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Echocardiographic Diagnosis of Idiopathic Hypertrophic Cardiomyopathy without Outflow Obstruction By A S. ABBASI, M.B., M.R.C.P.E., R. N. MACALPIN, M.D., L. M. EBER, M.D., AND M. L. PEARCE, M.D. Downloaded from http://circ.ahajournals.org/ by guest on June 14, 2017 SUMMARY The echocardiographic findings of eight patients with hypertrophic cardiomyopathy without outflow obstruction (HMC) and of 15 normal (Norm) individuals are presented. The characteristic features in HMC were: (1) interventricular septal width much greater than normal (HMC= 2.5 + 0.3 cm, Norm = 1.0 + 0.2 cm, P <0.005); (2) normal or only slightly increased posterior left ventricular wall thickness; (3) the ratio of interventricular septal to posterior wall thickness .2.0; (4) ejection fraction greater than normal (HMC = 0.76 0.08, Norm = 0.68 0.06, P < 0.025); (5) reduced velocity of the early diastolic closing motion of the anterior mitral leaflet (HMC = 60 23 mm/sec, Norm = 124 29 mm/sec, P < 0.005); (6) absence of abnormal systolic movement of the anterior mitral valve, as seen in hypertrophic obstructive cardiomyopathy. The diagnosis of hypertrophic cardiomyopathy can be made with echocardiography, even when outflow tract obstruction of the left ventricle is absent. Additional Indexing Words: Interventricular septal hypertrophy Idiopathic hypertrophic obtsructive cardiomy opathy HYPERTROPHIC cardiomyopathy is difficult to diagnose clinically. Cardiac catheterization, angiocardiography, and coronary arteriography are often necessary for the correct diagnosis of chest pain, dyspnea, an unexplained heart murmur, or an abnormal electrocardiogram with which these patients may present. When obstruction to the left ventricular outflow is present in this condition, systolic approximation of the mitral valve to the interventricular septum can be demonstrated as a characteristic deformity by echocardiography. 12 However, in nonobstructive hypertrophic cardiomyopathy,3-5 there is no gradient across the left ventricular outflow tract and an abnormal systolic movement of the mitral valve is not seen on echocardiography, even after amyl nitrite or isoproterenol provocation.6 The diagnosis in cases without outflow obstruction is based on angiocardiographic demonstration of left ventricular wall and interventricular septal hypertrophy, diminished end-systolic volume, and papillary muscle hypertrophy.7-8 In some cases the hypertrophy may be restricted to the ventricular septum.9-10 The ventricular septal hypertrophy is difficult to assess with isolated left ventricular angiograms and simultaneous left and right ventriculography has been advocated for this diagnosis." These procedures have From the Department of Medicine, University of California School of Medicine, Los Angeles, California. Supported in part by U. S. Public Health Service Grants HE-5056 and HE-05844. Address for reprints: A. S. Abbasi, M.D., Department of Medicine, UCLA Center for the Health Sciences, Los Angeles, California 90024. Received February 17, 1972; revision accepted for publication June 30, 1972. Circulation, Volume XLVI, November 1972 Mitral valve motion 897 ABBASI ET AL. 898 the obvious drawbacks of potential risk, expense, and patient inconvenience. Recent advances in ultrasound technics have made it possible to estimate left ventricular volume, left ventricular wall thickness, and width of the interventricular septum noninvasively. 12-15 We are reporting the ultrasound findings of eight patients with hypertrophic cardiomyopathy without left ventricular outflow obstruction. Downloaded from http://circ.ahajournals.org/ by guest on June 14, 2017 Methods Eight patients were studied; three were men and five were women. The ages of the patients ranged from 26 to 64 years with a mean age of 44 years. Four patients presented with chest pain as their leading symptom. Five patients had dyspnea on exertion; two were referred because of an abnormal electrocardiogram, suggesting old myocardial infarction in one and left ventricular hypertrophy in the other. Five patients had cardiac catheterization, angiocardiography, and coronary arteriography. None of these five patients had a gradient between the left ventricle and the brachial artery at rest, and only one had a gradient of 20 mm Hg during isoproterenol infusion. The diagnosis of hypertrophic cardiomyopathy in these patients was based on the angiocardiographic features described by Simon7 and Cohen.8 Three patients who did not have cardiac catheterization had clinical evidence suggesting hypertrophic cardiomyopathy.3, 5 The clinical features included evidence of left ventricular hypertrophy with normal blood pressure, rapid carotid upstroke, prominent fourth heart sound, and absence of ejection click. A short systolic murmur grade 12/6 was also present at the apex or lower left sternal border. Echocardiograms were also taken of 15 healthy volunteers. Echocardiographic Measurements Echocardiograms were performed using a commercially available ultrasonoscope* utilizing a 2.25 MHz, 0.5 cm-diameter transducer with a 10 cm focust and a repetition rate of 1,000 impulses per sec. The calibration dots on the oscilloscope were set so that adjacent dots were separated horizontally by 0.5 sec, and represented 1 cm tissue depth vertically. The technic of recording the echogram was that described by Feigenbaum et al.13 and Popp and Harrison.14 In brief, the left ventricular cavity size was determined by placing the transducer at the fourth or fifth left intercostal space, close to the sternum. The transducer was then rotated slightly superiorly and medially to localize the characteristic motion of the anterior mitral leaflet, and a record of its motion was made. From this position, the transducer was *Ekoline 20, Smith-Kline Instruments. tAerotech Laboratories. Table 1 Echocardiographic Measurements of Eight Patients with Hypertrophic Cardiomyopathy and 15 Normal Subjects BSA (in) Pt 1 2 3 4 et 6 7 8 Mean Sd (cm) Ss (cm) 1.58 1.88 3.8 4.0 2.2 2.4 5a7.5 1.51 1.65 1.66 4.4 5.0 3.0 89.2 130.9 2.05 4.5 5.0 1.68 1.79 4.8 5.2 3.a 3.0 2.2 3.0 3.0 EDV (ml) 67.1 95.4 130.9 101.8 147.2 (ml) EF IVS (cm) LVPW (cm) 11.2 14.4 28.2 44.8 28.2 11.2 28.0 28.0 24.3 0.81 0.79 3.0 2.2 1.5 0.68 0.66 0.70 2. 5 2.5 ESV 0.91 0.72 0.81 2.7 2.0 2.5 2.6 2.5 -0.3 1.1 1.2 1.0 1.2 0.8 1.0 (mm/see) 2.0 2.0 54 70 2.2 2.5 2.3 32 110 2.5 50 64 1.0 2.5 2.5 60 42 1.1 -0.21 2.33 -0.24 SD 1.73 -0.17 Normal: Mean SD . 1.85 125.4 40.1 0.68 1.0 0.9 1.17 0.16 -29.0 -14.0 -0.06 -0.2 -0.2 NS NS <0.025 <0.00t5 -0.14 <0.00 ` = P - 102.5 . 311.5 0.76 '-=0.08 MVDS IVS/LVPW NS 60 --23 124 -29 <0.00I5 Abbreviations: BSA = body surface area; Sd = end-diastolic short axis; Ss = end-systolic short axis; EDV = end-diastolic volume; ESV = end-systolic volume; EF = ejection fraction; IVS = interventricular septum: LVPW left ventricular posterior wall; MVDS = mitral valve diastolic slope; SD = standard deviation; NS = not significant. Circulation, Volume XLVI, November 1972 DIAGNOSIS OF CARDIOMYOPATHY 899 Downloaded from http://circ.ahajournals.org/ by guest on June 14, 2017 rotated slightly laterally and inferiorly to record the posterior left ventricular wall echo just behind the mitral echo. In order to delineate the ventricular septal echo, the depth compensation was adjusted so that maximum gain was placed close to 0 cm on the oscilloscope. In this way, the entire width of the septum was clearly seen separated from the anterior heart wall. All the patients had echocardiograms taken before and after inhalation of amyl nitrite. The left ventricular minor diameter was measured echocardiographically between the enidocardial echo of the posterior left ventricular wall and the endocardial border of the left septal surface. The minor diameter at end~diastole (Sd) was measured at the time of the R wave of the simultaneously recorded electrocardiogram, and that at end-systole (Ss) was taken at the poinlt where the endocardial surface of the septum and the posterior wall were nearest to each other. The ventricular volumes were estimated by: EDV = 1.047(Sd)3; ESV =1.047 (Ss)3, where EDV is end-diastolic volume and ESV is end-systolic volume.14 The ejection fraction (EF) was calculated -ES EF-ED EDV as: The estimation of the ventricular volume from the echocardiographic short axis of the left venitricle has been validated recently.'2-16 The reproducibility of the measurements among observers, and at different times in the same patient, has been documented by Pombo et al.17 The measurement of posterior left ventricular wall thickness and ventricular septal width was made at end-diastole. The early diastolic slope of the mitral valve was estimated as described by Edler.'s Results Table I shows echocardiographic features of the patients with nonobstructive hyper- ECG I.V. Septum -' 3[ Endocardium Epicardium Figure 1 Echocardiogram of a normal individual. The interventricular septal (I.V.Septum) width (0.8 cm), the short axis at end-diastole (Sd) and at end-systole (S,), the endocardium, and the epicardium are shown. Note the normal posterior left ventricular wall thickness (0.8 cm) between endocardium and epicardium. Circulation, Volume XLVI, November 1972 ABBASI ET AL. 900 Downloaded from http://circ.ahajournals.org/ by guest on June 14, 2017 trophic cardiomyopathy (HMC) and of 15 normal individuals. In patients with HMC, both end-diastolic and end-systolic volumes were relatively smaller than normal, but the difference was not statistically significant. The ejection fraction was significantly greater in HMC than in normal subjects (P < 0.025). The most striking difference was seen in the interventricular septal (IVS) width, which in 1MC was two and a half times greater than the normal (with no overlap of values between HMC and normals). There was no significant difference between posterior left ventricular wall thickness in the two groups. The early diastolic slope of the anterior leaflet of the mitral valve in HMC was significantly less than in the normal group (P<0.005); only one patient with HMC had a valve slope in the normal range. An abnormal systolic movement was not seen in any patient, even after amyl nitrite inhalation. Figure 1 shows an echocardiogram of a normal individual. The IVS (0.8 cm), the short axis at end-diastole (Sd) and at endsystole (Ss), the endocardium and epicardialpericardial junction of the posterior left ventricular wall, and its thickness (0.8 cm), are well seen. Figure 2 shows a record of a patient with HMC. Of note are the wide ventricular septum (2.5 cm), the relatively small left ventricular cavity size, and normal posterior left ventricular wall thickness (0.8 cm). Also of interest is the strong echo from the left side of the ventricular septum, suggesting a thickened endocardium. ECO i ^ I-le.V. Septum Endocard ium ' ---- r Epica-di§um a. 11A- S Figure 2 Echocardiogram of a patient with hypertrophic cardiomyopathy without outflow obstruction. Note the wide I.V.Septum (2.5 cm) and normal posterior left ventricular thickness (0.8 cm). Abbreviations same as in figtre 1. Circulaion, Volume XLVI, November 1972 DIAGNOSIS OF CARDIOMYOPATHY 901 Figure 3 shows the mitral valve motion of a patient with HMC without obstruction. Note the absence of aniterior systolic movement of the mitral valve. This echocardiogram was taken after inhalation of amyl nitrite. In conitrast to this, figure 4 shows the motion of the mitral valve of a patient with hypertrophic obstructive cardiomyopathy. The systolic anterior movement of the mitral valve toward the ventricular septum is quite well seen. Discussion Downloaded from http://circ.ahajournals.org/ by guest on June 14, 2017 Autopsy studies of patients with hypertrophic cardiomyopathy with or without outflow obstructioni have shown two types of myocardial hypertrophy. The most common variety is an asymmetric hyperthrophyj 19 22 mainly involving the interveentricular septum _~ W -- 1 - _k qb.q-. %~ and, to some extent, the free anterolateral wall of the left ventricle. The posterior left ventricular wall may be entirely normal. A much less common variety is that of diffuse hypertrophy21 22 involving the left ventricle in more or less symmetric fashion. Our cases appear to be of the asymmetric type, as the posterior left ventricular wall was within normal limits, except in one case. The ventricular septum, however, was consider- ably thicker than normal. Menges23 in his three autopsy cases of hypertrophic cardiomyopathy reported ventricular septal thicknesses of 2.8, 2.0, and 3.1 cm. In his series of normal cases, the thickness of the ventricular septum was 1.0-1.5 cm (average 1.3 cm). The values for ventricular septal width in the present study agree well -41- p- 1 .)Q ECG I.V. Septum -AML PM L L. V. post. wall Figure 3 The mitral valve of a patient wcith hbi ertrohic cardiomzyopathy teith1ou1t obstruction, after inhalatiotn of amiji niitrite. Bothl thlc anterior (AML) and posterior (PML) valtve lcaflets are seeni. Note the absence of sylstolic antetior motion of the mitral valve. Circulation, Volunet XLVI, Novem;ber 1972 ABBASI ET AL. 902 o_ ECG -IV. Septum AML SAM Downloaded from http://circ.ahajournals.org/ by guest on June 14, 2017 LV. post. wall Figure 4 The anterior mlitral leaflet (AML) of a patient with hypertrophic cardiomyopathy with outflow obstruction. Note the prominent systolic anterior movement of the nmitral valve (SAM). with those found by Menges. In the three cases of hypertrophic cardiomyopathy that Menges analyzed, the ratio of thickness of ventricular septum to that of free anterolateral wall ranged between 1.55 and 1.76. In 20 normal hearts, this ratio averaged 0.45 and in 50 hypertrophied hearts (other than hypertrophic cardiomyopathy) it averaged 0.98, exceeding 1.25 in only one instance. Thus, it seems that cardiac hypertrophy associated with hypertension and aortic stenosis is symmetric, affecting uniformly the left ventricular wall and the ventricular septum. However, in hypertrophic cardiomyopathy, the ventricular septum is disproportionately more hypertrophied. Since the posterior left ventricular wall is least affected by hypertrophy in hypertrophic cardiomyopathy, the ratio of the ventricular septum to the posterior left ventricular wall becomes even more significant. In our series of hypertrophic cardiomyopathy, this ratio was -2.0 in every case. The septal width, as measured by echocardiography, may overestimate the actual width of the septum because the ultrasound beam may not be directed at a right angle to the septal surfaces. However, the technic we employed was highly standardized and reproducible, and the empiric difference between the hypertrophic cardiomyopathy and normal is highly significant (P <0.005). The echocardiographic ventricular volumes in our normal series and in hypertrophic cardiomyopathy without outflow obstruction are in agreement with the angiocardiographic volumes in normal individuals and in hypertrophic cardiomyopathy with or without outflow obstruction reported by Conn and Circulation, Volumte XLVI, November 1972 DIAGNOSIS OF CARDIOMYOPATHY Downloaded from http://circ.ahajournals.org/ by guest on June 14, 2017 associates,24 particularly when corrected for body surface area. The rate of early diastolic closing motion of the anterior mitral leaflet was significantly reduced in all but one of the patients with hypertrophic cardiomyopathy. However, this has also been noted in patients with aortic stenosis,l and may reflect slow left ventricular filling due to reduced left ventricular distensibility in these patients.1 25 The hallmark of hypertrophic cardiomyopathy is an abnormally thick ventricular septum with a ratio of septum to posterior left ventricular wall thickness > 2.0. Unlike hypertrophic obstructive cardiomyopathy, there is no abnormal systolic movement of the mitral valve in hypertrophic cardiomyopathy without left ventricular outflow tract obstruction. Acknowledgment We wish to acknowledge the assistance of Mrs. Nancy Ellis, the senior echocardiography technician at UCLA. References 1. SHAH PM, GRAMIAK R, KRAMER DH: Ultra- sound localization of left ventricular outflow obstruction in hypertrophic obstructive cardiomyopathy. Circulation 40: 3, 1969 2. PoPP RL, HARRISON DC: Ultrasound in the diagnosis and evaluation of therapy of idiopathic hypertrophic subaortic stenosis. Circulation 40: 905, 1969 3. BRAUNWALD E, AYGEN MM: Idiopathic myocardial hypertrophy without congestive heart failure or obstruction to blood flow. Amer J Med 35: 7,1963 4. BRAUNWALD E, LAMBREW CT, ROCKOFF SD, Ross J, Moimow AG: Idiopathic hypertrophic subaortic stenosis. Circulation 30 (suppl IV): IV-1, 1964 5. NASEER WK, WILLIAM JF, MISHKIN ME, CHILDRESS RH, HELMEN C, MERRIT AD, GENOVESE PD: Familial myocardial disease with and without obstruction to left ventricular outflow. Circulation 35: 638, 1967 6. SHAH PM, GRAMIAK R, ADELMAN AG, WIGLE ED: Role of echocardiography in diagnostic and hemodynamic assessment of hypertrophic subaortic stenosis. Circulation 44: 891, 1971 7. SIMON AL, Ross J JR, GAvrr JH: Angiographic anatomy of the left ventricle and mitral valve in idiopathic hypertrophic subaortic stenosis. Circulation 36: 852, 1967 Circulation, Volume XLVI, November 1972 903 8. COHEN J, EFFAT H, GOODWIN JF, OAKLEY CM, STEINER RE: Hypertrophic obstructive cardiomyopathy. Brit Heart J 26: 16, 1964 9. TEARE D: A symmetrical hypertrophy of the heart in young adults. Brit Heart J 20: 1, 1958 10. WIGLE ED, HEIMBECKER RO, GUNTON RW: Idiopathic ventricular septal hypertrophy causing muscular subaortic stenosis. Circulation 26: 325, 1962 11. DESILETs DT, KADELL BM, RUlTENBERG HD, GOLDBERG SJ, MACALPIN RN: Angiographic demonstration of the ventricular septum. Radiology 91: 329, 1968 12. PoPP RL, WOLFE SB, HIRAT T, FEIGENBAUM A: Estimation of right and left ventricular size by ultrasound. Amer J Cardiol 24: 523, 1969 13. FEIGENBAUM H, PoPP RL, CmpI JN, HAINE C: Left ventricular wall thickness measured by ultrasound. Arch Intern Med (Chicago) 121: 391, 1968 14. PoPP RL, HARRISON DC: Ultrasonic cardiac echography for determining stroke volume and valvular regurgitation. Circulation 41: 493, 1970 15. TROY BL, RACKLEY CE: Measurement of the width of the interventricular septum by echocardiography. (Abstr) Circulation 43 (suppl II): II-52, 1971 16. FEIGENBAUM H, WOLFE SB, Popp RL, HAINE CL, DODGE HT: Correlation of ultrasound with angiocardiography in measuring left ventricular diastolic volume. Amer J Cardiol 23: 111, 1969 17. POMEO JF, TROY BL, RUSsELL RO: Left ventricular volumes and ejection fraction by echocardiography. Circulation 43: 480, 1970 18. EDLER I: Ultrasound cardiography in mitral valve stenosis. Amer J Cardiol 19: 18, 1967 19. WIGLE ED, HEIMBECKER RO, GUNTON RW: Idiopathic ventricular septal hypertrophy causing muscular subaortic stenosis. Circulation 26: 325, 1962 20. PARE JA, FRASER RG, PIROZYNSKI WJ, SHANKS JA, STUBINGTON D: Hereditary cardiovascular dysplasia: A form of familial cardiomyopathy. Amer J Med 31: 37, 1961 21. BERCU BA, DIETTERT GA, DANFORT HWH, PUND EE, AHLVAIN RC, BELLIvEAU R: Pseudoaortic stenosis produced by ventricular hypertrophy. Amer J Med 25: 814, 1958 22. BRoCK RC: Functional obstruction of the left ventricle. Guy Hosp Rep 106: 331, 1957 904 ABBASI ET AL. 23. MENGES H, BRANDERBURG RO, BROWN AL: The clinical, hemodynamic and pathologic diagnosis of muscular subaortic stenosis. Circulation 24: 1126, 1961 24. CONN RD, BLACKMON JR, FIGLEY MM, PAULSON PS, KENNEDY JW: Quantitative left ventricular angiography in idiopathic hypertrophic subaortic stenosis. Clin Res 14: 123, 1966 25. OAILEY C: Ventricular hypertrophy in cardiomyopathy. Brit Heart J 33: 179, 1971 Downloaded from http://circ.ahajournals.org/ by guest on June 14, 2017 Circulation, Volume XLVI, November 1972 Echocardiographic Diagnosis of Idiopathic Hypertrophic Cardiomyopathy without Outflow Obstruction A S. ABBASI, R. N. MACALPIN, L. M. EBER and M. L. PEARCE Downloaded from http://circ.ahajournals.org/ by guest on June 14, 2017 Circulation. 1972;46:897-904 doi: 10.1161/01.CIR.46.5.897 Circulation is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231 Copyright © 1972 American Heart Association, Inc. All rights reserved. Print ISSN: 0009-7322. Online ISSN: 1524-4539 The online version of this article, along with updated information and services, is located on the World Wide Web at: http://circ.ahajournals.org/content/46/5/897 Permissions: Requests for permissions to reproduce figures, tables, or portions of articles originally published in Circulation can be obtained via RightsLink, a service of the Copyright Clearance Center, not the Editorial Office. Once the online version of the published article for which permission is being requested is located, click Request Permissions in the middle column of the Web page under Services. 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