Guidelines for the Evaluation of Prosthetic Valve Function With Cardiovascular Imaging: A Report From the American Society of Echocardiography Developed in Collaboration With the Society for Cardiovascular Magnetic Resonance and the Society of Cardiovascular Computed Tomography.

In patients with significant cardiac valvular disease, intervention with either valve repair or valve replacement may be inevitable. Although valve repair is frequently performed, especially for mitral and tricuspid regurgitation, valve replacement remains common, particularly in adults. Diagnostic methods are often needed to assess the function of the prosthesis. Echocardiography is the first-line method for noninvasive evaluation of prosthetic valve function. The transthoracic approach is complemented with two-dimensional and three-dimensional transesophageal echocardiography for further refinement of valve morphology and function when needed. More recently, advances in computed tomography and cardiac magnetic resonance have enhanced their roles in evaluating valvular heart disease. This document offers a review of the echocardiographic techniques used and provides recommendations and general guidelines for evaluation of prosthetic valve function on the basis of the scientific literature and consensus of a panel of experts. This guideline discusses the role of advanced imaging with transesophageal echocardiography, cardiac computed tomography, and cardiac magnetic resonance in evaluating prosthetic valve structure, function, and regurgitation. It replaces the 2009 American Society of Echocardiography guideline on prosthetic valves and complements the 2019 guideline on the evaluation of valvular regurgitation after percutaneous valve repair or replacement.

Utility of Left and Right Ventricular Strain in Arrhythmogenic Right Ventricular Cardiomyopathy: A Prospective Multicenter Registry.

BACKGROUND: Imaging evaluation of arrhythmogenic right ventricular cardiomyopathy (ARVC) remains challenging. Myocardial strain assessment by echocardiography is an increasingly utilized technique for detecting subclinical left ventricular (LV) and right ventricular (RV) dysfunction. We aimed to evaluate the diagnostic and prognostic utility of LV and RV strain in ARVC. METHODS: Patients with suspected ARVC (n = 109) from a multicenter registry were clinically phenotyped using the 2010 ARVC Revised Task Force Criteria and underwent baseline strain echocardiography. Diagnostic performance of LV and RV strain was evaluated using the area under the receiver operating characteristic curve analysis against the 2010 ARVC Revised Task Force Criteria, and the prognostic value was assessed using the Kaplan-Meier analysis. RESULTS: Mean age was 45.3±14.7 years, and 48% of patients were female. Estimation of RV strain was feasible in 99/109 (91%), and LV strain was feasible in 85/109 (78%) patients. ARVC prevalence by 2010 ARVC Revised Task Force Criteria is 91/109 (83%) and 83/99 (84%) in those with RV strain measurements. RV global longitudinal strain and RV free wall strain had diagnostic area under the receiver operating characteristic curve of 0.76 and 0.77, respectively (both P<0.001; difference NS). Abnormal RV global longitudinal strain phenotype (RV global longitudinal strain > -17.9%) and RV free wall strain phenotype (RV free wall strain > -21.2%) were identified in 41/69 (59%) and 56/69 (81%) of subjects, respectively, who were not identified by conventional echocardiographic criteria but still met the overall 2010 ARVC Revised Task Force Criteria for ARVC. LV global longitudinal strain did not add diagnostic value but was prognostic for composite end points of death, heart transplantation, or ventricular arrhythmia (log-rank P=0.04). CONCLUSIONS: In a prospective, multicenter registry of ARVC, RV strain assessment added diagnostic value to current echocardiographic criteria by identifying patients who are missed by current echocardiographic criteria yet still fulfill the diagnosis of ARVC. LV strain, by contrast, did not add incremental diagnostic value but was prognostic for identification of high-risk patients.

Sex Differences in Extensive Mitral Annular Calcification With Associated Mitral Valve Dysfunction.

Mitral annular calcification (MAC)-related mitral valve (MV) dysfunction is an increasingly recognized entity, which confers a high burden of morbidity and mortality. Although more common among women, there is a paucity of data regarding how the phenotype of MAC and the associated adverse clinical implications may differ between women and men. A total of 3,524 patients with extensive MAC and significant MAC-related MV dysfunction (i.e., transmitral gradient ≥3 mm Hg) were retrospectively analyzed from a large institutional database, with the goal of defining gender differences in clinical and echocardiographic characteristics and the prognostic importance of MAC-related MV dysfunction. We stratified patients into low- (3 to 5 mm Hg), moderate- (5 to 10 mm Hg), and high- (≥10 mm Hg) gradient groups and analyzed the gender differences in phenotype and outcome. The primary outcome was all-cause mortality, assessed using adjusted Cox regression models. Women represented the majority (67%) of subjects, were older (79.3 ± 10.4 vs 75.5 ± 10.9 years, p <0.001) and had a lower burden of cardiovascular co-morbidities than men. Women had higher transmitral gradients (5.7 ± 2.7 vs 5.3 ± 2.6 mm Hg, p <0.001), more concentric hypertrophy (49% vs 33%), and more mitral regurgitation. The median survival was 3.4 years (95% confidence interval 3.0 to 3.6) among women and 3.0 years (95% confidence interval 2.6 to 4.5) among men. The adjusted survival was worse among men, and the prognostic impact of the transmitral gradient did not differ overall by gender. In conclusion, we describe important gender differences among patients with MAC-related MV dysfunction and show worse adjusted survival among men; although, the adverse prognostic impact of the transmitral gradient was similar between men and women.

Apical Aneurysms and Mid-Left Ventricular Obstruction in Hypertrophic Cardiomyopathy.

BACKGROUND: Apical left ventricular (LV) aneurysms in hypertrophic cardiomyopathy (HCM) are associated with adverse outcomes. The reported frequency of mid-LV obstruction has varied from 36% to 90%. OBJECTIVES: The authors sought to ascertain the frequency of mid-LV obstruction in HCM apical aneurysms. METHODS: The authors analyzed echocardiographic and cardiac magnetic resonance examinations of patients with aneurysms from 3 dedicated programs and compared them with 63 normal controls and 47 controls with apical-mid HCM who did not have aneurysms (22 with increased LV systolic velocities). RESULTS: There were 108 patients with a mean age of 57.4 ± 13.5 years; 40 (37%) were women. A total of 103 aneurysm patients (95%) had mid-LV obstruction with mid-LV complete systolic emptying. Of the patients with obstruction, 84% had a midsystolic Doppler signal void, a marker of complete flow cessation, but only 19% had Doppler systolic gradients ≥30 mm Hg. Five patients (5%) had relative hypokinesia in mid-LV without obstruction. Aneurysm size is not bimodal but appears distributed by power law, with large aneurysms decidedly less common. Comparing mid-LV obstruction aneurysm patients with all control groups, the short-axis (SAX) systolic areas were smaller (P < 0.007), the percent SAX area change was greater (P < 0.005), the papillary muscle (PM) areas were larger (P < 0.003), and the diastolic PM areas/SAX diastolic areas were greater (P < 0.005). Patients with aneurysms had 22% greater SAX PM areas compared with those with elevated LV velocities but no aneurysms (median: 3.00 cm2 [IQR: 2.38-3.70 cm2] vs 2.45 [IQR: 1.81-2.95 cm2]; P = 0.004). Complete emptying occurs circumferentially around central PMs that contribute to obstruction. Late gadolinium enhancement was always brightest and the most transmural apical of, or at the level of, complete emptying. CONCLUSIONS: The great majority (95%) of patients in the continuum of apical aneurysms have associated mid-LV obstruction. Further research to investigate obstruction as a contributing cause to apical aneurysms is warranted.

Frequency, predictors and cardiovascular outcomes associated with transthoracic echocardiographic findings during acute ischaemic stroke hospitalisation.

OBJECTIVE: To characterise the clinical utility of transthoracic echocardiography (TTE) at the time of acute ischaemic stroke (AIS). BACKGROUND: The utility of obtaining a TTE during AIS hospitalisation is uncertain. METHODS: We studied AIS hospitalisations at a single centre (2002-2016). TTE abnormalities were classified as findings associated with: high stroke risk (Category I), cardiac events (Category II) and of unclear significance (Category III). We performed logistic regressions to predict Category I, II and III abnormalities. The odds of 1 year recurrent stroke hospitalisation captured by ICD 9 and 10 codes as a function of Category I, II and III abnormalities were assessed. Improvement in predictive capacity for 1 year recurrent ischaemic stroke hospitalisation beyond stroke risk factors was evaluated by net reclassification improvement. RESULTS: There were 5523 AIS hospitalisations. Nearly 81% of admission TTEs were abnormal (18.7% Category I, 32.7% Category II, 72.8% Category III). Older patients with coronary artery disease, atrial fibrillation, hypertension, diabetes, and patent intracranial and extracranial vessels were likely to have an abnormal TTE. Category I finding was associated with lower odds of 1-year recurrent stroke hospitalisation (OR 0.54, 95% CI 0.30 to 0.96). Category I data significantly improved the predictive value for 1-year recurrent ischaemic stroke hospitalisation beyond stroke risk factors (net reclassification improvement 0.1563, 95% CI 0.0465 to 0.2661). CONCLUSIONS: TTE abnormalities associated with stroke and cardiac event risk were commonly detected during AIS hospitalisation. Detection of Category I TTE findings reduced the risk of recurrent stroke, potentially due to neutralisation of the cardioembolic source by targeted therapy, indicating the clinical utility of TTE.

Impact of Pulmonary Hypertension on Outcomes in Patients With Mitral Annular Calcium and Associated Mitral Valve Dysfunction.

The prevalence of mitral annular calcium (MAC) is increasing in our aging population. However, data regarding prognostication in MAC-related mitral valve (MV) disease remain limited. This retrospective observational study aims to explore the prognostic impact of systolic pulmonary artery pressure (SPAP) in MAC-related MV dysfunction and define its determinants. We identified 4,384 patients (mean age 78 ± 11 years and 69% female) with MAC-related MV dysfunction (documented transmitral gradient ≥3 mm Hg) from a large institutional echocardiographic database between 2001 and 2019. In Cox regression analysis, higher SPAP strongly associated with all-cause mortality, independent of cardiovascular risk factors and indices of MV dysfunction (adjusted hazard ratio 1.22 per 10 mm Hg SPAP increase, 95% confidence interval 1.17 to 1.27). Patients with SPAP ≥50 mm Hg had significantly higher mortality compared with SPAP <50 mm Hg (log-rank p <0.001), a finding that was consistent across different transmitral gradient subgroups (≤5, 5 to 10, and ≥10 mm Hg). Independent determinants of SPAP included the mean transmitral gradient, mitral regurgitation severity, left ventricular ejection fraction, and ≥moderate aortic stenosis (adjusted p <0.05), and atrial fibrillation and left atrial dimension. The impact of concomitant mitral regurgitation on SPAP decreased at higher transmitral gradients and was no longer significant at gradients ≥10 mm Hg (p = 0.100). In conclusion, SPAP strongly associates with mortality in MAC, independent of cardiovascular risk factors and indices of MAC-related MV dysfunction. These findings suggest an incremental role for SPAP in the risk stratification and prognostication in this increasingly prevalent condition with expanding the scope of possible interventions.

Acute Ischemic Stroke, Depressed Left Ventricular Ejection Fraction, and Sinus Rhythm: Prevalence and Practice Patterns.

BACKGROUND: There are limited data about the epidemiology and secondary stroke prevention strategies used for patients with depressed left ventricular ejection fraction (LVEF) and sinus rhythm following an acute ischemic stroke (AIS). We sought to describe the prevalence of LVEF ≤40% and sinus rhythm among patients with AIS and antithrombotic treatment practice in a multi-center cohort from 2002 to 2018. METHODS: This was a multi-center, retrospective cohort study comprised of patients with AIS hospitalized in the Greater Cincinnati Northern Kentucky Stroke Study and 4 academic, hospital-based cohorts in the United States. A 1-stage meta-analysis of proportions was undertaken to calculate a pooled prevalence. Univariate analyses and an adjusted multivariable logistic regression model were performed to identify demographic, clinical, and echocardiographic characteristics associated with being prescribed an anticoagulant upon AIS hospitalization discharge. RESULTS: Among 14 338 patients with AIS with documented LVEF during the stroke hospitalization, the weighted pooled prevalence of LVEF ≤40% and sinus rhythm was 5.0% (95% CI, 4.1-6.0%; I2, 84.4%). Of 524 patients with no cardiac thrombus and no prior indication for anticoagulant who survived postdischarge, 200 (38%) were discharged on anticoagulant, 289 (55%) were discharged on antiplatelet therapy only, and 35 (7%) on neither. There was heterogeneity by site in the proportion discharged with an anticoagulant (22% to 45%, P<0.0001). Cohort site and National Institutes of Health Stroke Severity scale >8 (odds ratio, 2.0 [95% CI, 1.1-3.8]) were significant, independent predictors of being discharged with an anticoagulant in an adjusted analysis. CONCLUSIONS: Nearly 5% of patients with AIS have a depressed LVEF and are in sinus rhythm. There is significant variation in the clinical practice of antithrombotic therapy prescription by site and stroke severity. Given this clinical equipoise, further study is needed to define optimal antithrombotic treatment regimens for secondary stroke prevention in this patient population.

A Case of Spontaneous Coronary Artery Dissection in a Patient with Severe Anorexia Nervosa.

There are a variety of cardiac complications of anorexia nervosa including arrythmias, valvopathies, and myopathies. Spontaneous coronary artery dissection (SCAD) has not been widely reported among this patient population. This case report describes a middle-aged female with severe anorexia nervosa, who presented after being found unconscious, and was later diagnosed with SCAD. A literature review revealed one previous case of SCAD in a patient with anorexia nervosa and prompted a discussion of a series of possible predisposing factors for SCAD in this patient population. Patients with anorexia nervosa may be at increased risk for SCAD due to their complex nutritional and endocrine imbalances. This case highlights a possible underdiagnosed cardiac complication of anorexia nervosa.

Left Atrial Mechanics Assessed Early during Hospitalization for Cryptogenic Stroke Are Associated with Occult Atrial Fibrillation: A Speckle-Tracking Strain Echocardiography Study.

BACKGROUND: Occult atrial fibrillation (AF) is an important contributor to cryptogenic stroke, yet remains difficult to unmask at presentation. This study investigated the predictive value of left atrial (LA) mechanics by strain echocardiography during stroke hospitalization for the presence of AF as detected on early 30-day monitoring and routine clinical follow-up. METHODS: Left atrial mechanics were studied by strain echocardiography in a retrospective cohort of 191 patients with cryptogenic stroke and 30-day mobile cardiac outpatient telemetry poststroke to diagnose AF. After this, AF was diagnosed via routine clinical follow-up. The independent and incremental value of measures of LA size and mechanics (i.e., strain and strain rate in the reservoir, conduit, and booster pump phase) to predict AF on top of clinical characteristics was assessed. RESULTS: Of 191 patients, 15% (n = 28) developed AF, of which 10 were observed during 30-day mobile cardiac outpatient telemetry and 18 were observed at a median follow-up of 25 (interquartile range, 10-43) months. Median time from embolic stroke to strain echocardiography was 1 day (interquartile range, 1-2 days). Left atrial mechanics were significantly worse in AF (P < .05 for all), despite largely similar baseline cardiovascular risk profile. Booster pump strain rate was the strongest predictor for AF, independent of age, LA volume index, E/e', and reservoir strain (odds ratio = 2.88 per SD increase; 95% confidence interval, 1.29-6.41; P = .010). Adding LA strain reservoir strain and booster pump function significantly enhanced a multivariate model to predict AF. Freedom from AF was significantly lower in subjects with a booster pump strain rate (at stroke presentation) worse than -0.67 sec-1, as derived from receiver operator curve analysis (P < .001). CONCLUSIONS: Left atrial mechanics and particularly the LA booster pump function assessed early during hospitalization for cryptogenic stroke can identify patients at greater likelihood of future diagnosis of AF. These findings could in part relate to LA mechanical stunning after spontaneous cardioversion, which-when identified by early strain echocardiography-can inform further risk stratification and decision-making.

A comparison of postprocedural anticoagulation in high-risk patients undergoing WATCHMAN device implantation.

BACKGROUND: Left atrial appendage closure (LAAC) is an alternative to long-term anticoagulation for thromboembolic protection in patients with atrial fibrillation (AF) and high bleeding risk. Short-term Warfarin use following LAAC is well-studied, while data pertaining to novel oral anticoagulant (NOAC) use in this setting is less robust. Specifically, data regarding the safety and efficacy of postprocedural NOAC use in high-risk patients is lacking. OBJECTIVE: To compare the safety and efficacy of Warfarin and NOAC use in a high-risk patient population undergoing LAAC with the WATCHMAN device. METHODS: From November 2015 to October 2017, 97 patients underwent LAAC with the WATCHMAN device. All patients were discussed at a multidisciplinary meeting prior to device implantation. Longitudinal data were collected and analyzed for a composite endpoint of stroke and death at 8 months, and major bleeding at 3 and 6 months. RESULTS: Among the 90 patients included in the safety and efficacy analysis, 43 were prescribed Warfarin and 47 were prescribed NOACs. Baseline characteristics were comparable between study groups. There were no procedural complications and no significant differences in the incidence of death and stroke at 8 months or major bleeding at 3 and 6 months. CONCLUSION: For patients with AF at high risk of both thromboembolic and hemorrhagic events, NOACs as compared to Warfarin, seem to be safe and effective for short-term anticoagulation following LAAC with the WATCHMAN device. Further validation in large randomized controlled trials is required.

Noninvasive Multimodality Imaging in ARVD/C.

Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a familial cardiomyopathy resulting in progressive right ventricular (RV) dysfunction and malignant ventricular arrhythmias. Although ARVD/C is generally considered an inherited cardiomyopathy, the arrhythmogenic nature of the disease is striking. Affected individuals typically present in the second to fourth decade of life with arrhythmias originating from the right ventricle. Over the past decade, pathogenic ARVD/C-causing mutations have been identified in 5 genes encoding the cardiac desmosome. Disruption of the desmosomal connection system between cardiomyocytes may be represented structurally by ventricular enlargement, global or regional contraction abnormalities, RV aneurysms, or fibrofatty replacement. These abnormalities are typically observed in predilection areas, including the subtricuspid region, basal RV free wall, and left ventricular posterolateral wall. As such, structural and functional abnormalities on cardiac imaging constitute an important diagnostic criterion for the disease. This paper discusses the current status and role of echocardiography, cardiac magnetic resonance imaging, and computed tomography for suspected ARVD/C.

Educational intervention to reduce outpatient inappropriate echocardiograms: a randomized control trial.

OBJECTIVES: This study sought to prospectively study the impact of an appropriate use criteria (AUC)-based educational intervention on outpatient transthoracic echocardiography (TTE) ordering by physicians-in-training. BACKGROUND: AUC were developed in response to concerns about inappropriate utilization. It is unknown whether an educational intervention can reduce inappropriate outpatient TTE. METHODS: We conducted a randomized control trial in which physicians-in-training were randomized to an AUC-based educational intervention or a control group at an academic medical center in Boston, Massachusetts. The primary endpoints were the rates of inappropriate and appropriate TTE. RESULTS: For the cardiology physicians-in-training, the proportion of inappropriate TTE was significantly lower in the intervention than in the control group (13% vs. 34%, p < 0.001). As a corollary, the proportion of appropriate TTE ordered by the intervention group was significantly higher than that of the control group (81% vs. 58%, p < 0.001). The odds of ordering an appropriate TTE in the cardiology intervention group was 2.7 (95% confidence interval [CI]: 1.5 to 5.1, p = 0.002) relative to the control group. The internal medicine physicians-in-training ordered a small number of TTE overall, and there was a trend toward significant odds of ordering an appropriate TTE in the intervention group relative to the control group (odds ratio [OR]: 8.1, 95% CI: 0.95 to 69.0, p = 0.055). Six clinical scenarios accounted for 75% of all inappropriate TTE, with the 3 most common inappropriate indications being routine surveillance (<1 year) of known cardiomyopathy without a change in clinical status, routine surveillance of known small pericardial effusion, and routine surveillance of ventricular function with known coronary artery disease and no change in clinical status. CONCLUSIONS: In cardiology fellows with a high rate of ordering inappropriate TTE, an AUC-based educational and feedback intervention reduced the proportion of inappropriate outpatient TTE and increased the proportion of appropriate outpatient TTE. (Educational Intervention to Reduce Outpatient Inappropriate Transthoracic Echocardiograms; NCT01944202).

Management strategy in 249 consecutive patients with obstructive hypertrophic cardiomyopathy referred to a dedicated program.

The likelihood of success of conservative management of obstructive hypertrophic cardiomyopathy (HC) and the predictors of failure of conservative therapy are not known. We therefore evaluated the efficacy of an algorithm for the management of symptoms and predictors of failed conservative therapy in 249 consecutive symptomatic patients with obstructive HC referred to a dedicated HC program for management in general or for septal reduction therapy (SRT) in particular. There was considerable practice variation in the extent to which conservative therapy was optimized before referral for SRT. Over 3.7 ± 2.9-year follow-up, symptoms resolved with addition of or increase in dosage of a ß blocker, calcium channel blocker, or disopyramide in 16%, 10%, and 10% of patients, respectively. Pacing with short atrioventricular delay controlled symptoms in 4 of 9 patients. In 63% of patients, conservative measures failed to control symptoms. Multivariate predictors of failure of conservative therapy were presence of New York Heart Association class III or IV symptoms (hazard ratio 2.0, 95% confidence interval 1.4 to 2.9, p = 0.001) and greater septal wall thickness (hazard ratio 1.06, 95% confidence interval 1.02 to 1.10, p = 0.003) at presentation. At time of presentation, 93 patients (37%) were already on optimal therapy and were referred for SRT. Of the remaining 156 patients who did not require immediate SRT, 93 (60%) were free from a recommendation for SRT at the end of the follow-up period. In conclusion, in symptomatic patients with obstructive HC, conservative therapy is successful in >1/3 of referred patients at 3.7-year follow-up, obviating SRT in these patients. Clinicians in programs offering SRT should optimize conservative therapy before recommending SRT.

Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the task force criteria.

BACKGROUND: In 1994, an International Task Force proposed criteria for the clinical diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) that facilitated recognition and interpretation of the frequently nonspecific clinical features of ARVC/D. This enabled confirmatory clinical diagnosis in index cases through exclusion of phenocopies and provided a standard on which clinical research and genetic studies could be based. Structural, histological, electrocardiographic, arrhythmic, and familial features of the disease were incorporated into the criteria, subdivided into major and minor categories according to the specificity of their association with ARVC/D. At that time, clinical experience with ARVC/D was dominated by symptomatic index cases and sudden cardiac death victims-the overt or severe end of the disease spectrum. Consequently, the 1994 criteria were highly specific but lacked sensitivity for early and familial disease. METHODS AND RESULTS: Revision of the diagnostic criteria provides guidance on the role of emerging diagnostic modalities and advances in the genetics of ARVC/D. The criteria have been modified to incorporate new knowledge and technology to improve diagnostic sensitivity, but with the important requisite of maintaining diagnostic specificity. The approach of classifying structural, histological, electrocardiographic, arrhythmic, and genetic features of the disease as major and minor criteria has been maintained. In this modification of the Task Force criteria, quantitative criteria are proposed and abnormalities are defined on the basis of comparison with normal subject data. CONCLUSIONS: The present modifications of the Task Force Criteria represent a working framework to improve the diagnosis and management of this condition. CLINICAL TRIAL REGISTRATION: URL: http://www.clinicaltrials.gov. Unique identifier: NCT00024505.

Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the Task Force Criteria.

BACKGROUND: In 1994, an International Task Force proposed criteria for the clinical diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) that facilitated recognition and interpretation of the frequently nonspecific clinical features of ARVC/D. This enabled confirmatory clinical diagnosis in index cases through exclusion of phenocopies and provided a standard on which clinical research and genetic studies could be based. Structural, histological, electrocardiographic, arrhythmic, and familial features of the disease were incorporated into the criteria, subdivided into major and minor categories according to the specificity of their association with ARVC/D. At that time, clinical experience with ARVC/D was dominated by symptomatic index cases and sudden cardiac death victims-the overt or severe end of the disease spectrum. Consequently, the 1994 criteria were highly specific but lacked sensitivity for early and familial disease. METHODS AND RESULTS: Revision of the diagnostic criteria provides guidance on the role of emerging diagnostic modalities and advances in the genetics of ARVC/D. The criteria have been modified to incorporate new knowledge and technology to improve diagnostic sensitivity, but with the important requisite of maintaining diagnostic specificity. The approach of classifying structural, histological, electrocardiographic, arrhythmic, and genetic features of the disease as major and minor criteria has been maintained. In this modification of the Task Force criteria, quantitative criteria are proposed and abnormalities are defined on the basis of comparison with normal subject data. CONCLUSIONS: The present modifications of the Task Force Criteria represent a working framework to improve the diagnosis and management of this condition. Clinical Trial Registration clinicaltrials.gov Identifier: NCT00024505.

Ventricular arrhythmia following alcohol septal ablation for obstructive hypertrophic cardiomyopathy.

We sought to assess the risk of sudden cardiac death (SCD) and ventricular arrhythmia after alcohol septal ablation (ASA) for obstructive hypertrophic cardiomyopathy. ASA is a nonsurgical alternative to septal myectomy for treatment of symptomatic, drug-refractory, obstructive hypertrophic cardiomyopathy. The effect of ASA on ventricular arrhythmia risk is not well established. We examined the rates of SCD among 89 patients treated with ASA. The secondary end point was ventricular tachycardia/ventricular fibrillation (VT/VF), appropriate implantable cardioverter defibrillator (ICD) therapy, or cardiac arrest after ASA among those with implanted ICDs or permanent pacemakers (n = 42). Patients were classified as either high-risk or low-risk on the basis of established clinical indications for ICD implantation. No mortality was attributable to SCD at a mean follow-up of 5.0 +/- 2.3 years in the entire cohort. Among the 42 patients with an ICD or permanent pacemaker, 9 had documented VT/VF, cardiac arrest, or appropriate ICD therapy, resulting in an annual event rate of 4.9%/year. The annual event rate for VT/VF, cardiac arrest, or appropriate ICD therapy was 2.8%/year (4 of 29 patients) in low-risk patients and 13.4% in high-risk patients (5 of 13 patients). A 10-mm Hg increase in the immediate post-ASA gradient was associated with a hazard ratio of 2.66 for arrhythmic events (95% confidence interval 1.55 to 4.56, p <0.001). In conclusion, ASA was performed in patients with highly symptomatic, drug-refractory hypertrophic cardiomyopathy with no mortality attributable to SCD and an annual rate of VT/VF, cardiac arrest, or appropriate ICD therapy of 4.9%/year.

Arrhythmogenic right ventricular cardiomyopathy/dysplasia clinical presentation and diagnostic evaluation: results from the North American Multidisciplinary Study.

BACKGROUND: Prior reports on patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) focused on individuals with advanced forms of the disease. Data on the diagnostic performance of various testing modalities in newly identified individuals suspected of having ARVC/D are limited. OBJECTIVE: The purpose of the Multidisciplinary Study of Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia was to study the clinical characteristics and diagnostic evaluation of a large group of patients newly identified with ARVC/D. METHODS: A total of 108 newly diagnosed patients with suspected ARVC/D were prospectively enrolled in the United States and Canada. The patients underwent noninvasive and invasive tests using standardized protocols that initially were interpreted by the enrolling center and adjudicated by blind analysis in six core laboratories. Patients were followed for a mean of 27 +/- 16 months (range 0.2-63 months). RESULTS: The clinical profile of these newly diagnosed patients differs from the profile of reported patients with more advanced disease. There was considerable difference in the initial and final classification of the presence of ARVC/D after the diagnostic tests were evaluated by the core laboratories. Final clinical diagnosis was 73 affected, 28 borderline, and 7 unaffected. Individual tests agreed with the final diagnosis in 50% to 70% of the 73 patients with a final classification of affected. CONCLUSION: The clinical profile of 108 newly diagnosed probands with suspected ARVC/D indicates that a combination of diagnostic tests is needed to evaluate the presence of right ventricular structural, functional, and electrical abnormalities. Echocardiography, right ventricular angiography, signal-averaged ECG, and Holter monitoring provide optimal clinical evaluation of patients suspected of ARVC/D.

Frequency and mechanism of persistent systolic anterior motion and mitral regurgitation after septal ablation in obstructive hypertrophic cardiomyopathy.

Relief of obstruction using ventricular septal ablation (VSA) may not eliminate systolic anterior motion (SAM) of the mitral valve and mitral regurgitation (MR) in patients with obstructive hypertrophic cardiomyopathy. The hypothesis was that persistent SAM after VSA was secondary to anterior papillary muscle displacement and malcoaptation of mitral valve leaflets and that these findings could predict persistence of SAM. Echocardiograms were examined from 37 patients with obstructive hypertrophic cardiomyopathy before and 12+/-3 months after VSA. Anterior leaflet malposition (anterior-to-posterior leaflet coaptation position ratio), papillary muscle malposition (septal-to-lateral/left ventricular internal diameter ratio), and anterior position of coaptation relative to the septum (coaptation-to-septal distance) were assessed. MR proximal jet width was also measured. Of 37 patients, 30 underwent successful VSA (left ventricular outflow tract gradient reduction>50%); 22 of 30 and 7 of 7 with <50% reduction (total 29 of 37; 78%) showed persistent SAM at 12+/-3 months. These patients had more anterior malposition of the mitral valve and less MR reduction than those without SAM: anterior-to-posterior leaflet coaptation position ratio 0.42+/-0.06 versus 0.56+/-0.09, septal-to-lateral/left ventricular internal diameter ratio 0.39+/-0.12 versus 0.55+/-0.12, coaptation-to-septal distance 1.8+/-0.42 versus 2.8+/-0.30 cm, and MR reduction by 29+/-22% versus 71+/-12% (p<0.0001). Gradients, both at rest and provokable, were higher (27+/-33 vs 4+/-5 mm Hg, p=0.0004; >45 mm Hg in 9 vs 0, p=0.03, respectively) in patients with persistent SAM. Anterior malposition was present before VSA, with anterior-to-posterior leaflet coaptation position ratio<0.5 predicting SAM after VSA (p<0.0001). In conclusion, SAM and MR were often not eliminated using VSA. Mitral valve malposition was a strong predictor of SAM and MR reduction after VSA and may need to be considered in optimizing results of this procedure.