DIAGNOSTIC METHODS CARDIOMYOPATHY Apical hypertrophic cardiomyopathy: evaluation by noninvasive and invasive techniques in 23 patients

Over a 3 year period we evaluated 23 patients (16 men, seven women) with apical hypertrophic cardiomyopathy by noninvasive and invasive methods. Sixteen patients had chest pain. In 17, results of cardiovascular examination were normal. The electrocardiogram showed precordial inverted T waves in all patients and these were of mild-to-moderate amplitude (<10 mm) in 18 and giant (>10 mm) in five. M mode echocardiography revealed a typical pattem of contraction and relaxation in the apical region of the left ventricle that was associated with significant hypertrophy. These findings were confirmed by two-dimensional echocardiography. Systolic anterior motion of the mitral valve was not observed nor was there any evidence of obstruction of the left ventricular outflow tract. Results of Doppler echocardiographic study of the mitral and aortic flow were normal in all patients but one who had mild mitral insufficiency. Radionuclide studies of 14 patients revealed a mean left ventricular ejection fraction of 66 + 6% (range 55% to 79%), with normal left ventricular contraction in all patients but two with apical hypokinesis. In all six patients who underwent catheterization a characteristic appearance of the left ventricle at end-systole as well as abnormal end-diastolic contour were noted on the left ventricular angiogram, but the "ace of spades" configuration was seen in only one. We conclude that the 23 patients studied form a homogeneous group of individuals with nonobstructive apical hypertrophic cardiomyopathy, which differs in many respects from cardiomyopathies reported by other investigators. Circulation 71, No. 1, 45-56, 1985. HYPERTROPHIC CARDIOMYOPATHY is defined as a heart muscle disorder of unknown origin that is characterized by hypertrophy of a nondilated left ventricle. ' The hypertrophy may be obstructive or nonobstructive and its distribution may exhibit marked variation.2 Recently, Japanese investigators34 described a form of nonobstructive hypertrophic cardiomyopathy characterized by disproportionate hypertrophy of the left ventricular apical region associated with giant precordial negative T waves and high QRS voltage. A characteristic "ace of spades" configuration at end-diastole was observed on left ventriculograms of patients with this disorder.5' 6 Reviewing their extensive experience with hypertrophic cardiomyopathy, Maron et al.7 reported seven patients who had a distribution of left ventricular hyFrom the Department of Cardiology, Ichilov Hospital, Tel Aviv Medical Center, and the Tel Aviv University Sackler School of Medicine, Tel Aviv. Address for correspondence: Shlomo Laniado, M.D., Department of Cardiology, Ichilov Hospital, 6 Weizmann St., Tel Aviv 64239, Israel. Received March 23, 1984; revision accepted Sept. 20, 1984. pertrophy similar to that observed by the Japanese investigators. However, the electrocardiograms of their patients showed relatively mild T wave inversion and left ventricular angiograms showed a characteristic appearance of mid left ventricular constriction and a small, often poorly contracting apical segment. No other extensive study of apical hypertrophic cardiomyopathy has appeared in the literature and only a few cases have been reported with findings similar to those of the Japanese group.tt-4 The present study reports a series of 23 patients with apical hypertrophic cardiomyopathy who were evaluated at our institution by both noninvasive and invasive techniques. To the best of our knowledge, this is the largest series of patients with this diagnosis reported so far outside of Japan. Methods Selection of patients. Over a 3 year period 23 patients with apical hypertrophic cardiomyopathy were studied in our institution. The diagnosis of apical hypertrophic cardiomyopathy was established with echocardiography by the demonstration of ventricular hypertrophy that was virtually confined to the apical part Vol. 71, No. 1, January 1985 45 by gest on A ril 5, 2017 http://ciajournals.org/ D ow nladed from