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Echocardiographic features of combined membranous subaortic stenosis and acquired calcific aortic valvulopathy
Case reports
Echocardiographic features of combined membranous subaortic stenosis and acquired calcific aortic valvulopathy Paul G. Hess, M.D. Navin C. Nanda, M.D. James A. DeWeese, M.D. William C. Reeves, M.D. Raymond Gramiak, M.D. Rochester, N. Y.
The combination of congenital fixed subaortic stenosis and acquired calcific aortic valvulopathy is an unusual cause of left ventricular outflow tract (LVOT) obstruction and aortic insufficiency in the middle-aged adult.' ^ We describe a 45-year-old white male with both a congenital subaortic diaphragm and a probably acquired aortic valve deformity leading to the clinical diagnosis of calcific aortic valvular stenosis and insufficiency. The abnormal structure and motion of the aortic valve on the M-mode echocardiogram suggested both aortic valvular and subvalvular lesions prior to surgery. Case report A.C., a-45-year old white male, was referred to Strong Memorial Hospital in January, 1974, for evaluation of cardiac murmurs. There was no history of rheumatic fever, infective endocarditis, or familial heart murmurs. Physical examination revealed a heart rate of 80 per minute and a blood pressure of 110/80 mm. Hg. The lungs were clear. Cardiac examination demonstrated a localized, sustained apical impulse in the normal position; a normal first and second heart sound; a Grade III/VI, harsh, crescendo-decrescendo systolic ejection murmur maximal at the apex with radiation to the entire precordium and both carotid arteries; a Grade II/VI, high-pitched, decrescendo, early diastolic murmur maximal at the left sternal border; normal carotid upstroke; and normal jugular venous pulses. The electrocardiograpi demonstrated left ventricular hypertrophy by precordial voltage criteria. The chest x-ray showed From the Departments of Medicine (Cardiology), Radiology, and Cardiothoracie Surgery, University of Rochester School of Medicine and Dentistry and Strong Memorial Hospital, Rochester, N. Y. Received for publication Aug. 30, 1978. Accepted tor publication Sept. 23, 1976. Reprint requests: Dr. Paul G. Hess, Cardiology Unit—Box 679, Strong Memorial Hospital, 601 Elmwood Ave., Rochester, N. Y. 14642.
September,
1977, Vol. 94, No. 3, pp.
349-352
a normal cardiac silhouette with prominence of the ascending aorta. M-mode cardiac ultrasonography was performed with a commercially available echograph (Picker) and a 2.25 mHz transducer focused at 7 cm. Continuous records were made on a 35 mm. film by means of a Fairchild oscilloscope record camera and a dual beam oscilloscope operating as a slave. The echocardiogram (Fig. 1) demonstrated a slightly en4arged aortic root (width = 40 mm.) containing multiple, central diastolic echoes consistent with a thickened, calcified, tricuspid aortic valve.' Both the anterior (right coronary) and posterior (non-coronary) cusps showed rapid opening movements toward the periphery of the aortic root in systole. The right coronary cusp, immediately after opening, executed an abrupt rapid movement toward closure and remained in the semi-closed position for the remainder of systole, a finding indicative of fixed subaortic left ventricular outflow tract obstruction. Ultrasound beam scanning from the mitral valve to the aortic root failed to show either narrowing of the LVOT or linear echoes in the LVOT, observed occasionally with subaortic membranes. The mitral valve was structurally normal, but demonstrated diastolic flutter of the anterior leaflet consistent with aortic insufficiency. Cardiac catheterization and angiography utilizing the transseptal approach demonstrated a peak systolic pressure gradient across the LVOT of 40 mm. Hg with a left ventricular cavity pressure of 155 /10 mm. Hg and systemic artery pressure of 115/60 mm. Hg, a moderate amount of calcium in the area of a tricuspid aortic valve, and moderate aortic insufficiency. No definite subaortic diaphragm was demonstrated. Coronary arteriograms were normal. Because his symptoms were not severe and since cardiac hemodynamics and angiography indicated a moderate degree of LVOT obstruction and aortic insufficiency, surgery was deferred. T h e patient returned to Strong Memorial Hospital in January, 1976, with symptoms of moderate dyspnea on exertion and anterior chest heaviness consistent with angina pectoris. Physical examination, electrocardiogram, chest xray, cardiac ultrasonography, and repeat coronary arteriography were unchanged from the studies of 1974. Repeat cardiac catheterization was not performed. In view of signifi-
American
Heart
Journal
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H e s s et al.
A¥
ECG Fig. 1, A and B. A, Aortic valve echocardiogram from patient A. C. demonstrating an early systolic, rapid movement toward closure of the anterior (right coronary) cusp, suggesting a subaortic diaphragm. B, Aortic valve echocardiogram from patient A. C. demonstrating multiple diastolic echoes consistent with aortic cusp thickening and calcification. AV = aortic valve; ECG = electrocardiogram.
Fig. 2, A, B, and C. A, Aortic valve echocardiogram from a young girl with a subaortic membrane and normal aortic valve demonstrating an early systolic, rapid movement toward closure of the anterior (right coronary) cusp. B {top). Aortic valve echocardiogram from a patient with hypertrophic obstructive cardiomyopathy (HOCM) showing early systolic, gradual movement toward closure with maintenance of a semi-closed position through midsystole and then a reopening movement in late systole of the anterior (right coronary) cusp. B (bottom), Simultaneous carotid pulse tracing and phonocardiogram. C, Aortic valve echocardiogram from a patient with mitral regurgitation and reduced forward cardiac output showing gradual systolic movement toward closure of both the anterior (right coronary) and posterior (non-coronary) cusps. A y = aortic valve; ECG = electrocardiogram; C = carotid pulse tracing; PCG = phonocardiogram. cant progression in the patient's symptoms, cardiac surgery was advised. At surgery, a deformed, tricuspid aortic valve was present. The cusp commissures were fused at their origins and the valve cusps were thickened with the free edges of the cusps folded back upon themselves to create a predominantly insufficient, minimally stenotic aortic valve. Mild cusp calcification was apparent on gross examination. No evidence of recent or healed Midocarditis was present. A discrete, fibrous diaphragm was located approximately 5 mm. below the aortic valve annulus. This diaphragm appeared stenotic, admitting only the tip of an index finger. The aortic valve cusps and the
350
diaphragm were excised and a cloth-covered Starr-Edwards prosthesis was implanted. The microscopic pathology of the aortic valve cusps revealed areas of fibrosis and calcification. The subaortic diaphragm was composed of dense connective tissue with no muscular elements.
Discussion
We present this case as an unusual example in the middle-aged adult of a congenital subaortic diaphragm causing fixed LVOT obstruction and a
September,
1977, Vol. 94, No. 3
Subaortic stenosis and acquired calcific aortic
probably acquired deformity of the aortic valve associated with moderate aortic insufficiency. We illustrate the difficulty in determining the precise location and nature of LVOT lesions preoperatively by clinical, hemodynamic, and angiographic methods. We emphasize the value of M-mode cardiac ultrasonography as a noninvasive method to evaluate the aortic valve and LVOT. The M-mode echocardiographic features of fixed subaortic LVOT obstruction have been delineated."' In these cases, an early systolic, rapid cusp movement toward closure, frequently with maintenance of this semi-closed position for the duration of systole, is present (Fig. 2A). Aortic valve structure is normal by echocardiography. In this patient, the aortic valve was structurally abnormal with multiple diastolic cusp echoes indicative of thickening and calcification. The preservation of systolic cusp opening, however, suggested the absence of a significant valvular systolic pressure gradient.* The remarkable feature is t h a t enough systolic cusp mobility remained to demonstrate an early and rapid systolic movement toward closure of the right coronary cusp. This early systolic preclosure of the right coronary cusp in the absence of clinical or echocardiographic features of hypertrophic obstructive cardiomyopathy (HOCM), mitral regurgitation, or ventricular septal defect with left-to-right shunting suggested fixed subaortic LVOT obstruction. The flutter of the anterior leaflet of the mitral valve confirmed aortic insufficiency, a condition frequently associated with congenital discrete subaortic stenosis." Various patterns of aortic valve pre-closure have been described with cardiac conditions other than congenital fixed subaortic obstruction.' * HOGM with dynamic LVOT obstruction may demonstrate rapid, full amplitude systolic opening of the aortic valve cusps followed by gradual movement of one or both cusps toward closure in early or mid-systole. The cusps maintain this semi-closed position for a variable time in midsystole and may demonstrate coarse systolic flutter. There is gradual reopening of the cusps in late systole (Fig. 2B). In some cases of mitral regurgitation with reduced cardiac output or ventricular septal defect with left-to-right shunting, the aortic valve cusps may show a full amplitude systolic cusp separation followed by a gradual drifting movement of the cusps toward closure
American Heart
Journal
valvulopathy
and fine systolic flutter for the remainder of systole (Fig. 2C). Gradual movement toward closure in early or mid-systole with gradual reopening for the remainder of systole of the right coronary cusp has been described with right coronary sinus of Valsalva fistulae.' In this patient, the process causing cusp thickening with mild calcification, commissural fusion, and folding back of the cusp free edges is not known. We speculate that the aortic valve deformity was acquired either through the long-terni effects of the subvalvular diaphragm with trauma to the aortic valve cusps from the high velocity jet striking the cusps or, perhaps, chronic rheumatic valvulitis. Aortic valve cusp thickening with rolling back of the edges but without commissural fusion has been described at the time of surgery or autopsy in older patients with discrete subaortic stenosis complicated by aortic valvular endocarditis.^ However, in previous echocardiographic descriptions of fixed subaortic stenosis, aortic valve structure has been normal. The localization of a subaortic diaphragm by cardiac catheterization depends on the precise localization of a fixed subvalvular systolic pressure gradient by catheter withdrawal across the LVOT." If the subaortic diaphragm is immediately subjacent to the aortic valve it may be difficult to distinguish a valvular from a subvalvular systolic pressure gradient. In this patient, the transseptal technique was performed without the benefit of withdrawal pressures across the LVOT. In addition, selective left ventricular angiography by the transseptal method with reflux of contrast dye into the left atrium did not allow optimal visualization of the LVOT to detect a subaortic diaphragm." In summary, we present a middle-aged white male with a congenital subaortic diaphragm and a probably acquired aortic valve deformity documented at surgery. The lesions were associated with moderate LVOT obstruction and aortic insufficiency. The abnormal structure of the aortic valve cusps with multiple diastolic echoes and the abnormal early systolic pre-closure movement of the right coronary cusp on M-mode echocardiography suggested both lesions preoperatively. We emphasize the value of noninvasive M-mode cardiac ultrasonography in the evaluation of the LVOT and aortic valve as an aid in the differential diagnosis of lesions associated with LVOT obstruction and aortic insufficiency.
351
H e s s et al.
Summary
The M-mode echocardiographic features of aortic valve structure and motion in a 45-year-old male with combined congenital subaortic diaphragm and acquired deformity of the aortic valve are described. Clinical, hemodynamic, and angiographic studies suggested calcific aortic valve disease with stenosis and insufficiency, but the additional presence of a subaortic diaphragm was not appreciated. Cardiac ultrasonography demonstrated multiple, central diastolic aortic valve cusp echoes consistent with a thickened, calcified, tricuspid aortic valve. Despite calcification of the cusps, however, enough systolic cusp excursion remained to demonstrate an early systolic, rapid movement toward closure of the right coronary cusp—a finding suggestive of fixed subvalvular obstruction. Surgery confirmed a discrete subaortic diaphragm and a tricuspid, thickened, mildly calcified aortic valve with fusion of the cusp commissures at their origins and rolling back of the cusp edges. The value of echocardiography in the evaluation of the left ventricular outflow tract and aortic valve is emphasized. The authors thank Dr. Arthur Moss for allowing us to study his patient and Mrs. Summer King for her secretarial assistance.
352
REFERENCES 1.
2. 3.
4.
5.
6.
7.
8. 9.
10.
Roberts, W. C : The structure of the aortic valve in clinically isolated aortic stenosis. An autopsy study of 162 patients over 15 years of age, Circulation 42:91, 1970. Morrow, A. G., Fort, L., I l l , Roberts, W. C , and Braunwald, E.: Discrete subaortic stenosis complicated by aortic valvular regurgitation, Circulation 31:163, 1965. Nanda, N. C , Gramiak, R., Manning, J., Mahoney, E. B., Lipchik, E. O., and DeWeese, J. A.: Echocardiographic recognition of the congenital bicuspid aortic valve. Circulation 49:870, 1974. Laurenceau, J.-L., Guay, J. M., and Gagne, S.: Echocardiography in the diagnosis of subaortic membranous stenosis (Abstr.), Circulation 4 7 and 4 8 (Suppl. IV):46, 1973. Davis, R. H., Feigenbaum, H., Chang, S., Konecke, L. L., and Dillon, J. C : Echocardiographic manifestations of discrete subaortic stenosis. Am. J. Cardiol. 33:277, 1974. Popp, R. L., Silverman, J. F., French, J. W., Stinson, E. B., and Harrison, D. C : Echocardiographic findings in discrete subvalvular aortic stenosis. Circulation 49:226, 1974. Johnson, M. L., Warren, S. G., Waugh, R. A., Kisslo, J. A., Sabiston, D. C , and Lester, R. G.: Echocardiography of the aortic valve in non-rheumatic left ventricular outflow tract lesions. Radiology 112:677, 1974. Gramiak, R., and Shah, P. M.: Cardiac ultrasonography: A review of current applications, Radiol. Clin. North Am. 9:469, 1971. Braunwald, E., Goldblatt, A., Aygen, R., Rockoff, S. D., and Morrow, A. G.: Congenital aortic stenosis I. Clinical and hemodynamic findings in 100 patients, Circulation 27:426, 1963. Deutsch, v., Shem-Tov, A., Yahini, J. H., and Neufeld, H. N.: Subaortic stenosis (discrete form). Radiology 101:275, 1971.
September,
1977, Vol. 94, No. 3
Echocardiographic features of combined membranous subaortic stenosis and acquired calcific aortic valvulopathy Paul G. Hess, M.D. Navin C. Nanda, M.D. James A. DeWeese, M.D. William C. Reeves, M.D. Raymond Gramiak, M.D. Rochester, N. Y.
The combination of congenital fixed subaortic stenosis and acquired calcific aortic valvulopathy is an unusual cause of left ventricular outflow tract (LVOT) obstruction and aortic insufficiency in the middle-aged adult.' ^ We describe a 45-year-old white male with both a congenital subaortic diaphragm and a probably acquired aortic valve deformity leading to the clinical diagnosis of calcific aortic valvular stenosis and insufficiency. The abnormal structure and motion of the aortic valve on the M-mode echocardiogram suggested both aortic valvular and subvalvular lesions prior to surgery. Case report A.C., a-45-year old white male, was referred to Strong Memorial Hospital in January, 1974, for evaluation of cardiac murmurs. There was no history of rheumatic fever, infective endocarditis, or familial heart murmurs. Physical examination revealed a heart rate of 80 per minute and a blood pressure of 110/80 mm. Hg. The lungs were clear. Cardiac examination demonstrated a localized, sustained apical impulse in the normal position; a normal first and second heart sound; a Grade III/VI, harsh, crescendo-decrescendo systolic ejection murmur maximal at the apex with radiation to the entire precordium and both carotid arteries; a Grade II/VI, high-pitched, decrescendo, early diastolic murmur maximal at the left sternal border; normal carotid upstroke; and normal jugular venous pulses. The electrocardiograpi demonstrated left ventricular hypertrophy by precordial voltage criteria. The chest x-ray showed From the Departments of Medicine (Cardiology), Radiology, and Cardiothoracie Surgery, University of Rochester School of Medicine and Dentistry and Strong Memorial Hospital, Rochester, N. Y. Received for publication Aug. 30, 1978. Accepted tor publication Sept. 23, 1976. Reprint requests: Dr. Paul G. Hess, Cardiology Unit—Box 679, Strong Memorial Hospital, 601 Elmwood Ave., Rochester, N. Y. 14642.
September,
1977, Vol. 94, No. 3, pp.
349-352
a normal cardiac silhouette with prominence of the ascending aorta. M-mode cardiac ultrasonography was performed with a commercially available echograph (Picker) and a 2.25 mHz transducer focused at 7 cm. Continuous records were made on a 35 mm. film by means of a Fairchild oscilloscope record camera and a dual beam oscilloscope operating as a slave. The echocardiogram (Fig. 1) demonstrated a slightly en4arged aortic root (width = 40 mm.) containing multiple, central diastolic echoes consistent with a thickened, calcified, tricuspid aortic valve.' Both the anterior (right coronary) and posterior (non-coronary) cusps showed rapid opening movements toward the periphery of the aortic root in systole. The right coronary cusp, immediately after opening, executed an abrupt rapid movement toward closure and remained in the semi-closed position for the remainder of systole, a finding indicative of fixed subaortic left ventricular outflow tract obstruction. Ultrasound beam scanning from the mitral valve to the aortic root failed to show either narrowing of the LVOT or linear echoes in the LVOT, observed occasionally with subaortic membranes. The mitral valve was structurally normal, but demonstrated diastolic flutter of the anterior leaflet consistent with aortic insufficiency. Cardiac catheterization and angiography utilizing the transseptal approach demonstrated a peak systolic pressure gradient across the LVOT of 40 mm. Hg with a left ventricular cavity pressure of 155 /10 mm. Hg and systemic artery pressure of 115/60 mm. Hg, a moderate amount of calcium in the area of a tricuspid aortic valve, and moderate aortic insufficiency. No definite subaortic diaphragm was demonstrated. Coronary arteriograms were normal. Because his symptoms were not severe and since cardiac hemodynamics and angiography indicated a moderate degree of LVOT obstruction and aortic insufficiency, surgery was deferred. T h e patient returned to Strong Memorial Hospital in January, 1976, with symptoms of moderate dyspnea on exertion and anterior chest heaviness consistent with angina pectoris. Physical examination, electrocardiogram, chest xray, cardiac ultrasonography, and repeat coronary arteriography were unchanged from the studies of 1974. Repeat cardiac catheterization was not performed. In view of signifi-
American
Heart
Journal
349
H e s s et al.
A¥
ECG Fig. 1, A and B. A, Aortic valve echocardiogram from patient A. C. demonstrating an early systolic, rapid movement toward closure of the anterior (right coronary) cusp, suggesting a subaortic diaphragm. B, Aortic valve echocardiogram from patient A. C. demonstrating multiple diastolic echoes consistent with aortic cusp thickening and calcification. AV = aortic valve; ECG = electrocardiogram.
Fig. 2, A, B, and C. A, Aortic valve echocardiogram from a young girl with a subaortic membrane and normal aortic valve demonstrating an early systolic, rapid movement toward closure of the anterior (right coronary) cusp. B {top). Aortic valve echocardiogram from a patient with hypertrophic obstructive cardiomyopathy (HOCM) showing early systolic, gradual movement toward closure with maintenance of a semi-closed position through midsystole and then a reopening movement in late systole of the anterior (right coronary) cusp. B (bottom), Simultaneous carotid pulse tracing and phonocardiogram. C, Aortic valve echocardiogram from a patient with mitral regurgitation and reduced forward cardiac output showing gradual systolic movement toward closure of both the anterior (right coronary) and posterior (non-coronary) cusps. A y = aortic valve; ECG = electrocardiogram; C = carotid pulse tracing; PCG = phonocardiogram. cant progression in the patient's symptoms, cardiac surgery was advised. At surgery, a deformed, tricuspid aortic valve was present. The cusp commissures were fused at their origins and the valve cusps were thickened with the free edges of the cusps folded back upon themselves to create a predominantly insufficient, minimally stenotic aortic valve. Mild cusp calcification was apparent on gross examination. No evidence of recent or healed Midocarditis was present. A discrete, fibrous diaphragm was located approximately 5 mm. below the aortic valve annulus. This diaphragm appeared stenotic, admitting only the tip of an index finger. The aortic valve cusps and the
350
diaphragm were excised and a cloth-covered Starr-Edwards prosthesis was implanted. The microscopic pathology of the aortic valve cusps revealed areas of fibrosis and calcification. The subaortic diaphragm was composed of dense connective tissue with no muscular elements.
Discussion
We present this case as an unusual example in the middle-aged adult of a congenital subaortic diaphragm causing fixed LVOT obstruction and a
September,
1977, Vol. 94, No. 3
Subaortic stenosis and acquired calcific aortic
probably acquired deformity of the aortic valve associated with moderate aortic insufficiency. We illustrate the difficulty in determining the precise location and nature of LVOT lesions preoperatively by clinical, hemodynamic, and angiographic methods. We emphasize the value of M-mode cardiac ultrasonography as a noninvasive method to evaluate the aortic valve and LVOT. The M-mode echocardiographic features of fixed subaortic LVOT obstruction have been delineated."' In these cases, an early systolic, rapid cusp movement toward closure, frequently with maintenance of this semi-closed position for the duration of systole, is present (Fig. 2A). Aortic valve structure is normal by echocardiography. In this patient, the aortic valve was structurally abnormal with multiple diastolic cusp echoes indicative of thickening and calcification. The preservation of systolic cusp opening, however, suggested the absence of a significant valvular systolic pressure gradient.* The remarkable feature is t h a t enough systolic cusp mobility remained to demonstrate an early and rapid systolic movement toward closure of the right coronary cusp. This early systolic preclosure of the right coronary cusp in the absence of clinical or echocardiographic features of hypertrophic obstructive cardiomyopathy (HOCM), mitral regurgitation, or ventricular septal defect with left-to-right shunting suggested fixed subaortic LVOT obstruction. The flutter of the anterior leaflet of the mitral valve confirmed aortic insufficiency, a condition frequently associated with congenital discrete subaortic stenosis." Various patterns of aortic valve pre-closure have been described with cardiac conditions other than congenital fixed subaortic obstruction.' * HOGM with dynamic LVOT obstruction may demonstrate rapid, full amplitude systolic opening of the aortic valve cusps followed by gradual movement of one or both cusps toward closure in early or mid-systole. The cusps maintain this semi-closed position for a variable time in midsystole and may demonstrate coarse systolic flutter. There is gradual reopening of the cusps in late systole (Fig. 2B). In some cases of mitral regurgitation with reduced cardiac output or ventricular septal defect with left-to-right shunting, the aortic valve cusps may show a full amplitude systolic cusp separation followed by a gradual drifting movement of the cusps toward closure
American Heart
Journal
valvulopathy
and fine systolic flutter for the remainder of systole (Fig. 2C). Gradual movement toward closure in early or mid-systole with gradual reopening for the remainder of systole of the right coronary cusp has been described with right coronary sinus of Valsalva fistulae.' In this patient, the process causing cusp thickening with mild calcification, commissural fusion, and folding back of the cusp free edges is not known. We speculate that the aortic valve deformity was acquired either through the long-terni effects of the subvalvular diaphragm with trauma to the aortic valve cusps from the high velocity jet striking the cusps or, perhaps, chronic rheumatic valvulitis. Aortic valve cusp thickening with rolling back of the edges but without commissural fusion has been described at the time of surgery or autopsy in older patients with discrete subaortic stenosis complicated by aortic valvular endocarditis.^ However, in previous echocardiographic descriptions of fixed subaortic stenosis, aortic valve structure has been normal. The localization of a subaortic diaphragm by cardiac catheterization depends on the precise localization of a fixed subvalvular systolic pressure gradient by catheter withdrawal across the LVOT." If the subaortic diaphragm is immediately subjacent to the aortic valve it may be difficult to distinguish a valvular from a subvalvular systolic pressure gradient. In this patient, the transseptal technique was performed without the benefit of withdrawal pressures across the LVOT. In addition, selective left ventricular angiography by the transseptal method with reflux of contrast dye into the left atrium did not allow optimal visualization of the LVOT to detect a subaortic diaphragm." In summary, we present a middle-aged white male with a congenital subaortic diaphragm and a probably acquired aortic valve deformity documented at surgery. The lesions were associated with moderate LVOT obstruction and aortic insufficiency. The abnormal structure of the aortic valve cusps with multiple diastolic echoes and the abnormal early systolic pre-closure movement of the right coronary cusp on M-mode echocardiography suggested both lesions preoperatively. We emphasize the value of noninvasive M-mode cardiac ultrasonography in the evaluation of the LVOT and aortic valve as an aid in the differential diagnosis of lesions associated with LVOT obstruction and aortic insufficiency.
351
H e s s et al.
Summary
The M-mode echocardiographic features of aortic valve structure and motion in a 45-year-old male with combined congenital subaortic diaphragm and acquired deformity of the aortic valve are described. Clinical, hemodynamic, and angiographic studies suggested calcific aortic valve disease with stenosis and insufficiency, but the additional presence of a subaortic diaphragm was not appreciated. Cardiac ultrasonography demonstrated multiple, central diastolic aortic valve cusp echoes consistent with a thickened, calcified, tricuspid aortic valve. Despite calcification of the cusps, however, enough systolic cusp excursion remained to demonstrate an early systolic, rapid movement toward closure of the right coronary cusp—a finding suggestive of fixed subvalvular obstruction. Surgery confirmed a discrete subaortic diaphragm and a tricuspid, thickened, mildly calcified aortic valve with fusion of the cusp commissures at their origins and rolling back of the cusp edges. The value of echocardiography in the evaluation of the left ventricular outflow tract and aortic valve is emphasized. The authors thank Dr. Arthur Moss for allowing us to study his patient and Mrs. Summer King for her secretarial assistance.
352
REFERENCES 1.
2. 3.
4.
5.
6.
7.
8. 9.
10.
Roberts, W. C : The structure of the aortic valve in clinically isolated aortic stenosis. An autopsy study of 162 patients over 15 years of age, Circulation 42:91, 1970. Morrow, A. G., Fort, L., I l l , Roberts, W. C , and Braunwald, E.: Discrete subaortic stenosis complicated by aortic valvular regurgitation, Circulation 31:163, 1965. Nanda, N. C , Gramiak, R., Manning, J., Mahoney, E. B., Lipchik, E. O., and DeWeese, J. A.: Echocardiographic recognition of the congenital bicuspid aortic valve. Circulation 49:870, 1974. Laurenceau, J.-L., Guay, J. M., and Gagne, S.: Echocardiography in the diagnosis of subaortic membranous stenosis (Abstr.), Circulation 4 7 and 4 8 (Suppl. IV):46, 1973. Davis, R. H., Feigenbaum, H., Chang, S., Konecke, L. L., and Dillon, J. C : Echocardiographic manifestations of discrete subaortic stenosis. Am. J. Cardiol. 33:277, 1974. Popp, R. L., Silverman, J. F., French, J. W., Stinson, E. B., and Harrison, D. C : Echocardiographic findings in discrete subvalvular aortic stenosis. Circulation 49:226, 1974. Johnson, M. L., Warren, S. G., Waugh, R. A., Kisslo, J. A., Sabiston, D. C , and Lester, R. G.: Echocardiography of the aortic valve in non-rheumatic left ventricular outflow tract lesions. Radiology 112:677, 1974. Gramiak, R., and Shah, P. M.: Cardiac ultrasonography: A review of current applications, Radiol. Clin. North Am. 9:469, 1971. Braunwald, E., Goldblatt, A., Aygen, R., Rockoff, S. D., and Morrow, A. G.: Congenital aortic stenosis I. Clinical and hemodynamic findings in 100 patients, Circulation 27:426, 1963. Deutsch, v., Shem-Tov, A., Yahini, J. H., and Neufeld, H. N.: Subaortic stenosis (discrete form). Radiology 101:275, 1971.
September,
1977, Vol. 94, No. 3