Hypertrophic cardiomyopathy. The importance of the site and the extent of hypertrophy. A review

HCM is a heterogeneous disease genotypically, phenotypically, pathophysiologically, clinically, and therapeutically. In decisions on the management of these patients, it is important to recognize this heterogeneity and to direct therapy at the predominant abnormalities.

Hypertrophic Cardiomyopathy Clinical Spectrum and Treatment

https://www.bktimes.net/data/board_notice/1361431162-67.pdf

Long-term effects of surgical septal myectomy on survival in patients with obstructive hypertrophic cardiomyopathy.

BACKGROUND Patients with obstructive hypertrophic cardiomyopathy (HCM) with symptoms refractory to drugs (beta-blockers or verapamil) are candidates for cardiac surgery (left ventricular septal myectomy or mitral valve replacement). The present study examines prospectively the ability of dual-chamber (DDD) pacing to improve symptoms and relieve left ventricular outflow obstruction in such patients. METHODS AND RESULTS Forty-four consecutive patients with obstructive HCM who had failed to benefit from pharmacotherapy underwent treadmill exercise tests, echocardiography, and cardiac catheterization before and 1.5-3 months after implantation of a DDD pacemaker. Symptoms (angina, dyspnea, palpitations, presyncope, and syncope), New York Heart Association functional class status (1.7 +/- 0.7 versus 3.4 +/- 0.5, p less than 0.00001), and exercise durations were improved at follow-up evaluation. This was associated with significant reduction in left ventricular outflow tract gradient (38 +/- 38 versus 87 +/- 43 mm Hg, p less than 0.0001) and significant increases in cardiac output and systemic arterial pressures. Notably, when pacing was discontinued and comparisons were made in sinus rhythm, treadmill exercise durations were greater and left ventricular outflow tract gradients were less at the follow-up evaluation compared with the baseline study. CONCLUSIONS DDD pacing is an effective alternative to surgery in most patients with obstructive HCM with drug-refractory symptoms. The beneficial effects of pacing continue to be evident when pacing is acutely discontinued.

Impact of dual-chamber permanent pacing in patients with obstructive hypertrophic cardiomyopathy with symptoms refractory to verapamil and beta-adrenergic blocker therapy.

Hypertrophic cardiomyopathy in tuscany: Clinical course and outcome in an unselected regional population

Since 1960, at the National Institutes of Health, ventricular septal myotomy and myectomy has been the mode of treatment for severely symptomatic patients with hypertrophic cardiomyopathy and obstruction to left ventricular outflow who do not respond to medical therapy. Our long-term results of operation for hypertrophic cardiomyopathy are reviewed in 240 patients operated upon prior to 1980. Postoperatively, most patients had improved symptomatically (i.e. 70%) and manifested marked reduction or abolition of the basal left ventricular outflow gradient (i.e. 98%). However, 8% of the patients died of causes related to operation, 9% had persistent or recurrent severe functional limitation, and 7% died up to 19 years postoperatively due to the underlying cardiomyopathy. Of 17 late postoperative deaths, eight were sudden and nine were due to chronic heart failure. In particular, postoperative atrial fibrillation was a significant contributing factor to poor clinical outcome. Hence, while ventricular septal myotomy-myectomy is not always a curative procedure for obstructive hypertrophic cardiomyopathy, the vast majority of patients who survive operation experience long-lasting clinical improvement.

Symptomatic status and prognosis of patients after operation for hypertrophic obstructive cardiomyopathy: efficacy of ventricular septal myotomy and myectomy

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Symptomatic status and prognosis of patients after operation for hypertrophic obstructive cardiomyopathy: efficacy of ventricular septal myotomy and myectomy