Comparison of Reported Outcomes With Percutaneous Versus Surgical Closure of Ruptured Sinus of Valsalva Aneurysm Emy M. Kuriakose, MD, Puneet Bhatla, MD, and Doff B. McElhinney, MD* Sinus of Valsalva aneurysm is a rare cardiac malformation that stems from incomplete fusion of the aortic media and the aortic valve annulus, a weakness that may result in rupture of the sinus, large left-to-right shunt, and severe congestive heart failure. Historically, this lesion has been repaired surgically, but percutaneous closure (PC) has emerged as a therapeutic intervention over the last 20 years. We review and contrast 34 studies detailing the PC approach with 16 studies on surgical closure (SC), together comprising a total of 877 patients who were treated for ruptured sinus of Valsalva aneurysm from 1956 to 2014. Both groups had similar sites of rupture, age distribution, and clinical symptoms at presentation. Selection bias ultimately prohibits a direct comparison between the 2 groups as patients who underwent SC often had worse aortic regurgitation and more complex associated lesions, including endocarditis, bicuspid aortic valve, tunnel-type fistulous connections, larger defect size, and multiple site of rupture. In conclusion, although SC is indicated and reserved for these more complicated patients, our review of previously published reports reveals that PC in patients who are too ill to undergo bypass, with mild or no aortic regurgitation and simple associated defects (muscular ventricular septal defects, secundum atrial septal defect, small patent ductus arteriosus), can be safe, effective, and practical. Ó 2015 Elsevier Inc. All rights reserved. (Am J Cardiol 2015;115:392e398) Sinus of Valsalva aneurysm (SOVA) is a rare cardiac malformation that stems from incomplete fusion of the aortic media and the aortic valve annulus, the cause of which is a deficiency in the elastin layer of the aortic wall. A ruptured aneurysm in this location has historically been repaired surgically. However, since Cullen et al1 described the first percutaneous closure (PC) in 1994, this less invasive approach has been increasingly used and can be a viable alternative to closure in select cases.2 The rarity of SOVA and its scattered multinational incidence make it a difficult entity to track and study. We present a systemic review of 37 retrospective case studies (1994 to 2014) with a total of 136 patients who underwent PC of ruptured SOVA. We also reviewed 16 studies (1988 to 2013) from surgical cohorts, representing 741 individual patients. Although selection bias, difference in era of intervention, and limitations in long-term follow-up inevitably prohibit a direct comparison of outcomes between the 2 groups, we offer a discussion of the results and potential guidelines for management. Pathophysiology Edwards and Burchell3 described SOVA in autopsy specimens as defects above the level of the aortic cusps in
which there is lack of continuity between the aortic media and the annulus fibrosis. This may later initiate aneurysm formation, leading to local flow turbulence, laminar flow separation, or dilatation of the annulus and valve cusps. Most commonly, the right coronary or noncoronary sinuses are implicated in rupture, communicating anteriorly with the right ventricle (through the outlet septum or crista supraventricularis) or posteriorly to the right atrium.4 Rupture may be due to a gradual enlargement of a congenital defect or may be secondary to bacterial endocarditis or atherosclerosis. The incidence of acute endocarditis associated with rupture is unknown, although between 2% and 30% of patients reported remote histories of endocarditis in larger surgical series.5e7 In patients undergoing open heart surgery, SOVA is more prevalent in Asian (1.2% to 4.9%) than in Western populations (0.14% to 1.5%).4e7 A ventricular septal defect is the most commonly associated defect (49% in surgical series), along with bicuspid aortic valve, infundibular pulmonary stenosis, patent ductus arteriosus, atrial septal defect, coarctation and aortic regurgitation (AR), or aortic stenosis.7e19 Asian populations more frequently had ventricular septal defects (VSDs) of the supracristal type.6,13,14,16 Surgical Repair of Ruptured SOVA
Department of Pediatric, New York University Langone Medical Center, New York, New York. Manuscript received August 1, 2014; revised manuscript received and accepted November 4, 2014. Funding: None. See page 396 for disclosure information. *Corresponding author: Tel: 650-739-6842. E-mail address: [email protected] (D.B. McElhinney). 0002-9149/14/$ - see front matter Ó 2015 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.amjcard.2014.11.013
Ruptured SOVA was always repaired, even if patients were asymptomatic and only diagnosed when a continuous murmur was found on routine examination. Although its natural history is not well understood, asymptomatic, unruptured SOVA, usually found during unrelated imaging studies, was almost always repaired in larger surgical series, www.ajconline.org
Review/Percutaneous Versus Surgical Closure of Ruptured SOVA
393
Figure 1. The characteristics of the ruptured and drainage sites for each repair group. LA ¼ left atrium; LC ¼ left cusp; LV ¼ left ventricle; NC ¼ noncoronary cusp; PA ¼ pulmonary artery; RA ¼ right atrium; RC ¼ right cusp; RV ¼ right ventricle. (Image reproduced in black and white in print; for full color, please see the electronic version.)
and documented anecdotal cases advocate for early intervention.4,11,15,17 For example, a Jehovah’s Witness with an unruptured aneurysm had refused surgery, but over the next 4 years, he developed severe AR, extension of the aneurysm to the right and left coronary sinuses, and dilatation of the aortic annulus.4 Vural et al17 proposed that patients with unruptured, stable aneurysms should be anticoagulated, followed at 6-month intervals, and then repaired if symptoms develop or the size of the sinus is >50% of the average size of the other 2 normal Valsalva sinuses, compresses or distorts surrounding chambers or tissues, or increases in consecutive echocardiographic examinations. For the purpose of this discussion, only surgical series that investigated ruptured SOVA (n ¼ 741, 67% men) were considered. The chambers of rupture and drainage are shown in Figure 1. Until the use of routine 2-dimensional echocardiography, most patients were diagnosed with angiography. Several investigators report an echocardiographic accuracy rate of >90%, with the most error occurring in the accurate identification of the chamber of drainage. In the current era, angiography is typically reserved for characterization of coronary artery disease in older patients.5,7,8,16,20,21 Of the 656 patients in whom symptoms were reported, 88% (n ¼ 583) presented with exertional dyspnea, palpitations, fever, chest pain, fatigability, or a combination of these.9e11 Symptom onset was further characterized in 328
patients, with 36% (n ¼ 121) of patients presenting suddenly, requiring early repair. The remaining patients were repaired electively within 2 to 4 months. New York Heart Association class was reported in 517 patients, with 52% (n ¼ 269) presenting in class 3and 4, and mean Qp:Qs (reported in 140 patients) was 2.35 (range 1.9 to 2.9). Investigators chose 1 of 3 accepted operative approaches to closure: through the ruptured cardiac chamber, through an aortotomy, or by a combined approach. The ruptured sites were closed directly or with prosthetic or pericardial patches. Some investigators describe a greater incidence of recurrent fistulas in patients who underwent direct closure, although others found no differences in recurrence or AR related to surgical approach, and this remains an area of contention.7,9,10,14e18 Of the 741 patients (Table 1), early mortality was low at 1.5% (n ¼ 11). The range of follow-up tracking late deaths was extremely variable, from several months to 37 years. Abe and Komatsu14 reported a late actuarial survival rate at 25 years of 86 7%. Typical causes of late death included anticoagulation-related hemorrhage, endocarditis, other organ failure, and congestive heart failure from myocardial disease. Total event-free survival has collectively improved over the last 2 decades.8,18 When taking only studies that reported the incidence of AR (n ¼ 696 patients), the occurrence was 34% (n ¼ 236). Of patients with AR, severity was quantified in 144 and was
394
The American Journal of Cardiology (www.ajconline.org)
Table 1 Demographics and outcomes for surgical repair of ruptured sinus of Valsalva aneurysm Publication (years of recruitment)
Number of patients
Age (years)
31 22
23 (3-58) 28.5 (15-45)
12 (38%) 8 (36%)
5 (16%) not reported 5 (9%) 0
53 34 104
36 (13-65) 31.6 (7-57) 27.5 (5-62)
23 (43%) 24 (70%) 45 (43%)
14 (26%) 0 11 (32%) not reported 27 (26%) 3 (2.9%)
Abe14 (1961-87) Agrawal13 (1979-90) Au22 (1978-96) Azakie10 (1970-98) Choudhary16 (1977-96) Dong C20 (1996-01) Dong R23 (1997-07) Hamid21 (1978-93) Jung24 (1990-06) Kirali8 (1985-99) Lin25 (1980-01) Lui26 (1993-03) Murashita18 (1963-98) van Son9 (1956-93) Wang7 (1988-06) Yan5 (1980-07) TOTAL
67 43 25 56 20 17 20 35
32 29 26 33.2 28.3 33.5 37.5 27.5
31 83 100 741
29 30.7 31 30.24
Aortic regurgitation
Aortic valve New aortic repair regurgitation
(2-57) 12 (17%) 12 (18%) (11-50) 15 (34%) not reported (11-49) not reported not reported (14-64) 8 (14%) 9 (16%) (14-55) 4 (20%) 4 (20%) (22-59) 8 (47%) 8 (47%) (16-58) not reported 0 (6-64) 9 (26%) 0
Follow up (years)
Re-operation for aortic regurgitation
Progressive aortic regurgitation
Early/Late Death
11.1 7.6 5 (0.5-10)
4 (13%) 0
4 (1.3%) 0
1/4 (3.2%/13%) 1/0 (4.5%/0%)
6.5 4.9 9.2 8.3 8.2 (1-20)
2 (3.8%) 6 (18%) 0
1 (1.9%) 6 (1.8%) 2 (1.9%)
0/5 (0%/9.4%) 0/4 (0%/12%) 2/1 (2%/2%)
0 2 (4.7%) 1 (4%) 4 (7.1%) 1 (5%) 1 (5.9%) 0 2 (5.7%)
1 (1.5%) 2 (4.7%) 3 (12%) 11 (20%) 0 1 (5.9%) 0 5 (14%)
not reported 2.1 (0.1-4.9) not reported 5.7 (1-10) not reported 5.5 (0.3-15) 5 (9%) 3.8 (0.1-15) 1 (5%) 4.4 (1-13) 0 11.5 (1-21) not reported 1.3 (1-20) 1 (2.9%) 16.2 9.4
1/1 (1.5%/1.5%) 0/0 1/1 (4%/4%) 0/2 (0%/3.6%) 1/0 (5%/0%) 0/1 (0%/5.9%) 1/0 (5%/0%) 0/1 (0%/2.9%)
(3-54) 13 (42%) 5 (1.6%) not reported 25.7 (0.3-37) 4 (13%) 3 (9.7%) 0/1 (0%/3.2%) (3-69) 21 (25%) 10 (8.3%) 0 9.6 3.8 0 not reported 0/1 (0%/1.2%) (14-57) 34 (34%) 22 (22%) not reported 15.6 3.9 not reported 6 (6%) 3/2 (3%/2%) (3-69) 236/696 132/673 7/390 (1.8%) 9 (0.1-37) 27/641 (4.2%) 45/658 (6.8%) 11/24 (34%) (20%) (1.5%/3.2%)
Table 2 Demographics and outcomes for percutaneous closure of ruptured sinus of Valsalva aneurysm Publication (years of recruitment)
Publication Type
Number of patients
Age (years)
Aortic regurgitation
Arora28 (1995-03) Chang29 (2003-04) Chen30 (2005-10) Guan31 (2005-10) Kerkar27 (2004-09) Liu26 (2003-13) Sen32 (2004-08) Sivadasanpillai2 (2006-08) Szkutnik33 (2007-08) Tong34 (2004-09) Zhao35 (2004-05) Zhao36 (2000-06) Multiple1,37e60 (1994-2013) TOTAL
Case Case Case Case Case Case Case Case
8 4 7 10 20 15 8 7
24.1 (14-35) 27.2 (18-47) 43.7 (25-65) 37.8 (19-63) 29.1 (17-52) 48 (24-74) 33þ/-10 44.8 (28-62)
Case Series Case Series Case Series Case Series Case Reports
5 13 4 10 25
32.2 28.7 37 37 33
Case Series 12 Case Reports 25
136
Series Series Series Series Series Series Series Series
(18-51) (18-38) (7-57) (7-69) (5-55)
34.9 (7-69)
New aortic regurgitation
Follow up (months)
Progressive aortic regurgitation
Re-operation
0 NR NR 2 (20%) 5 (25%) NR 1 (13%) 3 (43%)
0 0 0 1 (10%) 4 (20%) 2 0 0
2-96 3-18 6-12 22 (13-48) 24 (1-60) 1-60 11.3þ/-4.1 9.3 (8-17)
0 0 0 1 (0.07%) 0 0 0 0
1 Surgical (12.5%) none none none 1 Surgical (5%) 1 Surgical (6.7%) none none
NR 4 (30%) 2 (50%) 4 (40%) 1 (4.3%)
0 NR 0 NR 2 (8.7%)
14 (9-19) 43.4 (12-60) 3 3 0-18
0 0 0 0 1 (0.07%)
9/113 (8%)
0-60
2/136 (1.5%)
22/115 (19%)
moderate to severe in 74%, usually owing to cusp prolapse of the affected sinus. In the 716 patients for whom it was documented, 19% (n ¼ 132) required concurrent aortic valve repair or replacement, typically those with moderate or severe AR.11 Aortic valve procedures were reserved only for those patients with concurrent severe aortic root dilation, moderate-to-severe AR, fibrotic changes of the valve, or involvement of multiple sinuses. Investigators were reticent
none none none none 1 Aortic valve replacement (4.3%) 4/136 (2.9%)
to intervene on minor aortic valve abnormalities as repair has been shown to increase operative mortality.4,7,11,14 Taking the 641 patients for whom the incidence of reintervention was reported, only 3.2% (n ¼ 18) had reoperation for a recurrent aneurysm and 4.2% (n ¼ 27) had reintervention for AR. New or progressive AR was found in 6.8% (n ¼ 45). Several investigators noted no progression of mild AR that was not treated at the time of SOVA closure.13,18,19
Review/Percutaneous Versus Surgical Closure of Ruptured SOVA Table 3 Device type and frequency of use Device
Number*
Size (mm)*
ADO
55 (48%)
Other PDA Occluder
39 (34%)
mVSD Device
12 (11%)
6/4 8/6 10/8 12/10 14/12 10/8 12/10 13 14/12 16/14 4 6 7 8 10 12 14 6 14 16 12 17
ASO
4 (2.6%)
Rashkind
3 (2.6%)
Coil Total
2 (1.8%) 115
Number* 3 10 16 11 12 6 5 2 9 8 1 2 1 4 1 2 1 2 1 1 1 2
(2.6%) (8.8%) (14%) (8.8%) (10%) (5.3%) (4.4%) (1.8%) (7.9%) (7%) (0.9%) (1.8%) (0.9%) (3.5%) (0.9%) (1.8%) (0.9%) (1.8%) (0.9%) (0.9%) (0.9%) (1.8%)
ADO ¼ Amplatzer Ductal Occluder (St. Jude Medical, St. Paul, MN, USA); ASO ¼ Amplatzer Septal Occluder (St. Jude Medical, St. Paul, MN, USA); mVSD ¼ muscular VSD; PDA ¼ patent ductus arteriosus. * Size and number used only where reported.
Percutaneous Closure of Ruptured SOVA Patients (n ¼ 136, 62% men) were carefully selected before PC. Contraindications were twofold: those related to the characteristics of the aneurysm and their secondary complications (moderate-to-severe AR, large defect size, fistulous or tunnel-type burrowing of the aneurysmal tract, multiple sites of rupture) and those stemming from fixed, previously existing conditions (large VSD, active endocarditis, other congenital defects warranting surgical intervention).26,27 Characteristics of the ruptured sites were similar to surgical series, although the chamber of drainage was more equally distributed between the right ventricle and right atrium (Figure 1). Likely due to selection bias, there was only a 7.3% (n ¼ 10) incidence of associated VSD, and of 121 patients for whom it was reported, none had moderate or severe AR; only 17% (n ¼ 21) had preprocedural mild AR (Table 2). New York Heart Association class and Qp:Qs (mean 2.3; range 1.2 to 4.3) were similar to surgical series. Of the 77 patients for whom it was reported, 60% (n ¼ 56) were in New York Heart Association class 3 to 4, an indication that symptom severity did not dictate the choice of closure method. In fact, there is evidence showing that PC could be a viable option in severely ill patients as at least 3 successfully repaired patients in the PC group were first denied surgical intervention—1 due to multiple reoperations and 2 due to multiorgan system failure.28,32,37 Investigators elected to use small waist double-disk nitinol ductal occluders, of which Amplatzer ductal
395
occluders (ADO; St. Jude Medical, St. Paul, Minnesota) were the most commonly deployed, followed by devices with similar designs in countries where ADO devices were too expensive or unavailable. Other less frequently used devices included muscular VSD occluders, Amplatzer septal occluders (ASO), Rashkind umbrella devices, and, rarely, coils (Table 3). Citing a shortened procedure time, Cullen et al1 originally advocated for PC from a retrograde arterial approach, although most authors have since favored transvenous closure (95%). An exchange length wire is typically inserted in retrograde fashion through the aorta and snared from the venous side to create an arteriovenous loop to gain access to the defect from the venous side. A transvenous approach may be advantageous for several reasons: shorter length from access site to defect, avoiding a tortuous catheter course around the aortic arch (through which catheter maneuverability and control across the SOVA may be difficult), and device positioning with the septal surface resting in the sinus. The mean minimal diameter of the ruptured site was 7 mm (2 to 15 mm), and accordingly, device sizes ranged 6/ 4 to 16/14 mm. The lesions were sized during the case by transesophageal echocardiography (Figure 2), and investigators always elected to use devices that were at least 2 to 4 mm larger than the defect. Five device embolizations were reported; 3 of these were retrieved and subsequently closed with other devices, whereas the remaining 2 were converted to surgical closure (SC).27,38e41 One of the largest lesions (14 mm)28 could not be closed with ASO or ADO devices, but Guan et al31 successfully closed a 15-mm lesion with a patent ductus arteriosus occluder. Only one 9-mm lesion, perhaps due to irregularity in morphology, could not be closed with the largest ADO device (16/14 mm).27 Although failures of small defect closure are difficult to predict, we offer that a large (>12 mm) lesion on transthoracic echocardiogram should prompt further investigation with computed tomography or magnetic resonance imaging before attempted PC. Devices were only released after confirming precise placement, making certain there was no significant AR, tricuspid regurgitation, right ventricular outflow tract obstruction, arrhythmia, or coronary encroachment. Acute, new postprocedural AR occurred in 9 patients (7%). Dobarro et al42 described a patient in whom severe AR developed at 1-week follow-up and required surgical aortic valve replacement. AR was believed to be related to prolapse of the noncoronary cusp caused by the weight of the ADO device, although this was not seen in any other patients. Rather, several investigators reported stability and improvement in mild AR over time.27,31,42 Notably, a few patients in India and China with small perimembranous VSDs underwent concurrent PC of the septal defect. Liu et al26 went on to recommend percutaneous VSD and SOVA closure for patients with up to mild AR and perimembranous defects, while reserving SC for those with the subaortic variety. Devices were not released after placement in 2 cases; one with ST-segment changes and another with acute moderate AR.27,33 Arora et al28 and Liu et al26 each described 1 case in which device placement caused persistent shunts and hemolysis, and these patients ultimately underwent SC.
396
The American Journal of Cardiology (www.ajconline.org)
Figure 2. Intraprocedural transesophageal echocardiogram in short axis showing a typical windsock-like appearance of a ruptured sinus of Valsalva aneurysm of the right coronary cusp into the right atrium (A) with color Doppler (B). A 7-mm ASO device is placed in the ruptured aneurysm (C). Trivial residual shunt with color Doppler (D). AoV ¼ aortic valve; LA ¼ left atrium; RA ¼ right atrium; SOVA ¼ sinus of Valsalva aneurysm. (Image reproduced in black and white in print; for full color, please see the electronic version.)
Mean follow-up duration for the PC group was 15.5 months (0 to 92). Mild early residual shunting either remained stable or had completely disappeared by early follow-up (1 to 3 months). One death occurred 6 months after PC in a patient who failed to recover from pre-closure multiorgan system dysfunction secondary to severe congestive heart failure.28 Postintervention anticoagulation typically included aspirin for 6 months, with some investigators adding clopidogrel for the first 4 to 6 weeks.29,31,38,41,43 No reports clarified the duration of recommended dental prophylaxis. Because follow-up duration was limited in many studies, longer term outcomes are not defined. Newer devices not represented in this study that could also be useful in some patients might include the Amplatzer ADOII and AVPII devices. Symmetric or nearly symmetric devices, such as these and the Amplatzer ASO or cribriform devices, can be delivered either retrograde or anterograde, as long as a sufficient delivery sheath is available, but the ADO and similar devices, which have a distal skirt, are best delivered using a transvenous approach, with the retention disk on the higher pressure aortic side of the defect. Otherwise, specific anatomic features in a given case, including the potential effects of the device on the aortic valve and conduction system, for example, are important
factors that can help guide device selection. The studies reviewed in this report typically used a single or occasionally 2 types of devices and do not allow insight into relative utility or risks/benefits of different devices. Beyond efficacy of closure, the implications of device choice and sizing could potentially include differences in how the rigid device redistributes wall stresses or otherwise alters the mechanical behavior of the adjacent sinus wall, which may be intrinsically abnormal. We are not aware of any clinical or experimental data in this regard, and studies reviewed did not provide data on postclosure sinus remodeling, but it is reassuring that no recurrent ruptures were reported in the PC group. Although conventional SC under cardiopulmonary bypass offers excellent results for closure of ruptured SOVA, it is evident from our review that PC is generally a reasonable and safe alternative. Disclosures The authors have no conflicts of interest to disclose. 1. Cullen S, Somerville J, Redington A. Transcatheter closure of a ruptured aneurysm of the sinus of Valsalva. Br Heart J 1994;71: 479e480.
Review/Percutaneous Versus Surgical Closure of Ruptured SOVA 2. Sivadasanpillai H, Valaparambil A, Sivasubramonian S, Mahadevan KK, Sasidharan B, Namboodiri N, Thomas T, Jaganmohan T. Percutaneous closure of ruptured sinus of Valsalva aneurysms: intermediate term follow-up results. EuroIntervention 2010;6:214e219. 3. Edwards JE, Burchell HB. The pathological anatomy of deficiencies between aortic root and the heart including aortic sinus aneurysms. Thorax 1957;12:125e139. 4. Takach TJ, Reul GJ, Duncan JM, Cooley DA, Livesay JJ, Ott DA, Frazier OH. Sinus of Valsalva aneurysm or fistula: management and outcome. Ann Thorac Surg 1999;68:1573e1577. 5. Yan F, Huo Q, Qiao J, Murat V, Ma SF. Surgery for sinus of Valsalva aneurysm: 27-year experience with 100 patients. Asian Cardiovasc Thorac Ann 2008;16:361e365. 6. Chu SH, Hung CR, How SS, Chang H, Wang SS, Tsai CH, Liau CS, Tseng CD, Tseng YZ, Lee YT. Ruptured aneurysms of the sinus of Valsalva in Oriental patients. J Thorac Cardiovasc Surg 1990;99: 288e298. 7. Wang ZJ, Zou CW, Li DC, Li HX, Wang AB, Yuan GD, Fan QX. Surgical repair of sinus of Valsalva aneurysm in Asian patients. Ann Thorac Surg 2007;84:156e160. 8. Kirali K, Güler M, Daglar B, Yakut N, Mansuroglu D, Balkanay M, Berki T, Gürbüz A, Isik O, Yakut C. Surgical repair in ruptured congenital sinus of Valsalva aneurysms: a 13-year experience. J Heart Valve Dis 1999;8:424e429. 9. van Son JA, Danielson GK, Schaff HV, Orszulak TA, Edwards WD, Seward JB. Long-term outcome of surgical repair of ruptured sinus of Valsalva aneurysm. Circulation 1994;90:II20eII29. 10. Azakie A, David TE, Peniston CM, Rao V, Williams WG. Ruptured sinus of Valsalva aneurysm: early recurrence and fate of the aortic valve. Ann Thorac Surg 2000;70:1470e1471. 11. Moustafa S, Mookadam F, Cooper L, Adam G, Zehr K, Stulak J, Holmes D. Sinus of Valsalva aneurysms—47 years of a single center experience and systematic overview of published reports. Am J Cardiol 2007;99:1159e1164. 12. Adams JE, Sawyers JL, Scott HW Jr. Surgical treatment for aneurysms of the aortic sinuses with aorticoatrial fistula. Surgery 1957;14:26e42. 13. Agrawal NB, Khandeparkar JM, Oswal D, Hemantkumar CJ, Tendolkar AG, Magotra RA. Surgical correction of aneurysms of sinus of Valsalva—a report of 22 cases. Indian Heart J 1993;45:479e482. 14. Abe T, Komatsu S. Surgical repair and long-term results in ruptured sinus of Valsalva aneurysm. Ann Thorac Surg 1988;46:520e525. 15. Naka Y, Kadoba K, Ohtake S, Sawa Y, Hirata N, Nishimura M, Matuda H. The long-term outcome of a surgical repair of sinus of Valsalva aneurysm. Ann Thorac Surg 2000;70:727e729. 16. Choudhary SK, Bhan A, Sharma R, Airan B, Kumar AS, Venugopal P. Sinus of Valsalva aneurysms: 20 years’ experience. J Card Surg 1997;12:300e308. 17. Vural KM, Sener E, Tas¸demir O, Bayazit K. Approach to sinus of Valsalva aneurysms: a review of 53 cases. Eur J Cardiothorac Surg 2001;20:71e76. 18. Murashita T, Kubota T, Kamikubo Y, Shiiya N, Yasuda K. Long-term results of aortic valve regurgitation after repair of ruptured sinus of Valsalva aneurysm. Ann Thorac Surg 2002;73:1466e1471. 19. Taguchi K, Sasaki N, Matsuura Y, Uemura R. Surgical correction of aneurysm of the sinus of Valsalva: a report of forty-five consecutive patients including eight with total replacement of the aortic valve. Am J Cardiol 1969;23:180e191. 20. Dong C, Wu QY, Tang Y. Ruptured sinus of Valsalva aneurysm: a Beijing experience. Ann Thorac Surg 2002;74:1621e1624. 21. Hamid IA, Jothi M, Rajan S, Monro JL, Cherian KM. Transaortic repair of ruptured aneurysm of sinus of Valsalva: fifteen-year experience. J Thorac Cardiovac Surg 1994;107:1464e1468. 22. Au W, Chiu S, Mok C, Lee W, Cheung D, He G. Repair of ruptured sinus of Valsalva aneurysm: determinants of long-term survival. Ann Thorac Surg 1998;66:1604e1610. 23. Dong R, Chen BT, Meng X, Liu TS, Song Y, Zheng JB, He YH. Longterm results of surgical repair of ruptured sinus of aortic sinus aneurysm. Zhonghua Wai Ke Za Zhi 2008;46:1913e1915. 24. Jung SH, Yun TJ, Im YM, Park JJ, Song H, Lee JW, Seo DM, Lee MS. Ruptured sinus of Valsalva aneurysm: transaortic repair may cause sinus of Valsalva distortion and aortic regurgitation. J Thorac Cardiovasc Surg 2008;135:1153e1158. 25. Lin CY, Hong GJ, Lee KC, Tsai YT, Tsai CS. Ruptured congenital sinus of Valsalva aneurysms. J Card Surg 2004;19:99e102.
397
26. Liu S, Xu X, Ding X, Liu G, Zhao X, Qin Y. Comparison of immediate results and mid-term follow up of surgical and percutaneous closure of ruptured sinus of Valsalva aneurysm. J Cardiol 2014;63:239e243. 27. Kerkar PG, Lanjewar CP, Mishra N, Nyayadhish P, Mammen I. Transcatheter closure of ruptured sinus of Valsalva aneurysm using the Amplatzer duct occluder: immediate results and mid-term follow-up. Eur Heart J 2010;31:2881e2887. 28. Arora R, Trehan V, Rangasetty UM, Mukhopadhyay S, Thakur AK, Kalra GS. Transcatheter closure of ruptured sinus of Valsalva aneurysm. J Interv Cardiol 2004;17:53e58. 29. Chang CW, Chiu SN, Wu ET, Tsai SK, Wu MH, Wang JK. Transcatheter closure of a ruptured sinus of Valsalva aneurysm. Circ J 2006;70:1043e1047. 30. Chen SP, Bai Y, Zhao XX, Qin YW. [Safety and efficacy of a domestic made small-waist ventricular septal defect occluder for transcatheter closure of ruptured aneurysm of the sinus of Valsalva]. Zhonghua Xin Xue Guan Bing Za Zhi 2012;40:298e301. 31. Guan L, Zhou D, Zhang F, Pan W, Dong L, Chen H, Ge J. Percutaneous device closure of ruptured sinus of Valsalva aneurysm: a preliminary experience. J Invasive Cardiol 2013;25:492e496. 32. Sen S, Chattopadhyay A, Ray M, Bandyopadhyay B. Transcatheter device closure of ruptured sinus of Valsalva: immediate results and short term follow up. Ann Pediatr Cardiol 2009;2:79e82. 33. Szkutnik M, Kusa J, Glowacki J, Fiszer R, Bialkowski J. Transcatheter closure of ruptured sinus of Valsalva aneurysms with an Amplatzer occluder. Rev Esp Cardiol 2009;62:1317e1321. 34. Tong S, Zhong L, Liu J, Yao Q, Shu M, Song Z. The immediate and follow-up results of transcatheter occlusion of the ruptured sinus of Valsalva aneurysm with duct occluder. J Invasive Cardiol 2014;26: 55e59. 35. Zhao SH, Yan CW, Xu NX, Jiang SL, Xu ZY, Wang C, Wu WH, Li SG, Wang H, Ye ZK. Transcatheter closure of ruptured sinus of Valsalva aneurysm using Amplatzer duct occluder. Zhonghua Xin Xue Guan Bing Za Zhi 2006;34:240e242. 36. Zhao SH, Yan CW, Zhu XY, Li JJ, Xu NX, Jiang SL, Xu ZY, Wang C, Wu WH, Hai-Bo H, Li SG, Ye ZK, Wang H. Transcatheter occlusion of the ruptured sinus of Valsalva aneurysm with an Amplatzer duct occluder. Int J Cardiol 2008;129:81e85. 37. Bijulal S, Sivadasanpillai H, Valaparambil A. Nonsurgical closure of recurrent rupture of sinus of Valsalva aneurysm in the presence of aortic prosthesis. J Invasive Cardiol 2009;21:E42eE43. 38. Fedson S, Jolly N, Lang RM, Hijazi ZM. Percutaneous closure of a ruptured sinus of Valsalva aneurysm using the Amplatzer Duct Occluder. Catheter Cardiovasc Interv 2003;58:406e411. 39. Bialkowski J, Akdeniz C, Celebi A. Transcatheter closure in two rare cases of left-to-right shunt with Cardio-O-Fix occluders. Cardiol Young 2012;22:96e99. 40. Jayaranganath M, Subramanian A, Manjunath CN. Retrograde approach for closure of ruptured sinus of Valsalva. J Invasive Cardiol 2010;22:343e345. 41. Onorato E, Casilli F, Mbala-Mukendi M, Perlasca E, Santoro F, Bortone F, Arena V. Sudden heart failure due to a ruptured posterior Valsalva sinus aneurysm into the right atrium: feasibility of catheter closure using the Amplatzer duct occluder. Ital Heart J 2005;6: 603e607. 42. Dobarro D, Sánchez Recalde A, Martín Reyes R. Severe aortic regurgitation after the closure of a ruptured congenital aneurysm of sinus of Valsalva with an Amplatzer device. EuroIntervention 2010;6: 426e427. 43. Mehta NK, Mishra N, Kerkar P. Percutaneous closure of ruptured sinus of Valsalva aneurysm and atrial septal defect. J Invasive Cardiol 2010;22:E82eE85. 44. Mahimarangariah J, Kikkeri HS, Rai KM, Nanjappa MC. Combined transcatheter device closure of ruptured sinus of Valsalva and a postsurgical residual ventricular septal defect. Catheter Cardiovasc Interv 2013;82:E803eE808. 45. Kumar P, Banerji A. Transcatheter closure of ruptured sinus of Valsalva aneurysm. Catheter Cardiovasc Interv 2010;76:774e776. 46. Altekin RE, Karakas MS, Er A, Yanikoglu A, Ozbek S, Yilmaz H. Percutaneous closure of ruptured sinus of Valsalva aneurysm with Amplatzer ductal occluder. Acta Cardiol 2011;66:657e660. 47. Srivastava A, Radha AS. Transcatheter closure of ruptured sinus of Valsalva aneurysm into the left ventricle: a retrograde approach. Pediatr Cardiol 2012;33:347e350.
398
The American Journal of Cardiology (www.ajconline.org)
48. Jean WH, Kang TJ, Liu CM, Chang CW, Tsai SK, Wang JK. Transcatheter occlusion of ruptured sinus of Valsalva aneurysm guided by three-dimensional transesophageal echocardiography. J Formos Med Assoc 2004;103:948e951. 49. Kurs¸aklıo glu H, Barçın C, Baysan O, Iyisoy A, Celik T, Köse S. Transcatheter closure of a ruptured sinus Valsalva via retrograde approach. Anadolu Kardiyol Derg 2011;11:271e272. 50. Trehan VK, Mukhopadhyay S, UmaMahesh CR, Yusuf J, Arora R. Successful transcatheter closure of ruptured sinus of Valsalva aneurysm. Indian Heart J 2002;54:720e722. 51. Mandel L, Gakhal M, Hopkins J. Percutaneous closure of recurrent noncoronary sinus of Valsalva aneurysm rupture: utility of computed tomography in procedural planning. J Invasive Cardiol 2010;22:336e338. 52. Abidin N, Clarke B, Khattar RS. Percutaneous closure of ruptured sinus of Valsalva aneurysm using an Amplatzer occluder device. Heart 2005;91:244. 53. Santoro G, Pacileo G, Bigazzi MC, Russo MG, Caianiello G, Calabrò R. Transcatheter closure of ruptured sinus of Valsalva aneurysm causing Fontan circulation failure. J Cardiovasc Med 2007;8:470e472. 54. Anzai N, Yamada M, Tuchida K, Akiyama K, Iida Y. Rupture of an aneurysm of the aortic sinus of Valsalva into the right ventricle. A case presenting an unusual hemodynamic manifestation. Cardiology 1987;74:147e150.
55. Cui W, van Bergen AH, Patel D, Javois AJ, Roberson DA. Transcatheter closure of ruptured sinus of Valsalva aneurysm and secundum atrial septal defect with limited inferior rim. Echocardiography 2008;25:208e213. 56. Rao PS, Bromberg BI, Jureidini SB, Fiore AC. Transcatheter occlusion of ruptured sinus of Valsalva aneurysm: innovative use of available technology. Catheter Cardiovasc Interv 2003;58:130e134. 57. Mizia-Stec K, Haberka M, Mielczarek M, Szymanski L, Skiba W, Szkutnik M, Białkowski J, Gasior Z. [Recurrent rupture of a sinus of Valsalva aneurysm in a patient with a bicuspid aortic valve treated with percutaneous implantation of an Amplatzer occlude]. Kardiol Pol 2008;66:344e347. 58. Chen F, Li SH, Qin YW, Li P, Liu SX, Dong J, Zhao XX. Transcatheter closure of giant ruptured sinus of Valsalva aneurysm. Circulation 2013;128:e1ee3. 59. Gaio G, Santoro G, Iacono C, Carrozza M, Cappelli Bigazzi M, Giovanna Russo M, Calabrò R. Non-surgical treatment of ruptured sinus of Valsalva aneurysm. Int J Cardiol 2006;113:e44ee45. 60. Cullen S, Vogel M, Deanfield JE, Redington AN. Images in cardiovascular medicine. Rupture of aneurysm of the right sinus of Valsalva into the right ventricular outflow tract: treatment with Amplatzer atrial septal occluder. Circulation 2002;105: E1eE2.
which there is lack of continuity between the aortic media and the annulus fibrosis. This may later initiate aneurysm formation, leading to local flow turbulence, laminar flow separation, or dilatation of the annulus and valve cusps. Most commonly, the right coronary or noncoronary sinuses are implicated in rupture, communicating anteriorly with the right ventricle (through the outlet septum or crista supraventricularis) or posteriorly to the right atrium.4 Rupture may be due to a gradual enlargement of a congenital defect or may be secondary to bacterial endocarditis or atherosclerosis. The incidence of acute endocarditis associated with rupture is unknown, although between 2% and 30% of patients reported remote histories of endocarditis in larger surgical series.5e7 In patients undergoing open heart surgery, SOVA is more prevalent in Asian (1.2% to 4.9%) than in Western populations (0.14% to 1.5%).4e7 A ventricular septal defect is the most commonly associated defect (49% in surgical series), along with bicuspid aortic valve, infundibular pulmonary stenosis, patent ductus arteriosus, atrial septal defect, coarctation and aortic regurgitation (AR), or aortic stenosis.7e19 Asian populations more frequently had ventricular septal defects (VSDs) of the supracristal type.6,13,14,16 Surgical Repair of Ruptured SOVA
Department of Pediatric, New York University Langone Medical Center, New York, New York. Manuscript received August 1, 2014; revised manuscript received and accepted November 4, 2014. Funding: None. See page 396 for disclosure information. *Corresponding author: Tel: 650-739-6842. E-mail address: [email protected] (D.B. McElhinney). 0002-9149/14/$ - see front matter Ó 2015 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.amjcard.2014.11.013
Ruptured SOVA was always repaired, even if patients were asymptomatic and only diagnosed when a continuous murmur was found on routine examination. Although its natural history is not well understood, asymptomatic, unruptured SOVA, usually found during unrelated imaging studies, was almost always repaired in larger surgical series, www.ajconline.org
Review/Percutaneous Versus Surgical Closure of Ruptured SOVA
393
Figure 1. The characteristics of the ruptured and drainage sites for each repair group. LA ¼ left atrium; LC ¼ left cusp; LV ¼ left ventricle; NC ¼ noncoronary cusp; PA ¼ pulmonary artery; RA ¼ right atrium; RC ¼ right cusp; RV ¼ right ventricle. (Image reproduced in black and white in print; for full color, please see the electronic version.)
and documented anecdotal cases advocate for early intervention.4,11,15,17 For example, a Jehovah’s Witness with an unruptured aneurysm had refused surgery, but over the next 4 years, he developed severe AR, extension of the aneurysm to the right and left coronary sinuses, and dilatation of the aortic annulus.4 Vural et al17 proposed that patients with unruptured, stable aneurysms should be anticoagulated, followed at 6-month intervals, and then repaired if symptoms develop or the size of the sinus is >50% of the average size of the other 2 normal Valsalva sinuses, compresses or distorts surrounding chambers or tissues, or increases in consecutive echocardiographic examinations. For the purpose of this discussion, only surgical series that investigated ruptured SOVA (n ¼ 741, 67% men) were considered. The chambers of rupture and drainage are shown in Figure 1. Until the use of routine 2-dimensional echocardiography, most patients were diagnosed with angiography. Several investigators report an echocardiographic accuracy rate of >90%, with the most error occurring in the accurate identification of the chamber of drainage. In the current era, angiography is typically reserved for characterization of coronary artery disease in older patients.5,7,8,16,20,21 Of the 656 patients in whom symptoms were reported, 88% (n ¼ 583) presented with exertional dyspnea, palpitations, fever, chest pain, fatigability, or a combination of these.9e11 Symptom onset was further characterized in 328
patients, with 36% (n ¼ 121) of patients presenting suddenly, requiring early repair. The remaining patients were repaired electively within 2 to 4 months. New York Heart Association class was reported in 517 patients, with 52% (n ¼ 269) presenting in class 3and 4, and mean Qp:Qs (reported in 140 patients) was 2.35 (range 1.9 to 2.9). Investigators chose 1 of 3 accepted operative approaches to closure: through the ruptured cardiac chamber, through an aortotomy, or by a combined approach. The ruptured sites were closed directly or with prosthetic or pericardial patches. Some investigators describe a greater incidence of recurrent fistulas in patients who underwent direct closure, although others found no differences in recurrence or AR related to surgical approach, and this remains an area of contention.7,9,10,14e18 Of the 741 patients (Table 1), early mortality was low at 1.5% (n ¼ 11). The range of follow-up tracking late deaths was extremely variable, from several months to 37 years. Abe and Komatsu14 reported a late actuarial survival rate at 25 years of 86 7%. Typical causes of late death included anticoagulation-related hemorrhage, endocarditis, other organ failure, and congestive heart failure from myocardial disease. Total event-free survival has collectively improved over the last 2 decades.8,18 When taking only studies that reported the incidence of AR (n ¼ 696 patients), the occurrence was 34% (n ¼ 236). Of patients with AR, severity was quantified in 144 and was
394
The American Journal of Cardiology (www.ajconline.org)
Table 1 Demographics and outcomes for surgical repair of ruptured sinus of Valsalva aneurysm Publication (years of recruitment)
Number of patients
Age (years)
31 22
23 (3-58) 28.5 (15-45)
12 (38%) 8 (36%)
5 (16%) not reported 5 (9%) 0
53 34 104
36 (13-65) 31.6 (7-57) 27.5 (5-62)
23 (43%) 24 (70%) 45 (43%)
14 (26%) 0 11 (32%) not reported 27 (26%) 3 (2.9%)
Abe14 (1961-87) Agrawal13 (1979-90) Au22 (1978-96) Azakie10 (1970-98) Choudhary16 (1977-96) Dong C20 (1996-01) Dong R23 (1997-07) Hamid21 (1978-93) Jung24 (1990-06) Kirali8 (1985-99) Lin25 (1980-01) Lui26 (1993-03) Murashita18 (1963-98) van Son9 (1956-93) Wang7 (1988-06) Yan5 (1980-07) TOTAL
67 43 25 56 20 17 20 35
32 29 26 33.2 28.3 33.5 37.5 27.5
31 83 100 741
29 30.7 31 30.24
Aortic regurgitation
Aortic valve New aortic repair regurgitation
(2-57) 12 (17%) 12 (18%) (11-50) 15 (34%) not reported (11-49) not reported not reported (14-64) 8 (14%) 9 (16%) (14-55) 4 (20%) 4 (20%) (22-59) 8 (47%) 8 (47%) (16-58) not reported 0 (6-64) 9 (26%) 0
Follow up (years)
Re-operation for aortic regurgitation
Progressive aortic regurgitation
Early/Late Death
11.1 7.6 5 (0.5-10)
4 (13%) 0
4 (1.3%) 0
1/4 (3.2%/13%) 1/0 (4.5%/0%)
6.5 4.9 9.2 8.3 8.2 (1-20)
2 (3.8%) 6 (18%) 0
1 (1.9%) 6 (1.8%) 2 (1.9%)
0/5 (0%/9.4%) 0/4 (0%/12%) 2/1 (2%/2%)
0 2 (4.7%) 1 (4%) 4 (7.1%) 1 (5%) 1 (5.9%) 0 2 (5.7%)
1 (1.5%) 2 (4.7%) 3 (12%) 11 (20%) 0 1 (5.9%) 0 5 (14%)
not reported 2.1 (0.1-4.9) not reported 5.7 (1-10) not reported 5.5 (0.3-15) 5 (9%) 3.8 (0.1-15) 1 (5%) 4.4 (1-13) 0 11.5 (1-21) not reported 1.3 (1-20) 1 (2.9%) 16.2 9.4
1/1 (1.5%/1.5%) 0/0 1/1 (4%/4%) 0/2 (0%/3.6%) 1/0 (5%/0%) 0/1 (0%/5.9%) 1/0 (5%/0%) 0/1 (0%/2.9%)
(3-54) 13 (42%) 5 (1.6%) not reported 25.7 (0.3-37) 4 (13%) 3 (9.7%) 0/1 (0%/3.2%) (3-69) 21 (25%) 10 (8.3%) 0 9.6 3.8 0 not reported 0/1 (0%/1.2%) (14-57) 34 (34%) 22 (22%) not reported 15.6 3.9 not reported 6 (6%) 3/2 (3%/2%) (3-69) 236/696 132/673 7/390 (1.8%) 9 (0.1-37) 27/641 (4.2%) 45/658 (6.8%) 11/24 (34%) (20%) (1.5%/3.2%)
Table 2 Demographics and outcomes for percutaneous closure of ruptured sinus of Valsalva aneurysm Publication (years of recruitment)
Publication Type
Number of patients
Age (years)
Aortic regurgitation
Arora28 (1995-03) Chang29 (2003-04) Chen30 (2005-10) Guan31 (2005-10) Kerkar27 (2004-09) Liu26 (2003-13) Sen32 (2004-08) Sivadasanpillai2 (2006-08) Szkutnik33 (2007-08) Tong34 (2004-09) Zhao35 (2004-05) Zhao36 (2000-06) Multiple1,37e60 (1994-2013) TOTAL
Case Case Case Case Case Case Case Case
8 4 7 10 20 15 8 7
24.1 (14-35) 27.2 (18-47) 43.7 (25-65) 37.8 (19-63) 29.1 (17-52) 48 (24-74) 33þ/-10 44.8 (28-62)
Case Series Case Series Case Series Case Series Case Reports
5 13 4 10 25
32.2 28.7 37 37 33
Case Series 12 Case Reports 25
136
Series Series Series Series Series Series Series Series
(18-51) (18-38) (7-57) (7-69) (5-55)
34.9 (7-69)
New aortic regurgitation
Follow up (months)
Progressive aortic regurgitation
Re-operation
0 NR NR 2 (20%) 5 (25%) NR 1 (13%) 3 (43%)
0 0 0 1 (10%) 4 (20%) 2 0 0
2-96 3-18 6-12 22 (13-48) 24 (1-60) 1-60 11.3þ/-4.1 9.3 (8-17)
0 0 0 1 (0.07%) 0 0 0 0
1 Surgical (12.5%) none none none 1 Surgical (5%) 1 Surgical (6.7%) none none
NR 4 (30%) 2 (50%) 4 (40%) 1 (4.3%)
0 NR 0 NR 2 (8.7%)
14 (9-19) 43.4 (12-60) 3 3 0-18
0 0 0 0 1 (0.07%)
9/113 (8%)
0-60
2/136 (1.5%)
22/115 (19%)
moderate to severe in 74%, usually owing to cusp prolapse of the affected sinus. In the 716 patients for whom it was documented, 19% (n ¼ 132) required concurrent aortic valve repair or replacement, typically those with moderate or severe AR.11 Aortic valve procedures were reserved only for those patients with concurrent severe aortic root dilation, moderate-to-severe AR, fibrotic changes of the valve, or involvement of multiple sinuses. Investigators were reticent
none none none none 1 Aortic valve replacement (4.3%) 4/136 (2.9%)
to intervene on minor aortic valve abnormalities as repair has been shown to increase operative mortality.4,7,11,14 Taking the 641 patients for whom the incidence of reintervention was reported, only 3.2% (n ¼ 18) had reoperation for a recurrent aneurysm and 4.2% (n ¼ 27) had reintervention for AR. New or progressive AR was found in 6.8% (n ¼ 45). Several investigators noted no progression of mild AR that was not treated at the time of SOVA closure.13,18,19
Review/Percutaneous Versus Surgical Closure of Ruptured SOVA Table 3 Device type and frequency of use Device
Number*
Size (mm)*
ADO
55 (48%)
Other PDA Occluder
39 (34%)
mVSD Device
12 (11%)
6/4 8/6 10/8 12/10 14/12 10/8 12/10 13 14/12 16/14 4 6 7 8 10 12 14 6 14 16 12 17
ASO
4 (2.6%)
Rashkind
3 (2.6%)
Coil Total
2 (1.8%) 115
Number* 3 10 16 11 12 6 5 2 9 8 1 2 1 4 1 2 1 2 1 1 1 2
(2.6%) (8.8%) (14%) (8.8%) (10%) (5.3%) (4.4%) (1.8%) (7.9%) (7%) (0.9%) (1.8%) (0.9%) (3.5%) (0.9%) (1.8%) (0.9%) (1.8%) (0.9%) (0.9%) (0.9%) (1.8%)
ADO ¼ Amplatzer Ductal Occluder (St. Jude Medical, St. Paul, MN, USA); ASO ¼ Amplatzer Septal Occluder (St. Jude Medical, St. Paul, MN, USA); mVSD ¼ muscular VSD; PDA ¼ patent ductus arteriosus. * Size and number used only where reported.
Percutaneous Closure of Ruptured SOVA Patients (n ¼ 136, 62% men) were carefully selected before PC. Contraindications were twofold: those related to the characteristics of the aneurysm and their secondary complications (moderate-to-severe AR, large defect size, fistulous or tunnel-type burrowing of the aneurysmal tract, multiple sites of rupture) and those stemming from fixed, previously existing conditions (large VSD, active endocarditis, other congenital defects warranting surgical intervention).26,27 Characteristics of the ruptured sites were similar to surgical series, although the chamber of drainage was more equally distributed between the right ventricle and right atrium (Figure 1). Likely due to selection bias, there was only a 7.3% (n ¼ 10) incidence of associated VSD, and of 121 patients for whom it was reported, none had moderate or severe AR; only 17% (n ¼ 21) had preprocedural mild AR (Table 2). New York Heart Association class and Qp:Qs (mean 2.3; range 1.2 to 4.3) were similar to surgical series. Of the 77 patients for whom it was reported, 60% (n ¼ 56) were in New York Heart Association class 3 to 4, an indication that symptom severity did not dictate the choice of closure method. In fact, there is evidence showing that PC could be a viable option in severely ill patients as at least 3 successfully repaired patients in the PC group were first denied surgical intervention—1 due to multiple reoperations and 2 due to multiorgan system failure.28,32,37 Investigators elected to use small waist double-disk nitinol ductal occluders, of which Amplatzer ductal
395
occluders (ADO; St. Jude Medical, St. Paul, Minnesota) were the most commonly deployed, followed by devices with similar designs in countries where ADO devices were too expensive or unavailable. Other less frequently used devices included muscular VSD occluders, Amplatzer septal occluders (ASO), Rashkind umbrella devices, and, rarely, coils (Table 3). Citing a shortened procedure time, Cullen et al1 originally advocated for PC from a retrograde arterial approach, although most authors have since favored transvenous closure (95%). An exchange length wire is typically inserted in retrograde fashion through the aorta and snared from the venous side to create an arteriovenous loop to gain access to the defect from the venous side. A transvenous approach may be advantageous for several reasons: shorter length from access site to defect, avoiding a tortuous catheter course around the aortic arch (through which catheter maneuverability and control across the SOVA may be difficult), and device positioning with the septal surface resting in the sinus. The mean minimal diameter of the ruptured site was 7 mm (2 to 15 mm), and accordingly, device sizes ranged 6/ 4 to 16/14 mm. The lesions were sized during the case by transesophageal echocardiography (Figure 2), and investigators always elected to use devices that were at least 2 to 4 mm larger than the defect. Five device embolizations were reported; 3 of these were retrieved and subsequently closed with other devices, whereas the remaining 2 were converted to surgical closure (SC).27,38e41 One of the largest lesions (14 mm)28 could not be closed with ASO or ADO devices, but Guan et al31 successfully closed a 15-mm lesion with a patent ductus arteriosus occluder. Only one 9-mm lesion, perhaps due to irregularity in morphology, could not be closed with the largest ADO device (16/14 mm).27 Although failures of small defect closure are difficult to predict, we offer that a large (>12 mm) lesion on transthoracic echocardiogram should prompt further investigation with computed tomography or magnetic resonance imaging before attempted PC. Devices were only released after confirming precise placement, making certain there was no significant AR, tricuspid regurgitation, right ventricular outflow tract obstruction, arrhythmia, or coronary encroachment. Acute, new postprocedural AR occurred in 9 patients (7%). Dobarro et al42 described a patient in whom severe AR developed at 1-week follow-up and required surgical aortic valve replacement. AR was believed to be related to prolapse of the noncoronary cusp caused by the weight of the ADO device, although this was not seen in any other patients. Rather, several investigators reported stability and improvement in mild AR over time.27,31,42 Notably, a few patients in India and China with small perimembranous VSDs underwent concurrent PC of the septal defect. Liu et al26 went on to recommend percutaneous VSD and SOVA closure for patients with up to mild AR and perimembranous defects, while reserving SC for those with the subaortic variety. Devices were not released after placement in 2 cases; one with ST-segment changes and another with acute moderate AR.27,33 Arora et al28 and Liu et al26 each described 1 case in which device placement caused persistent shunts and hemolysis, and these patients ultimately underwent SC.
396
The American Journal of Cardiology (www.ajconline.org)
Figure 2. Intraprocedural transesophageal echocardiogram in short axis showing a typical windsock-like appearance of a ruptured sinus of Valsalva aneurysm of the right coronary cusp into the right atrium (A) with color Doppler (B). A 7-mm ASO device is placed in the ruptured aneurysm (C). Trivial residual shunt with color Doppler (D). AoV ¼ aortic valve; LA ¼ left atrium; RA ¼ right atrium; SOVA ¼ sinus of Valsalva aneurysm. (Image reproduced in black and white in print; for full color, please see the electronic version.)
Mean follow-up duration for the PC group was 15.5 months (0 to 92). Mild early residual shunting either remained stable or had completely disappeared by early follow-up (1 to 3 months). One death occurred 6 months after PC in a patient who failed to recover from pre-closure multiorgan system dysfunction secondary to severe congestive heart failure.28 Postintervention anticoagulation typically included aspirin for 6 months, with some investigators adding clopidogrel for the first 4 to 6 weeks.29,31,38,41,43 No reports clarified the duration of recommended dental prophylaxis. Because follow-up duration was limited in many studies, longer term outcomes are not defined. Newer devices not represented in this study that could also be useful in some patients might include the Amplatzer ADOII and AVPII devices. Symmetric or nearly symmetric devices, such as these and the Amplatzer ASO or cribriform devices, can be delivered either retrograde or anterograde, as long as a sufficient delivery sheath is available, but the ADO and similar devices, which have a distal skirt, are best delivered using a transvenous approach, with the retention disk on the higher pressure aortic side of the defect. Otherwise, specific anatomic features in a given case, including the potential effects of the device on the aortic valve and conduction system, for example, are important
factors that can help guide device selection. The studies reviewed in this report typically used a single or occasionally 2 types of devices and do not allow insight into relative utility or risks/benefits of different devices. Beyond efficacy of closure, the implications of device choice and sizing could potentially include differences in how the rigid device redistributes wall stresses or otherwise alters the mechanical behavior of the adjacent sinus wall, which may be intrinsically abnormal. We are not aware of any clinical or experimental data in this regard, and studies reviewed did not provide data on postclosure sinus remodeling, but it is reassuring that no recurrent ruptures were reported in the PC group. Although conventional SC under cardiopulmonary bypass offers excellent results for closure of ruptured SOVA, it is evident from our review that PC is generally a reasonable and safe alternative. Disclosures The authors have no conflicts of interest to disclose. 1. Cullen S, Somerville J, Redington A. Transcatheter closure of a ruptured aneurysm of the sinus of Valsalva. Br Heart J 1994;71: 479e480.
Review/Percutaneous Versus Surgical Closure of Ruptured SOVA 2. Sivadasanpillai H, Valaparambil A, Sivasubramonian S, Mahadevan KK, Sasidharan B, Namboodiri N, Thomas T, Jaganmohan T. Percutaneous closure of ruptured sinus of Valsalva aneurysms: intermediate term follow-up results. EuroIntervention 2010;6:214e219. 3. Edwards JE, Burchell HB. The pathological anatomy of deficiencies between aortic root and the heart including aortic sinus aneurysms. Thorax 1957;12:125e139. 4. Takach TJ, Reul GJ, Duncan JM, Cooley DA, Livesay JJ, Ott DA, Frazier OH. Sinus of Valsalva aneurysm or fistula: management and outcome. Ann Thorac Surg 1999;68:1573e1577. 5. Yan F, Huo Q, Qiao J, Murat V, Ma SF. Surgery for sinus of Valsalva aneurysm: 27-year experience with 100 patients. Asian Cardiovasc Thorac Ann 2008;16:361e365. 6. Chu SH, Hung CR, How SS, Chang H, Wang SS, Tsai CH, Liau CS, Tseng CD, Tseng YZ, Lee YT. Ruptured aneurysms of the sinus of Valsalva in Oriental patients. J Thorac Cardiovasc Surg 1990;99: 288e298. 7. Wang ZJ, Zou CW, Li DC, Li HX, Wang AB, Yuan GD, Fan QX. Surgical repair of sinus of Valsalva aneurysm in Asian patients. Ann Thorac Surg 2007;84:156e160. 8. Kirali K, Güler M, Daglar B, Yakut N, Mansuroglu D, Balkanay M, Berki T, Gürbüz A, Isik O, Yakut C. Surgical repair in ruptured congenital sinus of Valsalva aneurysms: a 13-year experience. J Heart Valve Dis 1999;8:424e429. 9. van Son JA, Danielson GK, Schaff HV, Orszulak TA, Edwards WD, Seward JB. Long-term outcome of surgical repair of ruptured sinus of Valsalva aneurysm. Circulation 1994;90:II20eII29. 10. Azakie A, David TE, Peniston CM, Rao V, Williams WG. Ruptured sinus of Valsalva aneurysm: early recurrence and fate of the aortic valve. Ann Thorac Surg 2000;70:1470e1471. 11. Moustafa S, Mookadam F, Cooper L, Adam G, Zehr K, Stulak J, Holmes D. Sinus of Valsalva aneurysms—47 years of a single center experience and systematic overview of published reports. Am J Cardiol 2007;99:1159e1164. 12. Adams JE, Sawyers JL, Scott HW Jr. Surgical treatment for aneurysms of the aortic sinuses with aorticoatrial fistula. Surgery 1957;14:26e42. 13. Agrawal NB, Khandeparkar JM, Oswal D, Hemantkumar CJ, Tendolkar AG, Magotra RA. Surgical correction of aneurysms of sinus of Valsalva—a report of 22 cases. Indian Heart J 1993;45:479e482. 14. Abe T, Komatsu S. Surgical repair and long-term results in ruptured sinus of Valsalva aneurysm. Ann Thorac Surg 1988;46:520e525. 15. Naka Y, Kadoba K, Ohtake S, Sawa Y, Hirata N, Nishimura M, Matuda H. The long-term outcome of a surgical repair of sinus of Valsalva aneurysm. Ann Thorac Surg 2000;70:727e729. 16. Choudhary SK, Bhan A, Sharma R, Airan B, Kumar AS, Venugopal P. Sinus of Valsalva aneurysms: 20 years’ experience. J Card Surg 1997;12:300e308. 17. Vural KM, Sener E, Tas¸demir O, Bayazit K. Approach to sinus of Valsalva aneurysms: a review of 53 cases. Eur J Cardiothorac Surg 2001;20:71e76. 18. Murashita T, Kubota T, Kamikubo Y, Shiiya N, Yasuda K. Long-term results of aortic valve regurgitation after repair of ruptured sinus of Valsalva aneurysm. Ann Thorac Surg 2002;73:1466e1471. 19. Taguchi K, Sasaki N, Matsuura Y, Uemura R. Surgical correction of aneurysm of the sinus of Valsalva: a report of forty-five consecutive patients including eight with total replacement of the aortic valve. Am J Cardiol 1969;23:180e191. 20. Dong C, Wu QY, Tang Y. Ruptured sinus of Valsalva aneurysm: a Beijing experience. Ann Thorac Surg 2002;74:1621e1624. 21. Hamid IA, Jothi M, Rajan S, Monro JL, Cherian KM. Transaortic repair of ruptured aneurysm of sinus of Valsalva: fifteen-year experience. J Thorac Cardiovac Surg 1994;107:1464e1468. 22. Au W, Chiu S, Mok C, Lee W, Cheung D, He G. Repair of ruptured sinus of Valsalva aneurysm: determinants of long-term survival. Ann Thorac Surg 1998;66:1604e1610. 23. Dong R, Chen BT, Meng X, Liu TS, Song Y, Zheng JB, He YH. Longterm results of surgical repair of ruptured sinus of aortic sinus aneurysm. Zhonghua Wai Ke Za Zhi 2008;46:1913e1915. 24. Jung SH, Yun TJ, Im YM, Park JJ, Song H, Lee JW, Seo DM, Lee MS. Ruptured sinus of Valsalva aneurysm: transaortic repair may cause sinus of Valsalva distortion and aortic regurgitation. J Thorac Cardiovasc Surg 2008;135:1153e1158. 25. Lin CY, Hong GJ, Lee KC, Tsai YT, Tsai CS. Ruptured congenital sinus of Valsalva aneurysms. J Card Surg 2004;19:99e102.
397
26. Liu S, Xu X, Ding X, Liu G, Zhao X, Qin Y. Comparison of immediate results and mid-term follow up of surgical and percutaneous closure of ruptured sinus of Valsalva aneurysm. J Cardiol 2014;63:239e243. 27. Kerkar PG, Lanjewar CP, Mishra N, Nyayadhish P, Mammen I. Transcatheter closure of ruptured sinus of Valsalva aneurysm using the Amplatzer duct occluder: immediate results and mid-term follow-up. Eur Heart J 2010;31:2881e2887. 28. Arora R, Trehan V, Rangasetty UM, Mukhopadhyay S, Thakur AK, Kalra GS. Transcatheter closure of ruptured sinus of Valsalva aneurysm. J Interv Cardiol 2004;17:53e58. 29. Chang CW, Chiu SN, Wu ET, Tsai SK, Wu MH, Wang JK. Transcatheter closure of a ruptured sinus of Valsalva aneurysm. Circ J 2006;70:1043e1047. 30. Chen SP, Bai Y, Zhao XX, Qin YW. [Safety and efficacy of a domestic made small-waist ventricular septal defect occluder for transcatheter closure of ruptured aneurysm of the sinus of Valsalva]. Zhonghua Xin Xue Guan Bing Za Zhi 2012;40:298e301. 31. Guan L, Zhou D, Zhang F, Pan W, Dong L, Chen H, Ge J. Percutaneous device closure of ruptured sinus of Valsalva aneurysm: a preliminary experience. J Invasive Cardiol 2013;25:492e496. 32. Sen S, Chattopadhyay A, Ray M, Bandyopadhyay B. Transcatheter device closure of ruptured sinus of Valsalva: immediate results and short term follow up. Ann Pediatr Cardiol 2009;2:79e82. 33. Szkutnik M, Kusa J, Glowacki J, Fiszer R, Bialkowski J. Transcatheter closure of ruptured sinus of Valsalva aneurysms with an Amplatzer occluder. Rev Esp Cardiol 2009;62:1317e1321. 34. Tong S, Zhong L, Liu J, Yao Q, Shu M, Song Z. The immediate and follow-up results of transcatheter occlusion of the ruptured sinus of Valsalva aneurysm with duct occluder. J Invasive Cardiol 2014;26: 55e59. 35. Zhao SH, Yan CW, Xu NX, Jiang SL, Xu ZY, Wang C, Wu WH, Li SG, Wang H, Ye ZK. Transcatheter closure of ruptured sinus of Valsalva aneurysm using Amplatzer duct occluder. Zhonghua Xin Xue Guan Bing Za Zhi 2006;34:240e242. 36. Zhao SH, Yan CW, Zhu XY, Li JJ, Xu NX, Jiang SL, Xu ZY, Wang C, Wu WH, Hai-Bo H, Li SG, Ye ZK, Wang H. Transcatheter occlusion of the ruptured sinus of Valsalva aneurysm with an Amplatzer duct occluder. Int J Cardiol 2008;129:81e85. 37. Bijulal S, Sivadasanpillai H, Valaparambil A. Nonsurgical closure of recurrent rupture of sinus of Valsalva aneurysm in the presence of aortic prosthesis. J Invasive Cardiol 2009;21:E42eE43. 38. Fedson S, Jolly N, Lang RM, Hijazi ZM. Percutaneous closure of a ruptured sinus of Valsalva aneurysm using the Amplatzer Duct Occluder. Catheter Cardiovasc Interv 2003;58:406e411. 39. Bialkowski J, Akdeniz C, Celebi A. Transcatheter closure in two rare cases of left-to-right shunt with Cardio-O-Fix occluders. Cardiol Young 2012;22:96e99. 40. Jayaranganath M, Subramanian A, Manjunath CN. Retrograde approach for closure of ruptured sinus of Valsalva. J Invasive Cardiol 2010;22:343e345. 41. Onorato E, Casilli F, Mbala-Mukendi M, Perlasca E, Santoro F, Bortone F, Arena V. Sudden heart failure due to a ruptured posterior Valsalva sinus aneurysm into the right atrium: feasibility of catheter closure using the Amplatzer duct occluder. Ital Heart J 2005;6: 603e607. 42. Dobarro D, Sánchez Recalde A, Martín Reyes R. Severe aortic regurgitation after the closure of a ruptured congenital aneurysm of sinus of Valsalva with an Amplatzer device. EuroIntervention 2010;6: 426e427. 43. Mehta NK, Mishra N, Kerkar P. Percutaneous closure of ruptured sinus of Valsalva aneurysm and atrial septal defect. J Invasive Cardiol 2010;22:E82eE85. 44. Mahimarangariah J, Kikkeri HS, Rai KM, Nanjappa MC. Combined transcatheter device closure of ruptured sinus of Valsalva and a postsurgical residual ventricular septal defect. Catheter Cardiovasc Interv 2013;82:E803eE808. 45. Kumar P, Banerji A. Transcatheter closure of ruptured sinus of Valsalva aneurysm. Catheter Cardiovasc Interv 2010;76:774e776. 46. Altekin RE, Karakas MS, Er A, Yanikoglu A, Ozbek S, Yilmaz H. Percutaneous closure of ruptured sinus of Valsalva aneurysm with Amplatzer ductal occluder. Acta Cardiol 2011;66:657e660. 47. Srivastava A, Radha AS. Transcatheter closure of ruptured sinus of Valsalva aneurysm into the left ventricle: a retrograde approach. Pediatr Cardiol 2012;33:347e350.
398
The American Journal of Cardiology (www.ajconline.org)
48. Jean WH, Kang TJ, Liu CM, Chang CW, Tsai SK, Wang JK. Transcatheter occlusion of ruptured sinus of Valsalva aneurysm guided by three-dimensional transesophageal echocardiography. J Formos Med Assoc 2004;103:948e951. 49. Kurs¸aklıo glu H, Barçın C, Baysan O, Iyisoy A, Celik T, Köse S. Transcatheter closure of a ruptured sinus Valsalva via retrograde approach. Anadolu Kardiyol Derg 2011;11:271e272. 50. Trehan VK, Mukhopadhyay S, UmaMahesh CR, Yusuf J, Arora R. Successful transcatheter closure of ruptured sinus of Valsalva aneurysm. Indian Heart J 2002;54:720e722. 51. Mandel L, Gakhal M, Hopkins J. Percutaneous closure of recurrent noncoronary sinus of Valsalva aneurysm rupture: utility of computed tomography in procedural planning. J Invasive Cardiol 2010;22:336e338. 52. Abidin N, Clarke B, Khattar RS. Percutaneous closure of ruptured sinus of Valsalva aneurysm using an Amplatzer occluder device. Heart 2005;91:244. 53. Santoro G, Pacileo G, Bigazzi MC, Russo MG, Caianiello G, Calabrò R. Transcatheter closure of ruptured sinus of Valsalva aneurysm causing Fontan circulation failure. J Cardiovasc Med 2007;8:470e472. 54. Anzai N, Yamada M, Tuchida K, Akiyama K, Iida Y. Rupture of an aneurysm of the aortic sinus of Valsalva into the right ventricle. A case presenting an unusual hemodynamic manifestation. Cardiology 1987;74:147e150.
55. Cui W, van Bergen AH, Patel D, Javois AJ, Roberson DA. Transcatheter closure of ruptured sinus of Valsalva aneurysm and secundum atrial septal defect with limited inferior rim. Echocardiography 2008;25:208e213. 56. Rao PS, Bromberg BI, Jureidini SB, Fiore AC. Transcatheter occlusion of ruptured sinus of Valsalva aneurysm: innovative use of available technology. Catheter Cardiovasc Interv 2003;58:130e134. 57. Mizia-Stec K, Haberka M, Mielczarek M, Szymanski L, Skiba W, Szkutnik M, Białkowski J, Gasior Z. [Recurrent rupture of a sinus of Valsalva aneurysm in a patient with a bicuspid aortic valve treated with percutaneous implantation of an Amplatzer occlude]. Kardiol Pol 2008;66:344e347. 58. Chen F, Li SH, Qin YW, Li P, Liu SX, Dong J, Zhao XX. Transcatheter closure of giant ruptured sinus of Valsalva aneurysm. Circulation 2013;128:e1ee3. 59. Gaio G, Santoro G, Iacono C, Carrozza M, Cappelli Bigazzi M, Giovanna Russo M, Calabrò R. Non-surgical treatment of ruptured sinus of Valsalva aneurysm. Int J Cardiol 2006;113:e44ee45. 60. Cullen S, Vogel M, Deanfield JE, Redington AN. Images in cardiovascular medicine. Rupture of aneurysm of the right sinus of Valsalva into the right ventricular outflow tract: treatment with Amplatzer atrial septal occluder. Circulation 2002;105: E1eE2.
