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

Clinical Implications of Midventricular Obstruction in Patients With Hypertrophic Cardiomyopathy

Transesophageal Doppler echocardiography in obstructive hypertrophic cardiomyopathy: clarification of pathophysiology and importance in intraoperative decision making.

0 bstruction to left ventricular outflow is a feature of hypertrophic cardiomyopathy (HC) that has been of great interest to clinicians and students of this disease for more than 25 years, and although generally regarded as a prominent and important component of HC, its clinical and pathophysiologic significance is the source of periodic and often intense disagreement and controversy.*-l7 The article of Grose et all8 in this issue of the Journal constitutes another piece of evidence supporting the concept that obstruction or impedance to left ventricular outflow occurs in some patients with HC. These authors report in a group of 10 patients with HC that the radiolucent line in left anterior oblique cineangiograms, representing systolic anterior motion of the mitral valve (SAM), occurs almost simultaneously with the onset of the subaortic pressure gradient measured by catheter micromanometry, at which time the left ventricle still has a considerable quantity of blood remaining to be ejected. This most recent observation must be considered in the context of studies that have spanned 25 years. In the early 196Os, a number of investigators observed that those patients with HC and a loud systolic heart murmur had a markedly increased systolic pressure and a pressure gradient recorded within the left ventricle at cardiac catheterization.5Jg-22 This subaortic gradient could be easily distinguished from gradients artifactually produced by physical entrapment of the catheter between left ventricular trabeculations.5 It was therefore concluded that obstruction to left ventricular outflow existed in these patients with HC.

Clinical significance and therapeutic implications of the left ventricular outflow tract pressure gradient in hypertrophic cardiomyopathy.

Forty-seven consecutive patients aged 1-38 years underwent operation for hypertrophic obstructive cardiomyopathy (HOCM) between 1972 and 1986. Isolated myectomy-myotomy was performed in 43 patients, three patients underwent myectomy and concomitant aortic valve repair, and one patient underwent concomitant mitral valve replacement. The peak systolic pressure gradient from the left ventricle to the aorta decreased from 70 +/- 33 mm Hg (mean +/- SEM) preoperatively to 10 +/- 15 mm Hg immediately after repair (p less than 0.001). Moderate or severe mitral insufficiency was identified in 16 patients preoperatively and was corrected by myectomy alone in 15. There was no operative mortality; two late deaths occurred during follow-up (median, 5 years; maximum, 16 years) for estimated 5- and 10-year survivals of 97 +/- 2% and 88 +/- 10%, respectively. Reoperation was required for aortic valve replacement (n = 2), remyectomy (n = 2), and permanent pacemaker implantation (n = 3). Preoperative symptoms were relieved in 24 of 29 (83%) patients with dyspnea, in 18 of 19 (95%) with angina, and in six of 10 (60%) with syncope. These results support myectomy-myotomy for symptomatic children and young adults with HOCM. Also, it appears that late survival after myectomy-myotomy in these young patients may be improved over that observed in historical controls treated with medications alone; operation should be considered for asymptomatic children and adults less than 40 years of age with large (greater than 80 mm Hg) left ventricular outflow gradients.

Results of operation for hypertrophic obstructive cardiomyopathy in children and adults less than 40 years of age.

The Dusseldorf surgical series of patients with hypertrophic obstructive cardiomyopathy (HOCM) (1963-82) comprises 137 patients, of whom 123 had typical HOCM and 14 atypical midventricular stenosis. Indications for surgery were unsatisfactory response to drug therapy and clinical symptoms according to at least functional class III (NYHA). The predominant approach for the relief of both types of HOCM was transaortic (112 cases; two deaths = 1.8%). The hospital mortality of the total series was 6.6% (nine of 137 patients). The most severe problems were caused by additional acquired mitral valve lesions. There is no special risk for patients with atypical midventricular HOCM. The systolic gradient between left ventricle and aorta could be removed or diminished in all cases.

Surgical treatment of hypertrophic obstructive cardiomyopathy with special reference to complications and to atypical hypertrophic obstructive cardiomyopathy.

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Surgical treatment of hypertrophic obstructive cardiomyopathy with special reference to complications and to atypical hypertrophic obstructive cardiomyopathy.