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BACKGROUND: We have previously reported our 3-step repair for obstructive hypertrophic cardiomyopathy (HCM) consisting of resection of the septum, horizontal plication of the anterior mitral leaflet (AML), and release of abnormal papillary muscle attachments. This article reviews our complete experience with surgical management of HCM to better understand the role and relevance of mitral plication. METHODS: From 1997 to 2011, 132 patients with HCM underwent surgical treatment at our institution. Eighty-two patients (62%) received AML plication based on selection criteria and were classified as group A; patients in group B did not receive plication. All patients underwent preoperative and postoperative echocardiography. Long-term clinical follow-up was obtained by review of scheduled echocardiograms and direct patient interview. RESULTS: The average age of all patients was 55.5 years. Operative mortality was 0%. The mean left ventricular outflow tract (LVOT) gradient decreased from 118+/-41 mm Hg to 6+/-13 mm Hg (p<0.0001). Mean mitral regurgitation improved from 2.4+/-1.0 to 0.5+/-0.7 (p<0.0001). Postoperatively, 96.2% of patients had no residual systolic anterior motion (SAM). Significant improvements in heart failure classification and quality of life scores were noted for all patients. Comparison of groups A and B showed no statistically significant differences in outcomes, complications, or survival. Survival at 1, 5, and 10 years was 98%, 98%, and 92%, respectively. CONCLUSIONS: The heterogeneity of the pathologic process in HCM supports detailed analysis of the septum, mitral leaflets, and subvalvular apparatus. Surgical management of HCM that includes horizontal plication of a lax and elongated AML is safe and results in durable clinical and echocardiographic improvement.

AIMS: Echocardiographic contrast (EC) improves the diagnostic accuracy of suboptimal echocardiograms. In October 2007, the Food and Drug Administration (FDA) placed a black box warning on the label of the perflutren-based agents Definity and Optison, contraindicating their use in patients with pulmonary hypertension (PHT) and unstable cardiopulmonary status, after serious cardiopulmonary reactions occurred in temporal relation to EC administration. In 2008 and 2011, the FDA revised the black box warning allowing their use in this same population. However, limited data exist regarding the safety profile of these agents in patients with PHT. METHODS AND RESULTS: Consecutive hospitalized patients with PHT who were referred for echocardiographic evaluation, but required the use of EC, were included. All our patients received the EC agent Definity. We evaluated these patients for serious adverse events (respiratory decompensation, hypotension, syncope, convulsions, arrhythmias, anaphylactic reactions, or death) occurring within 24 h of EC administration. The study group included 1513 patients (age 69 +/- 14 years, 55% males, BMI 33 +/- 9 kg/m(2)), of which 911 (60%) had mild PHT, 515 (34%) had moderate PHT, and 87 (6%) had severe PHT. The mean pulmonary artery systolic pressures (PASP) in the groups with mild, moderate, and severe PHT were 41 +/- 4 (range 35-49) mmHg, 55 +/- 5 (range 50-69) mmHg, and 78 +/- 9 (range 70-122) mmHg, respectively. The incidence of adverse events in all subgroups was rare (0.002%) and they were not attributed to EC because of temporal and clinical considerations. CONCLUSION: The use of the EC agent Definity is safe in hospitalized patients with PHT.

Unique genetic characteristics of hypertrophic cardiomyopathy (HCM), including heterogeneity and incomplete penetrance, have made making predictions about prognosis complex. We reviewed data from septal myectomy results as published from 1980 to 2011, most of which come from specialized tertiary care centers. We also performed a retrospective review of 132 consecutive patients who underwent HCM surgery at our institution. At a mean follow-up of 4.2 +/- 3.2 years (range, 3 days to 14.2 years), there were no deaths within 30 days of surgery for our cohort. Over the course of 15 years, 2 deaths occurred in older patients, both of whom had surgery for HCM along with additional cardiac procedures. Age, the presence of comorbidities, and concomitant cardiac procedures were not statistically significant risk factors for mortality. Overall survival at 1, 5, and 10 years was excellent: 99%, 99%, and 92%, respectively. Surgical myectomy has been proven to be a safe and effective procedure for symptomatic obstructive HCM, and it confers excellent survival similar to that of the healthy population.

Sudden cardiac death (SCD) in young athletes is relatively uncommon but tragic when it occurs. Many of these deaths can be prevented by pre-exercise screening to identify cardiac abnormalities and those at high risk. Although recent research has provided much needed information on SCD in athletes, there remain significant gaps in the knowledge needed to determine an optimal screening protocol. This review examines the incidence and demographics of SCD in athletes and the difficulties in determining whether changes in an athlete's heart are due to training or represent a potentially malignant congenital abnormality. Current guidelines for screening and the intense debate over the use of the 12-lead electrocardiogram are discussed. Lastly, the importance of a response plan to an apparent SCD event that includes on-site/on-field automated external defibrillators will be discussed. A case study that illustrates the challenges in screening is presented.

Pharmacologic therapy is the first line approach to relieve symptoms in obstructive hypertrophic cardiomyopathy. There are no randomized trials to evaluate their effect on prognosis. Gradient reduction by surgical septal myectomy is associated with excellent prognosis, but not all patients have symptoms severe enough to require surgery; and, guidelines recommend operation only for patients with high gradients and symptoms unresponsive to pharmacologic therapy. The combination of disopyramide and beta-blockade is effective in reducing resting gradients (though not to the extent of surgery). This review examines the question of whether pharmacologic reduction of gradient in asymptomatic patients or those with milder symptoms might decrease HCM-related mortality.

Hypertrophic cardiomyopathy (HCM) occurs in 1 in 500 individuals. Treatment options for HCM differ from those administered in coronary disease, heart failure, and valvular disease patients that comprise the core of many cardiology practices. In this article, we offer a concise summary of the therapeutic use of disopyramide for reducing gradients and relieving symptoms in obstructive HCM.

Treatments for hypertrophic cardiomyopathy are largely selected based on patient symptoms and echocardiographic findings. Moreover, all the advanced treatments for heart failure symptoms depend on such imaging for planning and monitoring response to therapy. Risk of sudden death correlates with maximum left ventricular (LV) wall thickness. Massive LV thickening of 30 mm or more is an indication for primary prevention of sudden death with an implanted defibrillator. In this review, we will underscore potential pitfalls in echocardiographic diagnosis. Also we will review, a newly appreciated pathophysiologic mechanism in obstruction dynamic systolic dysfunction due to gradient.