valve repair/replacement (n = 7, 24%), and ASD repair (n = 6, 21%). Mean bypass and cross-clamp times were 88.8 ± 51 minutes and 54.7 ± 67 minutes, respectively. There was 1 early death (3%). Postoperative morbidity occurred in 18 patients (62%), most commonly arrhythmias (n = 14, 48%) or respiratory insufficiency/pneumonia (n = 6, 21%). There were 2 early reoperations and 4 early readmissions. Univariate factors associated with morbidity included male gender (P = 0.03) and longer cross-clamp time (P = 0.02). Median length of hospital stay was 6 days (interquartile range 5-10.5 days). Patients with NS frequently have multiple cardiac lesions requiring a broad spectrum of operations. Early mortality is low despite procedure complexity. Although early postoperative morbidity is common, patients overall do well with reasonable hospital lengths of stay. Additional studies are needed to evaluate long-term outcomes and quality of life.

Fontan circulation leads to chronic elevation of central venous pressure. We sought to identify the incidence, risk factors, and survival among patients who developed acute kidney injury (AKI) after the Fontan operation. We retrospectively reviewed 1,166 patients who had Fontan operation/revision at Mayo Clinic Rochester from 1973 to 2017 and identified patients who had AKI (defined by AKI Network criteria) within 7 days of surgery. A total of 132 patients (11%) developed AKI after the Fontan operation with no significant era effect. Of those who developed AKI, severe (grade 3) kidney injury was present in 101 patients (76.5%). Multivariable risk factors for AKI were asplenia (odds ratio [OR] 4.2, p0.0001), elevated preoperative pulmonary artery pressure (per 1 mm Hg increase, OR 1.04, p = 0.0002), intraoperative arrhythmias (OR 1.9, p = 0.02), and elevated post-bypass Fontan pressure (per 1 mm Hg increase, OR 1.12, p = 0.0007). Renal replacement therapy (RRT) was used in 72 patients (54%), predominantly through peritoneal dialysis (n = 56, 78%). Multivariable risk factors for RRT were age ≤3 years (OR 9.7, p = 0.0004), female gender (OR 2.6, p = 0.02), and aortic cross-clamp time60 minutes (OR 3.1, p = 0.01). Patients with AKI had more postoperative complications, including bleeding, stroke, pericardial tamponade, low cardiac output state and cardiac arrest, than those without AKI. This resulted in longer intensive care unit stay (39 vs 17 days, p = 0.0001). In-hospital mortality was exceedingly higher among patients with AKI versus no AKI (58%, 76 of 132 vs 10%, 99 of 1,034, p0.0001); however, there was no significant difference based on the need for RRT. Recovery from AKI was observed in 56 patients (42%). Over 20-year follow-up, patients with AKI had a distinctly higher all-cause-mortality (82%) than those without AKI (35%). It is prudent to identity patients at a higher risk of developing postoperative AKI after Fontan operation to ensure renal protective strategies in the perioperative period. Postoperative AKI leads to substantial short and long-term morbidity and mortality, but the need for RRT does not affect the outcomes.

Patient-reported outcomes are important metrics of medical and surgical care. In this study, we investigated the prevalence and risk factors of patient-reported postdischarge atrial fibrillation (AF) after septal myectomy for obstructive hypertrophic cardiomyopathy.Patients undergoing transaortic septal myectomy from August 2001 to January 2017 were contacted regarding postdischarge AF through questionnaire-based surveys sent at 3, 5, and 10 years post procedure. For each patient, the most recent survey response was analyzed.Among 949 patients, 248 (26.1%) last responded at 3 years post procedure, 353 (37.2%) at 5 years, and 348 (36.7%) at 10 years. The overall incidence of patient-reported postdischarge AF was 34.4% (n = 326), and at 3, 5, and 10 years, the incidences were 22.2%, 34.8%, and 42.5% (P.001). After multivariable adjustment, history of preoperative AF (odds ratio [OR] 5.566, P.001), early postoperative AF within the first 30 days (OR 2.211, P.001), preoperative left atrial volume index (OR 1.014, P = .005), postoperative right ventricular systolic pressure (OR 1.021, P = .013), postoperative moderate or greater mitral valve regurgitation (OR 1.893, P = .022), and preoperative septal thickness (OR 1.043, P = .036) were independently associated with patient-reported postdischarge AF.The incidence of patient-reported postdischarge AF increases with increasing length of follow-up after septal myectomy. We identified several risk factors for late postdischarge AF that were associated with chronicity of left ventricular outflow tract obstruction, and earlier intervention may mitigate late atrial arrhythmias.

The Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) provides observed-to-expected (O/E) operative mortality ratios to more than 100 congenital heart centers in North America. We compared the current approach for estimating O/E ratios to approaches incorporating information on diagnosis as moderators of procedures, other unused risk factors, and additional variation in confidence interval construction to characterize center performance.Bayesian additive regression trees (BART) and lasso models linked operative mortality to diagnosis-procedure categories, procedure-specific risk factors, and syndromes/abnormalities. Bootstrapping accounted for variation in the STS-CHSD (STS bootstrap) and lasso CIs. We compared O/E estimates, interquartile range of CI widths, and concordance of center performance categorizations (worse-than-, as-, or better-than-expected mortality) of the new approaches to the STS-CHSD.In 110 surgical centers including 98,822 surgical operative encounters, there were 2818 (2.85%) operative mortalities (center range, 0.37%-10%). Compared with the STS-CHSD, BART- and lasso-estimated O/E ratios varied more and had narrower confidence intervals (interquartile range of confidence interval: STS-CHSD = 1.11, STS bootstrap = 0.98; lasso = 0.80; BART = 0.96). Concordance of performance categorization with the STS-CHSD ranged from 84% (lasso) to 91% (STS Bootstrap); more than 70% of discordant centers improved categories. Discordant centers had smaller volumes, fewer operative mortalities, and treated more patients with congenital lung abnormalities.Relative to the STS-CHSD, up to 16% of hospitals changed performance categories, most improving performance. Given the significance of quality reports for congenital heart centers, inclusion of additional risk factors and unaddressed variation should be considered.

The prevalence and clinical impact of mitral annular calcification (MAC) in patients with obstructive hypertrophic cardiomyopathy (HCM) are largely unknown.We reviewed 2113 HCM patients who underwent septal myectomy from January 2000 to April 2016. Preoperative and intraoperative echocardiograms along with operative notes were reviewed to identify MAC. Survival was estimated and compared using Kaplan-Meier analysis and the log-rank test. Cox regression analysis was used to identify factors independently associated with mortality.MAC was identified in 390 (18.5%) patients. Older age, female sex, and presence of mitral valve leaflet calcification were strongly associated with higher odds of having MAC. Patients with MAC had higher resting left ventricular outflow tract gradients, were more likely to have worse mitral regurgitation preoperatively, and were more likely to undergo a concomitant mitral valve replacement (6% vs 1%; P.001) compared with those without MAC. Postoperatively, patients with MAC had marginally higher residual mitral regurgitation (13% vs 8%). After a median follow-up of 6.95 (interquartile range, 3.7-12.1) years, survival of patients with MAC at 1, 5, and 10 years was 99%, 92%, and 69%, respectively. Adjusted analysis identified MAC as an independent predictor of poor survival (hazard ratio, 1.46; 95% confidence interval, 1.08-1.97; P = .014).MAC is a frequent finding in older patients with obstructive HCM, is more likely to be seen in females, and is associated with higher rates of concomitant mitral valve replacement. Despite the higher prevalence of comorbidities, MAC remained an independent predictor for overall mortality after septal myectomy.

Current bioprostheses are considered to have improved durability and better hemodynamic performance compared with previous designs, but there are limited comparative data on late outcomes.From 2009 through 2011, 300 adults with severe aortic valve stenosis undergoing aortic valve replacement (AVR) were randomly assigned to receive Edwards Magna, St Jude Epic, or Sorin Mitroflow bioprostheses (n = 100, n = 101, n = 99, respectively). Overall survival was analyzed using Kaplan-Meier and Cox proportional hazards methods, whereas competing risk analysis was used for all time-to-event outcomes. Serial echocardiographic data were fitted with longitudinal models stratified by implant valve size.During median follow-up of 9.8 years (interquartile range, 8.7-10.2), 10-year survival was 50% for the Magna group, 42% for the Epic group, and 41% for the Mitroflow group (P = .415). Cumulative risk of stroke was 9% at 10 years, and rates were comparable for the three groups. Indexed aortic valve area and mean gradients were similar among the three groups receiving 19 mm and 21 mm valves, but in larger (23 mm or more) prostheses, gradients were lower (P.001) and indexed aortic valve areas were higher in the Magna group (P.001). The 10-year risk of endocarditis differed by group (P = .033), with higher incidence in the Mitroflow vs the Magna group (7% vs 0%, P = .019). Late risk of reinterventions in the Mitroflow group was 22%, compared with 0% in the Magna group (P.001) and 5% in the Epic group (P = .008).The Magna valve had the lowest gradients and largest indexed aortic valve area with larger implant sizes. The Mitroflow bioprosthesis is associated with an increased rate of reintervention and possible increased risk of infection compared with Magna and Epic valves.

Substantial improvements in techniques of tracheal surgery for children have occurred in the past 20 years. Precise preoperative imaging with computed tomography clearly defines the anatomy for surgical planning and is assisted by on-the-table needle localization. The use of cardiopulmonary bypass greatly facilitates creation of an airtight, widely patent trachea. The use of Ciprodex as a postoperative nebulizer has significantly decreased granulation tissue along the suture line. Most important has been the adoption of slide tracheoplasty as the procedure of choice.

Objective: To identify risk factors for pediatric mechanical mitral valve replacement (mMVR) to improve management in this challenging population. Methods: From 1993 to 2019, 93 children underwent 119 mMVR operations (median age, 8.8 years [interquartile range [IQR]: 2.1-13.3], 54.6% females) at our institution. Twenty-six (21.8%) patients underwent mMVR at ≤2 years and 93 (78.2%) patients underwent mMVR at >2 years. Median follow-up duration was 7.6 years [IQR: 3.2-12.4]. Results: Early mortality was 9.7%, but decreased with time and was 0% in the most recent era (13.9% from 1993 to 2000, 7.3% from 2001 to 2010, 0% from 2011 to 2019, P = .04). It was higher in patients ≤2 years compared to patients >2 years (26.9% vs 2.2%, P < .01). On multivariable analysis for mitral valve reoperation, valve size 2 years and those with a prosthesis ≥23 mm. Median time to reoperation was 7 years (IQR: 4.5-9.1) in patients >2 years and 3.5 years (IQR: 0.6-7.1) in patients ≤2 years ( P = .0511), but was similar between prosthesis sizes ( P = .6). During follow-up period (median 7.6 years [IQR: 3.2-12.4], stroke occurred in 10%, prosthetic valve thrombosis requiring reoperation in 4%, endocarditis in 3%, and bleeding in 1%. Conclusion: Early and late outcomes of mMVR in children are improved when performed at age >2 years and with prosthesis size ≥23 mm. These factors should be considered in the timing of mMVR.

Surgical approaches for mitral valve (MV) disease have evolved with the aim of developing minimally invasive techniques. Although the safety of robotic procedures has been documented, there are limited data on long-term echocardiographic follow-up. This review demonstrates outcomes of 11 years of robotic MV repair at a single, tertiary institution.From 2008 to 2019, 843 patients underwent robotic MV repair at Mayo Clinic in Rochester, Minnesota. Repeated measures generalized least squares (GLS) modeling was used to assess the echocardiographic changes over time.The median age was 58 years (interquartile range, 50.8, 65.5 years), and 591 were male (70.1%). The mechanism of mitral regurgitation was posterior leaflet prolapse in 479 (56.8%) patients, bileaflet prolapse in 325 (38.6%), and anterior leaflet prolapse in 36 (4.3%). There were 3 early deaths (0.4%) and 24 early reoperations (2.8%). Echocardiographic follow-up demonstrated left ventricular end-systolic and end-diastolic dimensions, left atrial volume index, and pulmonary pressure all continuously improved up to 2 years postoperatively. Ejection fraction immediately declined postoperatively but then gradually improved to near normal over 2 years. Survival and freedom from reoperation at 10 years were 93% and 92.6%, respectively. When patients were surveyed after dismissal, 93.4% reported their activity level at or above their peers, and 93.3% reported no activity limitation from cardiac symptoms.Robotic MV repair is safe and effective with excellent long-term results, including echocardiographic data that demonstrated early improvement in cardiac chamber size and maintenance of postoperative cardiac function. Exceedingly low mortality rates and freedom from reoperation are comparable to those of the standard open repair.

The relationship between institutional volume and operative mortality after surgical aortic valve replacement (SAVR) remains unclear.From January 2013 to June 2018, 234 556 patients underwent isolated SAVR (n = 144 177) or SAVR with coronary artery bypass grafting (CABG) (n = 90 379) within the Society of Thoracic Surgeons Adult Cardiac Surgery Database. The association between annualized SAVR volume (group 1 [1-25 SAVRs], group 2 [26-50 SAVRs], group 3 [51-100 SAVRs], and group 4 [100 SAVRs]) and operative mortality and composite major morbidity or mortality was assessed. Random effects models were used to evaluate whether historical (2013-2015) SAVR volume or risk-adjusted outcomes explained future (2016-2018) risk-adjusted outcomes.The annualized median number of SAVRs per site was 35 (interquartile range, 22-59; isolated aortic valve replacement [AVR], 20; AVR with CABG, 13). Among isolated SAVR cases, the mean operative mortality and composite morbidity or mortality were 1.5% and 9.7%, respectively, at the highest-volume sites (group 4), with significantly higher rates among progressively lower-volume groups (P trend.001). After adjustment, lower-volume centers had increased odds of operative mortality (group 1 vs group 4 [reference]: adjusted odds ratio [AOR] for SAVR, 2.24 [95% CI, 1.91-2.64]; AOR for SAVR with CABG, 1.96 [95% CI, 1.67-2.30]) and major morbidity or mortality (AOR for SAVR, 1.53 [95% CI, 1.39-1.69]; AOR for SAVR with CABG, 1.46 [95% CI, 1.32-1.61]) compared with the highest-volume institutions. Substantial variation in outcomes was observed across hospitals within each volume category, and prior outcomes explained a greater proportion of hospital operative outcomes than did prior volume.Operative outcomes after SAVR with or without CABG is inversely associated with institutional procedure volumes; however, prior outcomes are more predictive of future outcomes than is prior volume. Given the excellent outcomes observed at many lower-volume hospitals, procedural outcomes may be preferable to procedural volumes as a quality metric.

Introduction: Ebstein anomaly (EA) is a rare congenital heart defect characterized by abnormalities of the tricuspid valve (TV) leaflets and right ventricular (RV) cardiomyopathy. The current treatment for most patients with EA is cone reconstruction (CR) surgery. However, timing of repair has been highly variable, ranging from infancy to adulthood. Historically, surgical intervention for EA in the presence of severe RV failure has been associated with a longer intensive care unit course, increased need for ECMO support and a longer duration of mechanical ventilation. We sought to evaluate contemporary early postoperative outcomes in all age groups of patients with Ebstein …

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.

Hypertrophic cardiomyopathy (HCM) is characterized by multiple pathological features including myocyte hypertrophy, myocyte disarray, and interstitial fibrosis.This study sought to correlate myocardial histopathology with clinical characteristics of patients with obstructive HCM and post-operative outcomes following septal myectomy.The authors reviewed the pathological findings of the myocardial specimens from 1,836 patients with obstructive HCM who underwent septal myectomy from 2000 to 2016. Myocyte hypertrophy, myocyte disarray, interstitial fibrosis, and endocardial thickening were graded and analyzed.The median age at operation was 54.2 years (43.5 to 64.3 years), and 1,067 (58.1%) were men. A weak negative correlation between myocyte disarray and age at surgery was identified (ρ = -0.22; p 0.001). Myocyte hypertrophy (p 0.001), myocyte disarray (p 0.001), and interstitial fibrosis (p 0.001) were positively associated with implantable cardioverter-defibrillator implantation. Interstitial fibrosis (p 0.001) and endocardial thickening (p 0.001) were associated with atrial fibrillation pre-operatively. In the Cox survival model, older age (p 0.001), lower degree of myocyte hypertrophy (severe vs. mild hazard ratio: 0.41; 95% confidence interval: 0.19 to 0.86; p = 0.040), and lower degree of endocardial thickening (moderate vs. mild hazard ratio: 0.75; 95% confidence interval: 0.58 to 0.97; p = 0.019) were independently associated with worse post-myectomy survival. Among 256 patients who had genotype analysis, patients with pathogenic or likely pathogenic variants (n = 62) had a greater degree of myocyte disarray (42% vs. 15% vs. 20%; p = 0.022). Notably, 13 patients with pathogenic or likely pathogenic genetic variants of HCM had no myocyte disarray.Histopathology was associated with clinical manifestations including the age of disease onset and arrhythmias. Myocyte hypertrophy and endocardial thickening were negatively associated with post-myectomy mortality.

PURPOSE The impact of postoperative complications on long-term survival is not well characterized. We sought to study the prevalence of postoperative complications after cardiac surgery and their impact on long-term survival. METHODS Operative survivors (n = 26,221) who underwent coronary artery bypass grafting (CABG) (n = 13,054, 49.8%), valve surgery (n = 8667, 33.1%) or combined CABG and valve surgery (n = 4500, 17.2%) from 1993 to 2019 were included in the study. Records were reviewed for postoperative complications and long-term survival. Propensity-match analysis was performed between patients who did and did not have a postoperative complication. The associations between postoperative complications and survival were assessed using a Cox-proportional model. RESULTS Complications occurred in 17,463 (66.6%) of 26,221 operative survivors. A total of 17 postoperative complications were analyzed. Postoperative blood product use was the commonest (n = 12,397, 47.3%), followed by atrial fibrillation (n = 8399, 32.0%), prolonged ventilation (n = 2336, 8.9%), renal failure (n = 870, 3.3%), reoperation for bleeding (n = 859, 3.3%) and pacemaker/ICD insertion (n = 795, 3.0%). Stroke (hazard ratio [HR]: 1.55; 95% confidence interval [CI]: 1.36-1.77), renal failure (HR: 1.45; 95% CI: 1.33-1.58) and pneumonia (HR: 1.23; 95% CI: 1.11-1.36) had the strongest impact on long-term survival. Long-term survival decreased as the number of postoperative complications increased. CONCLUSIONS Postoperative complications after cardiac surgery significantly impact outcomes that extend beyond the postoperative period. Stroke, renal failure, and pneumonia are particularly associated with poor long-term survival.

One in a hundred babies, or 1.3 million per year around the world, are born with congenital heart defects (CHD), of which over 70% will require medical or surgical treatment within their first year of life. In low- and middle-income countries, the majority does not receive the treatment they need or receive suboptimal care. Despite a higher pediatric cardiac surgical disease burden, low-income countries only have 0.07 pediatric cardiac surgeons per million pediatric population, compared to 9.51 per million in high-income countries. Here, we perform a stakeholder analysis of existing advocacy bodies within the field of pediatric global cardiac surgery and identify gaps and opportunities in advocating for cardiac care for children with CHD. We propose a framework to optimize civil society messaging in order to more effectively advocate domestically and internationally to hold national and international policy makers accountable for existing gaps and disparities in CHD care around the world.

Background: COVID-19 has changed the world as we know it, and the United States continues to accumulate the largest number of COVID-related deaths worldwide at the time of publication. There exists a paucity of data regarding the effect of COVID-19 on adult cardiac surgery trends and outcomes on a national level. Methods: The STS Adult Cardiac Surgery Database was queried and analyzed from January 1, 2018 to June 30, 2020. The Johns Hopkins COVID-19 database was queried from February 1, 2020 to January 1, 2021. Surgical and COVID-19 volumes, trends, and outcomes were analyzed on a national and regional level. Observed-to-expected ratios were used to analyze mortality for all risk-adjustable cardiac procedures. Findings: A total of 717,103 adult cardiac surgery patients and more than 20 million COVID-19 patients were included for analysis. The South Atlantic (21.1%) and Great Lakes (17.4%) regions contributed the most patients during the study period. Nationally, there was a 52.7% reduction in overall adult cardiac surgery volume, and a 65.5% reduction in elective cases. The Mid-Atlantic region was most affected by the first COVID-19 surge, with a 69.7% reduction in overall case volume and 80.0% reduction in elective cases. In the Mid-Atlantic and New England regions, the observed-to-expected mortality for isolated coronary bypass increased as much as 1.48 times (148% increase) pre-COVID rates. After the first COVID-19 surge, nationwide cardiac surgical case volumes increased but did not return to baseline, indicating a COVID-associated deficit of cardiac surgery patients. Interpretation: This study is the largest analysis of COVID-19 related impact on adult cardiac surgery volume, trends, and outcomes. During the COVID-19 pandemic, cardiac surgery volume suffered dramatically, particularly in the Mid-Atlantic and New England regions during the first surge of COVID-19 cases, with a concurrent significant increase in observed-to-expected 30-day mortality. Funding: None. Conflict of Interest: TCN – Consultant, Edwards LifeSciences, Abbott, LivaNova, CryoLife. VHT – Consultant/research, Abbott, Boston Scientific, Edwards LifeSciences, W. L. Gore & Associates, Jenavalve. APN – None. RHH – None. JAD – None. JAC – None. AR – None. HM – None.DMS – None. JPJ – None. VB – Consultant, Abbott.

OBJECTIVE The objective of this study was to evaluate outcomes of surgical management of lead-induced tricuspid regurgitation (TR) in patients with congenital heart disease. METHODS We analyzed data of 54 consecutive patients who underwent tricuspid valve (TV) surgery from 1998 to 2015 for lead-induced TR. Primary end points, including mortality, TV reinterventions, and longitudinal TR measurements, were analyzed with the Kaplan-Meier method or with repeated measures proportional odds modeling. RESULTS The median age of patients was 48.2 years (interquartile range, 37.3-59.0 years); 31 (57.4%) were female; 2 (3.7%) were children. Thirty patients (55.6%) underwent TV repair and 24 (44.4%) had replacement, and 52 underwent concomitant cardiac procedures. Thirty-day mortality was 1.9% (repair: 3.3%, replacement: 0.0%). Five-year survival was 80.4% overall and 79.7% and 81.4% for the repair and replacement groups, respectively. In response to surgery, TR improved in both groups (each P

Objective To determine the impact of sleep-disordered breathing (SDB) on survival in patients with hypertrophic cardiomyopathy (HCM) following septal myectomy. Patients and Methods Patients with obstructive HCM undergoing septal myectomy from 2007 to 2016 were reviewed. Those who had an overnight oximetry test within 6 months before myectomy were included in analysis. Oxygen desaturation index was examined continuously and also categorically (SDB [>5/h] and severe SDB [>15/h]). Results A total of 619 of 1500 patients undergoing septal myectomy had overnight oximetry tests. Sleep-disordered breathing (oxygen desaturation index >5/h) was identified in 338 (54.6%) patients, and among those patients, 117 (18.9%) were classified as severe. Patients with SDB were older, had greater body mass index and body surface area, were more likely to have arterial hypertension and atrial fibrillation, and had an increased E/e’ ratio on Doppler echocardiography. Notably, there was no difference in preoperative resting left ventricular outflow tract pressure gradient between patients with SDB and those with normal overnight oximetry (55 (interquartile range: 25 to 86) mm Hg versus 52 (interquartile range: 21 to 85) mm Hg; P=.29). There was no difference in age-adjusted survival among patients with normal oximetry compared with those with mild SDB (hazard ratio: 0.98; 95% CI: 0.45 to 2.17), and severe SDB (hazard ratio: 1.06, 95% CI 0.42 - 2.71). Conclusion Sleep-disordered breathing is present in more than half of patients with obstructive HCM in whom septal myectomy is indicated, and is mainly associated with aging, overweight, and male sex. However, SDB does not alter survival following septal myectomy.

Introduction: There are limited data on the impact of isolated tricuspid valve (TV) surgery on recovery of right ventricular (RV) function and RV reverse remodeling. Methods: Among 223 patients who had isolated TV procedures between 2001 and 2017, 60 (27%) underwent TV repair and 163 (73%) received TV replacement. RV reverse remodeling was assessed by echocardiography at a median of 11.3 months (IQR 5.9-13.5) post dismissal. Kaplan-Meier analysis and the log-rank test were used to estimate and create survival curves. Cox proportional hazards analysis was performed to study the association of clinical and echocardiographic parameters with mortality. Results: Mean age was 67.3 ± 13.7 years, and 57% were female. Indication for surgery was functional TR in 64% (n=143) patients. Overall 30-day mortality was 2.7%. After a median follow-up period of 9.5 years (IQR 3.6-12.9) there were a total of 122 (54.7%) deaths. Adjusted Cox regression analysis revealed comparable survival for TV repair and replacement and identified older age, and presence of RV dysfunction (HR 1.84, 95% CI 1.14-2.98; P=0.01) as independent predictors of poor survival. Impact of early RV reverse remodeling on survival after TV surgery was evaluated in 90 patients; In 39 RV function and/or RV size recovered, but in 51 RV dysfunction and/or RV enlargement persisted or worsened. Patients who exhibited RV reverse remodeling had significantly improved survival compared to those who did not (Log-Rank P=0.005) and reverse remodeling was independently associated with improved survival (HR 0.42, 95% CI 0.24-0.74; P=0.003). In a multivariable analysis lower preoperative right atrial pressure (OR 0.83, 95% CI 0.73-0.94; P=0.004) was an independent predictor of early right heart reverse remodeling. Conclusions: Isolated TV surgery can be performed with acceptable outcomes, and overall survival is best in patients who receive the operation before developing severe RV systolic dysfunction. Adjusted survival was similar for patients undergoing TV repair or replacement. Reverse remodeling of RV during the first 18 months after surgery appears to provide survival benefit. Preoperative RA pressure is an independent determinant of postoperative RV remodeling.

Background: Papillary fibroelastoma (PFE) is the most common benign cardiac tumor. Because PFEs have the potential to embolize, they typically are surgically excised. Prior studies have suggested that postoperative recurrence of PFE is rare or does not occur. Objectives: To determine the rate at which PFEs recurred after surgical removal and to identify any risk factors associated with recurrence. Methods: Patients with pathologically proven PFE were retrospectively identified from a single center. Patients were included in the study if they had an echocardiographic examination at least 1 year after surgery. Echocardiographic images obtained intraoperatively (after excision) and at dismissal were compared with those of the most recent examination to assess the possibility of PFE recurrence. Results: Ninety eight patients were identified. The mean (SD) duration of follow-up was 5.4 (3.7) years (range, 1-17 years). Twelve patients (12.2%) had echocardiographically proven PFE recurrence. Three patients had the recurrent lesion surgically re-excised, and pathologic analysis showed that 2 were recurrent PFEs and 1 was a Lambl excrescence. Clinical presentation of stroke or TIA was more common for the recurrence patients (for the first PFE) than for the nonrecurrence patients (83% vs 26%; P Conclusions: Contrary to published data, PFEs do recur after surgical excision. This emphasizes the importance of postoperative follow-up with transesophageal echocardiography for identifying recurrent masses. Table. Characteristics of Patients With Recurrent PFE Abbreviations: AV, aortic valve; LA, left atrium; LE, Lambl excrescence; LVOT, left ventricular outflow tract; MV, mitral valve; PV, pulmonary valve; RA, right atrium; RC, right cusp; TV, tricuspid valve. a Corresponds to the same location. b Corresponds to a different location .

Delayed sternal closure (DSC) is a management strategy for hemodynamic instability and severe coagulopathy after complex congenital heart surgery. We hypothesized that DSC results in better outcomes than perioperative sternal reopening.We reviewed patients aged18 years old undergoing cardiac surgery 2007-2017 at our institution. A total of 179 patients (3.8%) had primary DSC (PDSC, sternum left open after initial operation) and 45 patients (0.9%) had secondary DSC (SDSC, sternum closed primarily and reopened perioperatively). Perioperative characteristics and outcomes among PDSC ≤2 days (98 patients), PDSC2 days (81 patients), and SDSC (45 patients) were analyzed.Median age was 120 days (range, 3-6553 days) and median DSC duration was 2 days (range, 1-60 days). The PDSC2 days group was the youngest group, and the distribution of procedures was different between groups. Indications for DSC were hemodynamic instability in 152 patients (67.9%) and severe coagulopathy in 33 patients (14.7%), with no difference between groups (P = .141). Extracorporeal membrane oxygenation use was higher in the PDSC2 days group than the other groups (47.5% vs 7.1%, P.01 and 47.5% vs 28.9%, P = .02), respectively. Operative mortality was higher in SDSC compared to the other groups (17.8% vs 0% for PDSC ≤2 and 6.2% for PDSC2 days, P.01). Hospital stay was longer in SDSC (57 ± 7 days) than PDSC ≤2 days (22 ± 5 days) and PDSC2 days (44 ± 6, P = .01). Survival was better in PDSC regardless of duration than SDSC.PDSC demonstrated better outcomes than SDSC. Sternal reopening can be life-saving, but, when anticipated, PDSC can yield better outcomes.

Background Papillary fibroelastoma (PFE) is the most common primary benign cardiac tumor. Because PFEs have the potential to embolize, they often are surgically excised. Prior studies have suggested that postoperative recurrence of PFE is rare or does not occur. We aimed to determine the rate at which PFEs recurred after surgical removal and to identify any risk factors associated with recurrence. Methods We retrospectively identified all patients from a single center with pathologically proven PFE, treated from January 1995 through December 2018. Patients were included in the study if they had an echocardiographic examination at least 1 year after surgery. We compared echocardiographic images obtained intraoperatively (after excision) and at dismissal with those of the most recent examination to assess the possibility of PFE recurrence. Results We included 98 patients in the study. The mean (SD) duration of follow-up was 5.4 (3.7) years (range, 1-17 years); the median duration of follow-up was 4.3 years (interquartile range, 1.9-7.7 years). Twelve patients (12.2%) had echocardiographically supported PFE recurrence. Three patients had the recurrent lesion surgically re-excised, and pathologic analysis showed that 2 were recurrent PFEs and 1 was a Lambl excrescence. Initial clinical presentation of stroke or transient ischemic attack was more common for the recurrence group (for the first PFE) than for the nonrecurrence group (83% vs 26%; P Conclusions Contrary to findings from previous studies, PFEs do recur after surgical excision. These findings emphasize the importance of postoperative follow-up with transesophageal echocardiography for identifying recurrent masses.

BackgroundAlthough tricuspid valve surgery improves functional capacity in patients with Ebstein anomaly, it is not always associated with improvement in aerobic capacity. The purpose of this study was to identify the determinants of improved aerobic capacity after tricuspid valve surgery in adults with Ebstein anomaly with severe tricuspid regurgitation.MethodsRetrospective study of patients with severe tricuspid regurgitation due to Ebstein anomaly that had tricuspid valve surgery at Mayo Clinic Rochester (2000–2019) and had preoperative and postoperative cardiopulmonary exercise tests and echocardiograms. The patients were divided into aerobic capacity(+) and aerobic capacity(-) groups depending on whether they had postoperative improvement in %-predicted peak oxygen consumption (VO2).ResultsOf 76 patients with severe tricuspid regurgitation due to Ebstein anomaly, 28 (37%) and 48 (63%) were in aerobic capacity(+) and aerobic capacity(-) groups, respectively. The average improvement in peak VO2 was 2.1±1.4 mL/kg/min and −0.9±0.4 mL/kg/min in the in aerobic capacity(+) and aerobic capacity(-) groups, respectively. Although both groups had similar severity of residual tricuspid regurgitation, the aerobic capacity(+) group had more postoperative improvement in right atrial (RA) function, left atrial (LA) function and left ventricular preload and stroke volume. Of the preoperative variables analysed, RA reservoir strain (relative risk 1.12; 95% CI 1.06 to 1.18); LA reservoir strain (relative risk 1.09; 95% CI 1.04 to 1.14) and LV stroke volume index (OR 1.04; 95% CI 1.01 to 1.07) were predictors of postoperative improvement in peak VO2.ConclusionsOne-third of patients with severe tricuspid regurgitation due to Ebstein anomaly had postoperative improvement in aerobic capacity, and atrial function indices were the best predictors of postoperative improvement in aerobic capacity. These data provide new insight into the haemodynamic determinants of exercise capacity and lay the foundation for further studies to determine whether postoperative improvement in aerobic capacity translates to improved long-term survival, and whether timing of tricuspid valve surgery based on these echocardiographic indices will improve long-term outcomes.

Background In patients undergoing heart transplantation, significant allosensitization limits access to organs, resulting in longer wait times and high waitlist mortality. Current desensitization strategies are limited in enabling successful transplantation. Objectives The purpose of this study was to describe the cumulative experience of combined heart-liver transplantation using a novel heart-after-liver transplant (HALT) protocol resulting in profound immunologic protection. Methods Reported are the results of a clinical protocol that was instituted to transplant highly sensitized patients requiring combined heart and liver transplantation at a single institution. Patients were dual-organ listed with perceived elevated risk of rejection or markedly prolonged waitlist time due to high levels of allo-antibodies. Detailed immunological data and long-term patient and graft outcomes were obtained. Results A total of 7 patients (age 43 ± 7 years, 86% women) with high allosensitization (median calculated panel reactive antibody = 77%) underwent HALT. All had significant, unacceptable donor specific antibodies (DSA) (>4,000 mean fluorescence antibody). Prospective pre-operative flow cytometric T-cell crossmatch was positive in all, and B-cell crossmatch was positive in 5 of 7. After HALT, retrospective crossmatch (B- and T-cell) became negative in all. DSA fell dramatically; at last follow-up, all pre-formed or de novo DSA levels were insignificant at 1R rejection over a median follow-up of 48 months (interquartile range: 25 to 68 months). There was 1 death due to metastatic cancer and no significant graft dysfunction. Conclusions A heart-after-liver transplantation protocol enables successful transplantation via near-elimination of DSA and is effective in preventing adverse immunological outcomes in highly sensitized patients listed for combined heart-liver transplantation.

Background There are limited data on the impact of isolated tricuspid valve (TV) surgery on recovery of right ventricular (RV) function and RV reverse remodeling. Methods Among 223 patients who had isolated TV procedures between 2001 and 2017, 60 (27%) underwent TV repair and 163 (73%) received TV replacement. Indication for surgery was functional tricuspid valve regurgitation in 64%, lead induced in 18%, and primary leaflet dysfunction in 18%. RV reverse remodeling was assessed by echocardiography at a median of 11.3 months (interquartile range [IQR] 5.9-13.5) post-dismissal. Results Mean age was 67.3 ± 13.7 years, and 57% were female. Overall 30-day mortality was 2.7%. After a median follow-up period of 9.5 years (IQR 3.6-12.9), adjusted Cox regression analysis revealed comparable survival for TV repair and replacement and identified older age, and presence of RV dysfunction (hazard ratio [HR] 1.84, 95% confidence interval [CI] 1.14-2.98; P = .01), as independent predictors of poor survival. Patients who exhibited RV reverse remodeling within 18 months postoperatively had significantly improved survival compared with those who did not (log-rank P = .005), and reverse remodeling was independently associated with improved survival (HR 0.42, 95% CI 0.24-0.74; P = .003). Lower preoperative right atrial pressure (odds ratio 0.83, 95% CI 0.73-0.94; P = .004) was predictive of early RV reverse remodeling. Conclusions Isolated TV surgery can be performed with acceptable outcomes (early mortality 2.7%), and overall survival is best in patients who receive the operation before developing RV systolic dysfunction. Adjusted survival was similar for patients undergoing TV repair or replacement. Early reverse remodeling of RV after surgery is associated with survival benefit.

Whereas the prevalence and impact of atrioventricular valve (AVV) regurgitation in patients with single ventricle physiology has become increasingly apparent, the optimal timing for valve intervention is unclear. To investigate this, we performed a retrospective review of all 1,167 patients from the Mayo Clinic Fontan database. Thirteen percent (153 patients) had AVV repair or replacement during their staged single ventricle palliation. We found that patients with right ventricular morphology and common AVV were at increased risk for AVV intervention. Patients who underwent AVV intervention had increased risk of death/transplant compared with those who did not (hazards ratio [HR] = 1.75, 95% CI 1.37 to 2.23, p0.001). With respect to valve intervention timing, whereas AVV intervention before Fontan presented similar risk for death/transplant compared with no AVV intervention (HR = 0.85, 95% CI 0.32 to 2.27, p = 0.74), intervention at time of Fontan had a significantly higher risk (HR = 1.46, 95% CI 1.09 to 1.97, p = 0.01), and intervention after Fontan had a much more substantial risk (HR = 3.83, 95% CI 2.54 to 5.79, p0.001). AVV repair failure occurred in 11% of patients. In terms of relative risk of valve repair versus replacement, in post-Fontan AVV intervention patients, AVV replacement carried a 2.9 fold risk of death/transplant compared with AVV repair. In conclusion, AVV disease remains a considerable challenge for durable Fontan physiology. This data demonstrates that earlier intervention on valve pathology improves survival with the Fontan circulation. Continued surveillance of single ventricle patients and prompt referral of those with valve pathology can improve outcomes in this challenging population.

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.