Ann Thorac Surg 1998;65:735-740
© 1998 The Society of Thoracic Surgeons
Original Articles: Cardiovascular
Aneurysm of Sinus of Valsalva Dissecting Into Interventricular Septum
Shiv Kumar Choudhary, MCh,
Anil Bhan, MCh,
Subhash Chandra Bose Reddy, DM,
Rajesh Sharma, MCh,
Vivek Murari, FRCSEd,
Balram Airan, MCh,
Arkalgud Sampath Kumar, MCh,
Panangipalli Venugopal, MCh
Cardiothoracic Centre, All India Institute of Medical Sciences Ansari Nagar, New Delhi, India
Accepted for publication September 24, 1997.
Dr Bhan, Department of Cardiothoracic and Vascular Surgery, Cardiothoracic Sciences Centre, All India Institute of Medical Sciences, New Delhi 110029, India.
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Abstract
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Background. Dissection of interventricular septum by aneurysm of the sinus of Valsalva is extremely rare. We present our experience with the management of 10 patients with this condition.
Methods. Ten patients with aneurysm of the sinus of Valsalva dissecting into the interventricular septum were managed at All India Institute of Medical Sciences, New Delhi, between May 1987 and September 1996. Conduction abnormalities and aortic insufficiency dominated the clinical picture. Eight patients underwent surgical repair. Two patients refused operation, and only permanent pacemaker implantation was done for complete heart block in both these patients.
Results. There was no hospital mortality. Follow-up ranged from 1 to 9 years. There was one late death due to carcinoma of the larynx, and 1 patient required reoperation for persistent aortic insufficiency. All other patients who underwent operation are in New York Heart Association functional class I.
Conclusions. We recommend surgical repair of this condition to deal with aortic regurgitation and to avoid the potential risk of rupture, thromboembolism, and infective endocarditis. However, surgical repair offers no guarantee against arrhythmias and conduction abnormalities.
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Introduction
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Aneurysms of the sinus of Valsalva (ASVs) are rare and account for 0.14% to 1.5% of the cardiac surgical load [1][2][3], the incidence being higher in patients of Asian origin. Aneurysm of the sinus of Valsalva dissecting into the interventricular septum is even rarer, and to date only 26 cases have been reported in the English-language literature. This report presents clinical features, surgical outcome, and follow-up of 10 such patients.
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Patients and Methods
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From May 1987 through September 1996, 10 patients with ASV dissecting into the interventricular septum were seen. The clinical profile is shown in Table 1. All patients were male, and the age ranged from 15 to 50 years (mean, 30.2 years). Palpitations (70%), syncope (50%), and dyspnea (40%) were the most common presenting features. Eight of these patients had clinical features of moderate to severe aortic regurgitation. Electrocardiographic findings were most striking in these patients (Table 2). Nine of 10 patients (90%), at one stage or another, were having abnormalities in the form of complete heart block, 2:1 atrioventricular block, right or left bundle-branch block, intraventricular conduction delay, ventricular tachycardia, or ventricular ectopics.
Chest radiographs were mostly unremarkable. Echocardiographic and cineangiographic findings are shown in Table 3. On two-dimensional echocardiography, the aneurysm was seen as a cystic mass involving membranous or upper muscular interventricular septum (Fig 1Fig 2). Cineangiography also revealed the aneurysm and its septal extension (Fig 3Fig 4Fig 5). In all cases, the right coronary sinus was the site of origin. In 1 patient, in addition to the right coronary sinus, the left coronary sinus was also involved. In 4 patients, the aneurysm walls were calcified.
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Echocardiographic and Cineangiographic Findings in Patients With Intraseptal Extension of Aneurysm of Sinus of Valsalva
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Transesophageal echocardiograms in the midesophageal view in the transverse plane (0 to 180 degrees) showing the aortic sinuses and the mouth of the aneurysm (arrow). (B) The aneurysm is burrowing into the interventricular septum. (ANE = aneurysm; AV = aortic valve; LA = left atrium; LV = left ventricle; LVOT = left ventricular outflow tract; RA = right atrium; RV = right ventricle.)
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Multiplane transesophageal echocardiograms in vertical (A) and long-axis views (B) showing the dilated sinus of Valsalva and its extension into the interventricular septum. (ANE = aneurysm; AO = aorta; AV = aortic valve; LA = left atrium; LV = left ventricle; LVOT = left ventricular outflow tract.)
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(A) Aortic root angiogram in the right anterior oblique view showing (early phase) filling of aorta (AO) with faint opacification of the aneurysm of the sinus of Valsalva (ANE). Note the calcification of the walls of the aneurysm (arrows). (B) Left coronary angiogram in the right anterior oblique view of the same patient showing displacement of the left anterior descending artery (arrows) superiorly and to the left by the dilated sinus of Valsalva.
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Aortic root angiogram in the left anterior oblique view showing the aneurysm (ANE) from the right sinus of Valsalva protruding into the interventricular septum. The plane of the interventricular septum is represented by arrows. Note the filling of the left ventricle due to aortic regurgitation.
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(A) Aortic root angiogram in the left anterior oblique view showing the aneurysm (ANE) from the right coronary sinus dissecting into the interventricular septum (arrows). The left ventricle (LV) is also filled because of regurgitation. (B) Aortic root angiogram of the same patient in the right anterior oblique view. The image of the aneurysm (ANE) is superimposed on that of the left ventricle (LV). Straight arrows represent the outline of the left ventricle, whereas curved arrows represent the outline of the aneurysm.
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Two patients (patients 5 and 7) refused operation. Eight patients were operated on. Under cardiopulmonary bypass and moderate hypothermia the aorta was cross-clamped and opened transversely. Antegrade cardioplegia was delivered through the coronary ostia. The aneurysm opening was directly closed using pledgeted, interrupted sutures in 5 patients, whereas in 3 patients a Dacron patch (C.R. Bard, Inc, Haverhill, MA) was used. In addition, the aortic valve was replaced with a prosthetic valve in 4 patients. In 1 patient (patient 10), the redundant right coronary cusp was repaired in the manner described by Trusler and associates [4].
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Results
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There was no operative mortality. The early postoperative course was uncomplicated in all the patients. In 2 patients (patients 2 and 8), who required temporary pacemaker insertion preoperatively, complete heart block persisted postoperatively and required permanent pacemaker implantation (VVI type) on the 10th and 12th days, respectively. Two of the patients who were operated on already had a permanent pacemaker. The 4 other patients were kept on ventricular demand pacing during the early postoperative period. In 1 of these patients (patient 6), electrocardiography remained normal throughout the hospitalization. The preoperative right bundle-branch block in patient 3 persisted postoperatively. Electrophysiologic study and 24-hour Holter monitoring revealed no rhythm disturbance, and no treatment was given for right bundle-branch block. Left bundle-branch block developed postoperatively in 2 patients (patients 4 and 10). Holter monitoring done on the 10th postoperative day showed an additional intraventricular conduction defect and multiple ectopics in patient 10. In another patient (patient 4), Holter monitoring revealed no rhythm disturbances, and hence no active measures were taken for these electrophysiologic changes.
Duration of follow-up ranges from 1 year to 9 years (mean, 4.1 ± 2.3 years). One patient died of carcinoma of the larynx 5 years after the operation. One patient (patient 4) was readmitted 2 months after the operation for sustained ventricular tachycardia and required electrical cardioversion followed by lignocaine and amiodarone infusion. Twenty-four–hour Holter monitoring showed repeated episodes of ventricular tachycardia. Subsequently he required chronic oral amiodarone therapy. Presently, 1 year postoperatively, he is in New York Heart Association class II, with normal sinus rhythm and persisting left bundle-branch block. In the patient in whom a Trusler repair was performed (patient 10), severe aortic regurgitation developed postoperatively; this patient was reoperated on after an interval of 6 months. The remaining patients who underwent operation are in New York Heart Association class I. All patients who underwent operation were studied postoperatively by echocardiography and aortic root angiography. Aneurysm flow was not seen in any of these patients.
The two patients who refused operation were followed up for 2 years and 5 years, respectively. Both the patients were in New York Heart Association class III at the time of their last visit, 5 and 2 years ago. Late follow-up of these patients is not available.
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Comment
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Extension of ASV into the interventricular septum is extremely rare. Since it was first reported in 1947 by Warthen [5], only 26 cases [5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25][26] have been reported in the English-language literature. Four cases reported earlier from our center [27] are not included in this count. In 23 cases (88.5%), aneurysm originated in right coronary sinus of Valsalva, whereas in 2 cases both the right and left coronary sinuses of Valsalva were involved. In 1 case, the aneurysm originated from a noncoronary sinus. Aneurysm remained unruptured in 18 cases (69.2%). In 6 cases (23.1%), it ruptured into the left ventricular cavity, whereas in 1 case it communicated with the right ventricular outflow tract. In 1 case, aneurysm ruptured in both left and right ventricles. Clinical features and electrocardigraphic findings of these patients are shown in Table 4Table 5. Twenty patients (80%) had evidence of significant aortic regurgitation.
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Presenting Clinical Features of Reported Cases1 of Aneurysm of Sinus of Valsalva With Extension Into Interventricular Septum
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Electrocardiographic Features of Reported Cases1 of Aneurysm of Sinus of Valsalva With Extension Into Interventricular Septum
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The first successful surgical repair of this condition was reported in 1976 by Heydorn and associates [13]. Subsequently 11 more patients have been operated on [14][15][16][19][20][22][23][24][25][26]. Besides closing the mouth of the aneurysm, the operation included replacement of the aortic valve in 4 cases. Of the 12 patients operated on, 3 patients died of arrhythmias, 1 in the immediate postoperative period [22] and 2 after 3 and 6 months, respectively [13][14]. In addition, 1 patient died of hemorrhage in the early postoperative period [22]. Among patients who underwent operation, permanent pacemaker implantation for complete heart block was required in 5 patients: in 3 preoperatively [16][20][26] and in 2 postoperatively [14][23]. The remaining patients who underwent operation were doing well at follow-up 6 to 36 months postoperatively.
Among patients who were not operated on, 10 patients [5][6][7][8][9][10][11][12][22] were treated for congestive heart failure but none of them survived. One patient who presented with cerebral infarction also died. In 1 patient permanent pacemaker implantation for complete heart block [21] relieved his symptoms, and the patient was doing well after 4 months. Similarly 1 patient who presented with infective endocarditis [18] was treated with antibiotics alone. Subsequent follow-up at 9 months was uneventful. In 1 patient [17] the outcome was not mentioned.
In the majority of cases, congenital ASV develops as a diverticulum into one of the cardiac structures. Involvement of the interventricular septum can be ascribed to its proximity to the right coronary sinus, especially the middle part of the right coronary sinus. Sakakibara and Konno [28][29] believed that intraseptal extension is caused by intramural rupture of a congenital aneurysm with subsequent formation of a hematoma. They considered this type of case to be representative of a pseudoaneurysm. This opinion is also shared by others [5][8][9][10][22], as no true aneurysm wall was found during autopsy studies. In its natural course this pseudoaneurysm gradually enlarges and ultimately ruptures in one of the cardiac chambers. This is evident from the fact that majority of the patients with ruptured aneurysms had a long presenting history, whereas patients with unruptured aneurysms had symptoms of short duration.
Intraseptal extension has been seen to produce conduction disturbances. This is explicable by proximity of the aneurysm to the conduction tissue [6][8][16][29][30]. Direct pressure by the expanding aneurysm has been considered responsible for atrioventricular dissociation and various types of bundle-branch blocks. However, Lee and colleagues [8] found evidence of hemorrhage and low-grade inflammatory changes in conduction tissues present near the aneurysm, and held inflammatory changes responsible for conduction disturbances. In fact, they successfully reversed the heart block in a patient with cortisone therapy. Appearance of new heart block or aggravation of preexisting block in the postoperative period after successful repair of the aneurysm also points to another mechanism besides direct pressure on conduction tissues. The occurrence of ventricular tachycardia, as mentioned earlier [13][24][31] and also present postoperatively in 1 of our patients, remains unexplained. Yuksel and associates [24] suggested that it might result from either the hemodynamic changes caused by the aneurysm or the mass effect of the aneurysm.
Aortic regurgitation, minimal or severe, is usually present whether the ASV ruptures or not. In fact, the majority of patients presented with features of aortic regurgitation only. In the reported cases, 53.8% presented with congestive heart failure as a result of aortic regurgitation. In our series also, 80% of patients were having a moderate to severe degree of aortic regurgitation. The mechanism of regurgitation was variable, but in the majority of cases it was due to thickening and deformity of the aortic valve cusps. One of our patients had a redundant aortic cusp producing severe regurgitation. Besides this annular dilatation [16], congenital absence of right coronary cusp [9] and necrosis of one or more cusps [10] have also been reported.
Besides aortic regurgitation, aortocardiac fistula, and heart block, intraseptal extension of ASV may present as a source of cerebral embolization [22] or as a focus of infective endocarditis [18].
When an aneurysm presents with aortic regurgitation or aortocardiac fistula, surgical repair of the aneurysm is mandatory. But when a patient presents with other complications in the form of complete heart block or infective endocarditis, the indications for operation are debatable. One of our patients, who had complete heart block, remained asymptomatic for 20 years on a permanent pacemaker until significant aortic regurgitation developed. A similarly satisfactory result has been reported by Lokhandwala and associates [21], who implanted a permanent pacemaker for complete heart block without carrying out surgical repair of the aneurysm. In 1 more patient, who presented with infective endocarditis, antibiotic therapy resolved all the symptoms and no subsequent treatment was required [18]. However, if the aneurysm remains untreated, there is a potential danger of its expansion, rupture, infective endocarditis, and systemic embolization. Thus surgical repair for all cases of ASV with intraseptal extension may be suggested, but at the same time, surgical repair offers no guarantee against fatal ventricular arrhythmias, as observed by other investigators [13][14] and also present in 1 of our patients.
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Abstract
Introduction
