Jannet F. Lewis

Relationship of race to sudden cardiac death in competitive athletes with hypertrophic cardiomyopathy

OBJECTIVES The goal of this study was to determine the impact of race on identification of hypertrophic cardiomyopathy (HCM). BACKGROUND Sudden death in young competitive athletes is due to a variety of cardiovascular diseases (CVDs) and, most commonly, HCM. These catastrophes have become an important issue for African Americans, although HCM has been previously regarded as rare in this segment of the U.S. population. METHODS We studied the relationship of race to the prevalence of CVDs causing sudden death in our national athlete registry, and compared these findings with a representative multicenter hospital-based cohort of patients with HCM. RESULTS Of 584 athlete deaths, 286 were documented to be due to CVD at ages 17 +/- 3 years; 156 (55%) were white, and 120 (42%) were African American. Most were male (90%), and 67% participated in basketball and football. Among the 286 cardiovascular deaths, most were due to HCM (n = 102; 36%) or anomalous coronary artery of wrong sinus origin (n = 37; 13%). Of the athletes who died of HCM, 42 (41%) were white, but 56 (55%) were African American. In contrast, of 1,986 clinically identified HCM patients, only 158 (8%) were African American (p < 0.001). CONCLUSIONS In this autopsy series, HCM represented a common cause of sudden death in young and previously undiagnosed African American male athletes, in sharp contrast with the infrequent clinical identification of HCM in a hospital-based population (i.e., by seven-fold). This discrepancy suggests that many HCM cases go unrecognized in the African American community, underscoring the need for enhanced clinical recognition of HCM to create the opportunity for preventive measures to be employed in high-risk patients with this complex disease.

Pulmonary hypertension in sickle cell disease

Abstract Pulmonary hypertension is not uncommon in the echocardiograms of patients with sickle cell disease. Although a substantial number of these patients may not have symptoms of cardiac dysfunction, mortality is significantly increased compared with patients with sickle cell disease hut without pulmonary hypertension.

ACC/AHA clinical competence statement on echocardiography: A Report of the American College of Cardiology/American Heart Association/American College of Physicians–American Society of Internal Medicine Task Force on Clinical Competence Developed in Collaboration with the American Society of Echocardiography, the Society of Cardiovascular Anesthesiologists, and the Society of Pediatric Echocardiography

Preamble......688 References......708 The granting of clinical staff privileges to physicians is a primary mechanism used by institutions to uphold the quality of care. The Joint Commission on Accreditation of Health Care Organizations requires that the granting of continuing medical staff

Usefulness of Doppler echocardiographic assessment of diastolic filling in distinguishing "athlete's heart" from hypertrophic cardiomyopathy.

Objective—In some athletes with a substantial increase in left ventricular wall thickness, it may be difficult to distinguish with certainty physiological hypertrophy due to athletic training from hypertrophic cardiomyopathy. The purpose of the present investigation was to determine whether assessment of left ventricular filling could differentiate between these two conditions. Design—Doppler echocardiography was used to obtain transmitral flow velocity waveforms from which indices of left ventricular diastolic filling were measured. Normal values were from 35 previously studied control subjects. Setting—Athletes were selected mostly from the Institute of Sports Science (Rome, Italy), and patients with hypertrophic cardiomyopathy were studied at the National Institutes of Health (Bethesda, Maryland). Participants—The athlete group comprised 16 young competitive athletes with an increase in left ventricular wall thickness (range 13–16 mm; mean 14). For comparison, 12 symptom free patients with non-obstructive hypertrophic cardiomyopathy were selected because their ages and degree of hypertrophy were similar to those of the athletes. Results—In the athlete group, values for deceleration of flow velocity in early diastole, peak early and late diastolic flow velocities, and their ratio were not significantly different from those obtained in untrained normal subjects; furthermore, Doppler diastolic indices were normal in each of the 16 athletes. Conversely, in patients with hypertrophic cardiomyopathy, mean values for Doppler diastolic indices were significantly different from both normal subjects and athletics (p = 0·01 to 0·003), and one or more indices were abnormal in 10 (83%) of the 12 patients. Conclusions—Doppler echocardiographic indices of left ventricular filling may aid in distinguishing between pronounced physiological hypertrophy due to athletic training and pathological hypertrophy associated with hypertrophic cardiomyopathy.

Elderly patients with hypertrophic cardiomyopathy: a subset with distinctive left ventricular morphology and progressive clinical course late in life.

This report describes a subgroup of 52 elderly patients with obstructive hypertrophic cardiomyopathy in whom certain clinical and morphologic features differed importantly from those of many other patients with this disease. Ages ranged from 60 to 84 years (mean 69) and 45 [87%] were women. Echocardiographic examination showed a relatively small heart, having only modest ventricular septal hypertrophy associated with marked distortion of left ventricular outflow tract morphology. By virtue of selection, left ventricular outflow tract size at end-diastole was substantially reduced, and anterior displacement of the mitral valve within the left ventricular cavity was particularly marked. Sizable deposits of calcium in the region of the mitral anulus, posterior to the mitral valve, appeared to contribute to the outflow tract narrowing. Systolic anterior motion of the mitral valve was severe (with apposition of the mitral valve and ventricular septum) in 32 patients and more moderate in 20. The mechanism by which systolic contact between the mitral valve and septum occurred in most patients appeared to differ from that observed more typically in many other patients with hypertrophic cardiomyopathy; in most elderly study patients, anterior excursion of the mitral valve leaflets was relatively restricted, and systolic apposition between the mitral valve and septum resulted from a combination of anterior motion of the mitral valve and posterior excursion of the septum. The vast majority (50 of 52) of the patients remained asymptomatic (or only mildly symptomatic) for most of their lives and often did not develop severe and intractable symptoms until the 6th or 7th decade (ages 56 to 81 years; mean 66). Of the 49 patients with at least 1 year follow-up study, only 12 had improvement with pharmacologic therapy; however, 14 of the 18 patients who underwent ventricular septal myotomy-myectomy or mitral valve replacement obtained symptomatic benefit from operation. In conclusion, obstructive hypertrophic cardiomyopathy in many elderly (and predominantly female) patients may assume a distinctive morphologic appearance and a progressive clinical course. This subgroup of patients appears to constitute an important segment of the disease spectrum of hypertrophic cardiomyopathy of cardiac disease in the elderly that previously has not been precisely defined nor fully appreciated.

ACC/AHA clinical competence statement on echocardiography: a report of the American College of Cardiology/American Heart Association/American College of Physicians-American Society of Internal Medicine Task Force on Clinical Competence.

Preamble......688 References......708 The granting of clinical staff privileges to physicians is a primary mechanism used by institutions to uphold the quality of care. The Joint Commission on Accreditation of Health Care Organizations requires that the granting of continuing medical staff

Preparticipation echocardiographic screening for cardiovascular disease in a large, predominantly black population of collegiate athletes.

Sudden death in young competitive athletes is most commonly due to underlying cardiovascular disease. Echocardiography has the potential to identify structural cardiovascular abnormalities, such as hypertrophic cardiomyopathy (HC), that have been incriminated in such events. In this study, echocardiography (2-dimensional and M-mode) was used as a primary screening test to assess 265 Howard University collegiate athletes for cardiovascular disease; 262 (99%) were black. Most athletes (234, 88%) had no definitive echocardiographic evidence of HC or other major cardiovascular diseases, but 30 (11%) had mitral valve prolapse, and 1 other athlete had a small atrial septal defect. In addition, 4 athletes were identified as having mild systemic hypertension. Most athletes (236 of 265) showed normal left ventricular wall thickness of less than or equal to 12 mm, but an important minority (29, 11%) had maximal ventricular septal thicknesses of greater than or equal to 13 mm that could not always be distinguished (by morphology alone) from mild anatomic expressions of nonobstructive HC. Based on this experience, preparticipation athletic screening using echocardiography as the primary test does not appear to be justified on a cost-effective basis. In addition, the substantial minority of subjects with increased wall thickness made clinical interpretation of the echocardiographic findings difficult in individual athletes.

Discordance in degree of right and left ventricular dilation in patients with dilated cardiomyopathy: recognition and clinical implications.

OBJECTIVES The purpose of the present study was to assess the influence of variations in the relative degree of dilation of left and right ventricular chambers on the clinical outcome of patients with dilated cardiomyopathy. BACKGROUND Dilated cardiomyopathy, a primary myocardial disease characterized by ventricular dilation and systolic dysfunction, is generally associated with a poor prognosis. However, considerable variability has been observed in the clinical course and the morphologic and hemodynamic features in individual patients. METHODS We evaluated 67 consecutive patients with dilated cardiomyopathy and without evidence of ischemic or primary valvular heart disease. On the basis of diastolic ventricular chamber area measurements obtained by echocardiography, patients were classified into two groups: 38 patients with a relatively equal degree of left and right ventricular dilation (LV congruent to RV) and 29 patients with predominant and disproportionate dilation of the left ventricle (LV > RV). RESULTS The 67 patients ranged in age from 19 to 81 years (mean 56); 49 (73%) were male. The two subsets of patients with dilated cardiomyopathy did not differ with regard to age, left ventricular diastolic dimension, wall thickness and mass or ejection fraction. However, patients in the LV congruent to RV group showed more severe mitral and tricuspid regurgitation by Doppler echocardiography than did those in the LV > RV group (p = 0.01 for mitral and 0.004 for tricuspid regurgitation). Over the follow-up period of 2 to 60 months (mean 28), there were 19 deaths. Survival in the LV > RV group was significantly better than in the LV congruent to RV group (p = 0.03). CONCLUSIONS Patients with dilated cardiomyopathy represent a heterogeneous group with regard to both clinical outcome and the relative degree of left and right ventricular chamber dilation. Patients in the LV > RV subset appear to have better overall survival and less severe mitral and tricuspid regurgitation than do patients in the LV congruent to RV subset. Longitudinal studies are needed to determine whether these morphologic subsets in fact represent a continuum within the disease spectrum of dilated cardiomyopathy.

American College of Cardiology/American Heart Association Clinical Competence Statement on Echocardiography

Developed in Collaboration with the American Society of Echocardiography, the Society of Cardiovascular Anesthesiologists, and the Society of Pediatric Echocardiography

Interpretation of Remotely Downloaded Pocket-Size Cardiac Ultrasound Images on a Web-Enabled Smartphone: Validation Against Workstation Evaluation

BACKGROUND Pocket-size ultrasound has increased echocardiographic portability, but expert point-of-care interpretation may not be readily available. The aim of this study was to test the hypothesis that remote interpretation on a smartphone with dedicated medical imaging software can be as accurate as on a workstation. METHODS Eighty-nine patients in a remote Honduran village underwent echocardiography by a nonexpert using a pocket-size ultrasound device. Images were sent for verification of point-of-care diagnosis to two expert echocardiographers in the United States reading on a workstation. Studies were then anonymized, randomly ordered, and reinterpreted on a smartphone with a dedicated, Health Insurance Portability and Accountability Act-compliant application. Point-of-care diagnosis was considered accurate if any abnormal finding was matched and categorized at the same level of severity (mild, moderate, or severe) by either expert interpretation. RESULTS The mean age was 54 ± 23 years, and 57% of patients were women. The most common indications for echocardiography were arrhythmia (33%), cardiomyopathy (28%), and syncope (15%). Using the workstation, point-of-care diagnoses were changed in 38% of cases by expert overread (41% left ventricular function correction, 38% valvulopathy correction, 18% poor image quality). Expert interobserver agreement was excellent at 82%, with a Cohens κ value of 0.82 (95% confidence interval, 0.70-0.94). Intraobserver agreement comparing interpretations on workstations and smartphones was 90%, with a Cohens κ value of 0.86 (95% confidence interval, 0.76-0.97), signifying excellent intertechnology agreement. CONCLUSIONS Remote expert echocardiographic interpretation can provide backup support to point-of-care diagnosis by nonexperts when read on a dedicated smartphone-based application. Mobile-to-mobile consultation may improve access in previously inaccessible locations to accurate echocardiographic interpretation by experienced cardiologists.