Cardiology 60: 129-139 (1975)
Ruptured Aneurysms of the Aortic Sinus of Valsalva B o o n -L ock C h ia , N g o h -C huan T an , C h in -H ock L im , L enny K h e n g -A nn T a n , C harles C. S .T o h and A. J o h a n 1 Dcpartmcnl of Medicine, University of Singapore; Cardio-Thoracic Unit, Tan Tock Seng Hospital; Department of Radiology, and Medical Unit III, Outram Road General Hospital, Singapore
Key Words. Aneurysm of the sinus of Valsalva • Supracristal ventricular septal defect • Right coronary sinus aneurysm Abstract. Aneurysms of the aortic sinus of Valsalva (ASV) are uncommon. This study describes eight cases of ruptured aneurysms of the congenital variety observed over a 10-year period in Singapore. Although ASV is classically diagnosed at the time of rupture, only one patient in this scries presented with acute chest pain and dyspnea. All eight patients had continuous murmurs. The clinical diagnosis was confirmed by cardiac catheterization and angiography in eight patients and by thoracotomy in six. In six patients, the aneurysm had ruptured into the right ventricle and in two into the right atrium. In the five patients where the site of the aneurysm could be definitely determined, four arose from the right coronary and one from the noncoronary sinus. This marked preponderance of right coronary sinus over noncoronary sinus aneurysm in Singapore is more in accordance with the Japanese rather than the Western ex perience. A systolic gradient due to the aneurysm pressing on the right ventricular outflow tract was established in four patients during cardiac catheterization and angiography and confirmed in three patients at thoracotomy. Six patients underwent operation and were alive and well from 7 months to 3 years later.
Aneurysms of the aortic sinus of Valsalva (ASV) are uncommonly encountered. We describe here eight cases observed at the Cardiovascular Laboratory of the Outram Road General Hospital, Singapore, over the last 10 years. This is the only cardiovascular diagnostic center in this island state of 2.5 million people. During this period, a total of about 1,800 cardiac catheterizations were carried out for both congenital and acquired heart diseases in patients of all ages.
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1 The authors would like to thank Prof. C. S. Sf.ah and Prof. P. K. W ong for permission to publish cases from Medical Units III and I.
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Table 1 Symptoms
Thrills Collap Blood Apex beat sing LV+ RV+ syst. diast. si le pulse mm Hg Site
Murmurs character
Clinical diagnosis
site of max imum intensity
F
19
Chinese
progressive dyspnea
yes
150/50 5th LICS
no
no
yes
yes
2nd, 3rd and 4th LICSP
contin- 2nd, 3rd uous and 4th LICSP
RASV
2
M
23
Chinese
dyspnea
yes
120/30 6th LICS
-H-
no
yes
yes
2nd and 3rd LICSP
contin- 2nd and uous 3rd LICSP
7RASV ?VSD and AI
3
F
24
Chinese
dyspnea, edema of legs
yes
120/40 5th LICS
+
+
yes
no
4th, 5th contin- 4th, 5th LICSP uous RICSP and RICSP and LICSP
?VSD and AI
4
M
41
Indian
palpitations, no dyspnea
130/70 6th LICS
+
no
yes
no
5
M
33
Indian
sudden chest yes pain followed1 by dyspnea
115/50 not palpable
-
-
no
no
2nd and 3rd LICSP
7RASV
contin- 2nd and uous 3rd LICSP
?PDA 7RASV
contin- 2nd and uous 3rd LICSP
7PDA 7RASV
C hia/T an/L im/T an/T oh/J ohan
1
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Case Sex Age Race
6
M
24
Chinese
palpitations, yes dyspnea
115/40 5th LICS
+
yes
yes
3rd and 4th LICSP
contin- 3rd and uous 4th LICSP
?VSD and AI ?RASV
7
M
32
Chinese
dyspnea and yes tiredness
120/60 5th LICS
no
yes
no
4th and 5th LICSP
contin- 4th and uous 5th LICSP
7RASV ?VSD and AI
8
M
28
Chinese
dyspnea
140/40 5th LICS
no
no
no
4th and contin5th uous LICSP and R1CSP
yes
7RASV Aortic Sinus Aneurysms
5:h LICSP and RICSP
LICS = Left intercostal space; LICSP = left intercostal space parasternally; RICSP = right intercostal space parasternally; RASV = ruptured aneurysm sinus of Valsalva; VSD and AI = ventricular septal defect and aortic incompetence; PDA = patent ductus arteriosus. Downloaded by: King's College London 137.73.144.138 - 1/16/2019 2:28:35 AM
U>
C hia/T an/L im/T an/T oh/J ohan
132
Table II
Plethora
Case No.
C/T ratio Vo
RA
PA
AK
1 2 3 4 5 6 7 8
52 64 59 68 54 57 57 60
_
_
_
—
-
++ +
+ + —
+ ++ + + ++ + +
++ ++ + -
-
++ ++
C /T ratio = Cardiothoracic ratio; RA - right atrium; PA = pulmonary artery; AK = aortic knuckle
Table III Case No.
Rhythm
Mean frontal plane axis
LVH
RVH
Conduction defect
1 2 3 4 5 6 7 8
SR SR SR SR SR SR SR SR
+90° +65° +90° +45° +20° +90° +90° +30°
no yes no yes yes yes yes
no yes no no no no no
no no no complete RBBB no no no complete RBBB
SR Sinus rhythm; LVH left ventricular hypertrophy; RVH = right ventricular hypertrophy; RBBB = right bundle branch block.
The clinical, radiological, angiographic and electrocardiographic find ings and the hemodynamic data of the eight patients are summarized in tables I-IV. Six patients underwent thoracotomy and table V summarizes the operative findings, the surgical procedures and the outcome.
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Material and Results
Aortic Sinus Aneurysms
133
Table IV Case No.
Site of
Pressures, mm Hg
QP/QS
RA RV (mean)
PA
PeP
Ao
in oxygen saturation
1
9
85/18
54/30
13
137/67
RV
1.5:1
2
12
106/18
50/20
17
106/48
RV
2.0:1
3 4 5 6 7
7 It)
45/25 18/9
20 8
120/50 118/63
32/12 25/10
12 8
120/55 140/65
RA RV RV RV RV
4.0:1 1.5:1
6 5
45/5 44/11 38/10 45/5 65/5
8
12
40/10
36/16
14
120'60
RA
1.9:1
5.0:1 3.2:1
Aortogram
communication aorta to RV outflow tract large aneurysm RCS to RV outflow tract communication aorta to RA huge aneurysm RCS to RV communication aorta to RV communication aorta to RV aneurysm RCS to RV outflow tract aneurysm rupturing into RA
In 6 of the 8 patients who underwent thoracotomy the site of the aneurysm was in the right coronary sinus in 3 (cases 2, 4 and 6), in the noncoronary sinus in I (case 3), and unspecified in 2 (cases 1 and 5). In two patients only there was an associated ventricular septal defect, situated in the supracristal region in case 1 and in the infracristal region in case 6. For the two patients who did not undergo thoracotomy, aortography revealed that the aneurysm arose from the right coronary sinus in one case (case 7), but in the other (case 8) the site of origin of the aneurysm could not be determined from the aortogram. Hence, in 5 out of the 8 patients in this series, the site of the aneurysm could definitely be determined. In four patients the right coronary sinus was involved and in one the non coronary sinus. Classically, aneurysms of the sinus of Valsalva present with an acute history of chest pain, dyspnea or palpitations at the time of rupture. How ever, this is not invariable, as pointed out previously by various authors [O ram and E ast , 1955; W r ig h t , 1970], L ockha rt er al. [1964] stated that of 71 cases reviewed, only 37'Vo gave a history of abrupt onset. In this series of eight patients, only one (case 5, 12"/o) was acute, experiencing
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RA = Right atrium; RV = Right ventricle; PA = pulmonary artery; Pep = pulmonary capillary wedge; Ao = aorta; QP/QS = pulmonary blood flow/systemic blood flow; RCS = right coronary sinus.
134
C MIAA"An /L im /T an /T oh /J oh an
Table V
‘Wind sock' type of sinus of Valsalva aneurysm measuring 1 cm in diameter, rupturing into RV outflow tract just below pulmonary valve
associated lesions
Surgical procedure done
Results
Follow-up
VSD type I (supracristal) 1 cm in diameter
aneurysm ex cised; defect closed with Teflon felt; VSD closed
recovery
1 year later, symptomati cally well; no murmurs heard
aneurysm ex cised; defect closed with pericardium and graft
recovery
9 months later, well; no mur murs
fistula repaired with inter rupted sutures
recovery
2.5 years later, symptomati cally well; no murmurs
recovery aneurysm ex cised fistula re paired with Teflon felt
7 months later, asymptomatic; no murmurs
Right coronary sinus aneurysm 2.0 cm in diameter, rupturing into RV outflow tract
Middle third of noncoronary sinus of Valsalva ruptured into right atrium; fistula measured 1.0 cm in diameter
-
Hugh right coronary sinus aneurysm rupturing into right ventricle; ventricular aneurysm seen in posterior surface of left ventricle (incidental finding)
‘Wind sock' type sinus of Valsalva aneurysm rupturing into right ventricular outflow tract
-
aneurysm ex cised; defect repaired
Aneurysm of the right coronary sinus (1.5 x 1.0 cm) rupturing into RV outflow tract
small VSD type II (infracristal)
aneurysm ex recovery cised; fistula re paired with Teflon felt
recovery
3 years later, well
1 year 4 months later, well; no murmurs
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Case Operative findings No. ----------------------------------------description of aneurysm
Aortic Sinus Aneurysms
135
sudden chest pain followed soon after by progressive dyspnea. This patient and case 3 are the only 2 out of the 6 patients who at thoracotomy showed neither a ventricular septal defect nor a right ventricular outflow tract obstruction. Of the 2 patients who did not undergo operation, 1 (case 7) showed a right ventricular outflow tract obstruction of 40 mm at cardiac catheterization. This tends to support the belief that a preexisting pulmonary stenosis or ventricular septal defect minimizes the hemodynamic disturbances at the time of rupture of the aneurysm. Cardiac catheterization and angiography are necessary for confirmation of the diagnosis and to distinguish this condition from coronary arterio venous fistula or a ventricular septal defect with aortic incompetence. Oxygen sampling localized the site of shunt at the right ventricular level in 6 of our 8 patients and at the right atrial level in 2 (table IV). The pulmonary blood flow to systemic blood flow ratio varied from 1.5:1 to 5.0:1 (mean 2.7:1). A significant right ventricular outflow tract systolic gradient was seen in four patients, varying from 26 to 56 mm Hg. In three (cases 1, 2 and 4) the gradient was shown at thoracotomy to be due to the aneurysm pressing on the right ventricular outflow tract. This obstruction was clearly demonstrated before operation in the angiographicstudies of case 2. In this patient the aortogram revealed a huge aneurysm of the right sinus of Valsalva rupturing into the right ventricle. The right ventriculogram demonstrated that this aneurysm had compressed the right ventricular outflow tract causing a large filling defect (fig. I). In the remaining seven cases, aortography confirmed a communication between the aorta and the right ventricle in five patients (cases 1, 4—7) (fig. 2) and the aorta and the right atrium in two patients (cases 3 and 8) (fig. 3).
Discussion
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Aneurysms of the sinus of Valsalva are most commonly congenital, but may be associated with the Marfan and Ehlers-Danlos syndrome [H u d s o n , 1965], They can also be due to syphilis or infective endocarditis. All eight patients in this series were considered to have congenital aneurysms. Most of the cases of ruptured ASV were reported from Western coun tries [W r ig h t , 1970] except for isolated cases in India [R aman and M e n o n , 1949], Hongkong [B a r n es , 1968], Korea [H o n g et al., 1966], Thailand [K och a sen i et al., 1971], and two large series in Japan [S akaki-
136
C hia/T an/L im /T anAToh/J ohan
ba ra and K o n n o , 1962a; T a g u ch i ei at., 1969]. As far as we know, this is the largest documented study in the East apart from the two Japanese series. Differences were noted between the Japanese and the Western series. Based on studies from Europe and North America [B esterm a n et at., 1963; M a g id so n and K ay , 1963; A brams and E van s , 1964; P erasaeo et at., 1966], W r ig h t [1970] concluded that in the West aneurysms in volving the right coronary sinus of Valsalva are about twice as frequent as those involving the noncoronary sinus. This preponderance is, however, much greater in Japan. Sakakibara and K o nn o [1962a] found that of the cases documented in Japan up to 1962, 19 had involved the right coronary and only one the noncoronary sinus. In 1969, T a g u ch i et at. reported on a series of 45 patients operated by them in Hiroshima, Japan. They found
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Fig. I. Right ventriculogram of case 2 (lateral projection), showing a filling defect (arrow) caused by the aneurysm of the right sinus of Valsalva bulging on and com pressing the right ventricular outflow tract. Fig. 2 Aortogram of case 1 (lateral projection), showing reflux of contrast dye from aorta to right ventricle.
Aortic Sinus Aneurysms
137
Fig. 3. X-ray film obtained by subtraction technique. Aortogram of case 8 (antero posterior projection), showing aneurysm of the sinus of Valsalva (lower arrow) rupturing into the right atrium (upper arrow).
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that the right coronary sinus was involved in 89 %> and the noncoronary sinus in about 4°/o of the patients. This marked preponderance of right coronary over noncoronary sinus aneurysms and the corresponding rarity of noncoronary sinus aneurysms are linked with the relatively high in cidence in Japan of aneurysms of the anterior portion of the right coronary sinus, which are very frequently associated with a supracristal ventricular septal defect [W r ig h t , 1970]. Sakakibara and K c n n o [1962a] believe that the associated ventricular septal defect originates in a common con genital point of weakness with the right coronary sinus aneurysm. Although the present series is too small to enable any final conclusions, it neverthe less appears that the preponderance (4:1) of right coronary sinus aneu rysms over noncoronary sinus aneurysms in Singapore resembles more the Japanese than the Western experiences. Although systolic murmurs alone were documented in ruptured ASV [H o n g et al., 1966], the typical auscultatory findings was a continuous murmur. The character of this murmur may be modified by the presence of either a ventricular septal defect or aortic incompetence. Hence, the continuous murmur in ruptured ASV may be accentuated in either systole, diastole or both. Some authors do not consider the murmur.of ruptured ASV to be continuous although it extends throughout the cardiac cycle
C hia/T an/L im/T an/T oh/J ohan
138
and K o n n o , 1962b; F r ie d b e r g , 1966], This is because, un like the classical Gibson’s murmur of a patent ductus arteriosus, the maximal loudness of the murmur in ruptured ASV is not at the second heart sound of the cardiac cycle. However, we feel that this difficulty in description is largely a matter of semantics. In all eight patients of this series, clinical auscultation of the precordium revealed a long murmur which extended throughout systole and diastole and which we considered to be continuous. In six patients this murmur was loudest at either the second, third, fourth or fifth left intercostal spaces parasternally (table 1). In all these patients, the aneurysm had ruptured into the right ventricle. In the remaining two patients, where the aneurysm had ruptured into the right atrium, the continuous murmurs were loudest over the fourth and fifth intercostal spaces on both sides of the sternum. S akakibara and K o n n o [1962b] have suggested that when the aneurysm ruptures into the right atrium, the direction of the blood flow tends to project the murmur to the lower part or to the right of the sternum. The prognosis of patients with ruptured ASV, treated conservatively, depends largely on the size of the fistula, but it is generally poor. Surgical closure of the ruptured ASV under cardiopulmonary bypass is the treat ment of choice [S akakibara and K o n n o , 1963]. All six of our patients survived the operation and were found to be in good health when examined 7 months to 3 years later. [S akakibara
References A brams, L. D. and E vans, D. W.: Ruptured aortic sinus aneurysm. Q. Jl Med. 33:
285-295 (1964).
ruptured aneurysm of the aortic sinus. J. thorac. cardiovasc. Surg. 51: 507-514 (1966). H udson . R. E. B.: Cardiovascular pathology (Arnold, London 1965). K ochanseni, S. E.; H araphonages, M.; W ong T hongari, W„ and C hareonchob, E.: Ruptured aneurysm of sinus of Valsalva. J. med. Ass. Thailand 54: 203-212 (1971). Lockhart, A.; Scebat, L., and L enegre , J. (1964): Cited by W right (1970). M agidson, O. and K ay, J. H.: Ruptured aortic sinus aneurysm. Am. Heart J. 65: 597-606 (1963).
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Barnes, R. J.: Rupture sinus of Valsalva. Br. med. J. i: 683-684 (1968). Besterman. E.-. G oldberg , M., and Sellors , T.: Surgical repair of ruptured sinus of Valsalva. Br. med. J. if: 410-416 ( 1963). F riedberg , C. K.: Diseases of the heart (Saunders, Philadelphia 1966). H ong , P. W.: Lee, S. S.; K im, S. W., and C ha, H. D.: Unusual manifestations of
Aortic Sinus Aneurysms
139
O ram, S. and E ast, T.: Rupture of aneurysm of aortic sinus (of Valsalva) into the
right side of the heart. Br. Heart J. 17: 541-551 (1955). P erasalo, O.: H ai.onen , P. I.; Kyixonen , K. E„ and H eikkila, K. F.: Ruptured
aneurysm of the sinus of Valsalva. Acta chir. scand., suppl. 356B, pp. 55-64 (1966). R aman, T. K. and M enon . T. B.: Aneurysms of the sinuses of Valsalva. Indian Heart
J. I: 1-14(1949). Sakakibara. S. and Konno . S.: Congenital aneurysms of the sinus of Valsalva, anat
omy and classification. Am. Heart J. 63: 405-422 (1962a). Sakakibara, S. and Kon .no. S.: Congenital aneurysms of the sinus of Valsalva. A
clinical study. Am. Heart J. 63: 708-719 (1962b). T aguchi, K.: Sasaki, N.; Matsuura, V„ and U emura, R.: Surgical correction of
aneurysm of the sinus of Valsalva. Am. J. Cardiol. 23: 180-191 (1969). W right , J. S.: Ruptured aneurysm of the sinus of Valsalva. Q. Jl Med. 39: 501-513
(1970).
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Dr. B.-L. C hia. Department of Medicine. General Hospital. Sepoy Line, Singapore 3 (Malaysia)
Ruptured Aneurysms of the Aortic Sinus of Valsalva B o o n -L ock C h ia , N g o h -C huan T an , C h in -H ock L im , L enny K h e n g -A nn T a n , C harles C. S .T o h and A. J o h a n 1 Dcpartmcnl of Medicine, University of Singapore; Cardio-Thoracic Unit, Tan Tock Seng Hospital; Department of Radiology, and Medical Unit III, Outram Road General Hospital, Singapore
Key Words. Aneurysm of the sinus of Valsalva • Supracristal ventricular septal defect • Right coronary sinus aneurysm Abstract. Aneurysms of the aortic sinus of Valsalva (ASV) are uncommon. This study describes eight cases of ruptured aneurysms of the congenital variety observed over a 10-year period in Singapore. Although ASV is classically diagnosed at the time of rupture, only one patient in this scries presented with acute chest pain and dyspnea. All eight patients had continuous murmurs. The clinical diagnosis was confirmed by cardiac catheterization and angiography in eight patients and by thoracotomy in six. In six patients, the aneurysm had ruptured into the right ventricle and in two into the right atrium. In the five patients where the site of the aneurysm could be definitely determined, four arose from the right coronary and one from the noncoronary sinus. This marked preponderance of right coronary sinus over noncoronary sinus aneurysm in Singapore is more in accordance with the Japanese rather than the Western ex perience. A systolic gradient due to the aneurysm pressing on the right ventricular outflow tract was established in four patients during cardiac catheterization and angiography and confirmed in three patients at thoracotomy. Six patients underwent operation and were alive and well from 7 months to 3 years later.
Aneurysms of the aortic sinus of Valsalva (ASV) are uncommonly encountered. We describe here eight cases observed at the Cardiovascular Laboratory of the Outram Road General Hospital, Singapore, over the last 10 years. This is the only cardiovascular diagnostic center in this island state of 2.5 million people. During this period, a total of about 1,800 cardiac catheterizations were carried out for both congenital and acquired heart diseases in patients of all ages.
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1 The authors would like to thank Prof. C. S. Sf.ah and Prof. P. K. W ong for permission to publish cases from Medical Units III and I.
U» O
Table 1 Symptoms
Thrills Collap Blood Apex beat sing LV+ RV+ syst. diast. si le pulse mm Hg Site
Murmurs character
Clinical diagnosis
site of max imum intensity
F
19
Chinese
progressive dyspnea
yes
150/50 5th LICS
no
no
yes
yes
2nd, 3rd and 4th LICSP
contin- 2nd, 3rd uous and 4th LICSP
RASV
2
M
23
Chinese
dyspnea
yes
120/30 6th LICS
-H-
no
yes
yes
2nd and 3rd LICSP
contin- 2nd and uous 3rd LICSP
7RASV ?VSD and AI
3
F
24
Chinese
dyspnea, edema of legs
yes
120/40 5th LICS
+
+
yes
no
4th, 5th contin- 4th, 5th LICSP uous RICSP and RICSP and LICSP
?VSD and AI
4
M
41
Indian
palpitations, no dyspnea
130/70 6th LICS
+
no
yes
no
5
M
33
Indian
sudden chest yes pain followed1 by dyspnea
115/50 not palpable
-
-
no
no
2nd and 3rd LICSP
7RASV
contin- 2nd and uous 3rd LICSP
?PDA 7RASV
contin- 2nd and uous 3rd LICSP
7PDA 7RASV
C hia/T an/L im/T an/T oh/J ohan
1
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Case Sex Age Race
6
M
24
Chinese
palpitations, yes dyspnea
115/40 5th LICS
+
yes
yes
3rd and 4th LICSP
contin- 3rd and uous 4th LICSP
?VSD and AI ?RASV
7
M
32
Chinese
dyspnea and yes tiredness
120/60 5th LICS
no
yes
no
4th and 5th LICSP
contin- 4th and uous 5th LICSP
7RASV ?VSD and AI
8
M
28
Chinese
dyspnea
140/40 5th LICS
no
no
no
4th and contin5th uous LICSP and R1CSP
yes
7RASV Aortic Sinus Aneurysms
5:h LICSP and RICSP
LICS = Left intercostal space; LICSP = left intercostal space parasternally; RICSP = right intercostal space parasternally; RASV = ruptured aneurysm sinus of Valsalva; VSD and AI = ventricular septal defect and aortic incompetence; PDA = patent ductus arteriosus. Downloaded by: King's College London 137.73.144.138 - 1/16/2019 2:28:35 AM
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132
Table II
Plethora
Case No.
C/T ratio Vo
RA
PA
AK
1 2 3 4 5 6 7 8
52 64 59 68 54 57 57 60
_
_
_
—
-
++ +
+ + —
+ ++ + + ++ + +
++ ++ + -
-
++ ++
C /T ratio = Cardiothoracic ratio; RA - right atrium; PA = pulmonary artery; AK = aortic knuckle
Table III Case No.
Rhythm
Mean frontal plane axis
LVH
RVH
Conduction defect
1 2 3 4 5 6 7 8
SR SR SR SR SR SR SR SR
+90° +65° +90° +45° +20° +90° +90° +30°
no yes no yes yes yes yes
no yes no no no no no
no no no complete RBBB no no no complete RBBB
SR Sinus rhythm; LVH left ventricular hypertrophy; RVH = right ventricular hypertrophy; RBBB = right bundle branch block.
The clinical, radiological, angiographic and electrocardiographic find ings and the hemodynamic data of the eight patients are summarized in tables I-IV. Six patients underwent thoracotomy and table V summarizes the operative findings, the surgical procedures and the outcome.
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Material and Results
Aortic Sinus Aneurysms
133
Table IV Case No.
Site of
Pressures, mm Hg
QP/QS
RA RV (mean)
PA
PeP
Ao
in oxygen saturation
1
9
85/18
54/30
13
137/67
RV
1.5:1
2
12
106/18
50/20
17
106/48
RV
2.0:1
3 4 5 6 7
7 It)
45/25 18/9
20 8
120/50 118/63
32/12 25/10
12 8
120/55 140/65
RA RV RV RV RV
4.0:1 1.5:1
6 5
45/5 44/11 38/10 45/5 65/5
8
12
40/10
36/16
14
120'60
RA
1.9:1
5.0:1 3.2:1
Aortogram
communication aorta to RV outflow tract large aneurysm RCS to RV outflow tract communication aorta to RA huge aneurysm RCS to RV communication aorta to RV communication aorta to RV aneurysm RCS to RV outflow tract aneurysm rupturing into RA
In 6 of the 8 patients who underwent thoracotomy the site of the aneurysm was in the right coronary sinus in 3 (cases 2, 4 and 6), in the noncoronary sinus in I (case 3), and unspecified in 2 (cases 1 and 5). In two patients only there was an associated ventricular septal defect, situated in the supracristal region in case 1 and in the infracristal region in case 6. For the two patients who did not undergo thoracotomy, aortography revealed that the aneurysm arose from the right coronary sinus in one case (case 7), but in the other (case 8) the site of origin of the aneurysm could not be determined from the aortogram. Hence, in 5 out of the 8 patients in this series, the site of the aneurysm could definitely be determined. In four patients the right coronary sinus was involved and in one the non coronary sinus. Classically, aneurysms of the sinus of Valsalva present with an acute history of chest pain, dyspnea or palpitations at the time of rupture. How ever, this is not invariable, as pointed out previously by various authors [O ram and E ast , 1955; W r ig h t , 1970], L ockha rt er al. [1964] stated that of 71 cases reviewed, only 37'Vo gave a history of abrupt onset. In this series of eight patients, only one (case 5, 12"/o) was acute, experiencing
Downloaded by: King's College London 137.73.144.138 - 1/16/2019 2:28:35 AM
RA = Right atrium; RV = Right ventricle; PA = pulmonary artery; Pep = pulmonary capillary wedge; Ao = aorta; QP/QS = pulmonary blood flow/systemic blood flow; RCS = right coronary sinus.
134
C MIAA"An /L im /T an /T oh /J oh an
Table V
‘Wind sock' type of sinus of Valsalva aneurysm measuring 1 cm in diameter, rupturing into RV outflow tract just below pulmonary valve
associated lesions
Surgical procedure done
Results
Follow-up
VSD type I (supracristal) 1 cm in diameter
aneurysm ex cised; defect closed with Teflon felt; VSD closed
recovery
1 year later, symptomati cally well; no murmurs heard
aneurysm ex cised; defect closed with pericardium and graft
recovery
9 months later, well; no mur murs
fistula repaired with inter rupted sutures
recovery
2.5 years later, symptomati cally well; no murmurs
recovery aneurysm ex cised fistula re paired with Teflon felt
7 months later, asymptomatic; no murmurs
Right coronary sinus aneurysm 2.0 cm in diameter, rupturing into RV outflow tract
Middle third of noncoronary sinus of Valsalva ruptured into right atrium; fistula measured 1.0 cm in diameter
-
Hugh right coronary sinus aneurysm rupturing into right ventricle; ventricular aneurysm seen in posterior surface of left ventricle (incidental finding)
‘Wind sock' type sinus of Valsalva aneurysm rupturing into right ventricular outflow tract
-
aneurysm ex cised; defect repaired
Aneurysm of the right coronary sinus (1.5 x 1.0 cm) rupturing into RV outflow tract
small VSD type II (infracristal)
aneurysm ex recovery cised; fistula re paired with Teflon felt
recovery
3 years later, well
1 year 4 months later, well; no murmurs
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Case Operative findings No. ----------------------------------------description of aneurysm
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sudden chest pain followed soon after by progressive dyspnea. This patient and case 3 are the only 2 out of the 6 patients who at thoracotomy showed neither a ventricular septal defect nor a right ventricular outflow tract obstruction. Of the 2 patients who did not undergo operation, 1 (case 7) showed a right ventricular outflow tract obstruction of 40 mm at cardiac catheterization. This tends to support the belief that a preexisting pulmonary stenosis or ventricular septal defect minimizes the hemodynamic disturbances at the time of rupture of the aneurysm. Cardiac catheterization and angiography are necessary for confirmation of the diagnosis and to distinguish this condition from coronary arterio venous fistula or a ventricular septal defect with aortic incompetence. Oxygen sampling localized the site of shunt at the right ventricular level in 6 of our 8 patients and at the right atrial level in 2 (table IV). The pulmonary blood flow to systemic blood flow ratio varied from 1.5:1 to 5.0:1 (mean 2.7:1). A significant right ventricular outflow tract systolic gradient was seen in four patients, varying from 26 to 56 mm Hg. In three (cases 1, 2 and 4) the gradient was shown at thoracotomy to be due to the aneurysm pressing on the right ventricular outflow tract. This obstruction was clearly demonstrated before operation in the angiographicstudies of case 2. In this patient the aortogram revealed a huge aneurysm of the right sinus of Valsalva rupturing into the right ventricle. The right ventriculogram demonstrated that this aneurysm had compressed the right ventricular outflow tract causing a large filling defect (fig. I). In the remaining seven cases, aortography confirmed a communication between the aorta and the right ventricle in five patients (cases 1, 4—7) (fig. 2) and the aorta and the right atrium in two patients (cases 3 and 8) (fig. 3).
Discussion
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Aneurysms of the sinus of Valsalva are most commonly congenital, but may be associated with the Marfan and Ehlers-Danlos syndrome [H u d s o n , 1965], They can also be due to syphilis or infective endocarditis. All eight patients in this series were considered to have congenital aneurysms. Most of the cases of ruptured ASV were reported from Western coun tries [W r ig h t , 1970] except for isolated cases in India [R aman and M e n o n , 1949], Hongkong [B a r n es , 1968], Korea [H o n g et al., 1966], Thailand [K och a sen i et al., 1971], and two large series in Japan [S akaki-
136
C hia/T an/L im /T anAToh/J ohan
ba ra and K o n n o , 1962a; T a g u ch i ei at., 1969]. As far as we know, this is the largest documented study in the East apart from the two Japanese series. Differences were noted between the Japanese and the Western series. Based on studies from Europe and North America [B esterm a n et at., 1963; M a g id so n and K ay , 1963; A brams and E van s , 1964; P erasaeo et at., 1966], W r ig h t [1970] concluded that in the West aneurysms in volving the right coronary sinus of Valsalva are about twice as frequent as those involving the noncoronary sinus. This preponderance is, however, much greater in Japan. Sakakibara and K o nn o [1962a] found that of the cases documented in Japan up to 1962, 19 had involved the right coronary and only one the noncoronary sinus. In 1969, T a g u ch i et at. reported on a series of 45 patients operated by them in Hiroshima, Japan. They found
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Fig. I. Right ventriculogram of case 2 (lateral projection), showing a filling defect (arrow) caused by the aneurysm of the right sinus of Valsalva bulging on and com pressing the right ventricular outflow tract. Fig. 2 Aortogram of case 1 (lateral projection), showing reflux of contrast dye from aorta to right ventricle.
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Fig. 3. X-ray film obtained by subtraction technique. Aortogram of case 8 (antero posterior projection), showing aneurysm of the sinus of Valsalva (lower arrow) rupturing into the right atrium (upper arrow).
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that the right coronary sinus was involved in 89 %> and the noncoronary sinus in about 4°/o of the patients. This marked preponderance of right coronary over noncoronary sinus aneurysms and the corresponding rarity of noncoronary sinus aneurysms are linked with the relatively high in cidence in Japan of aneurysms of the anterior portion of the right coronary sinus, which are very frequently associated with a supracristal ventricular septal defect [W r ig h t , 1970]. Sakakibara and K c n n o [1962a] believe that the associated ventricular septal defect originates in a common con genital point of weakness with the right coronary sinus aneurysm. Although the present series is too small to enable any final conclusions, it neverthe less appears that the preponderance (4:1) of right coronary sinus aneu rysms over noncoronary sinus aneurysms in Singapore resembles more the Japanese than the Western experiences. Although systolic murmurs alone were documented in ruptured ASV [H o n g et al., 1966], the typical auscultatory findings was a continuous murmur. The character of this murmur may be modified by the presence of either a ventricular septal defect or aortic incompetence. Hence, the continuous murmur in ruptured ASV may be accentuated in either systole, diastole or both. Some authors do not consider the murmur.of ruptured ASV to be continuous although it extends throughout the cardiac cycle
C hia/T an/L im/T an/T oh/J ohan
138
and K o n n o , 1962b; F r ie d b e r g , 1966], This is because, un like the classical Gibson’s murmur of a patent ductus arteriosus, the maximal loudness of the murmur in ruptured ASV is not at the second heart sound of the cardiac cycle. However, we feel that this difficulty in description is largely a matter of semantics. In all eight patients of this series, clinical auscultation of the precordium revealed a long murmur which extended throughout systole and diastole and which we considered to be continuous. In six patients this murmur was loudest at either the second, third, fourth or fifth left intercostal spaces parasternally (table 1). In all these patients, the aneurysm had ruptured into the right ventricle. In the remaining two patients, where the aneurysm had ruptured into the right atrium, the continuous murmurs were loudest over the fourth and fifth intercostal spaces on both sides of the sternum. S akakibara and K o n n o [1962b] have suggested that when the aneurysm ruptures into the right atrium, the direction of the blood flow tends to project the murmur to the lower part or to the right of the sternum. The prognosis of patients with ruptured ASV, treated conservatively, depends largely on the size of the fistula, but it is generally poor. Surgical closure of the ruptured ASV under cardiopulmonary bypass is the treat ment of choice [S akakibara and K o n n o , 1963]. All six of our patients survived the operation and were found to be in good health when examined 7 months to 3 years later. [S akakibara
References A brams, L. D. and E vans, D. W.: Ruptured aortic sinus aneurysm. Q. Jl Med. 33:
285-295 (1964).
ruptured aneurysm of the aortic sinus. J. thorac. cardiovasc. Surg. 51: 507-514 (1966). H udson . R. E. B.: Cardiovascular pathology (Arnold, London 1965). K ochanseni, S. E.; H araphonages, M.; W ong T hongari, W„ and C hareonchob, E.: Ruptured aneurysm of sinus of Valsalva. J. med. Ass. Thailand 54: 203-212 (1971). Lockhart, A.; Scebat, L., and L enegre , J. (1964): Cited by W right (1970). M agidson, O. and K ay, J. H.: Ruptured aortic sinus aneurysm. Am. Heart J. 65: 597-606 (1963).
Downloaded by: King's College London 137.73.144.138 - 1/16/2019 2:28:35 AM
Barnes, R. J.: Rupture sinus of Valsalva. Br. med. J. i: 683-684 (1968). Besterman. E.-. G oldberg , M., and Sellors , T.: Surgical repair of ruptured sinus of Valsalva. Br. med. J. if: 410-416 ( 1963). F riedberg , C. K.: Diseases of the heart (Saunders, Philadelphia 1966). H ong , P. W.: Lee, S. S.; K im, S. W., and C ha, H. D.: Unusual manifestations of
Aortic Sinus Aneurysms
139
O ram, S. and E ast, T.: Rupture of aneurysm of aortic sinus (of Valsalva) into the
right side of the heart. Br. Heart J. 17: 541-551 (1955). P erasalo, O.: H ai.onen , P. I.; Kyixonen , K. E„ and H eikkila, K. F.: Ruptured
aneurysm of the sinus of Valsalva. Acta chir. scand., suppl. 356B, pp. 55-64 (1966). R aman, T. K. and M enon . T. B.: Aneurysms of the sinuses of Valsalva. Indian Heart
J. I: 1-14(1949). Sakakibara. S. and Konno . S.: Congenital aneurysms of the sinus of Valsalva, anat
omy and classification. Am. Heart J. 63: 405-422 (1962a). Sakakibara, S. and Kon .no. S.: Congenital aneurysms of the sinus of Valsalva. A
clinical study. Am. Heart J. 63: 708-719 (1962b). T aguchi, K.: Sasaki, N.; Matsuura, V„ and U emura, R.: Surgical correction of
aneurysm of the sinus of Valsalva. Am. J. Cardiol. 23: 180-191 (1969). W right , J. S.: Ruptured aneurysm of the sinus of Valsalva. Q. Jl Med. 39: 501-513
(1970).
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Dr. B.-L. C hia. Department of Medicine. General Hospital. Sepoy Line, Singapore 3 (Malaysia)
