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J Thorac Cardiovasc Surg 1994;107:1464-1468
© 1994 Mosby, Inc.
SURGERY FOR ACQUIRED HEART DISEASE |
Southampton, United Kingdom, and Madras, India
Received for publication, June 14, 1993. Accepted for publication Nov. 8, 1993. Address for reprints: K. M. Cherian, FRACS, Institute of Cardiavascular Diseases, 180, NSK Salai, Madras—600 026, India.
Abstract
Between 1978 and 1993 a total of 25 cases of ruptured aneurysm of the sinus of Valsalva underwent transaortic repair at the Railway Hospital and the Institute of Cardiovascular Diseases at Madras, India. The aneurysms ruptured into the right ventricle in 20 patients, into the right atrium in 2, into the left ventricle in 1, and into the interventricular septum in 2. They originated from the right coronary sinus in 22 patients and from the noncoronary sinus in 3. Associated congenital anomalies included ventricular septal defect in 21, tetralogy of Fallot in 1, and pulmonary stenosis in 1. All 25 patients underwent transaortic repair of the ruptured sinus of Valsalva, and in patients with a ventricular septal defect the defect also was closed via this route. The patient with associated pulmonary stenosis had a pulmonary valvotomy. The patient with tetralogy of Fallot had infundibular resection, ventricular septal defect closure via a transatrial route, and a right ventricular outflow patch. No intraoperative deaths occurred, but one early death resulted from septicemia and multiorgan failure after rupture of the aneurysm into the interventricular septum with consequent development of extensive intramyocardial and epicardial abscesses. One late death occurred as a result of recurrent subacute bacterial endocarditis and septicemia. Transaortic repair of ruptured aneurysm of the sinus of Valsalva can be accomplished with a low risk of mortality and has the advantage that an associated ventricular septal defect, which frequently accompanies this condition, can be conveniently closed via the same route. (J T HORAC CARDIOVASC SURG 1994;107:1464-8)
Aneurysm of the sinus of Valsalva is rare, but more common in the Orient than in the West.
1-5 It can produce various and sometimes unusual manifestations, 6,7 but rupture produces symptoms in 80% of patients, 8 although the severity of symptoms is modified to some extent by the presence of a ventricular septal defect (VSD) or aortic regurgitation. 9 Surgical resection of the aneurysm significantly lessens the symptoms. 10,11 Successful repair of ruptured aneurysms of the sinus of Valsalva was described by Lillehei, Stanley, and Varco. 12 Repair can be accomplished through the chamber into which the aneurysm ruptured, 7 through the aortic root, 13 or through both routes. 12,14-16 We describe our surgical technique and results in 25 patients in whom the defect was repaired primarily through the transaortic route.
PATIENTS AND METHODS
Patient profile
From 1978 until 1993, over a period of 15 years, 25 patients underwent repair of ruptured aneurysm of the sinus of Valsalva at the Railway Hospital and at the Institute of Cardiovascular Diseases at Madras, India. Eleven male and 14 female patients were included. The mean age at repair was 26 years (range 11 to 49 years). Major symptoms were dyspnea, chest pain, and palpitations, with the common clinical finding of a harsh systolic murmur at the left sternal border. Chest x-ray findings included an increased cardiothoracic ratio and plethoric lung fields. The majority of patients had left ventricular hypertrophy on the electrocardiogram. One patient had a high-grade fever and severe dyspnea after rupture of the aneurysm into the interventricular septum and subsequent development of intramyocardial abscesses. The aneurysm arose from the right coronary sinus in 22 patients and the noncoronary sinus in 3. It ruptured into the right ventricle in 20 patients, into the right atrium in 2, into the left ventricle in 1, and into the interventricular septum in 2. Twenty-one (84%) of these patients had an associated VSD, 1 had valvular pulmonary stenosis, and 1 had tetralogy of Fallot. Diagnosis was established by echocardiography. Cardiac catheterization was carried out in only two patients in whom the echocardiographic findings were doubtful. All patients had good left ventricular function except for the one patient with intramyocardial abscess, in whom left ventricular function was moderately impaired.
Operative technique
Cardiopulmonary bypass (CPB) was established after high aortic and right atrial cannulation. At moderate hypothermia, the aorta was crossclamped and the aorta opened via a low transverse incision (Fig. 1). Between 1978 and 1986 crystalloid cardioplegia was used to achieve diastolic arrest and myocardial protection. Blood cardioplegia has been used since 1986. The aortic root is visualized and the aneurysmal opening identified. The windsock of the aneurysm is inverted and excised at its edges. The resultant opening is closed transversely in a single layer with fine monofilament sutures (Fig. 2). Occasionally this opening may be large and a polytetrafluoroethylene (PTFE)patch is used to close the defect. By means of a stay suture, the right coronary cusp is elevated and the VSD visualized. Almost always this VSD is a semicircular, doubly committed subarterial type—the Asian form—rather than a conoventricular or perimembranous type. A pledget-supported fine monofilament suture is placed at the lowermost point of the VSD, on the right side of the septum, and injury to the bundles of His has been nil (Fig. 3, A to C). A piece of PTFE cut to a semicircular shape appropriate for the size of the VSD is used to close the VSD.
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RESULTS
No intraoperative deaths but one early death occurred. A 49-year-old man with acute dyspnea and fever was found to have rupture of the sinus of Valsalva aneurysm into the interventricular septum. At operation extensive intramyocardial and epicardial microabscesses were observed. The aortic valve was replaced after closure of the VSD via the transaortic route. Although the man was weaned from CPB without difficulty, he had a stormy postoperative period and died on the eighth day of septicemia and multiorgan failure. One late death also occurred. A 21-year-old man had subacute bacterial endocarditis and severe aortic regurgitation 2 months after repair. At reoperation the repair had dehisced, with pannus forming along the suture lines. The sutures on the VSD patch had also given way, resulting in a residual VSD. The VSD was repaired with a patch again and the aortic valve was replaced with a Starr-Edward valve (Baxter Healthcare Corp., Edwards Division, Santa Ana, Calif.). He recovered well and was discharged but returned 2 months later with residual aortic regurgitation and recurrent subacute bacterial endocarditis. He died 3 days later of fulminant septicemia.
The remaining patients are well and free of symptoms. The average duration of follow-up was 5
years (range 2 months to 15 years). Echocardiography was performed in 12 patients and demonstrated mild aortic regurgitation in two patients. None of these patients had any residual left-to-right shunts.
DISCUSSION
The prevalence of ruptured aneurysm of the sinus of Valsalva has variously been reported to be between 0.3% and 3.56% of all congenital heart diseases. 12,15,17 Chu and associates 1 reported that the condition was not only more common in Oriental patients than in the West, but that the disease pattern was much simpler than the more diverse entities in Western series. All the VSDs seen in this series have been doubly committed subarterial VSDs rather than conoventricular or perimembraneous, as seen in Western populations. Because of this peculiar type of VSD and the position of the bundles of His, no injuries to the bundle of His have been reported. A similar aortic approach either to conoventricular or perimembraneous types of VSD, which occur generally in non-Asian populations, might result in a complete heart block. Although ruptured sinus aneurysm can result from trauma, subacute bacterial endocarditis, syphilis, or cystic medial necrosis, the prevalence of associated VSDs in this condition, especially in Oriental patients, attests to the fact that a congenital cause could be a more plausible explanation. 1,14,18,19 Edwards and Burchell 20 attributed the cause to discontinuity between the aortic media and the anulus fibrosus of the aortic valve. 19 Others, however, have suggested that the primary lesion is a developmental structural defect of the anulus itself. Aortic regurgitation is possibly due to a lack of supporting tissues, 17 causing sagging of the aortic valve cusps. 21
The most effective treatment of ruptured sinus of Valsalva aneurysm is by surgical repair. Various surgical approaches have been described. Pan and associates 18 described repair via the cardiac chamber into which the aneurysm had ruptured; Spencer, Blake, and Bahnson 13 repaired the defect through the aortic root; and an aortocameral exposure has been described by others. 2,12,15,16,22 We advocate a transaortic repair because we believe visualization of the aortic cusps is vital to prevent leaflet distortion and consequent aortic regurgitation. An approach to the aneurysm alone via the affected chamber involves a risk of aortic cusp distortion if the sutures are placed too deeply 12 and especially if the aneurysm is closed directly. 19 An aortocameral approach has been used by others essentially for better exposure, exclusion, and closure of an associated VSD.
In our experience, a transaortic approach not only afforded good exposure of the aortic root and opening of the sinus, but also aided exclusion and subsequent closure of the VSD. An added advantage of this method compared with the aortocameral approach is the avoidance of a ventriculotomy, which may form a possible focus of arrhythmias in the future. Raffa, 23 Pasic, 24 and their associates have avoided resecting the aneurysmal sac, citing possible damage to the adjacent structures. We encountered no such difficulties even in the two patients in whom the sinus eroded into the interventricular septum.
An important modification in the closure of the VSD via this route is the creation of a short shelf of the PTFE patch used to close the VSD. This shelf supports the aortic cusp, and we believe this technique is important in reducing the possibility of aortic regurgitation. An interesting feature of this series is the rupture of the aneurysm into the interventricular septum in two patients. Rupture of the aneurysm into the interventricular septum has been reported to cause heart block. Although in one patient the rupture did not produce any untoward effect on the conduction system, in the other, as a result of accompanying subacute bacterial endocarditis, extensive microabscesses formed in the interventricular septum and the epicardium, and the patient eventually died.
In conclusion, ruptured aneurysm of the sinus of Valsalva is a rare condition that is frequently associated with VSD. The sinus and the associated VSD can be repaired through the transaortic route alone. If a patch is used to close the VSD and support the anulus, sagging of the cusp is prevented and the possibility of aortic regurgitation is thereby reduced.
Acknowledgments
Since submission of this article, we have used this technique to repair three more cases of ruptured aneurysm of the sinus of Valsalva.
Footnotes
From the Department of Cardiac Surgery, Southampton General Hospital, Southampton, United Kingdom,a and the Department of Cardiac Surgery, Institute of Cardiovascular Diseases, Madras, India.b 
*Gore-Tex patch, registered trademark of W. L. Gore & Associates, Inc., Elkton, Md.
References
This article has been cited by other articles:
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  F. Yan, Q. Huo, J. Qiao, V. Murat, and S.-F. Ma Surgery for Sinus of Valsalva Aneurysm: 27-Year Experience with 100 Patients Asian Cardiovasc Thorac Ann, October 1, 2008; 16(5): 361 - 365. [Abstract] [Full Text] [PDF]
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 Z.-j. Wang, C.-w. Zou, D.-c. Li, H.-x. Li, A.-b. Wang, G.-d. Yuan, and Q.-x. Fan Surgical Repair of Sinus of Valsalva Aneurysm in Asian Patients Ann. Thorac. Surg., July 1, 2007; 84(1): 156 - 160. [Abstract] [Full Text] [PDF]
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 G. Fazio, R. Zito, D. D. Dioco, C. Mussagy, S. Loredana, A. Damasceno, and S. Novo Rupture of a left sinus of Valsalva aneurysm into the pulmonary artery Eur J Echocardiogr, June 1, 2006; 7(3): 230 - 232. [Abstract] [Full Text] [PDF]
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 A. Maruo, T. Higami, H. Obo, and T. Shida Ruptured sinus of Valsalva aneurysm associated with aortic regurgitation caused by hemodynamic effect solely Eur. J. Cardiothorac. Surg., August 1, 2003; 24(2): 318 - 319. [Abstract] [Full Text] [PDF]
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T. Murashita, T. Kubota, Y. Kamikubo, N. Shiiya, and K. Yasuda Long-term results of aortic valve regurgitation after repair of ruptured sinus of valsalva aneurysm Ann. Thorac. Surg., May 1, 2002; 73(5): 1466 - 1471. [Abstract] [Full Text] [PDF]
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A. Azakie, T. E. David, C. M. Peniston, V. Rao, and W. G. Williams Ruptured sinus of valsalva aneurysm: early recurrence and fate of the aortic valve Ann. Thorac. Surg., November 1, 2000; 70(5): 1466 - 1470. [Abstract] [Full Text] [PDF]
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Y. Naka, K. Kadoba, S. Ohtake, Y. Sawa, N. Hirata, M. Nishimura, and H. Matuda The long-term outcome of a surgical repair of sinus of Valsalva aneurysm Ann. Thorac. Surg., September 1, 2000; 70(3): 727 - 729. [Abstract] [Full Text] [PDF]
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W.-K. Au, S.-W. Chiu, C.-K. Mok, W.-T. Lee, D. Cheung, and G.-W. He Repair of ruptured sinus of valsalva aneurysm: Determinants of long-term survival Ann. Thorac. Surg., November 1, 1998; 66(5): 1604 - 1610. [Abstract] [Full Text] [PDF]
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