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Coexisting idiopathic hypertrophic subaortic stenosis and coronary artery disease
J
THORAC CARDIOVASC SURG
82:278-280, 1981
Coexisting idiopathic hypertrophic subaortic stenosis and coronary artery disease Clinical implication and operative management Patients with either coronary artery disease (CAD) or idiopathic hypertrophic subaortic stenosis (IHSS) may have angina as a dominant symptom. It is also possible that these two diseases may coexist in the same patient. Such an association has been reported in 25% of patients with IHSS who are over 45 years of age. It is important that both entities be looked for in the evaluation of the patient with angina, particularly when operative management is contemplated. Treatment of one and not the other may leave the patient symptomatic. We have encountered three patients with both CAD and severe IHSS and have managed each with septal myectomy and coronary artery revascularization. Each has obtained significant symptomatic improvement.
Scott Stewart, M.D., and Bernard Schreiner, M.D., Rochester, N. Y.
An
association of idiopathic hypertrophic subaortic stenosis (IHSS) and coronary artery disease (CAD) in certain patients with angina has recently been demonstrated. \, 2 In the series of Walston and Behar, t that association occurred in 25% of patients with IHSS who were over 45 years of age. In a review of the literature, 14 patients with IHSS in association with CAD were found who had undergone operation. 2-8 However, only
seven had both a septal myectomy and coronary artery bypass grafting (CABG).3, 4, 6-8 Two of these seven also had aortic valve replacement. Twenty-eight percent (217) died following operation. This paper describes the University of Rochester Medical Center experience with the combined operation (septal myectomy and CABG) and discusses the clinical implication of IHSS and CAD occurring in the same patient. Methods and patients
From the Division of Cardiothoracic Surgery and the Cardiology Unit at the University of Rochester Medical Center, Rochester, N. Y. Received for publication Dec. 29, 1980. Accepted for publication Jan. 20, 1981. Address for reprints: Scott Stewart, M.D., Division of Cardiothoracic Surgery, University of Rochester Medical Center, Rochester, N. Y. 14642.
Between 1973 and 1980, 14 adult patients had a septal myectomy for IHSS at the University of Rochester Medical Center. An additional three patients also had aortic valve replacement for coexisting aortic valve disease." Coronary angiograms were performed in nine of these 17 patients and have been done in every patient
Table I. The clinical findings, operative management, and results of three patients with combined IHSS and CAD Symptoms
ECG
Patient
2
46
Consistent with subendocardial infarction L VH with strain
3
70
L VH with strain
49
Resting gradient (mm Hg)
IHSS
DOE, fatigue, palpitations, angina Angina
90
IHSS
Angina, syncope
35
IHSS
0
Provoked gradient (mm Hg)
EF
185
0.80 0.85
100
0.83
Legend: IHSS, Idiopathic hypertrophic subaortic stenosis. CAD, coronary artery disease. ECG, Electrocardiogram. ECHO, Echocardiogram. EF, Ejection fraction. DOE, Dyspnea on exertion. LVH, Left ventricular hypertrophy. RCA, Right coronary artery. LAD, Left anterior descending coronary artery. CABG, Coronary artery bypass grafting. CVA, Cerebrovascular accident.
278
0022-5223/81/080278+03$00.30/0 © 1981 The C. V. Mosby Co.
Volume 82
Coexisting IHSS and CAD
Number 2
279
August, 1981
with IHSS being considered for operation since 1974. Three patients with IHSS had significant CAD-two had two-vessel CAD and one had single-vessel CAD (Table I). Two of these patients had angina as their principal symptom. The third patient had dyspnea on exertion and easy fatigability as dominant symptoms but also experienced angina. Patient 1 had a welldocumented history of a previous myocardial infarction and an electrocardiogram consistent with an anterolateral subendocardial infarction. In the other two patients the electrocardiogram showed a pattern of left ventricular hypertrophy with strain. In each patient the preoperative echocardiogram showed systolic anterior motion of the mitral valve consistent with IHSS. At cardiac catheterization the resting gradient across the left ventricular outflow tract ranged between 0 and 90 mm Hg. A provoked gradient of 100 mm Hg was obtained in one patient and 185 mm Hg in another. No provocative maneuvers were performed in the patient with a 90 mm Hg resting gradient. Each had a high-normal ejection fraction. Each patient had a septal myectomy performed in the manner described by Morrow. 10 In addition, CABG was performed to significantly obstructed coronary arteries.
Results Each patient survived operation and had an .uncomplicated postoperative course. No patient is symptomatic with angina. Patient 3 does have mild dyspnea on exertion and Patient 1 did have palpitations which are now controlled with a small dose of propranolol.
Discussion Several authors have indicated that the incidence of CAD among those patients with IHSS who are older than 45 years is approximately 25%.1, 2 Despite the reported frequency of this association in two large series, only 14 patients have been described who have
Coronary angiogram
Two 90% marginal branch stenoses One 50% proximal RCA stenosis, one 95% proximal LAD stenosis One 75% proximal LAD stenosis
received operation. Surgical management has included CABG or gas endarterectomy alone, septal myectomy alone, CABG with septal myectomy and CABG, and septal myectomy and aortic valve replacement. Five patients have had CABG and septal myectomy. Two (40%) died after operation. Two other patients have had aortic valve replacement in addition to CABG and septal myectomy for concomitant aortic stenosis and both survived operation. A clinical diagnosis of coexisting IHSS and CAD may be very difficult to make, since the two diseases may share similar symptomatology and physical findings. Although angina is a major symptom of CAD, it may also occur in 80% of patients with IHSS and can be a dominant symptom in many of them. The murmur at the lower left sternal border or apex which occurs in IHSS may be improperly ascribed to papillary muscle dysfunction in a patient who is thought to have isolated CAD. The various maneuvers which affect the character of the murmur of IHSS may provide further clinical differentiation between IHSS and papillary muscle dysfunction. A history of syncope in a patient being evaluated for CAD who has an associated systolic murmur that changes at the bedside in response to the usual provocative maneuvers should arouse suspicion of IHSS. Failure of angina to respond to nitrates, or even a worsening of the angina, may occur in the presence of underlying IHSS since peripheral vasodilation, either arterial, venous, or both, may increase the left ventricular outflow tract obstruction. A history of protracted chest pain or myocardial infarction in a patient with IHSS strongly suggests the possibility of coexisting CAD. Patients with angina and suspected CAD should have an echocardiogram if they have atypical signs or symptoms such as syncope, a systolic murmur, or an unfavorable response to nitrate therapy. This simple, noninvasive examination usually will identify those patients with IHSS. Conversely, patients with IHSS
Operation
Result
Septal myectomy, sequential grafts to marginal vessels Septal myectomy, CABG to RCA and LAD Septal myectomy, CABG to LAD
Two-year follow-up, no angina, rare palpitations controlled with propranolol (Inderal) Four-year follow-up, no angina, died 4 years postop ofa CVA Two-year follow-up, no angina, some DOE
The Journal of
2 80 Stewart and Schreiner
and angina who do not respond to standard medical therapy or who are being considered for operation should have coronary angiograms to assess their coronary artery anatomy in addition to a standard right and left heart catheterization. Appropriate medical management of patients who have coexisting IHSS and CAD should generally include propranolol and should avoid the use of vasodilating drugs, f3-stimulating drugs, and aggressive diuresis. Propranolol has been very effective in the management of IHSS and CAD individually and, when they do coexist, has provided symptomatic relief in about half of the patients. 1 Operation is indicated for those patients whose symptoms are not adequately controlled on medical therapy. The type of operation performed has varied. Some groups have gone to extremes to avoid septal myectomy in the presence of CAD, including bypassing a right coronary artery obstruction in the beating heart and not treating a left anterior descending obstruction in order to avoid the use of cardiopulmonary bypass. 5 Others have believed that if the left ventricular outflow tract obstruction is mild, then septal myectomy is not indicated.! Most patients in this category have obtained a satisfactory result with CABG alone. We favor CABG alone if both the resting and the provoked gradient across the left ventricular outflow tract is small. If the gradient is more than 50 mm Hg, a septal myectomy should be done in addition to CABG. Although the risk of this combined procedure has been high in the past, in our experience it can be performed with minimal morbidity and mortality and with a high likelihood of achieving a satisfactory result.
Thoracic and Cardiovascular Surgery
2
3
4
5
6
7
8
9
10
REFERENCES Walston A, Behar VS: Spectrum of coronary artery disease in idiopathic hypertrophic subaortic stenosis. Am J Cardiol 38:12-16, 1976 Lardani H, Serrano JA, Villamil RJ: Hemodynamics and coronary angiography in idiopathic hypertrophic subaortic stenosis. Am J Cardiol 41:476-481, 1978 Gulotta SJ, Hamby RI, Aronson AL, Ewing K: Coexistent idiopathic hypertrophic subaortic stenosis and coronary arterial disease. Circulation 46:890-896, 1972 Marcus GB, Popp RL, Stinson EB: Coronary artery disease with idiopathic hypertrophic subaortic stenosis. Lancet 5:901-903, 1974 Oran E, Gupta S, Yeo B, Amari J, Lauterstein J, Potter R, Piccone VA: Surgical management of coexisting coronary artery disease and idiopathic hypertrophic subaortic stenosis. Ann Thorac Surg 16:11-18, 1973 Maron BJ, Merrill WH, Freier PA, Kent KM, Epstein SE, Morrow AG: Long-term clinical course and symptomatic status of patients after operation for hypertrophic subaortic stenosis. Circulation 57:1205-1213, 1978 Bensaid J: Idiopathic hypertrophic subaortic stenosis and associated coronary artery disease. Angiology 58:585593, 1979 Cohen 1M, Vieweg WVR, Alpert JS, Dennish GW, Forkerth TL, Hagan AD: Combined valvular aortic stenosis hypertrophic subaortic stenosis and coronary artery disease. Successful surgical correction. J Cardiovasc Surg 18:241-246, 1977 Stewart S, Nanda H, DeWeese JA: The simultaneous operative correction of aortic valve stenosis and idiopathic hypertrophic subaortic stenosis. Circulation 51, 52:Suppl 1:34-39, 1975 Morrow AG: Hypertrophic subaortic stenosis. J THORAC CARDIOVASC SURG 76:423-430, 1978
THORAC CARDIOVASC SURG
82:278-280, 1981
Coexisting idiopathic hypertrophic subaortic stenosis and coronary artery disease Clinical implication and operative management Patients with either coronary artery disease (CAD) or idiopathic hypertrophic subaortic stenosis (IHSS) may have angina as a dominant symptom. It is also possible that these two diseases may coexist in the same patient. Such an association has been reported in 25% of patients with IHSS who are over 45 years of age. It is important that both entities be looked for in the evaluation of the patient with angina, particularly when operative management is contemplated. Treatment of one and not the other may leave the patient symptomatic. We have encountered three patients with both CAD and severe IHSS and have managed each with septal myectomy and coronary artery revascularization. Each has obtained significant symptomatic improvement.
Scott Stewart, M.D., and Bernard Schreiner, M.D., Rochester, N. Y.
An
association of idiopathic hypertrophic subaortic stenosis (IHSS) and coronary artery disease (CAD) in certain patients with angina has recently been demonstrated. \, 2 In the series of Walston and Behar, t that association occurred in 25% of patients with IHSS who were over 45 years of age. In a review of the literature, 14 patients with IHSS in association with CAD were found who had undergone operation. 2-8 However, only
seven had both a septal myectomy and coronary artery bypass grafting (CABG).3, 4, 6-8 Two of these seven also had aortic valve replacement. Twenty-eight percent (217) died following operation. This paper describes the University of Rochester Medical Center experience with the combined operation (septal myectomy and CABG) and discusses the clinical implication of IHSS and CAD occurring in the same patient. Methods and patients
From the Division of Cardiothoracic Surgery and the Cardiology Unit at the University of Rochester Medical Center, Rochester, N. Y. Received for publication Dec. 29, 1980. Accepted for publication Jan. 20, 1981. Address for reprints: Scott Stewart, M.D., Division of Cardiothoracic Surgery, University of Rochester Medical Center, Rochester, N. Y. 14642.
Between 1973 and 1980, 14 adult patients had a septal myectomy for IHSS at the University of Rochester Medical Center. An additional three patients also had aortic valve replacement for coexisting aortic valve disease." Coronary angiograms were performed in nine of these 17 patients and have been done in every patient
Table I. The clinical findings, operative management, and results of three patients with combined IHSS and CAD Symptoms
ECG
Patient
2
46
Consistent with subendocardial infarction L VH with strain
3
70
L VH with strain
49
Resting gradient (mm Hg)
IHSS
DOE, fatigue, palpitations, angina Angina
90
IHSS
Angina, syncope
35
IHSS
0
Provoked gradient (mm Hg)
EF
185
0.80 0.85
100
0.83
Legend: IHSS, Idiopathic hypertrophic subaortic stenosis. CAD, coronary artery disease. ECG, Electrocardiogram. ECHO, Echocardiogram. EF, Ejection fraction. DOE, Dyspnea on exertion. LVH, Left ventricular hypertrophy. RCA, Right coronary artery. LAD, Left anterior descending coronary artery. CABG, Coronary artery bypass grafting. CVA, Cerebrovascular accident.
278
0022-5223/81/080278+03$00.30/0 © 1981 The C. V. Mosby Co.
Volume 82
Coexisting IHSS and CAD
Number 2
279
August, 1981
with IHSS being considered for operation since 1974. Three patients with IHSS had significant CAD-two had two-vessel CAD and one had single-vessel CAD (Table I). Two of these patients had angina as their principal symptom. The third patient had dyspnea on exertion and easy fatigability as dominant symptoms but also experienced angina. Patient 1 had a welldocumented history of a previous myocardial infarction and an electrocardiogram consistent with an anterolateral subendocardial infarction. In the other two patients the electrocardiogram showed a pattern of left ventricular hypertrophy with strain. In each patient the preoperative echocardiogram showed systolic anterior motion of the mitral valve consistent with IHSS. At cardiac catheterization the resting gradient across the left ventricular outflow tract ranged between 0 and 90 mm Hg. A provoked gradient of 100 mm Hg was obtained in one patient and 185 mm Hg in another. No provocative maneuvers were performed in the patient with a 90 mm Hg resting gradient. Each had a high-normal ejection fraction. Each patient had a septal myectomy performed in the manner described by Morrow. 10 In addition, CABG was performed to significantly obstructed coronary arteries.
Results Each patient survived operation and had an .uncomplicated postoperative course. No patient is symptomatic with angina. Patient 3 does have mild dyspnea on exertion and Patient 1 did have palpitations which are now controlled with a small dose of propranolol.
Discussion Several authors have indicated that the incidence of CAD among those patients with IHSS who are older than 45 years is approximately 25%.1, 2 Despite the reported frequency of this association in two large series, only 14 patients have been described who have
Coronary angiogram
Two 90% marginal branch stenoses One 50% proximal RCA stenosis, one 95% proximal LAD stenosis One 75% proximal LAD stenosis
received operation. Surgical management has included CABG or gas endarterectomy alone, septal myectomy alone, CABG with septal myectomy and CABG, and septal myectomy and aortic valve replacement. Five patients have had CABG and septal myectomy. Two (40%) died after operation. Two other patients have had aortic valve replacement in addition to CABG and septal myectomy for concomitant aortic stenosis and both survived operation. A clinical diagnosis of coexisting IHSS and CAD may be very difficult to make, since the two diseases may share similar symptomatology and physical findings. Although angina is a major symptom of CAD, it may also occur in 80% of patients with IHSS and can be a dominant symptom in many of them. The murmur at the lower left sternal border or apex which occurs in IHSS may be improperly ascribed to papillary muscle dysfunction in a patient who is thought to have isolated CAD. The various maneuvers which affect the character of the murmur of IHSS may provide further clinical differentiation between IHSS and papillary muscle dysfunction. A history of syncope in a patient being evaluated for CAD who has an associated systolic murmur that changes at the bedside in response to the usual provocative maneuvers should arouse suspicion of IHSS. Failure of angina to respond to nitrates, or even a worsening of the angina, may occur in the presence of underlying IHSS since peripheral vasodilation, either arterial, venous, or both, may increase the left ventricular outflow tract obstruction. A history of protracted chest pain or myocardial infarction in a patient with IHSS strongly suggests the possibility of coexisting CAD. Patients with angina and suspected CAD should have an echocardiogram if they have atypical signs or symptoms such as syncope, a systolic murmur, or an unfavorable response to nitrate therapy. This simple, noninvasive examination usually will identify those patients with IHSS. Conversely, patients with IHSS
Operation
Result
Septal myectomy, sequential grafts to marginal vessels Septal myectomy, CABG to RCA and LAD Septal myectomy, CABG to LAD
Two-year follow-up, no angina, rare palpitations controlled with propranolol (Inderal) Four-year follow-up, no angina, died 4 years postop ofa CVA Two-year follow-up, no angina, some DOE
The Journal of
2 80 Stewart and Schreiner
and angina who do not respond to standard medical therapy or who are being considered for operation should have coronary angiograms to assess their coronary artery anatomy in addition to a standard right and left heart catheterization. Appropriate medical management of patients who have coexisting IHSS and CAD should generally include propranolol and should avoid the use of vasodilating drugs, f3-stimulating drugs, and aggressive diuresis. Propranolol has been very effective in the management of IHSS and CAD individually and, when they do coexist, has provided symptomatic relief in about half of the patients. 1 Operation is indicated for those patients whose symptoms are not adequately controlled on medical therapy. The type of operation performed has varied. Some groups have gone to extremes to avoid septal myectomy in the presence of CAD, including bypassing a right coronary artery obstruction in the beating heart and not treating a left anterior descending obstruction in order to avoid the use of cardiopulmonary bypass. 5 Others have believed that if the left ventricular outflow tract obstruction is mild, then septal myectomy is not indicated.! Most patients in this category have obtained a satisfactory result with CABG alone. We favor CABG alone if both the resting and the provoked gradient across the left ventricular outflow tract is small. If the gradient is more than 50 mm Hg, a septal myectomy should be done in addition to CABG. Although the risk of this combined procedure has been high in the past, in our experience it can be performed with minimal morbidity and mortality and with a high likelihood of achieving a satisfactory result.
Thoracic and Cardiovascular Surgery
2
3
4
5
6
7
8
9
10
REFERENCES Walston A, Behar VS: Spectrum of coronary artery disease in idiopathic hypertrophic subaortic stenosis. Am J Cardiol 38:12-16, 1976 Lardani H, Serrano JA, Villamil RJ: Hemodynamics and coronary angiography in idiopathic hypertrophic subaortic stenosis. Am J Cardiol 41:476-481, 1978 Gulotta SJ, Hamby RI, Aronson AL, Ewing K: Coexistent idiopathic hypertrophic subaortic stenosis and coronary arterial disease. Circulation 46:890-896, 1972 Marcus GB, Popp RL, Stinson EB: Coronary artery disease with idiopathic hypertrophic subaortic stenosis. Lancet 5:901-903, 1974 Oran E, Gupta S, Yeo B, Amari J, Lauterstein J, Potter R, Piccone VA: Surgical management of coexisting coronary artery disease and idiopathic hypertrophic subaortic stenosis. Ann Thorac Surg 16:11-18, 1973 Maron BJ, Merrill WH, Freier PA, Kent KM, Epstein SE, Morrow AG: Long-term clinical course and symptomatic status of patients after operation for hypertrophic subaortic stenosis. Circulation 57:1205-1213, 1978 Bensaid J: Idiopathic hypertrophic subaortic stenosis and associated coronary artery disease. Angiology 58:585593, 1979 Cohen 1M, Vieweg WVR, Alpert JS, Dennish GW, Forkerth TL, Hagan AD: Combined valvular aortic stenosis hypertrophic subaortic stenosis and coronary artery disease. Successful surgical correction. J Cardiovasc Surg 18:241-246, 1977 Stewart S, Nanda H, DeWeese JA: The simultaneous operative correction of aortic valve stenosis and idiopathic hypertrophic subaortic stenosis. Circulation 51, 52:Suppl 1:34-39, 1975 Morrow AG: Hypertrophic subaortic stenosis. J THORAC CARDIOVASC SURG 76:423-430, 1978