Eur J Cardio-thorac
Surg (1992) 6:542-544
surgery
0 Springer-Verlag 1992
Ruptured congenital aneurysm of the sinus of Valsalva: surgical technique and long-term follow-up M. Pasic I, L. von Segesser ‘, Th. Carrel ‘, R. Jenni ‘, and M. Turina ’ 1 Clinic for Cardiovascular
Surgery and ’ Department
of Medicine, University
Hospital,
Zurich, Switzerland
Abstract. Congenital ruptured aneurysm of the sinus of Valsalva is a rare anomaly usually causing decrease of cardiac performance. Eight patients with a ruptured congenital aneurysm of the sinus of Valsalva were operated upon at the University Hospital Zurich between 1970 and 1991. There were four female and four male patients aged from 15 to 48 years (mean, 36 years). Three patients were asymptomatic and five symptomatic. Associated congenital cardiac defects were found in six patients. Surgical technique consisted of direct suture in seven patients and closure with a Dacron patch in one. A secondary Dacron patch closure was performed on the second postoperative day in a patient with suture insufficiency after direct closure. Associated operations were closure of ventricular septal defect in two patients, aortic valve replacement in two, aortic valve reconstruction in one and aortic valve commissurotomy in one patient. There were no operative deaths. The mean follow-up was 9 years, range 7 months to 17 years. There were two late deaths due to endocarditis and recurrent cerebral embolisation. An operation for a ruptured aneurysm of the sinus of Valsalva has a low operative risk, but patients remain prone to development of late valvular complications. (Eur J Cardio-thorac Surg (1992) 6:542-5441 Key words: Sinus of Valsalva aneurysm - Rupture - Aneurysm - Surgery
An aneurysm of the sinus of Valsalva is a rare anomaly which may be congenital or acquired [3]. The congenital aneurysm is more common than the acquired form. A special form of the congenital aneurysm is in the Marfan syndrome, usually with enlargement of all sinuses. The acquired aneurysm may result from trauma, endocarditis, syphilis and as a result of the aging process [7]. It appears in the aged [9, 211 as well as in a child [14]. Breviere et al. reported a closure of the sinus of Valsalva defect successfully performed on the third day of life [2]. An unruptured congenital aneurysm is usually asymptomatic, whereas rupture causes acute symptoms in about 35% of patients and mild symptoms in about 45%
WI. In this study we report the techniques and long-term results of surgical treatment of patients with ruptured aneurysm of the sinus of Valsalva operated upon at the University Hospital in Zurich, Switzerland between 1970 and 1991. Material and methods Eight patients with congenital ruptured aneurysm of the sinus of Valsalva were operated upon at the University Hospital Zurich Received for publication: Accepted for publication:
January 6, 1992 March 2, 1992
between 1970 and 1991. There were four females and four males, aged from 15 to 48 (mean, 36 years). Three patients were asymptomatic and five symptomatic. According to the classification of the New York Heart Association (NYHA), three patients were preoperatively in NYHA class I, two in NYHA class II, two in NYHA class III, and one in NYHA class IV. Acute symptoms were present in two patients. In three patients, the onset of symptoms was gradual with effort dyspnea, palpitations, and precordial or epigastric pain, and/or cardiac failure. The duration of symptoms was l-10 years before operation. One patient had hypertension, one patient recurrent pericarditis, and one had bacterial endocarditis 1 year before operation. In six patients, left-to-right shunt was found with an average value of 40% (range, 30X-70%). All patients had a classic continuous systolic-diastolic murmur along the left sternal border with maximum intensity at the third intercostal space, and various additional findings due to the associated abnormalities. Cardiomegaly was found in five patients. The diagnosis was made in all patients by echocardiography and/or cardiac catheterization. The origin of the ruptured aneurysm of the sinus of Valsalva was the right coronary sinus in five patients, the noncoronary sinus in two and the left coronary sinus in one. The aneurysm ruptured into the right ventricle in four patients (Fig. l), into the right atrium in one (Fig. 2), into both the right atrium and the right ventricle in one and into the left ventricle in two (Fig. 3). The size of the fistulous opening was between 2 mm and 12 mm (mean, 7 mm). Associated congenital defects were found in six patients. Associated cardiac anomalies included aortic regurgitation in four patients, ventricular septal defect in two, bicuspid aortic valve in three, mild pulmonary stenosis in two, and mild mitral stenosis in one. Mild mitral and tricuspidal insufficiency was found in two
’ Fig. 1. The aneurysm of the sinus of Valsalva from the right coronary sinus of Valsalva and rupturing into the right ventricle Fig. 2. The aortogram showing the ruptured aneurysm of the noncoronary sinus of Valsalva into the right atrium. Moderate aortic regurgitation is present Fig. 3. The aneurysm of the left coronary sinus of Valsalva rupturing into the left ventricle
patients. Two patients had a previous operation, both for a patent ductus Botalli and coarctation of the aorta. All patients were operated upon using cardiopulmonary bypass. The approach was an atriotomy alone in four patients and a double incision in four (aorta and ventricle in two, and aorta and the right atrium in two). Associated ventriculotomy was performed to treat associated congenital anomalies. Surgical technique consisted of direct suture in seven patients and closure with a Dacron patch in one. In two patients, closure was performed from both sides of the tistula. The aneurysm was totally resected in one patient, partially resected in one, and closure without resection was performed in six. Associated operations were closure of ventricular septal defect in two patients, resection of the infundibular pulmonary stenosis in one, aortic valve replacement in two, aortic valve reconstruction in one and aortic valve commissurotomy in one. The mean follow-up was 9 years, range 7 months to 17 years.
Results
was no operative death. During the early postoperative course, one patient was resuscitated because of ventricular tachycardia, and a second patient with associated aortic valve replacement required implantation of a permanent pacemaker due to complete heart block. One patient with direct closure of the rupture and associated aortic commisurotomy was reoperated upon on the second postoperative day with a Dacron patch closure because of a suture insufficiency. He underwent aortic valve replacement with a mechanical valve 4 years after operation because of severe aortic regurgitation and died 17 years after the first operation due to recurrent cerebral embolisation. During the follow-up, mild aortic regurgitation was diagnosed in three patients, mild mitral regurgitation in one, and mitral prolapse in one. In one patient, an arteriovenous fistula of the lienal artery was found. At the end of follow-up, all but one patient were in NYHA class I or II. There were two late deaths, 13 and 17 years, respectively after operation. The first patient had previous bacterial endocarditis and died from endocarditis, the second from recurrent cerebral embolization. There
Discussion
The right coronary sinus is the most frequent site of a sinus of Valsalva aneurysm, followed by the noncoronary sinus [3]. In our study, rupture of the right coronary sinus was found in five patients. An aneurysm of the left sinus is exceptional. The aneurysm ruptures usually into the right ventricle, the right atrium, or both into the right atrium and the ventricle [15]. Rupture into the left ventricle, intrapericardial, into the pulmonary artery, or into the interventricular septum [16] is very rare. Intrapericardial rupture of sinus of Valsalva aneurysm is usually fatal [I]. Ruptured aneurysm is either asymptomatic or causes symptoms. Rupture causes chest pain, dyspnea, occasionally arrhythmias and cardiac failure [I I]. The symptoms depend on the direction of the rupture. The size of the fistula influences the symptoms, but a very small opening can also produce a large shunt, such as in one of our patients. A 48-year-old woman with a rupture of the noncoronary sinus into the right atrium had a left-toright shunt of 60% and severe symptoms combined with heart enlargement although the size of the fistula on the atria1 side was only 2 mm. The aneurysm of the sinus of Valsalva may rupture spontaneously or secondary to bacterial endocarditis [I 31. Sometimes it is difficult to distinguish between cause and result, although it seems that endocarditis determines the onset of rupture [I I]. Rupture into the right ventricle or atrium usually causes a left-to-right shunt with cardiac failure [2, 211. The aneurysm can cause aortic regurgitation [6,21] or compression or obstruction of the adjacent structures [5, IO]. Aneurysm of the right coronary sinus can cause right ventricular outflow obstruction [9] and a left sinus aneurysm can produce compression of the left coronary artery [IO] or the left circumflex coronary artery [6]. It can produce unusual presentations such as mediastinal tumour [17] or mitral disease [5]. Rupture produces symptoms in about 80% of patients [12]. Acute symptoms at the time of rupture may be less frequent when there is also a ventricular septum defect [20], and more frequent when there is severe associated aortic valve regurgitation. Surgical resection of the aneurysm results in dramatic symptomatic improvement [2, 91. Surgical technique consists of exclusion of the aneurysm of the aortic sinus. The operative procedure depends on the diameter of the aneurysm. Direct suture is possible if the diameter is small. If the opening is large, it is necessary to close the defect of the aneurysm with a
544 Dacron patch [3, 151 or with autologous pericardium from inside the aorta [16], or from both sides of the tistula [ll, 131. Surgical technique in our patients consisted of direct suture in seven patients and closure with a Dacron patch in one. A modification of surgical technique is exclusion of the aneurysm by two thirds of the circumference of a tubular prosthesis [21]. It is not necessary to resect the aneurysmal sac or windsock because it can produce damage to adjacent structures [16]. Associated aortic valve pathology or other congenital abnormalities should be corrected simultaneously. Doppler, colour, and cross sectional echocardiography is a good diagnostic method for detection of an aneurysm of sinus of Valsalva [4, 81. The diagnosis should be confirmed by aortography. Examination by pulsed and continuous Doppler echocardiography shows continuous turbulence in patients with rupture into the right ventricle or the right atrium. Colour Doppler echocardiography shows turbulent flow [19]. Associated anomalies such as ventricular septal defect, aortic regurgitation, or bicuspid aortic valve can be also detected by echocardiography [14]. For diagnosis in a young patient, it is probably sufficient to perform echocardiography alone without catheterization [8,18]. Catheterization is mandatory to exclude changes of the coronary arteries, especially in an old patient. There is a high incidence of associated congenital cardiac anomalies such as ventricular septal defects and pathology of the aortic valve. The pathogenetic mechanism contributes to the development of aortic regurgitation [3]. Associated congenital defects were also usual tindings in our patients. Six of our eight patients with ruptured aneurysms of the sinus of Valsalva had different associated cardiovascular abnormalities. It is evident that differences exist between our and Oriental patients. We found the small ventricular septal defect only in two patients. Chu et al. [3] reported a higher incidence of associated ventricular septal defect (59O/) and very low incidence of other congenital cardiac abnormalities (4.1%) in Oriental people. Our two patients had a previous operation for a patent ductus Botalli and coarctation of the aorta. In four patients, there was severe regurgitation which necessitated a simultaneous operation on the aortic valve. The ruptured aneurysm of the sinus of Valsalva should be subjected to surgical correction when the diagnosis is made. Associated anomalies should be corrected simultaneously. Operation has a low risk, but patients remain prone to the development of late valvular complications such as endocarditis or thromboembolic complications. Acknowledgement. The authors gratefully acknowledge the assistance of Ms. Annette Bauer for help in preparing the manuscript.
References Brabham KR, Roberts WC (1990) Fatal intrapericardial rupture of sinus of Valsalva aneurysm. Am Heart J 120: 1455-1456 Breviere GM, Vaksmann G, Francart C (1990) Rupture of a sinus of Valsalva aneurysm in a neonate. Eur J Pediatr 149:603-604 Chu SH, Hung CR, How SS, Chang H, Wang SS, Tsai CH, Liau CS, Tseng CD, Tseng YZ, Lee YT, Lien WP, Lue HC, Lin
4.
5.
6.
7. 8.
9.
10.
11.
12. 13.
14.
15.
16.
17.
18.
19.
20.
21.
TY (1990) Ruptured aneurysms of the sinus of Valsalva in Oriental patients. J Thorac Cardiovasc Surg 99: 288-298 Dev V, Shrivastava S (1990) Echocardiographic diagnosis of unruptured aneurysm of the sinus of Valsalva dissecting into the ventricular septum. Am J Cardiol 66: 502- 503 Gander M, Goebel N, Jenni R, Turina M (1980) Grosses SinusValsalvae-Aneurysma mit dem klinischen Bild eines kombinierten Mitralvitiums. Fortsch Riintgenstr 133: 94-97 Glock Y, Ferrarini JM, Puel J, Fauvel JM, Bounhorne JP, Puel P (1990) Isolated aneurysm of the left sinus of Valsalva. Rupture into the left atrium, left ventricle and dynamic coronary constriction. J Cardiovasc Surg 31:235-238 Goldberg N, Krasnow N (1990) Sinus of Valsalva aneurysm. Clin Cardiol 13: 831-836 Goudevenos J, Kouvaras G, Chronopoulos DG, Ka Ratasakis G, Williams D, Reid P, Cokkinos D (1990) Colour Doppler echocardiography in the diagnosis of ruptured aneurysm of sinus of Valsalva. Eur Heart J 11: 666-669 Haraphongse M, Ayudhya RK, Jugdutt B, Rossall RE (1990) Isolated unruptured sinus of Valsalva aneurysm producing right ventricular outflow obstruction. Cathet Cardiovasc Diagn 19:98-102 Hinterauer L, Furger F, Steinbrunn W, Goebel N, Turina M (1982) Sinus-Valsalvae-Aneurysma mit subanullrer Ruptur in den linken Ventrikel, Kompression der linken Kranzarterie und rezidivierender Perikarditis. Fortschr Rijntgenstr 136:482-485 Jansen EWL, Nauta ILD, Lacquet LK (1984) Ruptured aneurysm of the sinus Valsalvae. Thorac Cardiovasc Surg 32: 148151 Kirklin JW, Barratt-Boyes BG, eds (1986) Cardiac surgery. Churchill Livingstone, New York, pp 669 Meyer J, Wukasch DC, Hallman GL, Cooley DA (1975) Aneurysm and listula of the sinus of Valsalva. Ann Thorac Surg 19: 170-179 Mohan JC (1990) Cross-sectional echocardiographic diagnosis of discrete subaortic stenosis, ruptured congenital aneurysm of the right sinus of Valsalva, unruptured aneurysm of non-coronary sinus and a rudimentary left aortic leaflet. Int J Cardiol 28: 122-125 Pan-Chih, Ching-Heng T, Chen-Chun, Chieh-Fu L (1981) Surgical treatment of the ruptured aneurysm of the aortic sinuses. Ann Thorac Surg 32: 162-166 Raffa H, Mosieri J, Sorefan AA, Kayali MT (1991) Sinus of Valsalve aneurysm eroding into the inter-ventricular septum. Ann Thorac Surg 51:996-998 Reid PG, Goudevenos JA, Hilton CJ (1990) Thrombosed saccular aneurysm of a sinus of Valsalva: unusual cause of a mediastinal mass. Br Heart J 63: 183 - 185 Rothbart RM, Chahine RA (1990) Left sinus of Valsalva aneurysm with rupture into the left ventricular outflow tract: diagnosis by color-encoded Doppler imaging. Am Heart J 120: 224227 Sahasakul Y, Panchavinnin P, Chaithiraphan S, Sakiyalak P (1990) Echocardiographic diagnosis of a ruptured aneurysm of the sinus of Valsalva: operation without catheterisation in seven patients. Br Heart J 64: 195-198 Sakakibara S, Konno S (1968) Congenital aneurysm of the sinus of Valsalva associated with ventricular septal defect: Anatomical aspects. Am Heart J 75: 595-601 Varin J, Donzeau-Gouge P, Forman J, Orion L, Gorce M, Chapsal J, Fouchard J (1990) Anevrisme calcilie non rompu du sinus de Valsalva avec angor et insuffrsance aortique. Cure chirurgicale. Ann Cardiol Angeiol 39:41 I-415
Dr. Miralem Pasic Klinik fur Herz- und Gefasschirurgie Universitatsspital Ramistrasse 100 CH-8091 Ziirich Switzerland
Surg (1992) 6:542-544
surgery
0 Springer-Verlag 1992
Ruptured congenital aneurysm of the sinus of Valsalva: surgical technique and long-term follow-up M. Pasic I, L. von Segesser ‘, Th. Carrel ‘, R. Jenni ‘, and M. Turina ’ 1 Clinic for Cardiovascular
Surgery and ’ Department
of Medicine, University
Hospital,
Zurich, Switzerland
Abstract. Congenital ruptured aneurysm of the sinus of Valsalva is a rare anomaly usually causing decrease of cardiac performance. Eight patients with a ruptured congenital aneurysm of the sinus of Valsalva were operated upon at the University Hospital Zurich between 1970 and 1991. There were four female and four male patients aged from 15 to 48 years (mean, 36 years). Three patients were asymptomatic and five symptomatic. Associated congenital cardiac defects were found in six patients. Surgical technique consisted of direct suture in seven patients and closure with a Dacron patch in one. A secondary Dacron patch closure was performed on the second postoperative day in a patient with suture insufficiency after direct closure. Associated operations were closure of ventricular septal defect in two patients, aortic valve replacement in two, aortic valve reconstruction in one and aortic valve commissurotomy in one patient. There were no operative deaths. The mean follow-up was 9 years, range 7 months to 17 years. There were two late deaths due to endocarditis and recurrent cerebral embolisation. An operation for a ruptured aneurysm of the sinus of Valsalva has a low operative risk, but patients remain prone to development of late valvular complications. (Eur J Cardio-thorac Surg (1992) 6:542-5441 Key words: Sinus of Valsalva aneurysm - Rupture - Aneurysm - Surgery
An aneurysm of the sinus of Valsalva is a rare anomaly which may be congenital or acquired [3]. The congenital aneurysm is more common than the acquired form. A special form of the congenital aneurysm is in the Marfan syndrome, usually with enlargement of all sinuses. The acquired aneurysm may result from trauma, endocarditis, syphilis and as a result of the aging process [7]. It appears in the aged [9, 211 as well as in a child [14]. Breviere et al. reported a closure of the sinus of Valsalva defect successfully performed on the third day of life [2]. An unruptured congenital aneurysm is usually asymptomatic, whereas rupture causes acute symptoms in about 35% of patients and mild symptoms in about 45%
WI. In this study we report the techniques and long-term results of surgical treatment of patients with ruptured aneurysm of the sinus of Valsalva operated upon at the University Hospital in Zurich, Switzerland between 1970 and 1991. Material and methods Eight patients with congenital ruptured aneurysm of the sinus of Valsalva were operated upon at the University Hospital Zurich Received for publication: Accepted for publication:
January 6, 1992 March 2, 1992
between 1970 and 1991. There were four females and four males, aged from 15 to 48 (mean, 36 years). Three patients were asymptomatic and five symptomatic. According to the classification of the New York Heart Association (NYHA), three patients were preoperatively in NYHA class I, two in NYHA class II, two in NYHA class III, and one in NYHA class IV. Acute symptoms were present in two patients. In three patients, the onset of symptoms was gradual with effort dyspnea, palpitations, and precordial or epigastric pain, and/or cardiac failure. The duration of symptoms was l-10 years before operation. One patient had hypertension, one patient recurrent pericarditis, and one had bacterial endocarditis 1 year before operation. In six patients, left-to-right shunt was found with an average value of 40% (range, 30X-70%). All patients had a classic continuous systolic-diastolic murmur along the left sternal border with maximum intensity at the third intercostal space, and various additional findings due to the associated abnormalities. Cardiomegaly was found in five patients. The diagnosis was made in all patients by echocardiography and/or cardiac catheterization. The origin of the ruptured aneurysm of the sinus of Valsalva was the right coronary sinus in five patients, the noncoronary sinus in two and the left coronary sinus in one. The aneurysm ruptured into the right ventricle in four patients (Fig. l), into the right atrium in one (Fig. 2), into both the right atrium and the right ventricle in one and into the left ventricle in two (Fig. 3). The size of the fistulous opening was between 2 mm and 12 mm (mean, 7 mm). Associated congenital defects were found in six patients. Associated cardiac anomalies included aortic regurgitation in four patients, ventricular septal defect in two, bicuspid aortic valve in three, mild pulmonary stenosis in two, and mild mitral stenosis in one. Mild mitral and tricuspidal insufficiency was found in two
’ Fig. 1. The aneurysm of the sinus of Valsalva from the right coronary sinus of Valsalva and rupturing into the right ventricle Fig. 2. The aortogram showing the ruptured aneurysm of the noncoronary sinus of Valsalva into the right atrium. Moderate aortic regurgitation is present Fig. 3. The aneurysm of the left coronary sinus of Valsalva rupturing into the left ventricle
patients. Two patients had a previous operation, both for a patent ductus Botalli and coarctation of the aorta. All patients were operated upon using cardiopulmonary bypass. The approach was an atriotomy alone in four patients and a double incision in four (aorta and ventricle in two, and aorta and the right atrium in two). Associated ventriculotomy was performed to treat associated congenital anomalies. Surgical technique consisted of direct suture in seven patients and closure with a Dacron patch in one. In two patients, closure was performed from both sides of the tistula. The aneurysm was totally resected in one patient, partially resected in one, and closure without resection was performed in six. Associated operations were closure of ventricular septal defect in two patients, resection of the infundibular pulmonary stenosis in one, aortic valve replacement in two, aortic valve reconstruction in one and aortic valve commissurotomy in one. The mean follow-up was 9 years, range 7 months to 17 years.
Results
was no operative death. During the early postoperative course, one patient was resuscitated because of ventricular tachycardia, and a second patient with associated aortic valve replacement required implantation of a permanent pacemaker due to complete heart block. One patient with direct closure of the rupture and associated aortic commisurotomy was reoperated upon on the second postoperative day with a Dacron patch closure because of a suture insufficiency. He underwent aortic valve replacement with a mechanical valve 4 years after operation because of severe aortic regurgitation and died 17 years after the first operation due to recurrent cerebral embolisation. During the follow-up, mild aortic regurgitation was diagnosed in three patients, mild mitral regurgitation in one, and mitral prolapse in one. In one patient, an arteriovenous fistula of the lienal artery was found. At the end of follow-up, all but one patient were in NYHA class I or II. There were two late deaths, 13 and 17 years, respectively after operation. The first patient had previous bacterial endocarditis and died from endocarditis, the second from recurrent cerebral embolization. There
Discussion
The right coronary sinus is the most frequent site of a sinus of Valsalva aneurysm, followed by the noncoronary sinus [3]. In our study, rupture of the right coronary sinus was found in five patients. An aneurysm of the left sinus is exceptional. The aneurysm ruptures usually into the right ventricle, the right atrium, or both into the right atrium and the ventricle [15]. Rupture into the left ventricle, intrapericardial, into the pulmonary artery, or into the interventricular septum [16] is very rare. Intrapericardial rupture of sinus of Valsalva aneurysm is usually fatal [I]. Ruptured aneurysm is either asymptomatic or causes symptoms. Rupture causes chest pain, dyspnea, occasionally arrhythmias and cardiac failure [I I]. The symptoms depend on the direction of the rupture. The size of the fistula influences the symptoms, but a very small opening can also produce a large shunt, such as in one of our patients. A 48-year-old woman with a rupture of the noncoronary sinus into the right atrium had a left-toright shunt of 60% and severe symptoms combined with heart enlargement although the size of the fistula on the atria1 side was only 2 mm. The aneurysm of the sinus of Valsalva may rupture spontaneously or secondary to bacterial endocarditis [I 31. Sometimes it is difficult to distinguish between cause and result, although it seems that endocarditis determines the onset of rupture [I I]. Rupture into the right ventricle or atrium usually causes a left-to-right shunt with cardiac failure [2, 211. The aneurysm can cause aortic regurgitation [6,21] or compression or obstruction of the adjacent structures [5, IO]. Aneurysm of the right coronary sinus can cause right ventricular outflow obstruction [9] and a left sinus aneurysm can produce compression of the left coronary artery [IO] or the left circumflex coronary artery [6]. It can produce unusual presentations such as mediastinal tumour [17] or mitral disease [5]. Rupture produces symptoms in about 80% of patients [12]. Acute symptoms at the time of rupture may be less frequent when there is also a ventricular septum defect [20], and more frequent when there is severe associated aortic valve regurgitation. Surgical resection of the aneurysm results in dramatic symptomatic improvement [2, 91. Surgical technique consists of exclusion of the aneurysm of the aortic sinus. The operative procedure depends on the diameter of the aneurysm. Direct suture is possible if the diameter is small. If the opening is large, it is necessary to close the defect of the aneurysm with a
544 Dacron patch [3, 151 or with autologous pericardium from inside the aorta [16], or from both sides of the tistula [ll, 131. Surgical technique in our patients consisted of direct suture in seven patients and closure with a Dacron patch in one. A modification of surgical technique is exclusion of the aneurysm by two thirds of the circumference of a tubular prosthesis [21]. It is not necessary to resect the aneurysmal sac or windsock because it can produce damage to adjacent structures [16]. Associated aortic valve pathology or other congenital abnormalities should be corrected simultaneously. Doppler, colour, and cross sectional echocardiography is a good diagnostic method for detection of an aneurysm of sinus of Valsalva [4, 81. The diagnosis should be confirmed by aortography. Examination by pulsed and continuous Doppler echocardiography shows continuous turbulence in patients with rupture into the right ventricle or the right atrium. Colour Doppler echocardiography shows turbulent flow [19]. Associated anomalies such as ventricular septal defect, aortic regurgitation, or bicuspid aortic valve can be also detected by echocardiography [14]. For diagnosis in a young patient, it is probably sufficient to perform echocardiography alone without catheterization [8,18]. Catheterization is mandatory to exclude changes of the coronary arteries, especially in an old patient. There is a high incidence of associated congenital cardiac anomalies such as ventricular septal defects and pathology of the aortic valve. The pathogenetic mechanism contributes to the development of aortic regurgitation [3]. Associated congenital defects were also usual tindings in our patients. Six of our eight patients with ruptured aneurysms of the sinus of Valsalva had different associated cardiovascular abnormalities. It is evident that differences exist between our and Oriental patients. We found the small ventricular septal defect only in two patients. Chu et al. [3] reported a higher incidence of associated ventricular septal defect (59O/) and very low incidence of other congenital cardiac abnormalities (4.1%) in Oriental people. Our two patients had a previous operation for a patent ductus Botalli and coarctation of the aorta. In four patients, there was severe regurgitation which necessitated a simultaneous operation on the aortic valve. The ruptured aneurysm of the sinus of Valsalva should be subjected to surgical correction when the diagnosis is made. Associated anomalies should be corrected simultaneously. Operation has a low risk, but patients remain prone to the development of late valvular complications such as endocarditis or thromboembolic complications. Acknowledgement. The authors gratefully acknowledge the assistance of Ms. Annette Bauer for help in preparing the manuscript.
References Brabham KR, Roberts WC (1990) Fatal intrapericardial rupture of sinus of Valsalva aneurysm. Am Heart J 120: 1455-1456 Breviere GM, Vaksmann G, Francart C (1990) Rupture of a sinus of Valsalva aneurysm in a neonate. Eur J Pediatr 149:603-604 Chu SH, Hung CR, How SS, Chang H, Wang SS, Tsai CH, Liau CS, Tseng CD, Tseng YZ, Lee YT, Lien WP, Lue HC, Lin
4.
5.
6.
7. 8.
9.
10.
11.
12. 13.
14.
15.
16.
17.
18.
19.
20.
21.
TY (1990) Ruptured aneurysms of the sinus of Valsalva in Oriental patients. J Thorac Cardiovasc Surg 99: 288-298 Dev V, Shrivastava S (1990) Echocardiographic diagnosis of unruptured aneurysm of the sinus of Valsalva dissecting into the ventricular septum. Am J Cardiol 66: 502- 503 Gander M, Goebel N, Jenni R, Turina M (1980) Grosses SinusValsalvae-Aneurysma mit dem klinischen Bild eines kombinierten Mitralvitiums. Fortsch Riintgenstr 133: 94-97 Glock Y, Ferrarini JM, Puel J, Fauvel JM, Bounhorne JP, Puel P (1990) Isolated aneurysm of the left sinus of Valsalva. Rupture into the left atrium, left ventricle and dynamic coronary constriction. J Cardiovasc Surg 31:235-238 Goldberg N, Krasnow N (1990) Sinus of Valsalva aneurysm. Clin Cardiol 13: 831-836 Goudevenos J, Kouvaras G, Chronopoulos DG, Ka Ratasakis G, Williams D, Reid P, Cokkinos D (1990) Colour Doppler echocardiography in the diagnosis of ruptured aneurysm of sinus of Valsalva. Eur Heart J 11: 666-669 Haraphongse M, Ayudhya RK, Jugdutt B, Rossall RE (1990) Isolated unruptured sinus of Valsalva aneurysm producing right ventricular outflow obstruction. Cathet Cardiovasc Diagn 19:98-102 Hinterauer L, Furger F, Steinbrunn W, Goebel N, Turina M (1982) Sinus-Valsalvae-Aneurysma mit subanullrer Ruptur in den linken Ventrikel, Kompression der linken Kranzarterie und rezidivierender Perikarditis. Fortschr Rijntgenstr 136:482-485 Jansen EWL, Nauta ILD, Lacquet LK (1984) Ruptured aneurysm of the sinus Valsalvae. Thorac Cardiovasc Surg 32: 148151 Kirklin JW, Barratt-Boyes BG, eds (1986) Cardiac surgery. Churchill Livingstone, New York, pp 669 Meyer J, Wukasch DC, Hallman GL, Cooley DA (1975) Aneurysm and listula of the sinus of Valsalva. Ann Thorac Surg 19: 170-179 Mohan JC (1990) Cross-sectional echocardiographic diagnosis of discrete subaortic stenosis, ruptured congenital aneurysm of the right sinus of Valsalva, unruptured aneurysm of non-coronary sinus and a rudimentary left aortic leaflet. Int J Cardiol 28: 122-125 Pan-Chih, Ching-Heng T, Chen-Chun, Chieh-Fu L (1981) Surgical treatment of the ruptured aneurysm of the aortic sinuses. Ann Thorac Surg 32: 162-166 Raffa H, Mosieri J, Sorefan AA, Kayali MT (1991) Sinus of Valsalve aneurysm eroding into the inter-ventricular septum. Ann Thorac Surg 51:996-998 Reid PG, Goudevenos JA, Hilton CJ (1990) Thrombosed saccular aneurysm of a sinus of Valsalva: unusual cause of a mediastinal mass. Br Heart J 63: 183 - 185 Rothbart RM, Chahine RA (1990) Left sinus of Valsalva aneurysm with rupture into the left ventricular outflow tract: diagnosis by color-encoded Doppler imaging. Am Heart J 120: 224227 Sahasakul Y, Panchavinnin P, Chaithiraphan S, Sakiyalak P (1990) Echocardiographic diagnosis of a ruptured aneurysm of the sinus of Valsalva: operation without catheterisation in seven patients. Br Heart J 64: 195-198 Sakakibara S, Konno S (1968) Congenital aneurysm of the sinus of Valsalva associated with ventricular septal defect: Anatomical aspects. Am Heart J 75: 595-601 Varin J, Donzeau-Gouge P, Forman J, Orion L, Gorce M, Chapsal J, Fouchard J (1990) Anevrisme calcilie non rompu du sinus de Valsalva avec angor et insuffrsance aortique. Cure chirurgicale. Ann Cardiol Angeiol 39:41 I-415
Dr. Miralem Pasic Klinik fur Herz- und Gefasschirurgie Universitatsspital Ramistrasse 100 CH-8091 Ziirich Switzerland
![[Ruptured aneurysm of the aortic sinus of Valsalva (author's transl)].](/assets/img/hold.png)
