hypertrophic subaortic stenosis. Am J Med 1960;29:924-945. 3. Cohen J, Effat H, Goodwin JF, Oakley CM, Steiner RE. Hypertrophic obstructive cardiomyopathy. Br Heart J 1964,26:16-32. 4. Wigle ED, Marquis Y, Auger P. Pharmacodynamics of mitral insufficiency in muscular subaortic stenosis. Can Med Assoc J 1967;97:299-301. 5. Helmcke F, Nanda NC, Hsiung MC, Soto B, Adey CK, Goyal RG, Gatewood RP. Color Doppler assessment of mitral regurgitation with orthogonal planes. Circulation 1987;75:175-183. 6. Kostucki W, Vandenbossche J-L, Friart A, Englert M. Pulsed Doppler regurgitant flow patterns of normal valves. Am J Cardiol 1986;58:309-313. 7. Berger M, Hecht SR, Van Tosh A, Lingam U. Pulsed and continuous wave Doppler echocardiographic assessment of valvular regurgitation in normal subjects. J Am Coil Cardiol 1989;13:1540-1545. 8. Choong CY, Abascal VM, Weyman J, Levine RA, Gentile F, Thomas JD, Weyman AE. Prevalence of valvular regurgitation by Doppler echocardiography in patients with structurally normal hearts by 2-D echocardiography. Am Heart J 1989;117:636-642. 9. Yoshida K, Yoshikawa J, Shakudo M, Akasaka T, Jyo Y, Takao S, Shiratori K, Koizumi K, Odumachi F, Kato H, Fukaya T. Color Doppler evaluation of valvular regurgitation in normal subjects. Circulation 1988;78:840-847.
Combined Obstructive Congenitally Bicuspid
Hypertrophic Aortic Valve
10. Simon AL, Ross J Jr, Gault JH. Angiographic anatomy of the left ventricle and mitral valve in idiopathic hypertrophic subaortic stenosis. Circulation 1967;36:852-864. 11. Hasegawa I, Sakamoto T, Yoshiyuki H, Takenaka K, Amano K, Takahashi H, Takahashi T, Suzuki J, Shiota T, Sugimoto T. J Am Sot Echo 1989;2:177186. 12. Kinoshita N, Yasuharu N, Okamoto M, Miyatake K, Nagata S, Sakakibara H. Mitral regurgitation in hypertrophic cardiomyopathy. Non-invasive study by two dimensional Doppler echocardiography. Br Heart J 1983;49:574-583. 13. Wigle ED, Adelman AG, Auger P, Marquis Y. Mitral regurgitation in muscular subaortic stenosis. Am J Cardiol 1969;24:698-706. 14. Yoshida K, Yoshikawa J, Jyo Y, Shakudo M, Akasaka T, Takao S, Shiratori K, Koizumi K, Okumachi F, Kato H, Fukaya T. Assessment of mitral regurgitation in hypertrophic obstructive cardiomyopathy by color Doppler and continuous-wave Doppler echocardiography. Am J Cardiac Imaging 1989;3:222-228. 15. Nishimura RA, Tajik AJ, Reeder GS, Seward JB. Evaluation of hypertrophic cardiomyopathy by Doppler color flow imaging: initial observations. Mayo Clin Proc 1986;61:631-639. 16. Tunick PA, Gindea A, Kronzon 1. Effect of aortic valve replacement for aortic stenosis on severity of mitral regurgitation. Am J Cardiol 1990;65:1219-1221.
Cardiomyopathy
and Stenotic
Paul S. Brown. Jr.. MD. Charles Stewart Roberts. MD, Charles L. McIntosh, MD, PhD, William C. Roberts, MD,’ and Richard E. Clark, MD ypertrophic cardiomyopathy (HC) is a congenital H heart disease.Personswith 1 type of congenital heart diseaseare more prone to a secondcongenital heart condition than are personswithout any congenital cardiac condition. The frequency of the congenitally bicuspid aortic valve in the general population is believed to be about l%.’ Therefore, it might be expectedto be present in an occasional patient with a more uncommon condition, such asHC. The presentreport describesfindings in 4 patients with coexistingHC and a congenitally bicuspid aortic valve. The records of the Surgery Branch, National Heart, Lung, and Blood Institute, were searched for all patients who had a cardiac operation for HC and who had coexisting aortic valve disease. From 1960 to 1990 a total of 525 patients with HC underwent ventricular septotomy and partial septectomy (hereafter referred to as myotomy and myectomy) and 29 (5%) of them had coexisting aortic valve disease: pure aortic regurgitation in 20 patients and aortic valve stenosis with or without regurgitation in 9patients. Of the latter 9patients, 5 had tricuspid aortic valves, and 4 had bicuspid aortic valves. This report focuses on the 4 patients with combined HC and stenotic congenitally bicuspid aortic valves. Pertinentfindings in the lpatients are summarized in Table I. All 4patients were in New York Heart Association junctional class III or IV. Patients 1 and 2 had other family members with HC. Grade 316 systolic precordial murmurs, loudest along the left sternal border, were audible in all 4patients, and the murmur was made louder by standing or by the Valsalva maneuver. Patients I, 3 and 4 had a second systolic murmur that
radiated to the neck, suggestive of valvular aortic stenosis. By echocardiogram the thickness of the ventricular septum was considerably greater (ratio >I .6) than that of the left ventricular free wall, and systolic anterior motion of the anterior mitral leaflet was present in all 4 patients. Thepeaksystolicpressure gradient between the left ventricle and ascending aorta was >70 mm Hg in all 4 patients (Figure I). At operation, all 4 patients had calci$ed, stenotic, congenitally bicuspid aortic valves (Figure 2). A typical mural endocardial plaque in apposition to the anterior mitral leaflet was present in all 4 patients. The ventricular septum by palpation was severely thickened and typical of that found in HC associated with a normal aortic valve. The portions of excised ventricular septum histologically had myofibril disorganization consistent with HC. All 4 patients had a lessening of their symptoms of cardiac dysfunction late postoperatively.
Coexisting WC and valvular aortic stenosishave been reported in at least 30 patients since 1960,2-4but usually the only evidenceof HC was a ventricular septum thicker than the left ventricular free wall. Although asymmetric septal hypertrophy is usually presentin HC, it also occurs in about 10%of patients with valvular aortic stenosis,and in about 10% of patients with systemic hypertension.5,6 Thus, to diagnose HC in the presenceof valvular aortic stenosis,findings other than a disproportionately thickenedventricular septum are necessary,and such was the case in the 4 patients described here. The congenitally bicuspid aortic valve appears to occur with the samefrequency in patients with obstructive HC asin the general population (each about 1%).At least 2 patients with HC and a congenitally bicuspid stenotic From the Surgery and Pathology Branches,National Heart, Lung, and aortic valve have been reported by others. One,3 a 52Blood Institute, National Institutes of Health, Room 2N244, Building 10, Bethesda, Maryland 20892. Manuscript received June 7, 1990; year-old man, had a 73-mm Hg peak systolic left ventricular outflow pressuregradient, a ventricular septum conrevisedmanuscript receivedand acceptedJuly 5, 1990. THE AMERICAN JOURNAL OF CARDIOLOGY NOVEMBER 15, 1990
siderably thicker than the left ventricular free wall, and systolic anterior motion of the anterior mitral leaflet; he underwent both aortic valve replacement and myotomy and myectomy. The second,4a 4%year-oldwomanwith a family history of HC, had a 120-mm Hg peak systolic pressure gradient between the left ventricle and aorta, and a ventricular septum considerably thicker than the left ventricular free wall; she underwent aortic valve replacement only. The operatively excisedvalvesin our 4 patientsactually appearedconsiderably more stenotic than was the degree of valvular stenosisfound at preoperative cardiac catheterization. In our 4 patients, however,valvular aortic stenosiswas anticipated becauseof the presenceof an additional precordial murmur, calcific deposits in the aortic valve on chest x-ray, and aortic regurgitation.
FIGURE 1. Left ventricle to ascending aorta pullback pressure tracings. The pressure gradient was primarily subvalvular with a 95%, and rates of recurrent pulmonary embolism of
Combined Obstructive Congenitally Bicuspid
Hypertrophic Aortic Valve
10. Simon AL, Ross J Jr, Gault JH. Angiographic anatomy of the left ventricle and mitral valve in idiopathic hypertrophic subaortic stenosis. Circulation 1967;36:852-864. 11. Hasegawa I, Sakamoto T, Yoshiyuki H, Takenaka K, Amano K, Takahashi H, Takahashi T, Suzuki J, Shiota T, Sugimoto T. J Am Sot Echo 1989;2:177186. 12. Kinoshita N, Yasuharu N, Okamoto M, Miyatake K, Nagata S, Sakakibara H. Mitral regurgitation in hypertrophic cardiomyopathy. Non-invasive study by two dimensional Doppler echocardiography. Br Heart J 1983;49:574-583. 13. Wigle ED, Adelman AG, Auger P, Marquis Y. Mitral regurgitation in muscular subaortic stenosis. Am J Cardiol 1969;24:698-706. 14. Yoshida K, Yoshikawa J, Jyo Y, Shakudo M, Akasaka T, Takao S, Shiratori K, Koizumi K, Okumachi F, Kato H, Fukaya T. Assessment of mitral regurgitation in hypertrophic obstructive cardiomyopathy by color Doppler and continuous-wave Doppler echocardiography. Am J Cardiac Imaging 1989;3:222-228. 15. Nishimura RA, Tajik AJ, Reeder GS, Seward JB. Evaluation of hypertrophic cardiomyopathy by Doppler color flow imaging: initial observations. Mayo Clin Proc 1986;61:631-639. 16. Tunick PA, Gindea A, Kronzon 1. Effect of aortic valve replacement for aortic stenosis on severity of mitral regurgitation. Am J Cardiol 1990;65:1219-1221.
Cardiomyopathy
and Stenotic
Paul S. Brown. Jr.. MD. Charles Stewart Roberts. MD, Charles L. McIntosh, MD, PhD, William C. Roberts, MD,’ and Richard E. Clark, MD ypertrophic cardiomyopathy (HC) is a congenital H heart disease.Personswith 1 type of congenital heart diseaseare more prone to a secondcongenital heart condition than are personswithout any congenital cardiac condition. The frequency of the congenitally bicuspid aortic valve in the general population is believed to be about l%.’ Therefore, it might be expectedto be present in an occasional patient with a more uncommon condition, such asHC. The presentreport describesfindings in 4 patients with coexistingHC and a congenitally bicuspid aortic valve. The records of the Surgery Branch, National Heart, Lung, and Blood Institute, were searched for all patients who had a cardiac operation for HC and who had coexisting aortic valve disease. From 1960 to 1990 a total of 525 patients with HC underwent ventricular septotomy and partial septectomy (hereafter referred to as myotomy and myectomy) and 29 (5%) of them had coexisting aortic valve disease: pure aortic regurgitation in 20 patients and aortic valve stenosis with or without regurgitation in 9patients. Of the latter 9patients, 5 had tricuspid aortic valves, and 4 had bicuspid aortic valves. This report focuses on the 4 patients with combined HC and stenotic congenitally bicuspid aortic valves. Pertinentfindings in the lpatients are summarized in Table I. All 4patients were in New York Heart Association junctional class III or IV. Patients 1 and 2 had other family members with HC. Grade 316 systolic precordial murmurs, loudest along the left sternal border, were audible in all 4patients, and the murmur was made louder by standing or by the Valsalva maneuver. Patients I, 3 and 4 had a second systolic murmur that
radiated to the neck, suggestive of valvular aortic stenosis. By echocardiogram the thickness of the ventricular septum was considerably greater (ratio >I .6) than that of the left ventricular free wall, and systolic anterior motion of the anterior mitral leaflet was present in all 4 patients. Thepeaksystolicpressure gradient between the left ventricle and ascending aorta was >70 mm Hg in all 4 patients (Figure I). At operation, all 4 patients had calci$ed, stenotic, congenitally bicuspid aortic valves (Figure 2). A typical mural endocardial plaque in apposition to the anterior mitral leaflet was present in all 4 patients. The ventricular septum by palpation was severely thickened and typical of that found in HC associated with a normal aortic valve. The portions of excised ventricular septum histologically had myofibril disorganization consistent with HC. All 4 patients had a lessening of their symptoms of cardiac dysfunction late postoperatively.
Coexisting WC and valvular aortic stenosishave been reported in at least 30 patients since 1960,2-4but usually the only evidenceof HC was a ventricular septum thicker than the left ventricular free wall. Although asymmetric septal hypertrophy is usually presentin HC, it also occurs in about 10%of patients with valvular aortic stenosis,and in about 10% of patients with systemic hypertension.5,6 Thus, to diagnose HC in the presenceof valvular aortic stenosis,findings other than a disproportionately thickenedventricular septum are necessary,and such was the case in the 4 patients described here. The congenitally bicuspid aortic valve appears to occur with the samefrequency in patients with obstructive HC asin the general population (each about 1%).At least 2 patients with HC and a congenitally bicuspid stenotic From the Surgery and Pathology Branches,National Heart, Lung, and aortic valve have been reported by others. One,3 a 52Blood Institute, National Institutes of Health, Room 2N244, Building 10, Bethesda, Maryland 20892. Manuscript received June 7, 1990; year-old man, had a 73-mm Hg peak systolic left ventricular outflow pressuregradient, a ventricular septum conrevisedmanuscript receivedand acceptedJuly 5, 1990. THE AMERICAN JOURNAL OF CARDIOLOGY NOVEMBER 15, 1990
siderably thicker than the left ventricular free wall, and systolic anterior motion of the anterior mitral leaflet; he underwent both aortic valve replacement and myotomy and myectomy. The second,4a 4%year-oldwomanwith a family history of HC, had a 120-mm Hg peak systolic pressure gradient between the left ventricle and aorta, and a ventricular septum considerably thicker than the left ventricular free wall; she underwent aortic valve replacement only. The operatively excisedvalvesin our 4 patientsactually appearedconsiderably more stenotic than was the degree of valvular stenosisfound at preoperative cardiac catheterization. In our 4 patients, however,valvular aortic stenosiswas anticipated becauseof the presenceof an additional precordial murmur, calcific deposits in the aortic valve on chest x-ray, and aortic regurgitation.
FIGURE 1. Left ventricle to ascending aorta pullback pressure tracings. The pressure gradient was primarily subvalvular with a 95%, and rates of recurrent pulmonary embolism of
