Ruptured Aneurysms of the Sinus of Valsalva

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1 Ruptured Aneurysms of the Sinus of Valsalva Ernst-Dietrich Mayer, M.D., Kai Ruffmann, M.D., Werner Saggau, M.D., Bernhard Butzmann, M.D., Karin Bernhardt-Mayer, M.D., Norbert Schatton, M.D., and Wolfgang Schmitz, M.D. ABSTRACT From 1964 to 1984,lO male and 5 female patients with ruptured aneurysms of the sinus of Valsalva (ASVs) underwent surgical correction. These procedures constituted 0.23% of the 6,350 surgical procedures that used cardiopulmonary bypass during this period. Five patients had an inflammatory condition (bacterial endocarditis, 4; syphilis, l), and 1 had an ASV relapse 5 years after her first operation. Coexistent lesions included aortic valve regurgitation in 5 patients, ventricular septal defect in 3 (1 of whom had mitral insufficiency), patent foramen ovale in 2, and atrial septal defect in 1. Ninety-three percent were symptomatic (sudden onset of symptoms, 5 patients; gradual onset, 9 patients), commonly with shortness of breath, fatigability, chest pain, and tachycardia. The following connections occurred: noncoronary sinus to right atrium (RA) (5 patients); right coronary sinus (RCS) to RA (5 patients); and RCS to right ventricle (5 patients). There were no early or late postoperative deaths. One patient underwent reoperation after an ASV relapse. The mean follow-up period (* standard deviation) was 7.9 years (range, 10 months to 20.1 years). Eighty percent of the patients were found to be in New York Heart Association class I, and 20% were in class 11. Apart from ASV relapse, late complications are determined by prosthetic valve dysfunction or evidence of valve disease. Early surgical intervention is justified in patients with ruptured ASV. Rupture of an aneurysm of the sinus of Valsalva (ASV), which to our knowledge was first reported in 1840 [l], is recognized as a rare event. Its incidence among all procedures requiring cardiopulmonary bypass (CPB) is reported to range from 0.14% [2] to 0.34% [3]. Most of these aneurysms are considered to be congenital, or to develop as a result of syndromes accompanied by degeneration of the connective tissue [4]. However, they also occur as a secondary effect of bacterial endocarditis [5] or rheumatoid heart disease [6]. ASVs are most commonly associated with other subvalvular defects, but some are isolated. ASVs have been reported in various locations and with various types of fistulas [4]. The onset of symptoms may be gradual or acute [2,4], and the rupture has even been found to be asymptomatic [7]. From the Departments of Cardiac Surgery and Cardiology, University of Heidelberg, Federal Republic of Germany. Accepted for publication Nov 16, Address reprint requests to Dr. Mayer, Pfuehlstrasse 69,7100 Heilbronn, Federal Republic of Germany. Medical management of isolated, unruptured ASV has been recommended unequivocally [8]. However, the prognosis for ruptured ASV is regarded as grave unless it is treated surgically [4, 91. Previous reports indicate that the management of this rare defect greatly affects late outcome. Material and Methods From October, 1964, through February, 1984,15 patients with ruptured ASV underwent cardiac surgical procedures at the Department of Cardiac Surgery, Heidelberg, Federal Republic of Germany. The mean age of these 10 male and 5 female subjects (-I- standard deviation) was 33.5 t 16.6 years (range, 4.5 to 60 years). Their operations constituted 0.23% of the 6,350 surgical procedures that used CPB during this 20-year period. In 4 patients (mean age, 40 years) bacterial endocarditis was present; syphilis was diagnosed in 1. In one 35-year-old woman with Marfan's syndrome, an ASV relapse occurred, 5 years after she had undergone ASV correction in another hospital. Isolated ASV occurred in 5 patients. Coexistent cardiac anomalies were present in 10 patients; 3 of these patients had an associated ventricular septal defect (VSD), one of which was combined with mitral valve regurgitation (grade 111). Aortic valve incompetence was coexistent with ASV in 5 patients (2 with grade 111 and 3 with grade IV). In 1 patient an atrial septal defect (ASD) of secundum type was found, and patent foramen ovale (PFO) was detected in 2 patients (once together with aortic valve incompetence). The fistula connected the noncoronary sinus and the right atrium in 5 patients. Perforation of the right coronary sinus was directed toward the right ventricle in 5 patients and toward the right atrium in 5 patients (Table). Except for one 34-year-old woman, all patients were symptomatic. Five patients (all with aortic valve regurgitation) experienced sudden onset of symptoms, including acute chest pain, dyspnea, fatigability, and intermittent tachycardia. In 3 of these patients these symptoms were followed by acute left heart failure. Gradual onset of symptoms occurred in 9 patients (60%), including shortness of breath and fatigability (9 patients), tachycardia (6), chest pain (3), and peripheral edema (3). In all patients a loud, classic, continuous "machinery" murmur was heard along the left sternal border, and was usually best heard at the third intercostal space. Palpitations were detected in 6 patients. Of the 5 patients with isolated ruptured ASV, 2 had normal chest roentgenograms; the other 3 had pulmonary plethora, 2 with obvious enlargement of the right side of the heart 81 Ann Thorac Surg 42:81-85, July 1986

2 82 The Annals of Thoracic Surgery Vol 42 No 1 July 1986 Data on 15 Patients with Ruptured Aneurysm of the Sinus of Valsalva ~~ Age Coexistent Preoperative Surgical Follow- Result Patient No. Sex (yr) Lesion Characteristics Fistula Approach up (yr) (NYHA) 1 M R-RV RV 20.1 I 2 M N-RA RA 18.1 I 3 M N-RA RA 15.5 I 4 M 37 VSD... R-RV RV 14.8 I1 5 M 35 VSD Relapse R-RV D 10.1 I 6 F 44 PF0,AI 4 IE N-RA D 8.8 I1 7 F R-RA D 7.7 I 8 F 21 PFO... R-RA A0 5.5 I 9 M 60 A1 4 IE N-RA D 4.9 I 10 F 57 ASD I1 Syphilis N-RA RA 4.8 I1 11 M 14 A1 3 IE R- RV A0 3.1 I 12 M 26 VSD,MI 3... N-RA RA 1.5 I 13 M 24 A1 3 IE R-RV A0 1.3 I 14 M 49 A R-RA A0 0.9 I 15 F R-RA RA 0.8 I M = male; F = female; VSD = ventricular septal defect; PFO = patent foramen ovale; A1 = aortic valve insufficiency (grades 3, 4); ASD I1 = atrial septal defect of secundum type; MI 3 = mitral valve incompetence, grade 3; IE = infective endocarditis; R-RV = from right coronary sinus to right ventricle; N-RA = from noncoronary sinus to right atrium; R-RA = from right coronary sinus to right atrium; RV = right ventriculotomy; RA = right atriotomy; D = double approach; A = aortotomy; NYHA = New York Heart Association functional class (I to IV). and prominence of the main pulmonary arterial trunk. In the 10 patients in whom further lesions were associated with ASV, the most common radiological findings included pulmonary plethora (7 patients) and rightsided cardiac enlargement (3 patients). Electrocardiographic findings in patients with isolated ruptured ASV were normal in 3 patients, but 2 patients showed signs of right ventricular strain. Coexistent lesions usually induced abnormal electrocardiograph patterns, and they led to right ventricular strain in 4 pa- Fig 1,, eft ventricular cif1eangiografn in a 57-year-old woman, showing ruptured ASV (fistula from noncoronary sinus to right atrium). (LV = left ventricle; A0 = aorta; AB = aortic bulbus; RA = right atrium; LCA = left coronary artery; RCA = right coronary artery; A = aneurysm.) tients, left ventricular strain in 2, and global ventricular strain in 3. Right-bundle branch block was detected twice, and left-bundle branch block was found in 1 patient who also had severe aortic incompetence. All patients had sinus rhythm. The mean time interval between onset of symptoms and cardiac catheterization, which had been performed preoperatively on all patients (Fig l), was years. Correct preoperative diagnosis was made in 14 (93.3%) of the patients (tentative diagnosis of VSD in 1 patient). If rupture had occurred without further coexistent shunting defect, mean left-to-right shunt was 44.3?L the shunt was 56.7 * if Other lesions with left-to-right shunt were associated. Mean pulmonary artery systolic pressure was 24.9 * 9.2 mm Hg. Mean preoperative systemic blood pressure was 133? 20.2 mm Hg (systolic)/58 lr mm Hg

3 83 Mayer, Ruffmann, Saggau, et al: Ruptured Aneurysms of Sinus of Valsalva (diastolic), and mean total pulmonary resistance was 87 * 31 dynes * sec-' * cmp5. The surgical procedures performed used CPB, moderate systemic hypothermia, and beginning in 1978, cold cardioplegia. The ruptured aneurysms were in a typical wind sock configuration in 8 patients, a funnel in 5, and a simple tract in 2. In 4 patients with isolated ruptured ASV, the aneurysm was approached through the chamber into which it was directed; closure was performed with three interrupted buttressed mattress sutures each. In 1 patient a double approach (aortotomy and right atriotomy) was performed to support repair by a further buttressed mattress suture, because the tissue of the aneurysmal edges appeared to be friable. In 5 patients needing aortic valve replacement, the surgical approach was through the aorta, using the same suture technique. In 1 patient with bacterial endocarditis, the aneurysm was also approached through atriotomy, and closure was supported with interrupted mattress sutures reinforced with Dacron pledgets. Aortic valves were replaced with Starr- Edwards, Bjork-Shiley, Carpentier-Edwards, St. Jude, and Hancock xenograft prostheses. ASV relapse was repaired with four patch sutures after right ventriculotomy, and in this instance, the VSD was closed with a Dacron patch. In 1 patient with ruptured ASV, VSD, and mitral incompetence, and in another with ruptured ASV and VSD, the aneurysm was approached in the same way (St. Jude prosthesis in the mitral position). In 1 patient with a fistula protruding into the right atrium and associated ASD, repair was performed after atriotomy by using three interrupted mattress sutures, and ASD was corrected by direct closure. In another patient with coexistent PFO, correction was made through the aorta, and PFO was also closed by direct suture. Results All 15 patients survived, and there were no early or late postoperative deaths. One female patient with a fistula terminating at the right atrium, with PFO, and in whom an aortic approach was used for repair, suffered from an ASV relapse one year after surgery. She underwent another operation in which a double approach was performed. Recovery was uneventful for all other subjects. Follow-up was continued through December, 1984, with physical, echocardiographic, electrocardiographic, and radiological examinations. The mean follow-up period was 7.9 * 6.3 years (range, 10 months to 20.1 years). Twelve patients (80%) were found to be in New York Heart Association (NYHA) functional class I (Fig 2). Seven of these 12 were working full time, 4 had returned to part-time work, and 1 had retired. Of the 3 patients in NYHA class 11, 1 man was working full time and 2 women were doing light chores. The cardiothoracic ratio was substantially reduced in all these patients (0.48 * 0.05, compared with a mean preoperative value of 0.55 * 0.06). Pathologic echocardiographic findings included septa1 hypokinesis in 2 pa- Fig 2. Changes in New York Heart Association (NYHA) functional classes from Preoperative to postoperative state in 15 patients (pts) with ruptured aneurysm of the sinus of Valsalva. tients. Aortic prosthetic insufficiency (grade I) was diagnosed in 1 patient, and moderate aortic and mitral insufficiency were suspected in another. Normal electrocardiographic patterns could be identified in 11 subjects (73%). Except for 1 patient with continuous arrhythmia, all had sinus rhythm. Left ventricular strain was observed twice; in 1 patient it was found together with right-bundle branch block. Left-bundle branch block occurred in 1 patient, and atrioventricular block (grade I) occurred in another. Mean systemic blood pressure was mm Hg (systolic)/83 * 10.6 mm Hg (diastolic). Comment ASV is regarded as a rare condition, and its incidence is reported to be even lower in the Western World than in oriental countries (9). ASV has been reported to represent less than 0.5% of all lesions requiring CPB for repair [2, 31. However, some authors have estimated that this anomaly occurs in up to 3.5% of all patients undergoing surgery for congenital heart disease [lo]. In the past the occurrence of these aneurysms was thought to be due only to inflammation. However, it is now suggested that either a congenital lack of continuity between the aortic media and the annulus fibrosus [ll], or a developmental structural defect in the aortic annulus itself [12], represents the determining factor in most cases of aneurysmal manifestation. Sometimes the cause is Marfan's syndrome or the Ehlers-Danlos syndrome [4]. Acquired types of ASV, resulting from specific inflammation with medial necrosis, play a secondary role in the era of modern antibiotic therapy. However, infective endocarditis may be the second most frequent cause of ASV. Occasionally, atherosclerosis and trauma may induce this aortic root defect [4], as may iatrogenic lesions following aortic valve replacement [13]. In 20% of our patients, rupture of ASV may have developed as a consequence of infection. Whether these aneurysms occurred before endocarditis or occurred secondary to an inflammatory condition is difficult to determine. Usually these aneurysms arise from the right sinus and erode to the right ventricle [4]. In 40% of them this constellation is reported to be associated with a supracristal VSD [7]. ASV arises most frequently from the right coronary sinus, less frequently from the noncoronary sinus, and least frequently from the left aortic sinus. If the noncoronary sinus is involved, 70% of the

4 84 The Annals of Thoracic Surgery Vol 42 No 1 July 1986 aneurysms have been observed to erode to the right atrium. In all 5 of our patients with ruptured noncoronary ASV, the aneurysm terminated in this cardiac chamber. One explanation for involvement of the left coronary sinus, an extremely rare condition, is that the left coronary artery is supporting the sinus wall by running intramurally just after its origin [4]. In patients with this condition, anginal complaints and even myocardial infarction caused by aneurysmal oppression of the proximal left coronary artery have been reported [ 141. Except for a right sinus-to-left atrial fistula, all possible variations have been described-even the formation of fistulas between an aortic sinus and more than one of the neighboring intracardiac structures [15]. In acquired ASV, extracardiac perforation may occur [16]. Symptoms caused by rupture usually depend on the direction of the fistula. Sudden onset of symptoms, including acute dyspnea and chest pain, does not represent the common clinical picture [7]. More than 50% of patients are reported to have a gradual onset of complaints, including shortness of breath, fatigability, palpitations, and tachycardia [2, 4, 7, 9, 17, 181. Rupture of ASV rarely occurs asymptomatically. In this series, 60% of the patients experienced a gradual onset of symptoms, i.e., over a period of months or years. However, the data are not sufficient to make a retrospective analysis of any relationship between physical stress and time of ASV rupture. Classically, rupture causes signs of a hyperdynamic cardiac state, with collapsing pulse and concomitant biventricular cardiac enlargement. However, considerable variations are possible. Usually a continuous murmur, best heard to the left side of the sternum, is present [15]. Radiological signs of pulmonary hypertension are frequently evident. Although rupture usually causes pathological changes, electrocardiographic findings are highly inconsistent [15]. However, in 40% of the fistulas forming toward the right atrium, right ventricular strain developed; this figure came up to most expectations. Ruptured ASVs are commonly diagnosed preoperatively. Recently, the combined use of various echocardiographical techniques has become more and more important in making an adequate diagnosis (151. Preoperative catheterization with routine aortic cineangiography, however, should still be obligatory. It is agreed that the prognosis of ASV rupture, if it is not treated surgically, is grave [9]. Some authors have suggested routine repair of the defect by using an approach through the aorta because this is the safest way to obtain closure at the aneurysmal base. Also, the risk of distortion in the aortic cusps or injury to a coronary artery is low, and accurate placement of each suture is possible [2, 71. Other authors prefer an approach through the chamber into which the fistula empties [19], and several advocate a double approach [3,4, 18). For the most part, our surgical strategy is individualized to each patient. If aortic valve incompetence necessitates aortotomy, the ASV is usually closed by using this approach. Sometimes, if infective endocarditis is present, an additional approach through the affected chamber may be necessary to support the aortal sutures. Isolated aorta-to-right atrial fistulas are repaired by using an approach through this chamber. Placement of the sutures requires great care because of the risk of aortic valve cusp injury or displacement with aortic incompetence. To avoid this, it has been suggested that all sutures be applied parallel to the long axis of the aorta [9]. Associated aortic regurgitation, which is said to be present frequently in ruptured or unruptured ASV and sometimes is due to ASV-induced displacement of aortic cusps, does not always necessitate valve replacement [12]. If the aneurysmal orifice is not too large and there is no need for patch closure, approximating the aneurysmal edges can occasionally effect a structural restoration of the aortic annulus. If the anatomical integrity of the cusps has been preserved, this procedure can make aortic valve replacement unnecessary [20]. In all our patients who had aortic incompetence, severe cusp destruction was found. Therefore, prostheses were inserted by using the prosthetic ring to reinforce the mouth of the aneurysmal sac after it had been closed. Patients with ruptured ASV are reported to have a normal life expectancy after repair [19]. This may be because no further complications related to the associated lesions that led to valve replacement would occur. Recurrence of rupture is rare, and recorrection, if performed immediately, is not known to be of higher risk than the first operation. Based on the excellent results that have been observed, even long after operation, we believe that early surgical intervention in patients with ruptured ASV is justified. References 1. Thurnham J: On aneurysms and especially spontaneous varicose aneurysms of the ascending aorta and sinuses of Valsalva. Med Chir Trans 23:323, Henze A, Huttunen H, Bjork VO: Ruptured sinus of Valsalva aneurysms. Scand J Thorac Cardiovasc Surg 17249, Meyer J, Wukasch DC, Hallman GL, et al: Aneurysm and fistula of the sinus of Valsalva. Ann Thorac Surg 19:170, Jansen EWL, Nauta ILD, Lacquet LK: Ruptured aneurysms of the Sinus Valsalvae. Thorac Cardiovasc Surg 32148, Shuhmaker HB: Aneurysms of the aortic sinuses of Valsalva due to bacterial endocarditis with special reference to their operative management. 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