Ann Thorac Surg 2002;73:1466-1471
© 2002 The Society of Thoracic Surgeons
Original article: cardiovascular
Long-term results of aortic valve regurgitation after repair of ruptured sinus of valsalva aneurysm
Toshifumi Murashita, MD, PhD*a,
Takehiro Kubota, MDa,
Yasuhiro Kamikubo, MD, PhDa,
Norihiko Shiiya, MD, PhDa,
Keishu Yasuda, MD, PhDa
a Department of Cardiovascular Surgery, Hokkaido University Hospital, Sapporo, Japan
Accepted for publication February 4, 2002.
* Address reprint requests to Dr Murashita, Department of Cardiovascular Surgery, Hokkaido University Hospital, Kita-14, Nishi-5, Kita-ku, Sapporo, 060-8648 Japan
e-mail: muratosh{at}med.hokudai.ac.jp
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Abstract
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Background. We reviewed our 35-year-experience to investigate the determinants of long-term results of aortic valve regurgitation (AR) after surgical repair of ruptured sinus of Valsalva aneurysms (RSVA).
Methods. Between 1963 and 1998, a total of 35 patients aged 7 to 64 years underwent surgery for RSVA. The aneurysms ruptured into the right ventricle (n = 24), right atrium (n = 10), and left atrium (n = 1). In all, 19 patients had VSD and 9 patients had AR. A combined approach through aortotomy and the involved chamber was used for 24 patients. Either direct (n = 19) or patch (n = 16) closure was used to close the rupture hole. The AR was graded on a scale of 0 to IV by angiographic or echographic evaluation.
Results. There were no early deaths. Late death occurred in 1 patient, whose AR deteriorated to grade III 20 years later. Two patients (5.7%) required reoperations on the aortic valve, because grade III AR was noted 8 and 26 years after operation, respectively. Freedom from postoperative grade III AR or higher was 93% at 10 years and was 87% at 20 years. Late AR was associated with preoperative and early postoperative AR (p < 0.05) but not with the presence of VSD, location of the fistula, surgical approach, or type of repair (direct vs patch). Multivariate analysis indicated that early postoperative AR was the only independent variable.
Conclusions. Late AR necessitating reoperation still confers significant risk in the long-term follow-up after repair of RSVA. No particular risk factor of preoperative conditions and surgical methods was elucidated in this study, and postoperative AR at discharge from the hospital was the only factor determining the long-term results of AR.
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Introduction
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The ruptured sinus of Valsalva aneurysm (RSVA) is a rare cardiac abnormality, although it has been reported to be relatively more common in Oriental populations [1, 2]. The RSVA usually originates in the right or noncoronary aortic sinus and communicates with a cardiac chamber, frequently right sided, producing an aorto-cardiac fistula. Associated cardiac abnormalities are common, including ventricular septal defects (VSD) and aortic regurgitation (AR). It has been demonstrated that surgical repair of RSVA is associated with low mortality and morbidity [2–6] since it was first repaired successfully by Lillehei and colleagues [7] in 1957. However, the long-term outcome after operation remains unclear. Recently, many reports have suggested AR still poses a risk in late follow-up, although the risk of a recurrent fistula or VSD is minimal in the current era. Preoperative endocarditis [8], coexistence of VSD [6, 9], a bicuspid aortic valve [10], and the surgical approach used [6] are reported to be risk factors influencing late AR. In this report, we review our entire experience with RSVA and analyze long-term results of AR after repair to determine factors influencing the progression of AR in late follow-up.
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Material and methods
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Between April 1963 and April 1998, a total of 35 patients (23 men and 12 women) with RSVA underwent operation at Hokkaido University Hospital, Sapporo, Japan. Patients with trauma, syphilis, or Marfan’s syndrome were excluded from this study. The mean age (± SD) was 27.5 ± 14.4 years, ranging from 6 to 64 years. At the time of admission, 22 patients (63%) were symptomatic and 7 patients (20%) were in New York Heart Association functional classes III and IV. The majority had the following symptoms: dyspnea (37%), palpitation (11%), and fatigue (6%). Heart murmur was heard in all patients, and 32 patients (91%) presented with loud continuous "machinery-type" murmurs. The results of chest x-rays were available for 24 patients, and 22 of them showed cardiomegaly (cadiothoracic ratio >0.5). With regard to electrocardiographic findings for these 24 patients with RSVA, 2 were normal and 9 had left ventricular hypertrophy, 3 had biventricular hypertrophy, 6 had right-axis deviation, and 2 had right bundle branch block. There were 2 patients (6%) with atrial fibrillation and the rest had sinus rhythm. A history of bacterial endocarditis was found in 3 patients (9%). A total of 31 patients had cardiac catheterization with or without echocardiography, whereas 4 patients had echocardiography only. The shunt ratio was available for 24 patients, with a mean value of 41.0% ± 24.1%.
A total of 32 patients (92%) were followed up either at our outpatient clinic or at other cardiac centers. Telephone calls were made and questionnaires were sent to patients for data collection when necessary. Preoperative and postoperative AR for both hospital discharge and late follow-up were evaluated; AR and was graded on a scale of 0 to IV by angiographic or echographic evaluation. Late results of AR by echocardiography were obtained for 32 patients (92%) and the follow-up periods for of surviving patients ranged from 2 to 34 years (mean 16.2 ± 9.4 years).
Operative procedure
Surgical repair was carried out by using cardiopulmonary bypass in all patients. The circulatory arrest technique was not necessary in our series. Moderate hypothermia was used between 1975 and 1994, while a tepid temperature has been used since 1995. Cold antegrade crystalloid cardioplegia was used until 1991. Since then, cold antegrade and retrograde blood cardioplegia have been used. In patients with significant shunting, such as a large ruptured sinus of Valsalva aneurysm, direct cannulation of the cardioplegic solution into coronary ostia was used. The mean aortic cross-clamp time was 59 ± 36 minutes (range 15 to 136 minutes) and the mean bypass time was 100 ± 44 minutes (range 42 to 183 minutes).
Surgical findings
The origins of the aneurysms of the sinus of Valsalva and the communicating cardiac chambers are shown in Table 1.
There was 1 patient whose aneurysm ruptured into the left atrium. One patient’s aneurysm originated from the right coronary sinus, which was bisaccular, but only one of the saccules ruptured. A total of 21 patients (60%) had coexisting cardiac lesions, including VSD in 19 and atrial septal defects (ASD) in 2. Of the 19 patients with VSD, 18 (95%) had a conus defect and one had a perimembranous defect. Significant tricuspid regurgitation was noted in 2 patients, necessitating annuloplasty. Three patients had had a cardiac operation before, including ASD closure in one, VSD closure in one, and ASD and VSD closures in one.
Surgical methods
Surgical approaches for the 35 patients were as shown in Table 2. In the early part of the series, 7 patients had repair of RSVA through the chamber of the rupture only, including right ventriculotomy in 6 and right atriotomy in 1. Beginning in 1968, a combined approach through the chamber of the rupture and aortotomy was used in all except 1 patient whose aneurysm ruptured into the left atrium. This patient had repair through aortotomy only. Eight of 27 patients with the combined approach had double fistula closure, one from the cardiac chamber and the other from the aortic side in the early years. The closure of RSVA and VSD is shown in Table 3.
Direct closure of RSVA was done in 19 patients in the early part of the series and VSD was closed directly in 4 patients with pledgeted mattress sutures. Since 1988, patch closure of RSVA has been used, whereas VSD has been closed with a patch of eitherTeflon (Impra Inc, subsidiary of L.R. Bard Tempe, AZ) or Dacron (C.R. Bard, Haverhill, PA) since 1978. For 2 patients, one patch was used to close both RSVA and VSD. No patient required aortic valve replacement (AVR) or repair, including patients with grade II AR before the initial operation. Other procedures associated with RSVA were tricuspid annuloplasty in 2 patients and ASD closure in 2 patients.
Statistical methods
The data were analyzed using StatView for Macintosh, version 5.01 (SAS Institute, Cary, NC). Time-related analyses of survival and freedom from AR grade III or more were performed with the Kaplan-Meier method. The log-rank test was used for statistical analysis, and p values of less than 0.05 were considered significant. For multivariate analyses, we used the Cox regression model with risk factors that were significant or those with p values of less than 0.1 obtained by univariate analysis. Data are presented as the mean ± standard deviation, and proportional data are presented with their 95% confidence intervals (CI).
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Results
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There was no early death after repair of RSVA. Late death occurred in 1 patient (2.8%), 20 years after operation (patient 4 in Table 4).
This patient was noted to have grade III AR, and died suddenly while waiting for reoperation. Two patients (5.7%) had reoperations because of deteriorated AR. Two patients had minor residual fistulas. The actuarial survival rate was 93% at 20 years.
Changes in aortic regurgitation and reoperation
Preoperative examination revealed that 2 patients had mild AR (grade II), whereas 7 patients had trivial to mild aortic regurgitation (grade I) and the rest of the patients had trivial or no aortic regurgitation. No patients required aortic valve replacement or repair at the initial operation. Postoperatively, AR deteriorated in 2 patients, 1 from grade 0 to II and the other from grade 0 to I. The former patient required AVR 21 years later (patient 2 in Table 4). In the rest of the patients, AR remained the same at hospital discharge, including grade II in 2 patients and grade I in 2. During the long-term follow-up, 5 patients were noted to have AR of more than grade III. Two patients had AR that increased from grade 0 at hospital discharge to I at late follow-up and 1 from grade I at hospital discharge to II at late follow-up, whereas the others have not shown increased AR during the follow-up so far. Table 4 represents 5 patients who developed hemodynamically significant AR (grade III or more) at late follow-up. Patient 1, with active infective endocarditis, had initial repair with patch closure of the defect, but required aortic valve repair 2 years later because the AR deteriorated from grade I to grade III and the recurrence of a minor fistula. Patient 2 was noted to have grade III AR 21 years after the initial repair and required AVR with a mechanical valve, as mentioned above. Patient 4 was noted to have grade III AR 20 years after surgery and this patient died suddenly while waiting for reoperation, presumably due to arrhythmia. Two other patients (patients 3 and 5) were noted have grade III AR at 26 and 7.5 years after surgery, and both are currently under observation in our outpatient clinic. Both patients had grade II AR before the initial operation and it remained at hospital discharge, but both patients’ AR deteriorated gradually. In the 5 patients whose AR deteriorated to grade III, the origin of the aneurysm was the right coronary sinus in 4 and left coronary sinus in 1, and the chamber that ruptured was the right ventricle in 4 and right atrium in 1. The RSVA was repaired by a combined approach through the ruptured chamber and the aorta. Actuarial freedom from postoperative AR grade III or more was 93% at 10 years and 87% at 20 years (Fig 1).
Actuarial freedom from events, including reoperation and cardiac death, was 97% at 10 years and 90% at 20 years (Fig 2).
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Fig 1. Freedom from aortic valve regurgitation (AR) grade III or greater after surgical repair of ruptured sinus of Valsalva aneurysm.
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Fig 2. Freedom from events, including reoperation and cardiac death, after surgical repair of ruptured sinus of Valsalva aneurysm.
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Factors influencing late aortic regurgitation
Tables 5 and 6
list factors that were found to influencing late aortic regurgitation. Age, sex, infective endocarditis, the functional class, shunt ratio, origin of the sinus, chamber ruptured, approach, method of closure, presence of VSD, and preoperative AR and postoperative AR at hospital discharge were analyzed with regard to long-term results of AR. Preoperative infective endocarditis was noted in 3 patients, 1 of whom had active endocarditis; of these patients, 2 had AR grade I (1 with active endocarditis) and the third did not have AR. The organisms of the bacterial endocarditis were staphylococcus epidermidis, streptococcus viridans, and the other was unknown. Two patients had VSD and the other had ASD, which were closed with patches. One patient required aortic valve repair 2 years after the initial repair as mentioned above, whereas no deterioration of AR was noted in the other 2 patients. As shown in Tables 5 and 6, univariate analysis revealed that preoperative AR and postoperative AR at hospital discharge were the only independent determinants for long-term results of AR, whereas other variables were not significantly correlated.
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Table 5. Univariate Relations Between Categoric Variables and Grade III Aortic Valve Regurgitation at Late Follow-Up
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Table 6. Univariate Relationships Between Continuous Variables and Grade III Aortic Valve Regurgitation at Late Follow-Up
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Three variables with p values of less than 0.1 were included in the Cox proportional hazards regression analysis: the method of RSVA closure, preoperative AR, and postoperative AR at hospital discharge. The results indicated that postoperative AR at hospital discharge (p = 0.048, odds ratio = 17.3, 95% CI = 1.02 to 192.2) was the only independent variable related to the late deterioration of AR after RSVA repair in this series.
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Comment
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The morphology of RSVA was described by Edward and associates [11] in 1957, showing the separation of the aortic media of the sinus from the media adjacent to the hinge line of the aortic valve cusp. The congenital weakness in this region, which results from the absence of these tissues, gradually gives way under aortic pressure to form an aneurysm. The operative results for RSVA have been excellent, as has been shown in our [4] and other reports [1, 5, 6, 8,12–17] since it was first repaired successfully by Lillehei and colleagues [7] in 1957. The postoperative long-term survival rate has also been excellent. In this study, we confirmed that the operative results and the postoperative long-term survival were both excellent. The risks for a recurrent fistula or VSD are reported to be minimal in the current era; however, some patients develop hemodynamically significant AR after repair of RSVA, and the recent literature [6, 8, 10] reported that 0% to 30% of operative survivors required reoperation on the aortic valve because of deteriorated AR. Therefore, the progression of AR is an important factor in determining long-term results. In our series, 5 of 35 patients developed grade III AR at late follow-up and three of them were noted to have grade III AR even 20 years after operation (Table 4). The gradual deterioration of AR was also suggested by another report [6] in which 2 patients required reoperation 25 and 31 years later.
Preoperative risk factors affecting late AR
Because the size of each study was small, the risk factors influencing late AR were not clarified. Preoperative infective endocarditis [8], the coexistence of VSD [6, 9], a bicuspid aortic valve [10], the surgical approach [6], and methods of closure [14, 17] have been reported to be possible risk factors affecting late AR or recurrence of the fistula. Some [6, 14] have suggested that aortic valve abnormalities and AR are common in patients with VSD, and Van Son and associates [6] reported that AR developed after surgery for sinus of Valsalva to right ventricle fistulas with associated VSD. Another report [8] stated that infective endocarditis may be a risk factor for late AR. One of the 3 patients with this condition in our series required urgent operation during the active stage. This patient had a recurrence of the fistula and AR deteriorated, requiring reoperation 2 years later, possibly due to inadequate debridement of infected tissue. As suggested by others [8, 10], debridement of infected tissue and pericardial patching of the attenuated sinus and annular tissue could potentially prevent recurrence of the fistula and AR.
In comparison with white patients, Oriental patients have a higher incidence of RSVA in general, a higher ratio of right sinus to right ventricle fistulas compared with right sinus to right atrium fistulas, and also a higher incidence of subarterial VSD, whereas the incidence of associated cardiac anomalies other than subarterial VSD is lower [1, 4]. In addition, recent reports have indicated that aortic valve abnormalities and incompetence are common in patients with RSVA. Van Son and associates [6] reported an incidence of bicuspid aortic valve of 12.9% and Azaki and associates [10] reported an incidence of bicuspid aortic valves of 21%, and the latter authors suggested there was a trend to late aortic valve repair failure in the presence of a bicuspid aortic valve. In our series, as reported by others for Oriental populations [1, 8], no patient noted to have a biscuspid aortic valve. Although it has not been pointed out, the incidence of bicuspid aortic valves may be significantly different between Oriental and Western populations.
Surgical procedure affecting late AR
Three different operative approaches have been described for correction of RSVA. It can be done through the cardiac chamber into which the aneurysm has ruptured [18] or by opening the aortic root only [5, 19]. However, the most widely accepted approach is through the aortic root and the chamber into which rupture has occurred [4, 6, 7, 20]. We performed repairs through right atriotomy or right ventriculotomy early in our series (n = 7); however, since 1968, we have used a combined approach [4]. Our current strategy is to use a combined approach and close the defect either from the involved chamber or the aortic root. Although the defect was closed from the involved chamber only in most cases, we believe that aortotomy is useful for inspection of the aortic root and valve so as to avoid distortion of the valve and a residual fistula.
With regard to the method of repair, some authors [6, 17, 21] recommended closing thin or large RSVAs with patches because of the histologic etiology of the lesion where the deficiency of elastic tissue between the annulus and aortic media extends 4 to 5 mm beyond the apparent rupture, whereas others [8] reported that patching is not always necessary, and that frequently a buttressed closure may be sufficient. In our experience, although we have routinely closed defects with patches since 1988, this retrospective study suggests that primary closure of the defect is not associated with a significantly higher incidence of recurrent fistulas or aortic regurgitation after RSVA repair.
AR at initial operation
Associated lesions are common in patients with congenital RSVA. Aortic valve regurgitation occurs in 17% to 75% of patients with RSVA, and VSD, either subarterial or perimembranous, occurs with comparable incidences of 30% to 50%. Most published reports indicate that the aortic valve requires replacement in 7% to 26% of cases of RSVA, and 18% to 60% of cases of RSVA with significant AR [2, 6, 8–10, 13]. In our series, however, grade I AR or higher occurred in 23% of all patients, and in 40% of patients with VSD, and no AVR was required. This low incidence and low grade of AR in patients with RSVA may be attributable to early diagnosis, or a deviation of the patient population in our series. Because the progression of untreated mild AR is so gradual and stable for more than 10 to 20 years in the presence of a pliable and normal cusp, mild AR may be left as such.
Conclusions
Both the operative results and long-term survival are excellent after repair of RSVA. The risk for a recurrent fistula is minimal; however, late AR necessitating reoperation is still a significant risk in the long-term follow-up. Although our study was clearly limited by the small sample size, no particular risk factor of preoperative conditions and surgical methods was elucidated, and postoperative AR at discharge from hospital was the only factor predicting the long-term results of AR. Because the progression of AR is so gradual, long-term follow-up of more than 20 to 30 years is mandatory.
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Abstract
Introduction
