- Email: [email protected]
Anomalous left main coronary artery originating from the right sinus of Valsalva
J THORAC CARDIOVASC SURG 80:198-205, 1980
Anomalous left main coronary artery originating from the right sinus of Valsalva Pathophysiology, angiographic definition, and surgical approaches The origin of the left main coronary artery (LMCA) from the right sinus 4 Valsalva, its course between the aorta and pulmonary artery. is a rare congenital coronary anomaly. seen in only 0.02% of all coronary arteriograms performed at the Cleveland Clinic. We describe the cases offour patients. aged /7 to 7/ years, with this anomaly. The l7-year-old patient had an acute anterolateral myocardial infarction with sudden cardiac collapse. In two patients. aged 58 and 7/ years. the diagnosis of the anomaly was made serendipitously during cardiac catheterization for arteriosclerotic coronary artery disease. The fourth patient. aged 58 years, combined aspects of both groups with clear evidence of anterior myocardial injury but without evidence offixed coronary obstruction. At operation. the coronary anomaly was carefully inspected in three of the four patients. We report the second surgical repair in a young individual with follow-up catheterization and define the preoperative angiographic approach for accurate diagnosis and definition of this lesion. On the bases of a careful inspection of the coronary anatomy at operation and our understanding of the pathophysiology leading to myocardial ischemia and sudden death. we advise a surgical approach for the repair of this rare and often lethal congenital coronary anomaly.
Douglas S. Moodie, M.D., Carl Gill, M.D., Floyd D. Loop, M.D., and William C. Sheldon, M.D., Cleveland, Ohio
T
he origin of the left main coronary artery (LMCA) from the right sinus of Valsalva, its course between the aorta and the pulmonary artery, is a rare congenital coronary anomaly. Several recent reviews concerning this rare condition have specifically referred to the incidence of sudden death in young, asymptomatic individuals following vigorous exercise. 1-12 Several mechanisms have been postulated to explain the cause of sudden death in these cases, the two most common being compression of the LMCA between the aorta and the pulmonary artery with exercise, or angulation and kinking of the origin of the LMCA with occlusion during exercise. Careful inspection of the anatomy of the anomaly at From the Department of Cardiology and the Department of Thoracic and Cardiovascular Surgery, The Cleveland Clinic Foundation, Cleveland, Ohio. Received for publication Nov. I, 1979. Accepted for publication Jan. 16, 1980. Address for reprints: Douglas S. Moodie, M.D., Cleveland Clinic, 9500 Euclid Ave., Cleveland, Ohio 44106.
198
operation has resulted in little or no verification of the proposed mechanisms for sudden death. In fact, there is only one published report" describing the surgical correction of this defect in young patients. Furthermore, angiographic demonstration of the anomaly and catheterization criteria for its definition have not been well defined. We recently have seen four cases in which the LMCA arose from the right sinus of Valsalva and coursed between the aorta and the pulmonary artery, and we report the second surgical repair in a young individual. On the basis of our four cases and a careful inspection of the coronary anatomy at operation, we have defined the angiographic criteria for diagnosis. We recommend a surgical approach based on our understanding of the pathophysiology leading to myocardial ischemia and sudden death.
Case reports CASE I. A 17-year-old high school basketball player, 197.5 cm tall and 99 kg in weight, was completely asymptomatic until he collapsed while jogging after a strenuous
0022-5223/80/080198+08$00.80/0 © 1980 The C. V. Mosby Co.
Volume 80
Origin of LMCA from right sinus of Valsalva
Number 2 August. 1980
I
199
AVR
Fig. lA. A twelve-lead electrocardiogram from Patient I shows findings compatible with an acute anteroseptal myocardial infarction.
I +--+---+--+--.---1,..,.------',...,.,.-----1rv----1r...,.--Jr----~--r~_r_
n
AVR
~---Jlr'"----IV'----JV'---..J~~-AVL ~
AVF
~
--Jl__----O\--,,----Jl__"'--.¥-
_
Fig. lB. Electrocardiogram 2 months after the electrocardiogram demonstrated in Fig. IA. The electrocardiogram returned to near normal with some lack of progression of the R wave from the right precordial leads across the precordium. basketball practice. He was resuscitated by a passerby and rushed to a local emergency room, where he was noted to have pulmonary edema with electrocardiographic evidence of an anterior infarction (Fig. IA). His recovery was uneventful, and a subsequent cardiac catheterization revealed an anomalous left coronary artery arising from the right sinus of Valsalva and directed between the pulmonary artery and the aorta. His activity was severely restricted and he was referred for further evaluation. Two months after the myocardial infarction, the electrocardiogram had improved with some loss of progression of the R waves in the right chest leads (Fig. IB). The patient was clinically asymptomatic, and results of his physical examination were normal. Results of an echocardiogram and resting thallium study were also normal. Surgical intervention was elected because of the obvious high risk of sudden death with any activity. The left internal mammary artery was anastomosed to the anterior descending coronary artery and a saphenous vein graft was constructed
between the aorta and the circumflex coronary artery. His postoperative course was uncomplicated. Two months later, a stress electrocardiogram showed a normal heart rate and blood pressure response; no S-T or T-wave abnormalities were noted. A considerable decrease in diastolic pressure occurred with exercise. Three months after operation, a repeat echocardiogram again was normal. A stress thallium study and the radioisotope ejection fraction (EF = 74%) were normal. Three months later, a second stress exercise test documented improved exercise performance with no evidence of S-T or T-wave abnormalities and a continued normal heart rate response. Cardiac catheterization 3 months after operation demonstrated widely patent internal mammary and vein grafts to the left anterior descending and circumflex arteries, respectively. The left ventricular chamber was elongated and there was mild impairment of the apical segment, which had been noted preoperatively. The anomalous LMCA was well visualized on the postoperative study (Fig. 2). The patient has
The Journal of
200 Moodie et al.
Thoracic and Cardiovascular Surgery
Fig. 2. Case I. Contrast visualization of the left coronary artery, opacified in a shallow left anterior oblique (LAO) projection, arising from the right sinus. continued to be asymptomatic with completely normal exercise performance. CAS E 2. A 71-year-old white man was doing well until 3 months prior to admission, when he awakened from sleep with difficulty breathing and had an episode of syncope that lasted for 30 seconds. An electrocardiogram done on the following day was read as normal. One month later, he had a similar episode of shortness of breath and diaphoresis. An electrocardiogram at that time was also reported as normal. He recalled previous episodes of sudden shortness of breath, but no history of chest pain. The physical examination revealed a blood pressure of 170 to 195 mm Hg systolic over a diastolic pressure of 80 mm Hg, with a Grade 2/6 systolic murmur at the right upper sternal border and left sternal border. Carotid bruits were audible bilaterally. The electrocardiogram revealed left anterior hemiblock with left atrial enlargement. Cardiac catheterization documented severe atherosclerotic coronary artery disease with total occlusion of both the right coronary artery and the proximal high lateral circumflex artery. The LMCA originated anomalously from the right coronary sinus and passed between the pulmonary artery and the aorta (Fig. 3, A and B). There was no angiographic evidence of arteriosclerotic disease in this vessel. Mitral regurgitation secondary to papillary muscle dysfunction was judged moderate. There were also moderate impairment of left ventricular contraction in the proximal half of the diaphragmatic wall and moderate impairment of the posterolateral wall with mild impairment of the anterolateral wall near the apex. A left internal mammary artery graft to the LMCA and saphenous vein grafts to the circumflex and right coronary arteries were performed. During operation there was no evidence of kinking, obstruction, or narrowing of the proximal portion of the anomalous LMCA as it coursed between the pulmonary artery and the aorta. The patient's postoperative course was uneventful and he currently has no cardiac symptoms 10 months following operation. CAS E 3. A 57-year-old white man had a I month history of chest pain. Results of a stress test were abnormal and chest
Fig. 3. A. Case 2. The catheter is in the right coronary sinus. The right coronary artery is totally occluded. The left coronary artery provides a main trunk which proceeds from the right side of the frame, provides a large first septal perforator, and then has the distribution of the left anterior descending artery. A small branch in the upper segment of the frame has the distribution of the circumflex coronary artery. B. Visualization of the coronary artery in the LAO projection. The left main coronary artery arises from the right sinus of Valsalva. pain developed with exercise. Five years previously he had had an episode of syncope while running. Abnormal physical findings included a blood pressure of 142/84 mm Hg with a murmur of mitral insufficiency. Cardiac catheterization demonstrated an anomalous origin of the LMCA (Fig. 4, A and B ) and 70% narrowing of the right coronary artery (Fig. 4, C). There was mild impairment of the anterior wall of the left ventricle with overall normal left ventricular function. Because of his poorly controlled angina, surgical intervention was elected, and in view of localized anterior wall infarction and the anomalous LMCA, bypass to the left anterior descending artery was planned. The anomalous origin of the left main trunk was confirmed at operation, but there was no evidence of kinking or compression of the artery. There was a small area of epicardial scar on the an-
Volume 80 Number 2
Origin of LMCA from right sinus of Valsalva
201
August , 1980
Fig 4. A. Case 3. Selective left coronary artery injection in the LAO projection , caudocranial view, demonstrating passage of the left coronary artery posterior to the conus branch, anterior to the aorta. B. Selective left coronary artery injection in the LAO projection, demon strating the origin of the left coronary artery from the right sinus with normal distribution of the left anterior descending and circumflex arteries, which arise from the left main coronary artery as it cour ses between the aorta and pulmonary artery . C. Right coronary artery injection once again demon strating passage of the left main coronary artery from the right sinus in the LAO view . The right coronary artery shows severe obstruction of about 90 % or more distall y. terior surface of the left ventricle. A left internal mammary artery was anastomosed to the left anterior descending artery, and a saphenous vein graft was constructed to the right coronary artery . His postoperative course was uncompli cated and he is currently free of angin a. CAS E 4. A 58-year-old white man underwent a bypass procedure with a right coronary artery endarterectomy and graft in December, 197 I. Mild aortic stenosis with an aortic gradient of 25 mm Hg was noted . Cardiac catheterization in 1972 demonstrated normal left ventricular function with a patent saphenous vein graft. No gradient was noted acros s the aortic valve . He did well until I year before admission to the Cleveland Clinic Hospital, when he noted increased dyspnea and chest pain with exertion. Abnormal findings on physical examination included a Grade 4/6 systolic ejection murmur at
the aortic area radiating to the neck and a fourth heart sound . There was a faint systolic thrill over the aortic area at end expiration . The electrocardiogram revealed left ventricular hypertrophy with nonspecific S-T and T-wave abnormalities . Catheterization data documented a 50 mm Hg aortic gradient across the aortic valve with a patent saphenous vein graft from the aorta to the right coronary artery . The LMCA arose from the right coronary sinus and cour sed anterior to the aorta and posterior to the pulmonary artery (Fig . 5, A to C). In comparison to his previous study , a severe obstruction had developed in the proximal third of the right coron ary artery and there had been progres sion in the degree of aortic stenosis. Moderately severe narrowing wa s present in the distal right coronary artery beyond the origin of the posterior descending artery . One month following catheterization , the
The Journal of
202 Moodie et al.
Thoracic and Cardiovascular Surgery
J"~~,, i):'~
Fig. SA. Case 4. Diagram of the coronary artery anatomy in Patient 4, demonstrating the origin of the left main coronary artery from the right sinus. The artery courses between the pulmonary artery and the aorta before branching into the left anterior descending and the left circumflex arteries. patient underwent aortic valve replacement with a No. 27 Hancock prosthesis. His postoperative course was uneventful and 2 months following operation he is doing well. Discussion Less than 60 cases of anomalous origin of the LMCA from the right sinus of Valsalva, the course being between the aorta and pulmonary artery, have been described. This anomaly has been an unusual finding in our patient population, with only 13 cases since 1961, an incidence of 0.02% although we saw four cases in the first 6 months of 1978. The angiographic interpretation and definition of this anomaly can be difficult. With injection into the left coronary sinus, one sees no opacification of the proximal LMCA. Direct visualization of the LMCA is noted from the right sinus of Valsalva. The best cardiac catheterization views for this anomaly are the right anterior oblique (RAO) and the lateral views; however, even in the RAO and lateral views, the course of the anomalous artery can be difficult to define. We recently have reviewed the cineangiograms of all our patients with an anomalous LMCA or left anterior descending artery arising from the right sinus. We find it difficult in
many cases to determine if the left coronary artery is running anterior or posterior to the pulmonary artery. For this reason, a balloon catheter should be placed in the pulmonary artery, as was done in Case 4. With injection into the proximal LMCA, one can see the course of this artery posterior to the balloon catheter. It is also important to try to identify the conus branch as it runs anterior to the anomalous vessel. Simultaneous injection in the right ventricle or pulmonary artery and the LMCA is the best method to demonstrate the position of the anomalous left coronary artery. Liberthson and associates! have pointed out that the plane of the anomalous left coronary artery is not at the level of the pulmonary artery, but at the level of the right ventricular infundibulum. Case 4, with simultaneous injection into the right ventricle, clearly demonstrates this finding. Table I lists the reported cases with this anomaly; they fit into two categories. Of the 30 patients, 19 were males younger than 22 years of age who died suddenly during or soon after vigorous exercise. The remaining eight patients compose a second group in whom the diagnosis of the anomaly has been made serendipitously during cardiac catheterization for acquired cardiac problems. The second group documents that the mere presence of an anomalous LMCA passing between the aorta and the pulmonary artery is not uniformly fatal, nor is it limited to the male population. In the first group, vigorous exercise in the presence of an anomalous LMCA passing between the aorta and pulmonary artery would certainly seem to be the precipitating factor in the sudden death of these young men. In our series of patients, Patient I fits into the first group, Patients 2 and 4 fit into the second group, and Patient 3 seems to combine aspects of both groups. He has clear evidence of anterior myocardial injury without evidence of fixed coronary obstruction, combined with a history of syncope during heavy exercise; yet he sought medical attention because of atherosclerotic coronary artery disease involving the right coronary artery. The case of sudden death or myocardial injury in these patients is unknown. Cohen and Shaw" postulated that compression of the anomalous coronary artery by the pulmonary artery and aorta during heavy exercise led to myocardial ischemia. Cheitlin and co-workers" reviewed cardiac specimens at the Armed Forces Institute of Pathology and believed that this theory was unlikely because of the relatively low pressure in the pulmonary arterial system. They alternatively proposed that the mechanism of myocardial ischemia in the distribution of the left coronary artery was related to angu-
Volume 80 Number 2
Origin of LMCA from right sinus of Valsalva
203
August. 1980
Fig. 5. B. Case 4 . Left coronary artery, 110 degree left posterior oblique position. In this view the left coronary artery can be clearl y seen to pass posterior to the Swan-Ganz catheter , which is positioned in the right ventricular outflow tract in the pulmonary artery . The conu s branch of the right coronary artery can be seen to pass anteriorly over the surface of the right ventricle anterior to the Swan-Ganz catheter. Thi s view demon strates that the left coronary artery passes between the pulmonary arter y and the right ventr icular outflow tract anteriorly and the aorta posteriorly. A rrows point to the Swan-Ganz catheter. C. Simultaneous right ventriculogram and left coronary artery arteriogram, 110 degree left posterior oblique projection , during right ventricular diastole. This frame demonstrates that the left coronary artery passes posterior to the right ventricular outflow tract and appears to pa ss immediately below the level of the pulmonary valve in the region of the right ventri cular infundibulum.
lation at the origin of the left coronary artery from the anterior sinus of Valsalva, occasioned by aortic distension during exercise. They advocated enlarging the origin of the LMCA to prevent sudden death and treated one patient in this fashion . First, from our experience and that of others, we
know that patients who have what appear to be normal right ventricles and normal pulmonary arterial vasculature may develop marked transient pulmonary hypertension immediately before and after an aortacoronary bypass procedure . Consequently , it does not seem impossible that this might occur during strenuous
The Journal of
204
Moodieetal.
Thoracic and Cardiovascular Surgery
Table I. Summary of reported patients with anomalous left coronary artery between the aorta and the pulmonary artery Case No.
I
I 2 3 4 5 6 76.7.10-12, 8 5,
9 10 II 12 13 14 15 16
174 , 18 191, 20 21 22"' 23 24 25 26 27 28 29 30
Age (yr.)
I
Sex
/--------------c-a-us-e-O-if-d-ea-r-h--------'-------
21 14 II 16 54 13 13 13 14 18 17 18 22 20 22 36
M M M M M M M M M M M M M M M M
17 II 49 39 70 I II 17
M M F M F M M M
Died after exercise Died after running Died after running, had myocardial infarction Died after basketball Died while shaving Died while running Died while playing basketball Massive myocardial infarction, died 4 hours later Died suddenly after exercise Died while running Died while running Died while running Died while running Died during forced march, had myocardial infarction Died while running Died suddenly after clutching chest and falling into a river; documented ventricular tachycardia in past Died during exercise Died during exercise Alive, mitral stenosis, asymptomatic Complete occlusion of proximal LAD, circumflex, and right coronary arteries; alive 90% stenosis of right coronary artery, 65% stenosis of circumflex Died while crying Died during exercise Died while playing soccer
36-70
4 M, 1 F
All alive 4 - atherosclerotic disease 1- aortic stenosis 2-operated upon with graft to the LAD and circumflex arteries
Legend: LAD. Left anterior descending coronary artery.
• Reference number.
exercise and might well cause embarrassment of blood flow through a vessel passing between the pulmonary artery and the aorta. Second, the normal responses to exercise (as seen in Patient I)-an increase in systolic pressure associated with an increase in heart ratecombined to increase myocardial oxygen demands while decreasing diastolic coronary perfusion pressure and time. These factors, combined with a small impediment to coronary flow, might be sufficient to produce myocardial ischemia. Patients I and 3 in this series clearly demonstrate myocardial injury in the distribution of the anomalous coronary arteries without fixed obstruction of the coronary arteries or evidence of kinking, narrowing, or flap formation at the origin of the LMCA. With these thoughts in mind, we believed the most prudent course was to provide an effective coronary blood flow; consequently, aorta-coronary bypass grafting was performed on the symptomatic patients.
Patient I is the first in whom successful revascularization for this anomaly has been reported in a person younger than the age of 20 years. Young patients, particularly those who have experienced symptoms of syncope or chest pain associated with exercise with this anomaly, should undergo bypass grafting. As the circumflex artery and the LMCA arise immediately upon passage of the LMCA between the aorta and pulmonary artery, it would seem appropriate that grafts be placed to the left anterior descending artery as well as the circumflex. The longterm results in this condition with performance of coronary artery vein bypass grafts are not known, but it is certainly gratifying that our young patient has completely normal exercise dynamics postoperatively with patent grafts. The question of competitive flow and relatively normal anatomic coronary arteries is unresolved and will await further evaluation.
Volume 80
Origin of LMCA from right sinus of Valsalva
Number 2
205
August, 1980
Our four cases indicate to us that an operation for this lesion can be done safely in young individuals who are symptomatic or who have had an infarction, as well in adults who are symptomatic. On the basis of our four cases, we have listed our angiographic definition of this anomaly, and on the basis of our angiographic findings and observations at operation, we have tried to define the possible pathophysiology of this lesion. We report the second successful repair in a young individual who had had a myocardial infarction. REFERENCES
2
3
4
5
Chaitman BR, Lesperance 11, Saltiel 1, Bourassa MG: Clinical, angiographic, and hemodynamic findings in patients with anomalous origin of the coronary arteries. Circulation 53: 122-131, 1976 Levin DC, Fellows KE, Abrams HL: Hemodynamically significant primary anomalies of the coronary arteries. Circulation 58:25-33, 1978 Kimbiris D, Iskandrian AS, Segal BL, Bemis CE: Anomalous aortic origin of coronary arteries. Circulation 58:606-614, 1978 Liberthson RR, Dinsmore RE, Bharati S, et al: Aberrant coronary artery origin from the aorta. Circulation 50: 774-779, 1974 Cheitlin MD, DeCastro CM, McAllister HA: Sudden
6 7
8
9
10
II
12 13
death as the complication of anomalous left coronary origin from the anterior sinus of Valsalva. Circulation 50:780-787, 1974 Adams RA, Sato K: Sudden death during exercise in an II-year-old boy. 1 Pediatr 85:731,1974 Liberthson RR, Dinsmore RE, Block PC, Pohort GM, Strauss HW: Myocardial compromise and sudden death in aberrant coronary artery origin from the aorta (abstr). Am 1 CardioI41:378, 1978 Liberthson RR, Dinsmore RE, Fallon JT: Aberrant coronary artery origin from the aorta. Circulation 59:748-754, 1979 Cohen LS, Shaw LD: Fatal myocardial infarction in an II-year-old boy associated with a unique coronary artery anomaly. Am 1 Cardiol 19:420-423, 1967 lokI C, McClellan IT, Ross GD: Congenital anomaly of left coronary artery in young athletes. lAMA 182:572573, 1962 Benson PA: Anomalous aortic origin of coronary artery with sudden death. Case report and review. Am Heart 1 79:254-257, 1970 Benson PA, Lack AR: Anomalous aortic origin of left coronary artery. Arch Pathol 86:214-216, 1968 Sacks IH, Londe SP, Rosenbluth A, Zalis EG: Left main coronary bypass for aberrant (aortic) intramural left coronary artery. 1 THoRAc CARDIOVASC SURG 73:733-737, 1977
Copyright information The appearance of a code at the bottom of the first page of an original article in this journal indicates the copyright owner's consent that copies of the article may be made for personal or internal use, or for the personal or internal use of specific clients. This consent is given on the condition, however, that the copier pay the stated per copy fee through the Copyright Clearance Center, Inc., 21 Congress Street, Salem, Mass. 01970, 617-7443350, for copying beyond that permitted by Sections 107 or 108 of the U. S. Copyright Law. This consent does not extend to other kinds of copying, such as copying for general distribution, for advertising or promotional purposes, for creating new collective works, or for resale.
Anomalous left main coronary artery originating from the right sinus of Valsalva Pathophysiology, angiographic definition, and surgical approaches The origin of the left main coronary artery (LMCA) from the right sinus 4 Valsalva, its course between the aorta and pulmonary artery. is a rare congenital coronary anomaly. seen in only 0.02% of all coronary arteriograms performed at the Cleveland Clinic. We describe the cases offour patients. aged /7 to 7/ years, with this anomaly. The l7-year-old patient had an acute anterolateral myocardial infarction with sudden cardiac collapse. In two patients. aged 58 and 7/ years. the diagnosis of the anomaly was made serendipitously during cardiac catheterization for arteriosclerotic coronary artery disease. The fourth patient. aged 58 years, combined aspects of both groups with clear evidence of anterior myocardial injury but without evidence offixed coronary obstruction. At operation. the coronary anomaly was carefully inspected in three of the four patients. We report the second surgical repair in a young individual with follow-up catheterization and define the preoperative angiographic approach for accurate diagnosis and definition of this lesion. On the bases of a careful inspection of the coronary anatomy at operation and our understanding of the pathophysiology leading to myocardial ischemia and sudden death. we advise a surgical approach for the repair of this rare and often lethal congenital coronary anomaly.
Douglas S. Moodie, M.D., Carl Gill, M.D., Floyd D. Loop, M.D., and William C. Sheldon, M.D., Cleveland, Ohio
T
he origin of the left main coronary artery (LMCA) from the right sinus of Valsalva, its course between the aorta and the pulmonary artery, is a rare congenital coronary anomaly. Several recent reviews concerning this rare condition have specifically referred to the incidence of sudden death in young, asymptomatic individuals following vigorous exercise. 1-12 Several mechanisms have been postulated to explain the cause of sudden death in these cases, the two most common being compression of the LMCA between the aorta and the pulmonary artery with exercise, or angulation and kinking of the origin of the LMCA with occlusion during exercise. Careful inspection of the anatomy of the anomaly at From the Department of Cardiology and the Department of Thoracic and Cardiovascular Surgery, The Cleveland Clinic Foundation, Cleveland, Ohio. Received for publication Nov. I, 1979. Accepted for publication Jan. 16, 1980. Address for reprints: Douglas S. Moodie, M.D., Cleveland Clinic, 9500 Euclid Ave., Cleveland, Ohio 44106.
198
operation has resulted in little or no verification of the proposed mechanisms for sudden death. In fact, there is only one published report" describing the surgical correction of this defect in young patients. Furthermore, angiographic demonstration of the anomaly and catheterization criteria for its definition have not been well defined. We recently have seen four cases in which the LMCA arose from the right sinus of Valsalva and coursed between the aorta and the pulmonary artery, and we report the second surgical repair in a young individual. On the basis of our four cases and a careful inspection of the coronary anatomy at operation, we have defined the angiographic criteria for diagnosis. We recommend a surgical approach based on our understanding of the pathophysiology leading to myocardial ischemia and sudden death.
Case reports CASE I. A 17-year-old high school basketball player, 197.5 cm tall and 99 kg in weight, was completely asymptomatic until he collapsed while jogging after a strenuous
0022-5223/80/080198+08$00.80/0 © 1980 The C. V. Mosby Co.
Volume 80
Origin of LMCA from right sinus of Valsalva
Number 2 August. 1980
I
199
AVR
Fig. lA. A twelve-lead electrocardiogram from Patient I shows findings compatible with an acute anteroseptal myocardial infarction.
I +--+---+--+--.---1,..,.------',...,.,.-----1rv----1r...,.--Jr----~--r~_r_
n
AVR
~---Jlr'"----IV'----JV'---..J~~-AVL ~
AVF
~
--Jl__----O\--,,----Jl__"'--.¥-
_
Fig. lB. Electrocardiogram 2 months after the electrocardiogram demonstrated in Fig. IA. The electrocardiogram returned to near normal with some lack of progression of the R wave from the right precordial leads across the precordium. basketball practice. He was resuscitated by a passerby and rushed to a local emergency room, where he was noted to have pulmonary edema with electrocardiographic evidence of an anterior infarction (Fig. IA). His recovery was uneventful, and a subsequent cardiac catheterization revealed an anomalous left coronary artery arising from the right sinus of Valsalva and directed between the pulmonary artery and the aorta. His activity was severely restricted and he was referred for further evaluation. Two months after the myocardial infarction, the electrocardiogram had improved with some loss of progression of the R waves in the right chest leads (Fig. IB). The patient was clinically asymptomatic, and results of his physical examination were normal. Results of an echocardiogram and resting thallium study were also normal. Surgical intervention was elected because of the obvious high risk of sudden death with any activity. The left internal mammary artery was anastomosed to the anterior descending coronary artery and a saphenous vein graft was constructed
between the aorta and the circumflex coronary artery. His postoperative course was uncomplicated. Two months later, a stress electrocardiogram showed a normal heart rate and blood pressure response; no S-T or T-wave abnormalities were noted. A considerable decrease in diastolic pressure occurred with exercise. Three months after operation, a repeat echocardiogram again was normal. A stress thallium study and the radioisotope ejection fraction (EF = 74%) were normal. Three months later, a second stress exercise test documented improved exercise performance with no evidence of S-T or T-wave abnormalities and a continued normal heart rate response. Cardiac catheterization 3 months after operation demonstrated widely patent internal mammary and vein grafts to the left anterior descending and circumflex arteries, respectively. The left ventricular chamber was elongated and there was mild impairment of the apical segment, which had been noted preoperatively. The anomalous LMCA was well visualized on the postoperative study (Fig. 2). The patient has
The Journal of
200 Moodie et al.
Thoracic and Cardiovascular Surgery
Fig. 2. Case I. Contrast visualization of the left coronary artery, opacified in a shallow left anterior oblique (LAO) projection, arising from the right sinus. continued to be asymptomatic with completely normal exercise performance. CAS E 2. A 71-year-old white man was doing well until 3 months prior to admission, when he awakened from sleep with difficulty breathing and had an episode of syncope that lasted for 30 seconds. An electrocardiogram done on the following day was read as normal. One month later, he had a similar episode of shortness of breath and diaphoresis. An electrocardiogram at that time was also reported as normal. He recalled previous episodes of sudden shortness of breath, but no history of chest pain. The physical examination revealed a blood pressure of 170 to 195 mm Hg systolic over a diastolic pressure of 80 mm Hg, with a Grade 2/6 systolic murmur at the right upper sternal border and left sternal border. Carotid bruits were audible bilaterally. The electrocardiogram revealed left anterior hemiblock with left atrial enlargement. Cardiac catheterization documented severe atherosclerotic coronary artery disease with total occlusion of both the right coronary artery and the proximal high lateral circumflex artery. The LMCA originated anomalously from the right coronary sinus and passed between the pulmonary artery and the aorta (Fig. 3, A and B). There was no angiographic evidence of arteriosclerotic disease in this vessel. Mitral regurgitation secondary to papillary muscle dysfunction was judged moderate. There were also moderate impairment of left ventricular contraction in the proximal half of the diaphragmatic wall and moderate impairment of the posterolateral wall with mild impairment of the anterolateral wall near the apex. A left internal mammary artery graft to the LMCA and saphenous vein grafts to the circumflex and right coronary arteries were performed. During operation there was no evidence of kinking, obstruction, or narrowing of the proximal portion of the anomalous LMCA as it coursed between the pulmonary artery and the aorta. The patient's postoperative course was uneventful and he currently has no cardiac symptoms 10 months following operation. CAS E 3. A 57-year-old white man had a I month history of chest pain. Results of a stress test were abnormal and chest
Fig. 3. A. Case 2. The catheter is in the right coronary sinus. The right coronary artery is totally occluded. The left coronary artery provides a main trunk which proceeds from the right side of the frame, provides a large first septal perforator, and then has the distribution of the left anterior descending artery. A small branch in the upper segment of the frame has the distribution of the circumflex coronary artery. B. Visualization of the coronary artery in the LAO projection. The left main coronary artery arises from the right sinus of Valsalva. pain developed with exercise. Five years previously he had had an episode of syncope while running. Abnormal physical findings included a blood pressure of 142/84 mm Hg with a murmur of mitral insufficiency. Cardiac catheterization demonstrated an anomalous origin of the LMCA (Fig. 4, A and B ) and 70% narrowing of the right coronary artery (Fig. 4, C). There was mild impairment of the anterior wall of the left ventricle with overall normal left ventricular function. Because of his poorly controlled angina, surgical intervention was elected, and in view of localized anterior wall infarction and the anomalous LMCA, bypass to the left anterior descending artery was planned. The anomalous origin of the left main trunk was confirmed at operation, but there was no evidence of kinking or compression of the artery. There was a small area of epicardial scar on the an-
Volume 80 Number 2
Origin of LMCA from right sinus of Valsalva
201
August , 1980
Fig 4. A. Case 3. Selective left coronary artery injection in the LAO projection , caudocranial view, demonstrating passage of the left coronary artery posterior to the conus branch, anterior to the aorta. B. Selective left coronary artery injection in the LAO projection, demon strating the origin of the left coronary artery from the right sinus with normal distribution of the left anterior descending and circumflex arteries, which arise from the left main coronary artery as it cour ses between the aorta and pulmonary artery . C. Right coronary artery injection once again demon strating passage of the left main coronary artery from the right sinus in the LAO view . The right coronary artery shows severe obstruction of about 90 % or more distall y. terior surface of the left ventricle. A left internal mammary artery was anastomosed to the left anterior descending artery, and a saphenous vein graft was constructed to the right coronary artery . His postoperative course was uncompli cated and he is currently free of angin a. CAS E 4. A 58-year-old white man underwent a bypass procedure with a right coronary artery endarterectomy and graft in December, 197 I. Mild aortic stenosis with an aortic gradient of 25 mm Hg was noted . Cardiac catheterization in 1972 demonstrated normal left ventricular function with a patent saphenous vein graft. No gradient was noted acros s the aortic valve . He did well until I year before admission to the Cleveland Clinic Hospital, when he noted increased dyspnea and chest pain with exertion. Abnormal findings on physical examination included a Grade 4/6 systolic ejection murmur at
the aortic area radiating to the neck and a fourth heart sound . There was a faint systolic thrill over the aortic area at end expiration . The electrocardiogram revealed left ventricular hypertrophy with nonspecific S-T and T-wave abnormalities . Catheterization data documented a 50 mm Hg aortic gradient across the aortic valve with a patent saphenous vein graft from the aorta to the right coronary artery . The LMCA arose from the right coronary sinus and cour sed anterior to the aorta and posterior to the pulmonary artery (Fig . 5, A to C). In comparison to his previous study , a severe obstruction had developed in the proximal third of the right coron ary artery and there had been progres sion in the degree of aortic stenosis. Moderately severe narrowing wa s present in the distal right coronary artery beyond the origin of the posterior descending artery . One month following catheterization , the
The Journal of
202 Moodie et al.
Thoracic and Cardiovascular Surgery
J"~~,, i):'~
Fig. SA. Case 4. Diagram of the coronary artery anatomy in Patient 4, demonstrating the origin of the left main coronary artery from the right sinus. The artery courses between the pulmonary artery and the aorta before branching into the left anterior descending and the left circumflex arteries. patient underwent aortic valve replacement with a No. 27 Hancock prosthesis. His postoperative course was uneventful and 2 months following operation he is doing well. Discussion Less than 60 cases of anomalous origin of the LMCA from the right sinus of Valsalva, the course being between the aorta and pulmonary artery, have been described. This anomaly has been an unusual finding in our patient population, with only 13 cases since 1961, an incidence of 0.02% although we saw four cases in the first 6 months of 1978. The angiographic interpretation and definition of this anomaly can be difficult. With injection into the left coronary sinus, one sees no opacification of the proximal LMCA. Direct visualization of the LMCA is noted from the right sinus of Valsalva. The best cardiac catheterization views for this anomaly are the right anterior oblique (RAO) and the lateral views; however, even in the RAO and lateral views, the course of the anomalous artery can be difficult to define. We recently have reviewed the cineangiograms of all our patients with an anomalous LMCA or left anterior descending artery arising from the right sinus. We find it difficult in
many cases to determine if the left coronary artery is running anterior or posterior to the pulmonary artery. For this reason, a balloon catheter should be placed in the pulmonary artery, as was done in Case 4. With injection into the proximal LMCA, one can see the course of this artery posterior to the balloon catheter. It is also important to try to identify the conus branch as it runs anterior to the anomalous vessel. Simultaneous injection in the right ventricle or pulmonary artery and the LMCA is the best method to demonstrate the position of the anomalous left coronary artery. Liberthson and associates! have pointed out that the plane of the anomalous left coronary artery is not at the level of the pulmonary artery, but at the level of the right ventricular infundibulum. Case 4, with simultaneous injection into the right ventricle, clearly demonstrates this finding. Table I lists the reported cases with this anomaly; they fit into two categories. Of the 30 patients, 19 were males younger than 22 years of age who died suddenly during or soon after vigorous exercise. The remaining eight patients compose a second group in whom the diagnosis of the anomaly has been made serendipitously during cardiac catheterization for acquired cardiac problems. The second group documents that the mere presence of an anomalous LMCA passing between the aorta and the pulmonary artery is not uniformly fatal, nor is it limited to the male population. In the first group, vigorous exercise in the presence of an anomalous LMCA passing between the aorta and pulmonary artery would certainly seem to be the precipitating factor in the sudden death of these young men. In our series of patients, Patient I fits into the first group, Patients 2 and 4 fit into the second group, and Patient 3 seems to combine aspects of both groups. He has clear evidence of anterior myocardial injury without evidence of fixed coronary obstruction, combined with a history of syncope during heavy exercise; yet he sought medical attention because of atherosclerotic coronary artery disease involving the right coronary artery. The case of sudden death or myocardial injury in these patients is unknown. Cohen and Shaw" postulated that compression of the anomalous coronary artery by the pulmonary artery and aorta during heavy exercise led to myocardial ischemia. Cheitlin and co-workers" reviewed cardiac specimens at the Armed Forces Institute of Pathology and believed that this theory was unlikely because of the relatively low pressure in the pulmonary arterial system. They alternatively proposed that the mechanism of myocardial ischemia in the distribution of the left coronary artery was related to angu-
Volume 80 Number 2
Origin of LMCA from right sinus of Valsalva
203
August. 1980
Fig. 5. B. Case 4 . Left coronary artery, 110 degree left posterior oblique position. In this view the left coronary artery can be clearl y seen to pass posterior to the Swan-Ganz catheter , which is positioned in the right ventricular outflow tract in the pulmonary artery . The conu s branch of the right coronary artery can be seen to pass anteriorly over the surface of the right ventricle anterior to the Swan-Ganz catheter. Thi s view demon strates that the left coronary artery passes between the pulmonary arter y and the right ventr icular outflow tract anteriorly and the aorta posteriorly. A rrows point to the Swan-Ganz catheter. C. Simultaneous right ventriculogram and left coronary artery arteriogram, 110 degree left posterior oblique projection , during right ventricular diastole. This frame demonstrates that the left coronary artery passes posterior to the right ventricular outflow tract and appears to pa ss immediately below the level of the pulmonary valve in the region of the right ventri cular infundibulum.
lation at the origin of the left coronary artery from the anterior sinus of Valsalva, occasioned by aortic distension during exercise. They advocated enlarging the origin of the LMCA to prevent sudden death and treated one patient in this fashion . First, from our experience and that of others, we
know that patients who have what appear to be normal right ventricles and normal pulmonary arterial vasculature may develop marked transient pulmonary hypertension immediately before and after an aortacoronary bypass procedure . Consequently , it does not seem impossible that this might occur during strenuous
The Journal of
204
Moodieetal.
Thoracic and Cardiovascular Surgery
Table I. Summary of reported patients with anomalous left coronary artery between the aorta and the pulmonary artery Case No.
I
I 2 3 4 5 6 76.7.10-12, 8 5,
9 10 II 12 13 14 15 16
174 , 18 191, 20 21 22"' 23 24 25 26 27 28 29 30
Age (yr.)
I
Sex
/--------------c-a-us-e-O-if-d-ea-r-h--------'-------
21 14 II 16 54 13 13 13 14 18 17 18 22 20 22 36
M M M M M M M M M M M M M M M M
17 II 49 39 70 I II 17
M M F M F M M M
Died after exercise Died after running Died after running, had myocardial infarction Died after basketball Died while shaving Died while running Died while playing basketball Massive myocardial infarction, died 4 hours later Died suddenly after exercise Died while running Died while running Died while running Died while running Died during forced march, had myocardial infarction Died while running Died suddenly after clutching chest and falling into a river; documented ventricular tachycardia in past Died during exercise Died during exercise Alive, mitral stenosis, asymptomatic Complete occlusion of proximal LAD, circumflex, and right coronary arteries; alive 90% stenosis of right coronary artery, 65% stenosis of circumflex Died while crying Died during exercise Died while playing soccer
36-70
4 M, 1 F
All alive 4 - atherosclerotic disease 1- aortic stenosis 2-operated upon with graft to the LAD and circumflex arteries
Legend: LAD. Left anterior descending coronary artery.
• Reference number.
exercise and might well cause embarrassment of blood flow through a vessel passing between the pulmonary artery and the aorta. Second, the normal responses to exercise (as seen in Patient I)-an increase in systolic pressure associated with an increase in heart ratecombined to increase myocardial oxygen demands while decreasing diastolic coronary perfusion pressure and time. These factors, combined with a small impediment to coronary flow, might be sufficient to produce myocardial ischemia. Patients I and 3 in this series clearly demonstrate myocardial injury in the distribution of the anomalous coronary arteries without fixed obstruction of the coronary arteries or evidence of kinking, narrowing, or flap formation at the origin of the LMCA. With these thoughts in mind, we believed the most prudent course was to provide an effective coronary blood flow; consequently, aorta-coronary bypass grafting was performed on the symptomatic patients.
Patient I is the first in whom successful revascularization for this anomaly has been reported in a person younger than the age of 20 years. Young patients, particularly those who have experienced symptoms of syncope or chest pain associated with exercise with this anomaly, should undergo bypass grafting. As the circumflex artery and the LMCA arise immediately upon passage of the LMCA between the aorta and pulmonary artery, it would seem appropriate that grafts be placed to the left anterior descending artery as well as the circumflex. The longterm results in this condition with performance of coronary artery vein bypass grafts are not known, but it is certainly gratifying that our young patient has completely normal exercise dynamics postoperatively with patent grafts. The question of competitive flow and relatively normal anatomic coronary arteries is unresolved and will await further evaluation.
Volume 80
Origin of LMCA from right sinus of Valsalva
Number 2
205
August, 1980
Our four cases indicate to us that an operation for this lesion can be done safely in young individuals who are symptomatic or who have had an infarction, as well in adults who are symptomatic. On the basis of our four cases, we have listed our angiographic definition of this anomaly, and on the basis of our angiographic findings and observations at operation, we have tried to define the possible pathophysiology of this lesion. We report the second successful repair in a young individual who had had a myocardial infarction. REFERENCES
2
3
4
5
Chaitman BR, Lesperance 11, Saltiel 1, Bourassa MG: Clinical, angiographic, and hemodynamic findings in patients with anomalous origin of the coronary arteries. Circulation 53: 122-131, 1976 Levin DC, Fellows KE, Abrams HL: Hemodynamically significant primary anomalies of the coronary arteries. Circulation 58:25-33, 1978 Kimbiris D, Iskandrian AS, Segal BL, Bemis CE: Anomalous aortic origin of coronary arteries. Circulation 58:606-614, 1978 Liberthson RR, Dinsmore RE, Bharati S, et al: Aberrant coronary artery origin from the aorta. Circulation 50: 774-779, 1974 Cheitlin MD, DeCastro CM, McAllister HA: Sudden
6 7
8
9
10
II
12 13
death as the complication of anomalous left coronary origin from the anterior sinus of Valsalva. Circulation 50:780-787, 1974 Adams RA, Sato K: Sudden death during exercise in an II-year-old boy. 1 Pediatr 85:731,1974 Liberthson RR, Dinsmore RE, Block PC, Pohort GM, Strauss HW: Myocardial compromise and sudden death in aberrant coronary artery origin from the aorta (abstr). Am 1 CardioI41:378, 1978 Liberthson RR, Dinsmore RE, Fallon JT: Aberrant coronary artery origin from the aorta. Circulation 59:748-754, 1979 Cohen LS, Shaw LD: Fatal myocardial infarction in an II-year-old boy associated with a unique coronary artery anomaly. Am 1 Cardiol 19:420-423, 1967 lokI C, McClellan IT, Ross GD: Congenital anomaly of left coronary artery in young athletes. lAMA 182:572573, 1962 Benson PA: Anomalous aortic origin of coronary artery with sudden death. Case report and review. Am Heart 1 79:254-257, 1970 Benson PA, Lack AR: Anomalous aortic origin of left coronary artery. Arch Pathol 86:214-216, 1968 Sacks IH, Londe SP, Rosenbluth A, Zalis EG: Left main coronary bypass for aberrant (aortic) intramural left coronary artery. 1 THoRAc CARDIOVASC SURG 73:733-737, 1977
Copyright information The appearance of a code at the bottom of the first page of an original article in this journal indicates the copyright owner's consent that copies of the article may be made for personal or internal use, or for the personal or internal use of specific clients. This consent is given on the condition, however, that the copier pay the stated per copy fee through the Copyright Clearance Center, Inc., 21 Congress Street, Salem, Mass. 01970, 617-7443350, for copying beyond that permitted by Sections 107 or 108 of the U. S. Copyright Law. This consent does not extend to other kinds of copying, such as copying for general distribution, for advertising or promotional purposes, for creating new collective works, or for resale.