Search

Bevacizumab is now an emerging treatment option for severe hereditary hemorrhagic telangiectasia–related bleeding including epistaxis and gastrointestinal tract bleeding. The impact of long-term intravenous bevacizumab therapy on cardiac structure and function is unknown. We describe 3 patients receiving intravenous bevacizumab therapy for severe hereditary hemorrhagic telangiectasia–related bleeding who were found to have abnormal mobile masses on the mitral valve (n=2) and aortic valve (n=1). The clinical impact of these findings is unknown and requires further study.

Severe coronary artery disease is associated with disproportionately increased risks of death in patients with hypertrophic cardiomyopathy. There is a paucity of data on the long-term effect of coronary revascularization at the time of myectomy. Between January 1, 1961, and October 31, 2017, 2913 adult patients underwent transaortic septal myectomy at Mayo Clinic. Concomitant coronary artery bypass grafting (CABG) was performed in 246 (8.4%). We compared baseline characteristics of patients who underwent septal myectomy with and without CABG and assessed the effect of surgical revascularization on the risk of all-cause mortality. Patients who underwent concomitant CABG were older (median [interquartile range], 66.3 [59.8-72.1] years vs 54.4 [43.5-64.8] years; P

ObjectiveA subset of patients at the time of transcatheter mitral valve repair (TMVR) will have normal left atrial pressure (LAP) (MethodsA single-centre retrospective cohort of consecutive patients who underwent transcatheter edge-to-edge mitral valve clip and continuous LAP monitoring between 5/1/2014 and 5/1/2018 was analysed. One-year mortality was compared by Kaplan–Meier survival curves. Multivariable analysis was performed to identify predictors of normal LAP and 1 year mortality.ResultsOf the 204 patients undergoing TMVR, 65% were men and the mean age was 81. Of these patients, 31 (15%) had normal LAP (mean LAP 10.5 mm Hg, mean V wave 16.5 mm Hg) and 173 had elevated LAP (mean LAP 19 mm Hg, mean V wave 32.5 mm Hg). The prevalence of severe MR was not different between groups, although the normal LAP group had significantly lower effective regurgitant orifice area and regurgitant volume. Other notable baseline characteristics including prior cardiac surgery, atrial fibrillation, hypertension, diabetes, congestive heart failure, body mass index, mechanism of MR and ejection fraction were similar between groups. However, there was an increased prevalence of chronic lung disease (CLD) (45.2% vs 17.3%, pConclusionNormal LAP at the time of TMVR is associated with a higher incidence of CLD which independently predicts increased 1-year mortality. In patients with CLD and apparently severe MR, measurement of LAP may help identify those with lower likelihood of benefit from TMVR.

Patients with obstructive hypertrophic cardiomyopathy may have occult gastrointestinal bleeding. In this study, we analyzed outcomes of septal myectomy in patients who had a history of gastrointestinal bleeding preoperatively to understand patient characteristics and impact of septal reduction on recurrent gastrointestinal bleeding.We analyzed 73 adult patients who had a history of gastrointestinal bleeding before transaortic septal myectomy for obstructive hypertrophic cardiomyopathy and compared outcomes to 219 patients without gastrointestinal bleeding preoperatively.Patients with preoperative history of gastrointestinal bleeding were older (median (IQR) age, 65 (59-69) years, P .001) and were more likely to have systemic hypertension (70% vs 53%, P = .020) and coronary artery disease (25% vs 13%, P = .026). Preoperatively, patients with gastrointestinal bleeding had a larger left atrial volume index (median, 53 mL/mPatients with a preoperative history of gastrointestinal bleeding have favorable short- and long-term outcomes after septal myectomy for obstructive hypertrophic cardiomyopathy. Remission of gastrointestinal bleeding was observed in 85% of patients postprocedure, and only 8% of the patients had recurrent gastrointestinal bleeding due to angiodysplasia or unknown causes.

Objectives Elongation of mitral valve leaflets is a phenotypic feature of hypertrophic cardiomyopathy, and some surgeons advocate plication of the anterior leaflet at the time of septal myectomy. The present study investigates mitral valve leaflet length and outcomes of patients undergoing septal myectomy for obstructive hypertrophic cardiomyopathy. Methods We reviewed the records and echocardiograms of 564 patients who underwent transaortic septal myectomy for obstructive hypertrophic cardiomyopathy between February 2015 and April 2018. Extended septal myectomy without plication of the anterior leaflet was the standard procedure. From intraoperative prebypass transesophageal echocardiograms, we measured anterior and posterior mitral valve leaflets and their coaptation length. For comparison, we performed these mitral valve leaflet measurements in 90 patients who underwent isolated coronary artery bypass grafting and 92 patients undergoing aortic valve replacement in the same period. Among patients with hypertrophic cardiomyopathy undergoing septal myectomy, we assessed left ventricular outflow tract gradient relief and 1-year survival in relation to leaflet length. Results Median patient age (interquartile range) was 60.3 (50.2-67.7) years, and 54.1% were male. Concomitant mitral valve repair was performed in 36 patients (6.4%), and mitral valve replacement was performed in 8 patients (1.4%), primarily for intrinsic mitral valve disease. Patients in the hypertrophic cardiomyopathy cohort had significantly longer mitral valve leaflet measurements compared with patients undergoing coronary artery bypass grafting or aortic valve replacement (P Conclusions Our study confirms previous findings that patients with hypertrophic cardiomyopathy have slight (5 mm) elongation of mitral valve leaflets. In contrast to other reports, increased anterior mitral valve leaflet length was not associated with higher left ventricular outflow tract gradients. Importantly, we found no significant relationship between anterior mitral valve leaflet length and postoperative left ventricular outflow tract resting gradients or gradient relief. Thus, in the absence of intrinsic mitral valve disease, transaortic septal myectomy with focus on extending the excision beyond the point of septal contact is sufficient for almost all patients.

Background: Investigational transcatheter edge-to-edge repair (TEER) for severe tricuspid regurgitation (TR) has shown promise as an alternative to surgery, but factors influencing outcomes, optimal patient selection, and procedural timing remain incompletely defined. Given the limitations of determining TR severity by conventional echocardiography, our objectives were to determine whether invasive right atrial (RA) pressures performed during the procedure are related to patient outcomes. Methods: This study was a retrospective review of patients who underwent off-label tricuspid TEER using MitraClip (Abbott Vascular, Menlo Park, CA) for significant TR at a single institution. Intraprocedural mean RA pressure, RA peak V-wave, RA pressure nadir, and systolic increase in RA pressure (XV height) were recorded. Results: Thirty-eight patients underwent tricuspid TEER; 33 underwent concomitant mitral TEER for mitral regurgitation. The study cohort was 39% female with a mean age of 78.6±14.3 years. Median follow-up was 339 days (interquartile range, 100–601). Any reduction in mean RA pressure, RA peak V-wave, RA nadir, and XV height occurred in 74%, 82%, 45%, and 87% of patients, respectively. At 1 year, event-free survival was 47%. Postprocedure XV height correlated with TR severity as determined by echocardiography ( P 8 mm Hg) had worse event-free survival compared with those who had concluding XV height ≤8 mm Hg ( P =0.02). Attainment of a concluding XV height less than or equal to median value was associated with a lower creatinine the next day (1.27±0.47 versus 1.64±0.47 mg/dL, P =0.04). Conclusions: Intraprocedural XV height correlates with TR severity after tricuspid TEER, and lower concluding pressures are associated with improved outcomes. Analysis of RA pressures may serve as a complementary tool for the evaluation of disease severity and procedural guidance.

Some patients with hypertrophic cardiomyopathy (HCM) present with reduced left ventricular (LV) stroke volume and elongated systolic cavity obliteration due to symmetric LV hypertrophy. In this report, we detail our experience with transapical septal myectomy to enlarge the LV volume and to relieve cavity obliteration in this unique subgroup of patients with HCM.We analyzed 38 patients with HCM who had extended symmetric LV hypertrophy and underwent transapical septal myectomy to enlarge the LV cavity from February 2001 to May 2021.At the time of evaluation for operation, 84.2% (n = 32) of the patients were in New York Heart Association class III/IV. The peak oxygen consumption was 51.5% (44.0%-58.0%) of the normal predicted values on the preoperative exercise stress test (n = 16). Preoperative left atrial sizes in this cohort were enlarged (left atrial volume index, 39.0 [33.5-51.5] mL/mTransapical myectomy to enlarge LV cavity volume can be performed safely with good early survival and functional results. This procedure is an important alternative to cardiac transplantation for HCM patients with systolic cavity obliteration and progressive heart failure.

Obesity is an independent risk factor for heart failure in patients with hypertrophic cardiomyopathy (HC). In this study, we examined national trends and early outcomes of bariatric surgery for obesity in patients with HC. Using the weighted discharge data from the National Inpatient Sample, we identified adult patients with HC who underwent elective bariatric surgery for obesity between 2011 and 2017. A total of 443 obese patients with HC were identified, and 42% (n = 185) had obstructive HC. The annual number of patients increased from 18 in 2011 to 130 in 2017. Overall, the median (interquartile range) age was 50 (43 to 57) years, and 85 patients (19%) were 60 years or older. Approximately 20% (n = 90) of the patients had heart failure at the time of operation. Atrial fibrillation was present in 83 patients (19%), and 22% (n = 95) of the cohort had a pacemaker or automatic cardiac defibrillator implanted before the operation. Laparoscopic sleeve gastrectomy (72%, n = 318) and laparoscopic Roux-en-Y gastric bypass (25%, n = 110) were the most commonly performed bariatric procedures. Overall, patients stayed in the hospital for a median (interquartile range) of 2 (1 to 2) days. During the hospital stay, there were no deaths, myocardial infarctions, or documented episodes of thromboembolism. In conclusion, bariatric surgery in patients with HC is performed more frequently in recent years and is safe and associated with few perioperative complications. Because of the impact of obesity on long-term survival, clinicians should strongly consider bariatric surgery for obese HC patients who do not respond to conservative weight loss measures.

This investigation analyzed clinical characteristics of patients with hypertrophic cardiomyopathy (HCM) and latent left ventricular outflow tract (LVOT) and outcomes following septal myectomy.We reviewed patients with HCM and LVOT obstruction undergoing septal myectomy from 2001 to 2016 at our center. Follow-up data on functional status were obtained through mailed survey questionnaires.There were 629 (31.8%) patients with latent obstruction (resting LVOT gradientlt;30 mm Hg, provoked gradientgt;30 mm Hg) among 1981 patients undergoing septal myectomy. Patients with latent obstruction were more likely to be male (65.7% vs 51.8%, P lt; .001), but there were no important differences in other clinical characteristics. The New York Heart Association functional classes and measured/predicted maximal oxygen consumption (62 [51, 72] vs 60 [48, 72], P = .158) in cardiopulmonary exercise tests were comparable between the 2 groups. Patients with latent obstruction had both lower septal thickness and lower posterior wall thickness. Median intraoperative provoked pressure gradient decreased from 96 (68, 126) mm Hg to 0 (0, 6) mm Hg after myectomy (P lt; .001). There was no difference in early (lt;30 days) deaths (3/629 vs 5/1352, P = .726) and long-term survival between patients with latent obstruction and resting obstruction. In follow-up, both general health status and New York Heart Association functional class were significantly improved following septal myectomy.Patients with HCM and latent LVOT obstruction generally have milder left ventricular hypertrophy but similarly impaired functional capacity compared to those with resting obstruction. Septal myectomy improves functional capacity and symptoms.

Objective This study was conducted to determine the influence of early septal reduction therapy (SRT) after referral on survival in patients with obstructive hypertrophic cardiomyopathy. Methods We reviewed the patients with obstructive hypertrophic cardiomyopathy (resting pressure gradient ≥30 mm Hg or provoked pressure gradient ≥50 mm Hg) who were evaluated at our clinic from 2000 to 2012. Early SRT was defined as undergoing septal myectomy or alcohol septal ablation during the 6 months after index evaluation. Survival after the 6-month landmark period was analyzed in a multivariable Cox model. Results A total of 1351 patients were included in the landmark analysis. Patients who were more symptomatic and had received more medical treatment at index evaluation were more likely to undergo early SRT. Over a median follow-up period of 10.2 years, the survival was comparable (P = .207) but patients undergoing early SRT had, on average, improved survival compared with the medical treatment group (hazard ratio, 0.66; 95% confidence interval, 0.48-0.90) after adjustment by age and comorbidities. Further analysis revealed significant treatment heterogeneity, with increased benefit of early SRT seen in women (hazard ratio, 0.51; 95% confidence interval, 0.35-0.75), those who are in New York Heart Association functional class III or IV (hazard ratio, 0.52; 95% confidence interval, 0.36-0.76), and patients without diabetes (hazard ratio, 0.59; 95% confidence interval, 0.42-0.82). Conclusions In experienced hypertrophic cardiomyopathy centers, early SRT is similar to continued medical treatment for patients with obstructive hypertrophic cardiomyopathy. It appears to improve survival of female patients and those who are in New York Heart Association functional class III or IV.

Hypertrophic cardiomyopathy (HCM), a relatively common, globally distributed, and often inherited primary cardiac disease, has now transformed into a contemporary highly treatable condition with effective options that alter natural history along specific personalized adverse pathways at all ages. HCM patients with disease-related complications benefit from: matured risk stratification in which major markers reliably select patients for prophylactic defibrillators and prevention of arrhythmic sudden death; low risk to high benefit surgical myectomy (with percutaneous alcohol ablation a selective alternative) that reverses progressive heart failure caused by outflow obstruction; anticoagulation prophylaxis that prevents atrial fibrillation-related embolic stroke and ablation techniques that decrease the frequency of paroxysmal episodes; and occasionally, heart transplant for end-stage nonobstructive patients. Those innovations have substantially improved outcomes by significantly reducing morbidity and HCM-related mortality to 0.5%/y. Palliative pharmacological strategies with currently available negative inotropic drugs can control symptoms over the short-term in some patients, but generally do not alter long-term clinical course. Notably, a substantial proportion of HCM patients (largely those identified without outflow obstruction) experience a stable/benign course without major interventions. The expert panel has critically appraised all available data and presented management insights and recommendations with concise principles for clinical decision-making.

Objective The objective of this study was to determine the characteristics of longitudinal strain and its effect on outcomes in patients with obstructive hypertrophic cardiomyopathy (HCM) who underwent septal myectomy. Methods We reviewed patients with obstructive HCM who underwent septal myectomy at our clinic from 2007 to 2016. Data of those who had strain echocardiography within 6 months before isolated myectomy were analyzed. Results The median age of the 857 patients studied was 55 (interquartile range [IQR], 44-63) years, and 451 (52.6%) were male. Left ventricular ejection fraction was 71% (IQR, 67%-74%), and the resting peak outflow tract gradient was 58 (IQR, 27-85) mm Hg. The median global longitudinal strain (GLS) was −14.6% (IQR, −12.0% to −17.3%). Regional longitudinal strain was nonuniform as reflected by more normal values in apical segments and more abnormal in basal segments. Moreover, GLS correlated poorly with ejection fraction and outflow tract gradient. In 64 patients who had postoperative strain echocardiography, GLS was comparable before and after septal myectomy, but regional strain was more uniform after myectomy. Over a follow-up of 8.3 (IQR, 6.5-10.3) years, when patients were equally stratified according to GLS (cutoff, −14.64%), the group with worse GLS had significantly poorer survival compared with the better GLS group (P = .002). Left ventricular ejection fraction had no association with survival. Conclusions Left ventricular longitudinal strain is nonuniform and might be significantly reduced in patients with obstructive HCM. Septal myectomy does not impair GLS but is associated with more uniform regional strains. Most importantly, reduced GLS preoperatively is strongly and independently associated with increased all-cause mortality after septal myectomy for obstructive HCM.

Residual or recurrent symptoms after septal reduction therapy are most often related to inadequate relief of left ventricular outflow gradients. We recently encountered a 71-year-old woman with hypertrophic cardiomyopathy and prior alcohol septal ablation who had a unique constellation of findings causing her symptoms. She was found to have four potential causes for her symptoms, residual midventricular obstruction, apical distribution of hypertrophy reducing end-diastolic volume, constrictive pericarditis, and marked arterial stiffness, as reflected by aortic atherosclerosis. She underwent complete pericardiectomy, transaortic septal myectomy, transapical myectomy, and replacement of a heavily calcified ascending aorta.

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.

Cardiac filling pressures may be elevated due to abnormalities in the myocardium, heightened pericardial restraint, or both. The authors hypothesized that the relative contributions due to myocardium and pericardium could be estimated by the ratio between right atrial pressure and pulmonary arterial wedge pressure (RAP/PAWP), which would enable better discrimination of the extent of myocardial disease in patients with constrictive pericarditis (CP).This study investigated the relationships between RAP/PAWP and the pericardial thickness as well as echocardiographic parameters of myocardial function and assessed the prognostic implications of RAP/PAWP for long-term mortality in primary and mixed CP patients who underwent pericardiectomy.A total of 113 surgically confirmed CP patients who underwent echocardiography and cardiac catheterization within 7 days of each other between 2005 and 2013 were included in the study. The patients were classified into a high RAP/PAWP group (≥0.77; n = 56) or a low RAP/PAWP group (0.77; n = 57) according to the median RAP/PAWP value. The primary outcome was prognostic implication of RAP/PAWP on long-term mortality and assessment of the relationship between RAP/PAWP and Doppler echocardiographic parameters in primary and mixed CP. In addition, the relationship between RAP/PAWP and the pericardial thickness was assessed.RAP/PAWP was directly correlated with pericardial thickness (regression coefficient [β] = 8.34; p 0.001). RAP/PAWP had a significant direct correlation with early diastolic velocity of medial mitral annulus (e') (β = 10.69; p 0.001) and inverse relationship with early transmitral diastolic velocity (E) (β = -105.15; p 0.001), resulting in an inverse relationship with the ratio of E/e' (β = -23.53; p 0.001). Patients with high RAP/PAWP ratio had a better survival rate compared with those with low RAP/PAWP ratio (p = 0.01). Its prognostic value was significant in primary CP (p = 0.03) but not in mixed CP with concomitant myocardial disease (p = 0.89).The RAP/PAWP ratio can reflect the degree of pericardial restraint versus restrictive myocardium and was associated with the long-term survival after pericardiectomy.

This statement was commissioned as a Multisociety Expert Consensus Systems of Care Document by the American Association for Thoracic Surgery (AATS), American College of Cardiology (ACC), American Society of Echocardiography (ASE), Society for Cardiovascular Angiography and Interventions (SCAI), and

Diastolic filling of the heart is a complex sequence of multiple inter-related events consisting of processes such as ventricular relaxation, erectile coronary effect, visco-elastic forces of the myocardium, ventricular interaction, myocardial stress strain relationships, pericardial restraint, passive filling, and atrial contraction. However, in order to understand diastolic filling from a clinical aspect, a simplified foundation can be used which divides the cardiac cycle into contraction, relaxation, passive filling, and filling at atrial contraction. The mitral flow velocity curves are representative of the relative driving pressure between left atrium and left ventricle and allow one to grade the progression of diastolic dysfunction which occurs in disease states. Doppler tissue imaging is necessary as a surrogate of ventricular relaxation to further determine the stages of diastolic dysfunction in patients with preserved ejection fraction. These Doppler flow velocity curves can be applied to understanding diastolic filling of the heart in patients with both reduced ejection fraction and preserved ejection fraction.

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.

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.

Objective To investigate provider opinions regarding a clinical decision support (CDS) system for cardiovascular risk assessment and for the creation of a replacement system. Methods From March to April 2018, an invitation letter with a link to a self-administered web-based survey was sent via e-mail to 279 providers with primary appointment in the Department of Cardiovascular Medicine, Mayo Clinic, Rochester. The e-mail was sent to providers on March 8, 2018 and the survey closed on April 16, 2018. Results One hundred providers responded to the survey yielding an overall response rate of 35.8%. Of these, 52 (52%) indicated they had used the cardiovascular (CV) risk profile CDS system and were classified as users and prompted to continue the survey. Among users, 42 (80.8%) indicated use of the CDS was either important (25; 48.1%) or very important (17; 32.7%) in their clinical practice; 45 (86.5%) responded that the system was very easy (17; 32.7%) or easy (28; 53.8%) to use. In addition, 48 (96.0%) users indicated that the CV risk profile supported their thought process at the point-of-care; 47 (97.9%) users indicated similar functionalities should be implemented into the new electronic health record system and 41 (85.4%) users reported new functionalities should also be incorporated. Conclusions For most users, the CDS system was easy to use and supported clinical thought process at the point-of-care. Users also felt their practice was supported and should continue to be supported by CDS systems providing individualized patient information at the point-of-care.

To assess the frequency and implications of mitral valve (MV) surgery at the time of septal myectomy (SM) for hypertrophic cardiomyopathy (HCM) in a national cohort.The National Inpatient Sample (NIS) was used to analyze surgical outcomes in patients with HCM undergoing SM from January 1, 2003, through December 31, 2014. Univariate analyses were used to compare patients undergoing SM with vs without concomitant procedures, and logistic regression was used to determine factors associated with prolonged length of stay (LOS) and in-hospital mortality. Numeric values of 10 or less were not reported per NIS data use agreements.The national cohort included 1174 adults with a primary diagnosis of HCM undergoing SM. Overall mean ± SD age was 54.4±14.5 years, and 45% of patients (n=529) were male. Isolated SM was performed in 67% (n=786), and the remainder had concomitant cardiac procedures, most frequently MV repair/replacement (22%, n=257). Median LOS was increased in those with concomitant MV surgery, 7 days, compared with isolated SM, 6 days (P.001). Overall hospital mortality was 2.9% (n=34) and was lowest in those undergoing isolated SM (1%; P.001). In otherwise isolated SM, MV replacement increased likelihood of in-hospital death (odds ratio, 12.0; 95% CI, 3.9-36.5; P.001) on a univariate basis.Intervention on the MV is more common nationally than in specialized centers, and the addition of MV replacement and other concomitant cardiac procedures was associated with increased rates of hospital mortality and LOS compared with patients undergoing isolated SM. These results suggest that concomitant MV intervention is associated with increased risk.

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 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.

Recent advances in transcatheter interventions have refueled the interest in utilizing invasive hemodynamics in the catheterization laboratory. The authors review contemporary invasive techniques used to confirm valve disease and guide transcatheter valve interventions. They also discuss the available data and the remaining questions on the role of invasive hemodynamics in current practice and in the future.

Hypertrophic cardiomyopathy (HCM) is a relatively common often inherited global heart disease, with complex phenotypic and genetic expression and natural history, affecting both genders and many races and cultures. Prevalence is 1:200-1:500, largely based on the disease phenotype with imaging, inferring that 750,000 Americans may be affected by HCM. However, cross-sectional data show that only a fraction are clinically diagnosed, suggesting under-recognition, with most clinicians exposed to small segments of the broad disease spectrum. Highly effective HCM management strategies have emerged, altering clinical course and substantially lowering mortality and morbidity rates. These advances underscore the importance of reliable HCM diagnosis with echocardiography and cardiac magnetic resonance. Family screening with noninvasive imaging will identify relatives with the HCM phenotype, while genetic analysis recognizes preclinical sarcomere gene carriers without left ventricular hypertrophy, but with the potential to transmit disease. Comprehensive initial patient evaluations are important for reliable diagnosis, accurate portrayal of HCM and family history, risk stratification, and distinguishing obstructive versus nonobstructive forms.

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.

Aim: This executive summary of the valvular heart disease guideline provides recommendations for clinicians to diagnose and manage valvular heart disease as well as supporting documentation to encourage their use. Methods: A comprehensive literature search was conducted from January 1, 2010, to March 1, 2020, encompassing studies, reviews, and other evidence conducted on human subjects that were published in English from PubMed, EMBASE, Cochrane, Agency for Healthcare Research and Quality Reports, and other selected database relevant to this guideline. Structure: Many recommendations from the earlier valvular heart disease guidelines have been updated with new evidence and provides newer options for diagnosis and treatment of valvular heart disease. This summary includes only the recommendations from the full guideline which focus on diagnostic work-up, the timing and choice of surgical and catheter interventions, and recommendations for medical therapy. The reader is referred to the full guideline for graphical flow charts, text, and tables with additional details about the rationale for and implementation of each recommendation, and the evidence tables detailing the data considered in developing these guidelines.

Background Whether the poor outcomes of isolated tricuspid valve surgery are related to the operation itself or to certain patient characteristics including late referral is unknown. Methods and Results Adult patients who underwent isolated tricuspid valve surgery were identified in the Nationwide Readmissions Database (2016–2017). Patients who had redo tricuspid valve surgery, endocarditis, or congenital heart disease were excluded. Multivariable logistic regression was performed to identify contributors to postoperative mortality. A total of 1513 patients were included (mean age 55.7±16.6 years, 49.6% women). Surrogates of late referral were frequent: 41% of patients were admitted with decompensated heart failure, 44.3% had a nonelective surgery status, 16.8% had advanced liver disease, and 31% had an unplanned hospitalization in the prior 90 days. The operation was performed on day 0 to 1 of the hospitalization in only 50% of patients, and beyond day 10 in 22% of patients. In‐hospital mortality occurred in 8.7% of patients. Median length of stay was 14 days (7–35 days), and median cost was $87 223 ($43 122–$200 872). In multivariable logistic regression analysis, surrogates for late referrals (acute heart failure decompensation, nonelective surgery status, or advanced liver disease) were the strongest predictors of in‐hospital mortality (odds ratio [OR], 4.75; 95% CI, 2.74–8.25 [ P P Conclusions The poor outcomes of isolated tricuspid valve surgery may be largely explained by the late referral for intervention. Studies are needed to determine the role of early intervention for severe isolated tricuspid regurgitation.

Given the universal presence of cardiac catheterization laboratories across the United States, it is difficult to imagine that for many years only a few institutions were able to perform invasive hemodynamic procedures. Since its inception more than seven decades ago, the Mayo Clinic Cardiac Catheterization Laboratory has played a pivotal role in advancing the field as well supporting the clinical practice. We provide an overview of Mayo’s invasive hemodynamic laboratory from its early work in congenital heart disease under the direction of Drs. Earl Wood and Jeremy Swan and the seminal studies in ischemic heart disease performed by Dr. Robert Frye, to classic simultaneous echocardiography-catheterization studies that became the hallmark of the clinic’s research endeavors from the 1980s to 2000s and provided the foundation for the widespread use of Doppler echocardiography in the noninvasive assessment of hemodynamics and valvular disease.

Invasive hemodynamic assessment is a crucial component of the diagnostic evaluation of the complex patient with structural heart disease. In this chapter, we will review the indications for invasive assessment and interpretation of data obtained from a right and left heart catheterization in a patient with structural heart disease.

To evaluate if there are sex and race disparities in use of implantable cardioverter-defibrillator (ICD) devices for prevention of sudden cardiac death in patients with hypertrophic cardiomyopathy (HCM).Using the National Inpatient Sample from January 2003 through December 2014, we identified all adult admissions with a diagnosis of HCM and an ICD implantation. Race was classified as White versus non-White. Trends in ICD use, predictors of ICD implantation, device-related complications, hospitalization costs, and lengths of stay were evaluated.Among a total of 23,535 adult hospitalizations for HCM, ICD implantation was performed in 3954 (16.8%) admissions. Over the study period, there was an overall increasing trend in ICD use (11.6% in 2003 to 17.0% in 2014, P.001). Compared with admissions not receiving an ICD, those receiving an ICD had shorter median lengths of in-hospital stay but higher hospitalization costs (P.001). Compared with men and White race, female sex (odds ratio, 0.72; 95% CI, 0.66 to 0.78; P.001) and non-White race (odds ratio, 0.87; 95% CI, 0.79 to 0.96; P.001) were associated with lower adjusted odds of receiving an ICD. Women and non-White hospitalizations had higher rates of device related complications, longer lengths of in-hospital stay, and higher hospitalization costs compared with men and White race, respectively (all P.01).Among HCM hospitalizations, ICD devices are underused in women and racial minorities independent of demographics, hospital characteristics, and comorbidities. Women and racial minorities also had higher rates of complications and greater resource use compared with men and those belonging to the White race, respectively.

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.

Until recently, there has been little attention paid to the treatment of tricuspid regurgitation (TR) aside from small series of patients with congenital disease or infective endocarditis. This reflects both the relative rarity of ‘primary TR’, as well as the perception that most TR was felt to be ‘secondary’ to pulmonary hypertension or a cardiomyopathic process, in which case the treatment was directed towards the underlying cause. However, there is now recognition of an increasing number of patients who present with ‘isolated’ TR. ‘Isolated TR’ is defined as the absence of primary tricuspid leaflet pathology, the absence of pulmonary hypertension and normal or near-normal left ventricular (LV) systolic function: in such cases, it is the TR itself that results in the signs and symptoms of right heart failure (HF). Such isolated TR is commonly associated with (1) chronic atrial fibrillation, (2) prior left heart surgery or (3) pacemaker device leads. Even with increased recognition of isolated TR, there is a reluctance to operate, in part due to a high reported operative mortality. Notably, despite increasing numbers of operations across the country, a high mortality rate of 10%–15% persisted in several studies.1 2 Therefore, patients are often conservatively managed or referred only at a late stage for surgery with advanced right HF and end-organ damage; and this approach has not unsurprisingly been associated with poor outcomes. In a recent cohort study from the national readmission database (2016–2017), >40% of patients who underwent isolated tricuspid valve (TV) surgery were referred late for surgery, with a fourfold higher operative mortality compared with those who had a timely referral.3 In this setting, Park et al 4 have contributed to an important observational study of 238 patients undergoing isolated TV surgery over nearly two decades at a large medical centre in South Korea. …

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.

Importance Volumetric measurements by transthoracic echocardiogram may better reflect left ventricular (LV) remodeling than conventional linear LV dimensions. However, the association of LV volumes with mortality in patients with chronic hemodynamically significant aortic regurgitation (AR) is unknown. Objective To assess whether LV volumes and volume-derived LV ejection fraction (Vol-LVEF) are determinants of mortality in AR. Design, Setting, and Participants This cohort study included consecutive asymptomatic patients with chronic moderately severe to severe AR from a tertiary referral center (January 2004 through April 2019). Exposures Clinical and echocardiographic data were analyzed retrospectively. Aortic regurgitation severity was graded by comprehensive integrated approach. De novo disk-summation method was used to derive LV volumes and Vol-LVEF. Main Outcome and Measures Associations between all-cause mortality under medical surveillance and the following LV indexes: linear LV end-systolic dimension index (LVESDi), linear LVEF, LV end-systolic volume index (LVESVi), and Vol-LVEF. Results Of 492 asymptomatic patients (mean [SD] age, 60 [17] years; 425 men [86%]), ischemic heart disease prevalence was low (41 [9%]), and 453 (92.1%) had preserved linear LVEF (≥50%) with mean (SD) LVESVi of 41 (15) mL/m2. At a median (interquartile range) of 5.4 (2.5-10.1) years, 66 patients (13.4%) died under medical surveillance; overall survival was not different than the age- and sex-matched general population (P = .55). Separate multivariate models, adjusted for age, sex, Charlson Comorbidity Index, and AR severity, demonstrated that in addition to linear LVEF and LVESDi, LVESVi and Vol-LVEF were independently associated with mortality under surveillance (all P

Objective Determine if information regarding hypertrophic cardiomyopathy (HCM) can be accurately retrieved from cardiac magnetic resonance (CMR) reports using natural language processing (NLP). Background CMR imaging is used for diagnosis and risk stratification of HCM. Manual annotation of information from CMR is time-consuming. NLP is an artificial intelligence method for automating extraction of information from narrative text. Methods We identified 200 HCM patients who had CMR reports from 1998 to 2018. These patients were randomly allocated into training (100 patients with 185 CMR reports) and testing sets (100 patients with 206 reports). An NLP system with 2 tiers was developed; the first extracted information regarding HCM diagnosis while second extracted categorical or numeric concepts for HCM classification. NLP performance was compared with gold-standard manual annotation. Results NLP algorithms achieved very high performance across all concepts with mean positive predictive value (PPV) = 0.96. An outlier was the performance for abstracting the presence of an apical pouch from CMR reports, which had noticeably lower PPV= 0.78 which be attributed to the low number of cases with this finding. Conclusions The algorithms developed can be translated to clinical decision support systems to increase efficiency and contribute to improved quality of care. Funding Acknowledgement Type of funding source: Other. Main funding source(s): Study supported by the National Heart, Lung and Blood Institute of National Institutes of Health (K01HL124045), the Mayo Clinic Center for Clinical and Translational Science (CCaTS), and the Mayo Clinic K2R award. Content is solely the responsibility of authors and does not necessarily represent official views of the National Institutes of Health.