Abstract Objectives We sought to assess the long-term outcomes in patients with hypertrophic cardiomyopathy and severe left ventricular outflow tract obstruction, in whom the decision regarding ...surgery (vs conservative management) was based on assessment of symptoms or exercise capacity. Methods This was an observational study of 1530 patients with hypertrophic cardiomyopathy (aged 50 ± 13 years, 63% were men) with severe left ventricular outflow tract obstruction (excluding those aged <18 years, with left ventricular ejection fraction <50%, and with left ventricular outflow tract gradient <30 mm Hg). A composite end point of death (excluding noncardiac causes) and/or implantable defibrillator discharge was assessed. Results Coronary artery disease, family history of hypertrophic cardiomyopathy, and syncope were present in 15%, 17%, and 18% of patients, respectively, whereas 73% patients were in New York Heart Association class II or greater. Mean left ventricular ejection fraction, basal septal thickness, and left ventricular outflow tract gradient (resting or provocable) were 62% ± 5%, 2.2 ± 1 cm, and 101 ± 39 mm Hg, respectively. A total of 858 patients (56%) underwent exercise echocardiography, of whom 503 (59%) had exercise capacity impairment. At 8.1 ± 6 years, 990 patients (65%) underwent surgical relief of left ventricular outflow tract obstruction, and 540 patients (35%) did not. There were 156 events (10%) (134 deaths), with 0% 30-day mortality in the surgical group. On multivariable Cox proportional hazard analysis, increasing age (hazard ratio HR, 1.20), coronary artery disease (HR, 1.68), worse New York Heart Association class (HR, 1.46), and atrial fibrillation (HR, 1.90) predicted higher events, whereas surgery (time-dependent covariate HR, 0.57) was associated with improved event-free survival (all P < .01). Conclusions In patients with hypertrophic cardiomyopathy and severe left ventricular outflow tract obstruction, in whom the decision regarding surgery was based on the presence of intractable symptoms and impaired exercise capacity, surgery was associated with significant improvement in long-term composite outcomes.
Left ventricular dysfunction is characterized by systolic and diastolic parameters, leading to heart failure (HF) with reduced or preserved ejection fraction (EF), respectively. The goal of this ...study is to examine the impact of left ventricular systolic and diastolic dysfunction (DD) on patient outcomes.
Two cohorts were used in this analysis: Cohort A included 136 455 patients with EF ≥50%, stratified by the presence and grade of DD. Cohort B included 16 850 patients with EF <50%, stratified by EF quartiles. Patients were followed to the end points of all-cause death and cardiovascular, HF, or cardiac arrest hospitalizations. Over a median follow-up of 3.42 years, 23 946 (16%) patients died and 31 113 (20%), 13 305 (9%), and 1269 (1%) were hospitalized for cardiovascular, HF, or cardiac arrest causes, respectively. With adjustment for comorbidities, the risk of all-cause mortality and of cardiovascular and HF hospitalizations increased steadily with increasing grade of DD in patients with normal EF, and even more so in patients with worsening EF. The risk of hospitalization for cardiac arrest in patients with grade III DD, however, was comparable to that of patients with EF <25% (hazard ratio, 1.00 95% CI, 0.98-1.01) and worse than that of patients in better EF quartiles.
Although systolic dysfunction is associated with a greater risk of overall death and HF hospitalizations than DD, the risk of cardiac arrest in patients with grade II and III DD is comparable to that of patients with moderate and severe systolic dysfunction, respectively. Future studies are needed to examine treatment strategies than can improve these outcomes.
This study sought to evaluate direct oral anticoagulant (DOAC) outcomes (vs. warfarin) in patients with atrial fibrillation (AF) across body mass index (BMI) categories, including ≥40 ...and <18.5 kg/m2.
Clinical trials have not systematically tested the fixed DOAC dosing in underweight and morbidly obese patients.
We retrospectively included consecutive patients with nonvalvular AF with CHA2DS2-VASc (Congestive heart failure, Hypertension, Age ≥75, Diabetes, Stroke/transient ischemic attack/systemic thromboembolism, Vascular disease, Age 65-74, Sex) of ≥1 receiving OACs at our hospital system (2010–2018). Patients were categorized into groups 1 (underweight: BMI of <18.5 kg/m2), 2 (normal/overweight: BMI of 18.5 to <30 kg/m2), 3 (grade 1/2 obesity: BMI of 30 to <40 kg/m2), and 4 (grade 3 obesity: BMI of ≥40 kg/m2). We further classified patients by DOAC versus warfarin use. Outcomes were ischemic stroke, significant bleeding events (i.e., resulting in hospitalization), and mortality.
We included 36,094 patients with a mean age of 74 ± 11 years and CHA2DS2-VASc of 3.4 ± 1.5. Groups 1 through 4 included 455 (1.3%), 18,339 (50.8%), 13,376 (37.1%), and 3,924 (10.9%) patients, respectively. DOAC use ranged from 49% to 56%. At 3.8 follow-up years, with multivariable Cox regression, DOACs (vs. warfarin) were associated with lower risk of ischemic stroke, bleeding, and mortality across all BMI groups, with hazard ratios (HRs) of 0.73 (95% confidence interval CI: 0.63 to 0.85), 0.75 (95% CI: 0.64 to 0.87), 0.75 (95% CI: 0.65 to 0.88), and 0.75 (95% CI: 0.64 to 0.87) (p < 0.001 for all) for ischemic stroke; 0.42 (95% CI: 0.19 to 0.92), 0.41 (95% CI: 0.19 to 0.89), 0.45 (95% CI: 0.20 to 1.00), and 0.43 (95% CI: 0.20 to 0.94) (p < 0.05 for all) for bleeding; and 0.90 (95% CI: 0.68 to 1.19; p = 0.5), 0.70 (95% CI: 0.66 to 0.75; p < 0.0001), 0.65 (95% CI: 0.60 to 0.71; p < 0.0001), and 0.66 (95% CI: 0.56 to 0.77; p < 0.0001) for mortality, in groups 1 to 4, respectively.
In patients with nonvalvular AF, DOACs compared to warfarin were associated with better safety and effectiveness across all BMI categories, including underweight and morbidly obese patients.
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Background Hypertrophic cardiomyopathy (HCM) is histopathologically characterized by myocyte hypertrophy, disarray, interstitial fibrosis, and small intramural coronary arteriole dysplasia, which ...contribute to disease progression. Longitudinal systolic and early diastolic strain rate (SR) measurements by speckle tracking echocardiography are sensitive markers of regional myocardial function. We sought to determine the association between septal SR and histopathologic findings in symptomatic HCM patients who underwent surgical myectomy. Methods We studied 171 HCM patients (documented on histopathology) who underwent surgical myectomy to relieve left ventricular outflow tract obstruction. Various clinical and echocardiographic parameters were recorded. Segmental longitudinal systolic and early diastolic SRs (of the septal segment removed at myectomy) were measured from apical 4- and 2-chamber views (VVI 2.0; Siemens, Erlangen, Germany). Histopathologic myocyte hypertrophy, disarray, small intramural coronary arteriole dysplasia, and interstitial fibrosis were classified as none, mild (1%-25%), moderate (26%-50%), and severe (>50%). Results The mean age was 53 ± 14 years (52% men, ejection fraction 62% ± 5%, mean left ventricular outflow tract gradient 102 ± 39 mm Hg, and basal septal thickness of 2.2 ± 0.5 cm). Mean longitudinal systolic and early diastolic SRs were −0.91 ± 0.5 and 0.82 ± 0.5 (1/s), respectively. There was an inverse association between systolic and early diastolic septal SR and degree of myocyte hypertrophy, disarray, and interstitial fibrosis (all P < .05). There was no association between histopathologic characteristics and other echocardiography parameters. On multivariable regression analysis, myocyte disarray and echocardiographic septal hypertrophy were associated with systolic and early diastolic septal SR ( P < .05). Conclusion In HCM patients, there is inverse association between various histopathologic findings and septal SR. Strain rate might potentially provide further insight into HCM pathophysiology.
Background Over the next few years, atrial fibrillation (AF)-related morbidity and costs will increase significantly. Thus, it is prudent to examine the impact of AF treatment on health care resource ...use. This study examined the impact of AF ablation on hospitalization, length of stay, and resource use for patients undergoing AF ablation in a multihospital system. Methods and Results In an observational analysis, outcomes of total, cardiovascular, and AF hospitalizations, emergency department visits, and length of stay were compared for 3417 patients between 12 months before and 24 months following AF ablation. Use of electrical cardioversions and antiarrhythmic use were also compared 1 year before to 2 years after AF ablation. There were fewer total (0.7±1.3 versus 0.3±0.7;
<0.001), cardiovascular (0.7±1.2 versus 0.2±0.6;
<0.001), and AF (0.6±1.1 versus 0.1±0.3;
<0.001) hospitalizations and emergency department visits (0.8±2.1 versus 0.4±0.9;
<0.001) per patient-year for the 2 years following AF ablation compared with 1 year before. Average length of stay per patient-year (1.4±7.9 versus 3.6±5.3 days;
<0.0001), the percentage of patients on antiarrhythmic therapy (21.2% versus 58.5%;
<0.0001), and those undergoing electrical cardioversions (16.1% versus 28.1%;
<0.0001) were lower 2 years following AF ablation versus 1 year before. Conclusions We noted a decrease in total, cardiovascular, and AF hospitalizations and health care resource use during the 2-year period after index AF ablation, compared with the 1 year before. AF ablation may portend a decline in patient morbidity and health care costs.
The clinical profile of arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) patients with late presentation is unknown.
The purpose of this study was to characterize the genotype, ...cardiac phenotype, and long-term outcomes of ARVC/D patients with late presentation (age ≥50 years at diagnosis).
Five hundred two patients with an ARVC/D diagnosis from Johns Hopkins and Utrecht Registries were studied and long-term clinical outcomes ascertained.
Late presentation was seen in 104 patients (21%; 38% PKP2 carriers); 3% were ≥65 years at diagnosis. Sustained ventricular tachycardia was the major (43%) mode of presentation in patients with late presentation, whereas cardiac syncope was infrequent (P <.001). Those with late presentation were significantly less likely to harbor a known pathogenic mutation (53%; P = .005), have less precordial T-wave repolarization changes (P <.001), and have lower ventricular ectopy burden (P = .026). Over median 6-year follow-up, 68 patients with late presentation (65%) experienced sustained ventricular arrhythmias, with similar arrhythmia-free survival at 5-year follow up (P = .48). Left ventricular dysfunction and heart failure were seen in 24 (32%) and 15 patients (14%), respectively, without need for cardiac transplantation. In the late presentation cohort, male sex, pathogenic mutation, right ventricular structural disease, lack of family history, and electrophysiologic study inducibility were associated with increased arrhythmic risk.
One-fifth of all ARVC/D patients present after age 50 years, often with sustained ventricular tachycardia, and are less likely to have prior syncope, ECG changes, ventricular ectopy, or identifiable pathogenic mutation. In ARVC/D, late presentation does not confer a benign prognosis and is associated with high arrhythmic risk.
Background Despite growing use of the subcutaneous implantable cardioverter-defibrillator (S- ICD ), its clinical role in arrhythmogenic right ventricular cardiomyopathy/dysplasia ( ARVC /D) patients ...remains undefined. We aim to elucidate the cardiac phenotype, implant characteristics, and long-term efficacy regarding appropriate therapy and complications in ARVC /D patients with an S- ICD implant. Methods and Results A transatlantic cohort of ARVC /D patients who underwent S- ICD implantation was analyzed for clinical characteristics, S- ICD therapy, and long-term outcome including device-related complications. The cohort included 29 patients (52% male, 76% probands, 59% with ARVC /D-associated mutation, 59% primary prevention no prior sustained ventricular arrhythmias, and 45% first-generation S- ICD devices). At implant, all inducible patients (27/29) had conversion of induced ventricular fibrillation. Two patients (7%) had superficial infections of the incision site that were treated conservatively. Over a median follow-up of 3.16 years (interquartile range: 2.21-4.51 years), all episodes (6 patients, 4% per year) of sustained ventricular arrhythmias were appropriately detected and treated. Six patients (21%) experienced 39 inappropriate shocks, with 3 requiring device explantation. Oversensing of noncardiac signal (n=4; especially myopotentials) and cardiac signal (n=4) was the most frequent etiology. No lead or device dislodgement, infection, skin erosion, or explantation related to need for antitachycardia pacing was noted. Conclusions S- ICD can effectively treat both induced and spontaneous ventricular arrhythmias in patients with ARVC /D. The rate of inappropriate shocks, although considerable, is comparable to that in ARVC /D patients treated with transvenous ICD s. When they occurred, inappropriate shocks were primarily due to cardiac and, uniquely, noncardiac oversensing. We suggest potential strategies for minimizing inappropriate therapy.
Highlights • Cardiac amyloid (CAm) may be not be recognized in patients with normal EF. • Independent predictors of CAm: older age, lower BMI, neuropathy, low voltage QRS • CAm is an independent ...predictor of mortality among patients with normal EF.
In Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C), a normal electrocardiogram (ECG) is considered reassuring. However, some patients with ARVD/C experiencing ventricular ...arrhythmias have a normal ECG.
To estimate how often patients with ARVD/C experiencing ventricular arrhythmias have a normal ECG during sinus rhythm, and to provide a clinical profile of these patients.
We included 145 patients with ARVD/C experiencing a documented sustained ventricular arrhythmia. Conventional 12-lead sinus rhythm ECGs within 6 months of the event were reviewed for diagnostic Task Force Criteria (TFC). ECGs were classified as abnormal (≥1 TFC), nonspecific (abnormal, no TFC), or normal. Cardiologic investigations within 6 months of the event were evaluated as per TFC in those with a nonspecific or normal ECG.
The ECG was nonspecific or normal in 17 of 145 (12%) subjects. Mean age of these patients was 41.3 ± 12.4 years and 14 (82%) were men, comparable to those with an abnormal ECG. Most patients with a nonspecific or normal ECG showed ≥1 TFC on Holter monitoring (n = 9 of 10) and signal-averaged ECG (n = 4 of 5), and all had nonsustained ventricular tachycardia recorded. Among 15 patients who underwent structural evaluation, 11 (73%) showed structural TFC (9 major and 2 minor).
Although most patients with ARVD/C experiencing arrhythmias have an abnormal ECG, a nonspecific or normal ECG does not preclude ARVD/C diagnosis. All patients with a nonspecific or normal ECG had alternative evidence of disease expression. These results alert the physician not to rely exclusively on ECG in ARVD/C, but to assess arrhythmic risk by comprehensive clinical evaluation.
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