Aneurvsm and Fistula of the Sinus of Valsalva J
Clinical Considerations and Surgical Treatment i n 45 Patients Joseph Meyer, M.D., Don C. Wukasch, M.D.,
Grady L. Hallman, M.D., and Denton A. Cooley, M.D. ABSTRACT Aneurysms and fistulas of the sinus of Valsalva, although rare, present a challenging surgical problem. Forty-five patients with this lesion have undergone operative treatment during the last 17 years. T h e series includes 32 male and 13 female patients ranging in age from 2 to 68 years with a mean age of 35.3 years. Only 1 early death occurred in the series, a hospital mortality of 2.2%. Diagnosis of the aneurysm was made preoperatively in 30 patients and discovered at operation in 15. The lesion involved the right coronary sinus in 28 patients, the noncoronary sinus in 19, and the left coronary sinus in 5. The aneurysm had not ruptured in 22 patients and had formed a fistulous communication between the right coronary sinus and the right ventricle in 13. Acute rupture occurred in 10 patients (22.2%). Long-term follow-up data were obtained in 38 patients after 1 to 15 years. Late mortality is low. A nonruptured aneurysm of the sinus of Valsalva should be managed conservatively when it occurs as an isolated lesion. Our experience supports the concept that perforated aneurysms and fistulas of the sinus of Valsalva, even if asymptomatic, should be treated operatively.
A
neurysms and fistulas of the sinus of Valsalva are rare. Among 10,370 patients who underwent cardiac procedures employing temporary cardiopulmonary bypass at the Texas Heart Institute from April, 1956, through September, 1973,45 had aneurysms or fistulas, or both, of the sinus of Valsalva; this represents 0.43% of the procedures utilizing cardiopulmonary bypass for that period of time. Although uncommon, this entity is important because it often poses difficult technical problems.
Clinical Material From April, 1956, through September, 1973,45 patients with aneurysms or fistulas, or both, of the aortic sinus underwent operation. Of these, 32 patients were male and 13 were female; they ranged in age from 2 to 68 years with a median age of 35.3 years. T h e right coronary sinus was involved in 28 patients, the noncoronary sinus in 19, and the left coronary sinus in 5, From the Division of Surgery, Texas Heart Institute of St. Luke’s Episcopal and Texas Children’s Hospitals, Houston, Tex. Accepted for publication Sept. 27, 1974. Address reprint requests to Dr. Cooley, Texas Heart Institute, P.O. Box 20345, Houston, Tex. 77025.
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Aneurysm and Fistula of the Sinus of Valsalva
accounting for a total of 52 instances of aneurysmal or ruptured sinus of Valsalva in 45 patients (Table 1). In the group of 22 patients whose aneurysms had not ruptured, 3 had two dilated aortic sinuses and 2 had three dilated sinuses. A fistula was present in 23 patients, either as part of a ruptured aneurysm or as a simple fistulous tract without aneurysmal dilatation. Communication between an aortic sinus and a cardiac chamber occurred as follows: sixteen times to the right ventricle, four times to the right atrium, once to the left ventricle, and once to the left atrium; one perforation occurred into the pericardium (Fig. 1). In 10 patients there was a reported history of rheumatic fever and in 17 patients an episode of bacterial endocarditis. Twenty-five patients were believed to have a congenital aneurysm oE the aortic sinus. One patient suffered from systemic lupus erythematosus, and in another the fistula was probably iatrogenic following cardiac catheterization for aortic valve disease caused by rheumatic fever. Six of the 25 patients with congenital lesions had typical Marfan’s syndrome. All 45 patients had associated anomalies. In 32 patients some pathological involvement of the aortic valve was present: 5 had combined aortic stenosis and aortic insufficiency, 2 had isolated aortic stenosis, and 25 had moderate to severe aortic insufficiency. Mitral insufficiency was present in 4 patients, ventricular septa1 defect in 5, and infundibular pulmonary stenosis in 3. One patient had an atrioventricular canal. According to the Functional Classification of the New York Heart Association, only 4 patients were asymptomatic at the time of operation: 23 were in Class 11, 11 were in Class 111, and 7 were in Class IV. Easy fatigability, shortness of breath, chest pain, and palpitations were the most common subjective symptoms. Sudden onset of manifestations was reported by 8 patients, all of whom had a ruptured aneurysm; in 5 the rupture had occurred during or following an episode of bacterial endocarditis. TABLE 1. ANATOMY OF ANEURYSM OR FISTULA OF T H E SINUS OF VAISALVA IN 45 PATIENTS
Site of
Rupture Not ruptured
Ruptured into right ventricle Ruptured into right atrium Ruptured into left atrium Ruptured into left ventricle Ruptured into pericardium Total
Right Coronary Sinus
Left Coronary Sinus
10 16
...
...
1
Noncoronary Sinus
Total
14
29
...
3
4
...
...
1
1
1
...
...
1
...
...
1
1
28
5
19
52
5
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FIG. 1. Origin and entry of
fi-
tulous communication; nuhbers indicate numbers of patients. (NCS = noncoronary sinus; RCS = right coronary sinus; LCS = left coron a y sinus.)
A “machinery type” murmur along the left sternal border was heard in only 9 patients, all of whom had a fistula from the aorta to the right ventricle. Cardiac enlargement was evident on chest roentgenogram in 25 patients and was most pronounced when associated with aortic insufficiency. Electrocardiogram revealed left ventricular hypertrophy in 17 patients and some form of conduction defect or arrhythmia in 12. Cardiac catheterization was performed in 36 patients and angiography in 28, with visualization of the aneurysm or fistula in 16. In 2 patients the catheter passed directly through the fistulous tract. A correct preoperative diagnosis of aneurysm or fistula, or both, of the sinus of Valsalva was made in 30 patients. Involvement of a sinus of Valsalva was an intraoperative finding in 15 patients. Comparison of the preoperative diagnosis and actual operative findings in these 15 patients is shown in Table 2.
Operatiue Techniques All patients but 1 underwent operation using temporary cardiopulmonary bypass and, since 1972, moderate hypothermia (30°C;. T h e defect was corrected in 44 patients. An exploratory thoracotomy alone was performed in 1 patient. This last patient underwent operation in 1965 and had a densely calcified aneurysm of the left coronary sinus of Valsalva that partially occluded the left main coronary artery; the aneurysm could not be repaired. T h e standard incision was a median sternotomy. Arterial cannulation was carried out through the right femoral artery in 32 patients
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THE ANNALS OF THORACIC SURGERY
Aneurysm and Fistula of the Sinus of Valsalua TABLE 2. PREOPERATIVE DIAGNOSIS COMPARED W I T H OPERATIVE FINDINGS IN 15 PATIENTS W I T H ANEURYSM OR FISTULA OF T H E SINUS OF VALSALVA
Patient’s Age (yr) Year of & Sex Operation
Preoperative Diagnosis
Operative Findings
F M M M M F
1956 1957 1959 1960 1960 1961
VSD IPS & left-to-right shunt VSD VSD A1 & VSD Tetralogy of Fallot
36, M 61, F
1961 1964
31, M 27, M
1965 1966
51, M 2, M
1966 1967
52, M
1968
20, M
1969
VSD or CA-to-RV fistula AS & A1 with left-to-right shunt VSD & A1 Massive AI, acute endocarditis, no cardiac cath. (Class IV) A1 Severe A1 (112 mm Hg gradient) Severe AI, rapidly progressive CHF, no cardiac cath. (Class I11 to IV) VSD, AI, AV canal
43, F
1969
18, 38, 17, 22, 34, 31,
Acute bacterial endocarditis, AS, AI, & MI
RC sinus-RV fistula RC sinus-RV fistula & IPS RC sinus-RV fistula RC sinus-RV fistula RC sinus-RV fistula Tetralogy & RC sinus-RV fistula VSD & RC sinus-RV fistula AS & AI; iatrogenic noncoronary sinus-LA fistula RC sinus-RV fistula Massive A1 with destroyed valve, aneurysm of noncoronary sinus AI, aneurysm of RC sinus Severe AS & aneurysm of noncoronary sinus A1 due to annular dilatation by aneurysm of RC sinus VSD, AI, AV canal, & non-
coronary sinus aneurysm AS & MI, noncoronary sinus aneurysm with abscess & perforation into pericardium
RC = right coronary artery; RV = right ventricle; VSD = ventricular septa1 defect; IPS = infundibular pulmonic stenosis; A1 = aortic insufficiency; AS = aortic stenosis; LA = left atrium; AV = atrioventricular; MI = mitral insufficiency; CHF = congestive heart failure; CA = coronary artery.
and in the distal ascending aorta in the last 12. A sump suction was placed through either the apex in the left ventricle (before 1967) or the right superior pulmonary vein in the left atrium (after 19G7). Of the 22 nonruptured aneurysms, 18 were totally resected. Aortic continuity was obtained by direct suture in 4 patients and through insertion of a Dacron patch in 11 (Fig. 2); direct closure was accomplished with interrupted simple or mattress sutures buttressed with felt when necessary. Three patients who had one or more massive aneurysms of the aortic sinus also had an aneurysm of the ascending aorta. Aneurysms of the sinus of Valsalva were resected together with the ascending aorta and replaced with a Dacron tube graft. Both coronary arteries were implanted in the tube graft in 2 patients (Fig. 3). I n another patient only the right coronary artery was reimplanted in the tube graft; the left coronary ostium with its rim of aortic wall was left attached to the aortic annulus. I n 3 patients the aneurysm was left in place and the aortic opening was closed with a Dacron patch. In 1 other patient a small aneurysmal dilatation
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associated with severe aortic stenosis and insufficiency was closed during implantation of a prosthetic aortic valve. Of the 23 patients with fistulas of the sinus of Valsalva, 21 underwent repair by direct closure and 2 by use of a Dacron patch. I n 10 patients a double approach-through the aorta and the involved cardiac chamber-was used, most frequently after 1965. A right ventriculotomy was performed in 13 patients and a right atriotomy in 3. Operative treatment of associated anomalies is shown in Table 3. Among 30 patients with aortic insufficiency, 24 underwent aortic valve replacement and 3 had aortic valvotomy (all in 1960). In the remaining 3 patients aortic insufficiency was not hemodynamically significant. I n 2 patients isolated stenosis was corrected by aortic valvotomy. An associated ventricular septa1 defect was closed with mattress sutures in 2 patients and with a Dacron patch in 3. In 1 patient with associated atrioventricular canal and cleft mitral valve the operative procedure consisted of resection and Dacron patch closure of a nonruptured aneurysm of the noncoronary sinus, direct closure of the mitral cleft, and Dacron patch repair of the atrioventricular canal. One patient underwent mitral replacement, 1 had mitral annuloplasty, and another had pulmonary infundibulectomy.
FIG. 2. Resection of nonruptured sinus of Valsalva aneurysm (a) and surgical repair by ( b ) direct closure or (c) insertion of a Dacron patch graft.
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Aneurysm and Fistula of the Sinus of Valsalva
Operative Results Of the 45 patients, 44 survived the operative procedure and 37 experienced an uneventful postoperative course. The average time of hospitalization was 15.1 days. Early postoperative complications necessitated four operations. In 2 patients reoperation was performed early for evacuation of a hemothorax followed by both patients' complete recovery. One patient, operated on in 1964 for aortic valve disease with an aneurysm of the noncoronary sinus, underwent a second operation nine days after the first procedure because of recurrence of the aneurysm and aortic insufficiency. A larger artificial valve (Magovern No. 4) was implanted and the recurrent aneurysm was repaired. He did well for about five months, then died because of formation of a massive thrombus on the artificial valve that had partially occluded both coronary arteries. The fourth patient who
MEYER ET AL. required reoperation suffered from Marfan’s syndrome with right coronary sinus aneurysm. T h e aneurysm was resected and repaired with a Dacron patch. On the second postoperative day the patient developed noticeable aortic valve insufficiency which led to reoperation and replacement of the aortic valve thirteen days after the first procedure. This patient also had an aneurysm of the aortic arch which was not resected and led to his sudden death at home three weeks later because of acute dissection and rupture into the esophagus. T h e patient who died immediately after operation in 1963 also had Marfan’s syndrome. A fistula from the aorta to the right atrium was closed through a right atriotomy with interrupted mattress sutures, and he died on the first day after operation of pulmonary edema caused by recurrence of the fistula with rupture of most of the stitches due to poor tissue. Microscopical examination revealed complete absence of elastic tissue in the wall of the aortic sinus. Other immediate postoperative complications included atrial arrhythmia (2 patients) and urinary tract infection (1 patient). T h e patient with a sinus of Valsalva aneurysm that had ruptured into the pericardium was treated by aortic valve replacement and direct closure of the opening of the fistula. T h e patient had a completely uneventful postoperative course and was doing well 2 years after operation. Late follow-up data included information on 38 patients for a period of 1 to 15 years with an average follow-up of 4 years. Three patients were lost to follow-up and 4 died during the first postoperative year: 1 patient immediately after operation, 1 after three weeks due to rupture of a thoracic aneurysm, 1 after five months from thrombosis of an artificial valve, and 1 TABLE 9. ASSOCIATED ANOMALIES AND OPERATIVE TECHNIQUES USED ~
~
Anomalies and Techniques Aortic insufficiency Aortic valve replacement Aortic valvotomy Isolated aortic stenosis Aortic valvotomy VSD Direct suture Dacron patch Mitral insufficiency Mitral annuloplasty Mitral valve replacement Infundibular pulmonic stenosis Pulmonary infundibulectomy Atrioventricular canal Dacron patch Total
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THE ANNALS OF THORACIC SURGERY
No. of Patients 24 3
2 2 3 1 1
1 1 38
30 2
5 4
3 1
45
Aneurysm and Fistula of the Sinus of Valsalva TABLE 4. CAUSE OF LATE DEATH IN 5 PATIENTS WHO UNDERWENT SURGICAL CORRECTION OF A FISTULA OF T H E SINUS OF VALSALVA ~~
Patient’s Age (yr) Year of & Sex Operation ~~
Year of Death
Interval (yr)
Operative Procedure
Cause of Death
Closure RC sinusRV fistula Closure RC sinusRC fistula, right ventriculotomy Suture of noncoronary sinus, fistula to LA, AVR AVR, mitral annuloplasty Excision of noncoronary sinus aneurysm & direct closure, AVR (SmeloffCutter)
Bacterial endocarditis Unknown
18, F
1956
1968
12
34, M
1960
1962
2
67, F
1964
1969
5
61, M
1966
1968
2
54, M
1967
1968
1
Stroke, CVI
CAOD Massive cerebral embolus
RC =right coronary; RV = right ventricle; AVR = aortic valve replacement; LA = left atrium; CVI = cerebrovascular insufficiency; CAOD = coronary artery occlusive disease.
after six months from systemic lupus erythematosus. T h e first patient in our series who underwent successful correction of a fistula from the right coronary sinus to the right ventricle in 1956 died 12 years later from a fulminating vegetative endocarditis. T o date, 4 other deaths have been recorded (Table 4).
Comment Aneurysms and fistulas of the sinus of Valsalva are rare and represent only 0.43y0 of the procedures using cardiopulmonary bypass performed at our institutions between April, 1956, and September, 1974. As reported in other series, most of our patients were young adult men [l, 3, 71. T h e right coronary sinus was most frequently involved (64y0in our series), followed by the noncoronary sinus. Fistulas or aneurysms of the left coronary sinus are rare. A perforation or fistula connected most frequently with the right ventricle and rarely with the right atrium, the proportion being 4 to 1. A fistula into the left atrium, left ventricle, or pericardium was the exception. A review of our series and those of others [l-31 does not provide a definitive etiology for this anomaly. There is strong indication that the aneurysm was congenital in origin in more than half of the patients in this series. This impression was drawn from the history, operative findings, and histological examination. T h e medical history of 12 patients supported an
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infectious etiology: in 5 an episode of bacterial endocarditis was responsible for the perforation of what we believed to be a preexisting congenital aneurysm of the aortic sinus. Most patients in our series were symptomatic, but in view of the frequency of associated anomalies this finding does not appear to be of specific value. T h e characteristic to-and-fro, machinery type murmur was heard less often in our series than in others [3, 51 and appears to characterize a fistula from the aorta to the right ventricle. Roentgenograms of the chest and electrocardiograms were not particularly valuable in our series. At the present time it should be possible to obtain a correct diagnosis in most of these patients with the help of cardiac catheterization and cineangiocardiography; however, two possible diagnostic difficulties remain: (1) the differential diagnosis between a fistula connecting the right coronary sinus to the right ventricle and a high ventricular septa1 defect; and (2) diagnosis of an asymptomatic small- to moderate-sized aneurysm of the noncoronary sinus of Valsalva. An operative classification of sinus of Valsalva lesions must include the following three entities: (1) nonruptured aneurysm of the aortic sinus of Valsalva, (2) aneurysm of the aortic sinus with intracardiac or extracardiac rupture, and (3) intracardiac or extracardiac fistula of the sinus of Valsalva. An unruptured aneurysm of the aortic sinus can easily remain undiagnosed unless it is associated with a hemodynamically significant cardiac lesion (most frequently involving the aortic valve), is sufficiently large to be noticed on a chest roentgenogram, or produces symptoms through compression of the right atrium or right ventricle. In our series an associated cardiac lesion whose hemodynamic properties indicated the need for operation was found in 21 of the 22 patients with nonruptured sinus of Valsalva aneurysms. We agree with Howard and colleagues [3] that patients with isolated nonruptured aneurysms of the sinus of Valsalva should be followed closely rather than operated upon. T h e 23 patients in our series who had perforated aneurysms or fistulas included 10 with windsock, or balloon type, aneurysms with the perforation at the tip of the aneurysm and 13 with a simple fistulous communication without aneurysmal dilatation. This differentiation is important because of the technical implications. Resection of a perforated aneurysm is a more difficult procedure, often necessitating a patch repair, while a simple fistulous tract without aneurysm can often be closed directly with a few interrupted mattress sutures. A ruptured aneurysm or fistula of the aortic sinus always constitutes an indication for operation since it imposes the constant strain of a left-to-right shunt and may cause acute pulmonary edema through a sudden increase in the diameter of the communication. With today’s advanced techniques in anesthesiology and cardiopulmonary bypass and with the feasibility of
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THE ANNALS OF THORACIC SURGERY
Aneurysm and Fistula of the Sinus of Valsalva aggressive aortic root operations using Dacron tube grafts, prosthetic aortic valves, and aortocoronary saphenous vein bypass grafts [4, 61, it should be possible to resect and replace most aneurysms of the sinus of Valsalva successfully.
References 1. Bonfils-Roberts, E. A., DuShane, J. W., McGoon, D. C., and Danielson, G. K.
2. 3.
4.
5. 6.
7.
Aortic sinus fistula: Surgical considerations and results of operation. Ann Thorac Surg 12:492, 1971. Chapman, D. W., Beazley, H. L., Peterson, P. K., Webb, J. A., and Cooley, D. A. Annulo-aortic ectasia with cystic medial necrosis. Am J Cardiol 16:679, 1965. Howard, R. J., Moller, J., Castaneda, A. R., Varco, R. L., and Nicoloff, D. M. Surgical correction of sinus of Valsalva aneurysm. J Thorac Cardiovasc Surg 66:420, 1973. Meyer, J., and Cooley, D. A. Surgical management of patients with pathological changes involving the aortic root: Review of present techniques and proposal of a modified surgical approach. Cardiovasc Dis (Bull Tex Heart Znst) l:lOl, 1974. Morgan, J. R., Rogers, A. K., and Fosburg, R. G. Ruptured aneurysms of the sinus of Valsalva. Chest 61:640, 1972. Najafi, H. Aneurysm of cystic medionecrotic aortic root: A modified surgical approach. J Thorac Cardiounsc Surg 66:71, 1973. Sawyers, J. L., Adams, J. E., and Scott, H. W. Surgical treatment for aneurysms of the aortic sinuses with aorticoatrial fistula. Surgery 41 :26, 1957.
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Clinical Considerations and Surgical Treatment i n 45 Patients Joseph Meyer, M.D., Don C. Wukasch, M.D.,
Grady L. Hallman, M.D., and Denton A. Cooley, M.D. ABSTRACT Aneurysms and fistulas of the sinus of Valsalva, although rare, present a challenging surgical problem. Forty-five patients with this lesion have undergone operative treatment during the last 17 years. T h e series includes 32 male and 13 female patients ranging in age from 2 to 68 years with a mean age of 35.3 years. Only 1 early death occurred in the series, a hospital mortality of 2.2%. Diagnosis of the aneurysm was made preoperatively in 30 patients and discovered at operation in 15. The lesion involved the right coronary sinus in 28 patients, the noncoronary sinus in 19, and the left coronary sinus in 5. The aneurysm had not ruptured in 22 patients and had formed a fistulous communication between the right coronary sinus and the right ventricle in 13. Acute rupture occurred in 10 patients (22.2%). Long-term follow-up data were obtained in 38 patients after 1 to 15 years. Late mortality is low. A nonruptured aneurysm of the sinus of Valsalva should be managed conservatively when it occurs as an isolated lesion. Our experience supports the concept that perforated aneurysms and fistulas of the sinus of Valsalva, even if asymptomatic, should be treated operatively.
A
neurysms and fistulas of the sinus of Valsalva are rare. Among 10,370 patients who underwent cardiac procedures employing temporary cardiopulmonary bypass at the Texas Heart Institute from April, 1956, through September, 1973,45 had aneurysms or fistulas, or both, of the sinus of Valsalva; this represents 0.43% of the procedures utilizing cardiopulmonary bypass for that period of time. Although uncommon, this entity is important because it often poses difficult technical problems.
Clinical Material From April, 1956, through September, 1973,45 patients with aneurysms or fistulas, or both, of the aortic sinus underwent operation. Of these, 32 patients were male and 13 were female; they ranged in age from 2 to 68 years with a median age of 35.3 years. T h e right coronary sinus was involved in 28 patients, the noncoronary sinus in 19, and the left coronary sinus in 5, From the Division of Surgery, Texas Heart Institute of St. Luke’s Episcopal and Texas Children’s Hospitals, Houston, Tex. Accepted for publication Sept. 27, 1974. Address reprint requests to Dr. Cooley, Texas Heart Institute, P.O. Box 20345, Houston, Tex. 77025.
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Aneurysm and Fistula of the Sinus of Valsalva
accounting for a total of 52 instances of aneurysmal or ruptured sinus of Valsalva in 45 patients (Table 1). In the group of 22 patients whose aneurysms had not ruptured, 3 had two dilated aortic sinuses and 2 had three dilated sinuses. A fistula was present in 23 patients, either as part of a ruptured aneurysm or as a simple fistulous tract without aneurysmal dilatation. Communication between an aortic sinus and a cardiac chamber occurred as follows: sixteen times to the right ventricle, four times to the right atrium, once to the left ventricle, and once to the left atrium; one perforation occurred into the pericardium (Fig. 1). In 10 patients there was a reported history of rheumatic fever and in 17 patients an episode of bacterial endocarditis. Twenty-five patients were believed to have a congenital aneurysm oE the aortic sinus. One patient suffered from systemic lupus erythematosus, and in another the fistula was probably iatrogenic following cardiac catheterization for aortic valve disease caused by rheumatic fever. Six of the 25 patients with congenital lesions had typical Marfan’s syndrome. All 45 patients had associated anomalies. In 32 patients some pathological involvement of the aortic valve was present: 5 had combined aortic stenosis and aortic insufficiency, 2 had isolated aortic stenosis, and 25 had moderate to severe aortic insufficiency. Mitral insufficiency was present in 4 patients, ventricular septa1 defect in 5, and infundibular pulmonary stenosis in 3. One patient had an atrioventricular canal. According to the Functional Classification of the New York Heart Association, only 4 patients were asymptomatic at the time of operation: 23 were in Class 11, 11 were in Class 111, and 7 were in Class IV. Easy fatigability, shortness of breath, chest pain, and palpitations were the most common subjective symptoms. Sudden onset of manifestations was reported by 8 patients, all of whom had a ruptured aneurysm; in 5 the rupture had occurred during or following an episode of bacterial endocarditis. TABLE 1. ANATOMY OF ANEURYSM OR FISTULA OF T H E SINUS OF VAISALVA IN 45 PATIENTS
Site of
Rupture Not ruptured
Ruptured into right ventricle Ruptured into right atrium Ruptured into left atrium Ruptured into left ventricle Ruptured into pericardium Total
Right Coronary Sinus
Left Coronary Sinus
10 16
...
...
1
Noncoronary Sinus
Total
14
29
...
3
4
...
...
1
1
1
...
...
1
...
...
1
1
28
5
19
52
5
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FIG. 1. Origin and entry of
fi-
tulous communication; nuhbers indicate numbers of patients. (NCS = noncoronary sinus; RCS = right coronary sinus; LCS = left coron a y sinus.)
A “machinery type” murmur along the left sternal border was heard in only 9 patients, all of whom had a fistula from the aorta to the right ventricle. Cardiac enlargement was evident on chest roentgenogram in 25 patients and was most pronounced when associated with aortic insufficiency. Electrocardiogram revealed left ventricular hypertrophy in 17 patients and some form of conduction defect or arrhythmia in 12. Cardiac catheterization was performed in 36 patients and angiography in 28, with visualization of the aneurysm or fistula in 16. In 2 patients the catheter passed directly through the fistulous tract. A correct preoperative diagnosis of aneurysm or fistula, or both, of the sinus of Valsalva was made in 30 patients. Involvement of a sinus of Valsalva was an intraoperative finding in 15 patients. Comparison of the preoperative diagnosis and actual operative findings in these 15 patients is shown in Table 2.
Operatiue Techniques All patients but 1 underwent operation using temporary cardiopulmonary bypass and, since 1972, moderate hypothermia (30°C;. T h e defect was corrected in 44 patients. An exploratory thoracotomy alone was performed in 1 patient. This last patient underwent operation in 1965 and had a densely calcified aneurysm of the left coronary sinus of Valsalva that partially occluded the left main coronary artery; the aneurysm could not be repaired. T h e standard incision was a median sternotomy. Arterial cannulation was carried out through the right femoral artery in 32 patients
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THE ANNALS OF THORACIC SURGERY
Aneurysm and Fistula of the Sinus of Valsalua TABLE 2. PREOPERATIVE DIAGNOSIS COMPARED W I T H OPERATIVE FINDINGS IN 15 PATIENTS W I T H ANEURYSM OR FISTULA OF T H E SINUS OF VALSALVA
Patient’s Age (yr) Year of & Sex Operation
Preoperative Diagnosis
Operative Findings
F M M M M F
1956 1957 1959 1960 1960 1961
VSD IPS & left-to-right shunt VSD VSD A1 & VSD Tetralogy of Fallot
36, M 61, F
1961 1964
31, M 27, M
1965 1966
51, M 2, M
1966 1967
52, M
1968
20, M
1969
VSD or CA-to-RV fistula AS & A1 with left-to-right shunt VSD & A1 Massive AI, acute endocarditis, no cardiac cath. (Class IV) A1 Severe A1 (112 mm Hg gradient) Severe AI, rapidly progressive CHF, no cardiac cath. (Class I11 to IV) VSD, AI, AV canal
43, F
1969
18, 38, 17, 22, 34, 31,
Acute bacterial endocarditis, AS, AI, & MI
RC sinus-RV fistula RC sinus-RV fistula & IPS RC sinus-RV fistula RC sinus-RV fistula RC sinus-RV fistula Tetralogy & RC sinus-RV fistula VSD & RC sinus-RV fistula AS & AI; iatrogenic noncoronary sinus-LA fistula RC sinus-RV fistula Massive A1 with destroyed valve, aneurysm of noncoronary sinus AI, aneurysm of RC sinus Severe AS & aneurysm of noncoronary sinus A1 due to annular dilatation by aneurysm of RC sinus VSD, AI, AV canal, & non-
coronary sinus aneurysm AS & MI, noncoronary sinus aneurysm with abscess & perforation into pericardium
RC = right coronary artery; RV = right ventricle; VSD = ventricular septa1 defect; IPS = infundibular pulmonic stenosis; A1 = aortic insufficiency; AS = aortic stenosis; LA = left atrium; AV = atrioventricular; MI = mitral insufficiency; CHF = congestive heart failure; CA = coronary artery.
and in the distal ascending aorta in the last 12. A sump suction was placed through either the apex in the left ventricle (before 1967) or the right superior pulmonary vein in the left atrium (after 19G7). Of the 22 nonruptured aneurysms, 18 were totally resected. Aortic continuity was obtained by direct suture in 4 patients and through insertion of a Dacron patch in 11 (Fig. 2); direct closure was accomplished with interrupted simple or mattress sutures buttressed with felt when necessary. Three patients who had one or more massive aneurysms of the aortic sinus also had an aneurysm of the ascending aorta. Aneurysms of the sinus of Valsalva were resected together with the ascending aorta and replaced with a Dacron tube graft. Both coronary arteries were implanted in the tube graft in 2 patients (Fig. 3). I n another patient only the right coronary artery was reimplanted in the tube graft; the left coronary ostium with its rim of aortic wall was left attached to the aortic annulus. I n 3 patients the aneurysm was left in place and the aortic opening was closed with a Dacron patch. In 1 other patient a small aneurysmal dilatation
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associated with severe aortic stenosis and insufficiency was closed during implantation of a prosthetic aortic valve. Of the 23 patients with fistulas of the sinus of Valsalva, 21 underwent repair by direct closure and 2 by use of a Dacron patch. I n 10 patients a double approach-through the aorta and the involved cardiac chamber-was used, most frequently after 1965. A right ventriculotomy was performed in 13 patients and a right atriotomy in 3. Operative treatment of associated anomalies is shown in Table 3. Among 30 patients with aortic insufficiency, 24 underwent aortic valve replacement and 3 had aortic valvotomy (all in 1960). In the remaining 3 patients aortic insufficiency was not hemodynamically significant. I n 2 patients isolated stenosis was corrected by aortic valvotomy. An associated ventricular septa1 defect was closed with mattress sutures in 2 patients and with a Dacron patch in 3. In 1 patient with associated atrioventricular canal and cleft mitral valve the operative procedure consisted of resection and Dacron patch closure of a nonruptured aneurysm of the noncoronary sinus, direct closure of the mitral cleft, and Dacron patch repair of the atrioventricular canal. One patient underwent mitral replacement, 1 had mitral annuloplasty, and another had pulmonary infundibulectomy.
FIG. 2. Resection of nonruptured sinus of Valsalva aneurysm (a) and surgical repair by ( b ) direct closure or (c) insertion of a Dacron patch graft.
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Aneurysm and Fistula of the Sinus of Valsalva
Operative Results Of the 45 patients, 44 survived the operative procedure and 37 experienced an uneventful postoperative course. The average time of hospitalization was 15.1 days. Early postoperative complications necessitated four operations. In 2 patients reoperation was performed early for evacuation of a hemothorax followed by both patients' complete recovery. One patient, operated on in 1964 for aortic valve disease with an aneurysm of the noncoronary sinus, underwent a second operation nine days after the first procedure because of recurrence of the aneurysm and aortic insufficiency. A larger artificial valve (Magovern No. 4) was implanted and the recurrent aneurysm was repaired. He did well for about five months, then died because of formation of a massive thrombus on the artificial valve that had partially occluded both coronary arteries. The fourth patient who
MEYER ET AL. required reoperation suffered from Marfan’s syndrome with right coronary sinus aneurysm. T h e aneurysm was resected and repaired with a Dacron patch. On the second postoperative day the patient developed noticeable aortic valve insufficiency which led to reoperation and replacement of the aortic valve thirteen days after the first procedure. This patient also had an aneurysm of the aortic arch which was not resected and led to his sudden death at home three weeks later because of acute dissection and rupture into the esophagus. T h e patient who died immediately after operation in 1963 also had Marfan’s syndrome. A fistula from the aorta to the right atrium was closed through a right atriotomy with interrupted mattress sutures, and he died on the first day after operation of pulmonary edema caused by recurrence of the fistula with rupture of most of the stitches due to poor tissue. Microscopical examination revealed complete absence of elastic tissue in the wall of the aortic sinus. Other immediate postoperative complications included atrial arrhythmia (2 patients) and urinary tract infection (1 patient). T h e patient with a sinus of Valsalva aneurysm that had ruptured into the pericardium was treated by aortic valve replacement and direct closure of the opening of the fistula. T h e patient had a completely uneventful postoperative course and was doing well 2 years after operation. Late follow-up data included information on 38 patients for a period of 1 to 15 years with an average follow-up of 4 years. Three patients were lost to follow-up and 4 died during the first postoperative year: 1 patient immediately after operation, 1 after three weeks due to rupture of a thoracic aneurysm, 1 after five months from thrombosis of an artificial valve, and 1 TABLE 9. ASSOCIATED ANOMALIES AND OPERATIVE TECHNIQUES USED ~
~
Anomalies and Techniques Aortic insufficiency Aortic valve replacement Aortic valvotomy Isolated aortic stenosis Aortic valvotomy VSD Direct suture Dacron patch Mitral insufficiency Mitral annuloplasty Mitral valve replacement Infundibular pulmonic stenosis Pulmonary infundibulectomy Atrioventricular canal Dacron patch Total
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No. of Patients 24 3
2 2 3 1 1
1 1 38
30 2
5 4
3 1
45
Aneurysm and Fistula of the Sinus of Valsalva TABLE 4. CAUSE OF LATE DEATH IN 5 PATIENTS WHO UNDERWENT SURGICAL CORRECTION OF A FISTULA OF T H E SINUS OF VALSALVA ~~
Patient’s Age (yr) Year of & Sex Operation ~~
Year of Death
Interval (yr)
Operative Procedure
Cause of Death
Closure RC sinusRV fistula Closure RC sinusRC fistula, right ventriculotomy Suture of noncoronary sinus, fistula to LA, AVR AVR, mitral annuloplasty Excision of noncoronary sinus aneurysm & direct closure, AVR (SmeloffCutter)
Bacterial endocarditis Unknown
18, F
1956
1968
12
34, M
1960
1962
2
67, F
1964
1969
5
61, M
1966
1968
2
54, M
1967
1968
1
Stroke, CVI
CAOD Massive cerebral embolus
RC =right coronary; RV = right ventricle; AVR = aortic valve replacement; LA = left atrium; CVI = cerebrovascular insufficiency; CAOD = coronary artery occlusive disease.
after six months from systemic lupus erythematosus. T h e first patient in our series who underwent successful correction of a fistula from the right coronary sinus to the right ventricle in 1956 died 12 years later from a fulminating vegetative endocarditis. T o date, 4 other deaths have been recorded (Table 4).
Comment Aneurysms and fistulas of the sinus of Valsalva are rare and represent only 0.43y0 of the procedures using cardiopulmonary bypass performed at our institutions between April, 1956, and September, 1974. As reported in other series, most of our patients were young adult men [l, 3, 71. T h e right coronary sinus was most frequently involved (64y0in our series), followed by the noncoronary sinus. Fistulas or aneurysms of the left coronary sinus are rare. A perforation or fistula connected most frequently with the right ventricle and rarely with the right atrium, the proportion being 4 to 1. A fistula into the left atrium, left ventricle, or pericardium was the exception. A review of our series and those of others [l-31 does not provide a definitive etiology for this anomaly. There is strong indication that the aneurysm was congenital in origin in more than half of the patients in this series. This impression was drawn from the history, operative findings, and histological examination. T h e medical history of 12 patients supported an
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infectious etiology: in 5 an episode of bacterial endocarditis was responsible for the perforation of what we believed to be a preexisting congenital aneurysm of the aortic sinus. Most patients in our series were symptomatic, but in view of the frequency of associated anomalies this finding does not appear to be of specific value. T h e characteristic to-and-fro, machinery type murmur was heard less often in our series than in others [3, 51 and appears to characterize a fistula from the aorta to the right ventricle. Roentgenograms of the chest and electrocardiograms were not particularly valuable in our series. At the present time it should be possible to obtain a correct diagnosis in most of these patients with the help of cardiac catheterization and cineangiocardiography; however, two possible diagnostic difficulties remain: (1) the differential diagnosis between a fistula connecting the right coronary sinus to the right ventricle and a high ventricular septa1 defect; and (2) diagnosis of an asymptomatic small- to moderate-sized aneurysm of the noncoronary sinus of Valsalva. An operative classification of sinus of Valsalva lesions must include the following three entities: (1) nonruptured aneurysm of the aortic sinus of Valsalva, (2) aneurysm of the aortic sinus with intracardiac or extracardiac rupture, and (3) intracardiac or extracardiac fistula of the sinus of Valsalva. An unruptured aneurysm of the aortic sinus can easily remain undiagnosed unless it is associated with a hemodynamically significant cardiac lesion (most frequently involving the aortic valve), is sufficiently large to be noticed on a chest roentgenogram, or produces symptoms through compression of the right atrium or right ventricle. In our series an associated cardiac lesion whose hemodynamic properties indicated the need for operation was found in 21 of the 22 patients with nonruptured sinus of Valsalva aneurysms. We agree with Howard and colleagues [3] that patients with isolated nonruptured aneurysms of the sinus of Valsalva should be followed closely rather than operated upon. T h e 23 patients in our series who had perforated aneurysms or fistulas included 10 with windsock, or balloon type, aneurysms with the perforation at the tip of the aneurysm and 13 with a simple fistulous communication without aneurysmal dilatation. This differentiation is important because of the technical implications. Resection of a perforated aneurysm is a more difficult procedure, often necessitating a patch repair, while a simple fistulous tract without aneurysm can often be closed directly with a few interrupted mattress sutures. A ruptured aneurysm or fistula of the aortic sinus always constitutes an indication for operation since it imposes the constant strain of a left-to-right shunt and may cause acute pulmonary edema through a sudden increase in the diameter of the communication. With today’s advanced techniques in anesthesiology and cardiopulmonary bypass and with the feasibility of
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Aneurysm and Fistula of the Sinus of Valsalva aggressive aortic root operations using Dacron tube grafts, prosthetic aortic valves, and aortocoronary saphenous vein bypass grafts [4, 61, it should be possible to resect and replace most aneurysms of the sinus of Valsalva successfully.
References 1. Bonfils-Roberts, E. A., DuShane, J. W., McGoon, D. C., and Danielson, G. K.
2. 3.
4.
5. 6.
7.
Aortic sinus fistula: Surgical considerations and results of operation. Ann Thorac Surg 12:492, 1971. Chapman, D. W., Beazley, H. L., Peterson, P. K., Webb, J. A., and Cooley, D. A. Annulo-aortic ectasia with cystic medial necrosis. Am J Cardiol 16:679, 1965. Howard, R. J., Moller, J., Castaneda, A. R., Varco, R. L., and Nicoloff, D. M. Surgical correction of sinus of Valsalva aneurysm. J Thorac Cardiovasc Surg 66:420, 1973. Meyer, J., and Cooley, D. A. Surgical management of patients with pathological changes involving the aortic root: Review of present techniques and proposal of a modified surgical approach. Cardiovasc Dis (Bull Tex Heart Znst) l:lOl, 1974. Morgan, J. R., Rogers, A. K., and Fosburg, R. G. Ruptured aneurysms of the sinus of Valsalva. Chest 61:640, 1972. Najafi, H. Aneurysm of cystic medionecrotic aortic root: A modified surgical approach. J Thorac Cardiounsc Surg 66:71, 1973. Sawyers, J. L., Adams, J. E., and Scott, H. W. Surgical treatment for aneurysms of the aortic sinuses with aorticoatrial fistula. Surgery 41 :26, 1957.
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