Percutaneous Closure of Ruptured Sinus of Valsalva Aneurysm and Atrial Septal Defect

ABSTRACT: Ruptured sinus of Valsalva aneurysm (SOVA) is a rare albeit well-described entity and coexisting defects like ventricular septal defect, aortic regurgitation and, infrequently, atrial septal defect (ASD) have been reported. Until recently, open surgical closure with cardiopulmonary bypass was the mainstay of treatment. However, transcatheter closure of isolated ruptured SOVA defects has been encouraging. We report percutaneous closure of ruptured noncoronary SOVA to the right atrium and a coexisting secundum ASD with deficient aortic margin. Our experience suggests that successful percutaneous closure of ruptured SOVA and coexisting ASD is a safe alternative to open surgery with satisfactory mid-term follow up. J INVASIVE CARDIOL 2010;22:E82–E85 Key words: transcatheter, intervention, transesophageal echocardiography, duct occluder, septal occluder Ruptured sinus of Valsalva aneurysm (SOVA) is a relatively uncommon clinical entity with a preponderance in the Asian population.¹ It occurs invariably due to a congenital weakness of tissues at the junction of the aortic media with annulus fibrosus. Depending on the site of rupture, most patients present with acute or subacute hemodynamic decompensation leading to congestive heart failure or infrequently with cardiac tamponade. Anomalies such as ventricular septal defect (VSD) and aortic regurgitation (AR) are commonly seen with ruptured SOVA. However, for unclear reasons, atrial septal defect (ASD) infrequently coexists with ruptured SOVA. Open surgical management of ruptured SOVA, especially with coexisting defects, has been the mainstay for treatment. ² Recent advances using transcatheter approaches have yielded success in selected cases of isolated ruptured SOVA. ^3–5 To our knowledge, percutaneous closure of SOVA and coexisting ASD has been reported just once. ⁶ The case described here demonstrates successful transcatheter closure of SOVA with ASD in a 37-year-old female as a staged procedure. Case Report. A 37-year-old female presented with a history of chest pain and progressively increasing dyspnea of 10 days’ duration, and on admission, was found to be in congestive heart failure (New York Heart Association [NYHA] Class IV). On physical examination, she was in respiratory distress (respiration rate: 32/minute), tachycardic (heart rate: 110 beats/minute) with wide pulse pressure. Jugular venous distension with prominent “V” waves was present. Fine crepitations were heard over both lung bases. Auscultation revealed continuous grade V/VI murmur over the lower left parasternal area. The electrocardiogram was unremarkable. A chest x-ray showed cardiomegaly with increased pulmonary vascularity. Transthoracic echocardiography followed by intraoperative transesophageal echocardiography (TEE) confirmed the presence of a ruptured noncoronary SOVA draining into the right atrium (Figures 1A and B), a large secundum ASD (22 mm at its widest dimension) with a deficient aortic rim. (Figure 2A), large combined left-to-right shunt and mild pulmonary hypertension. There was no evidence of AR or VSD. The ruptured SOVA tract measured 10 mm at the aortic end and 7 mm at the right atrial end. Since a ruptured SOVA was responsible for acute decompensation of the patient’s clinical status, transcatheter closure of this defect was prioritized. Routine laboratory investigations revealed no abnormalities and informed consent was obtained. The procedure was performed under general anesthesia with TEE guidance. On catheterization, right-heart pressures were elevated (right atrium mean 14 mmHg, right ventricle 45/16 mmHg and pulmonary artery pressure 45/25, mean 32 mmHg). Aortic pressures were 110/60 mmHg. Aortic root angiography clearly defined the SOVA defect in a RAO view (Figure 3A). As described earlier,3 the procedure entailed crossing the defect from the left side, establishing the standard arteriovenous wire loop and deploying the 12 mm x 14 mm Amplatzer duct occluder (ADO) (AGA Medical Corp., Plymouth, Minnesota) from the venous side under fluoroscopic and TEE guidance. The device was positioned across the defect, with precautions to avoid encroachment of the device on the aortic valve and coronary ostia using real-time TEE and angiography. There was no evidence of AR or residual shunt after placement of the device (Figure 1C). Anticipating a reduction in the ASD size following ruptured SOVA closure and to avoid the difficulties and risks in one-step repair of these defects, ASD closure was deferred. In the interim, our patient improved symptomatically (NYHA Class II), with a reduction in heart size and normal lung fields on follow-up chest X-rays. After 6 weeks, TEE revealed a decrease in the ASD size (from 22 mm to 18 mm) and closure was performed using a 24 mm Amplatzer septal occluder (ASD) (Figure 2B), with a balloon assist technique7 owing to the deficient aortic rim. At this time, aortic root angiography revealed no residual shunt across the device (Figure 3B). The ruptured SOVA and ASD closure procedures took 60 and 55 minutes, respectively. Owing to the proximity of the device to the coronary circulation, our patient empirically received 6 weeks of aspirin (150 mg/day) with clopidogrel (75 mg/day), followed by a 6-month prescription of aspirin (150 mg/day). Her recovery was uneventful and at a 4-month follow up, a chest X-ray (Figure 4) and TEE showed the devices in good position with no residual shunts and no evidence of any valvular regurgitation. Discussion. Ruptured SOVA is a rare clinical entity with a surprisingly high preponderance in the Asian population. ¹ Most patients present with an acute or subacute hemodynamic decompensation leading to congestive heart failure. Left untreated, such patients are likely to die. Traditionally surgery has been the mainstay of therapy for ruptured SOVA. ² Over the last four decades, we have come a long way from open surgical repair of SOVA in 1956⁸ to the first transcatheter closure using an umbrella device in 1994. ⁹ Ever since, there have been only anecdotal case reports and small series describing successful transcatheter closure of ruptured SOVA. ^3–5 However, except for the report by Cui et al, ⁶ all these have dealt with isolated ruptured SOVA without associated defects. The present case is unique in that an associated large secundum ASD with deficient aortic rim was also closed nonsurgically as a staged procedure. Echocardiography played an important role in the decision to perform transcatheter closure of both the defects. Since the aortic rim of the ASD was deficient it was evident that the ASD would straddle the aortic root and there was concern that the right atrial disk may encroach on the right atrial end of the ADO used for ruptured SOVA closure. However, echocardiography clearly demonstrated a good distance between the two defects, the right atrial rupture site of the ruptured SOVA being lower than the anteroinferior margin of the ASD. Moreover, the two defects could never be visualized in the same plane of echocardiographic viewing (Figures 1 and 2). The procedures were staged and not performed at the same time for the following reasons: i) since a ruptured SOVA was responsible for the acute hemodynamic decompensation, its closure was prioritized; ii) we anticipated a decrease in the cardiomegaly and the size of the ASD after ruptured SOVA closure and hence using a smaller ASD (the maximum ASD size did decrease from 22 mm to 18 mm); iii) waiting for 6 weeks would allow time for endothelialization of the ADO; iv) most importantly, staging decreased the complexity of the procedure. If both interventional procedures were performed at the same time, we would have required a third venous access, two for the devices and a third for the balloon assistance (in view of the deficient aortic rim). Both defects would have had to be dealt with simultaneously rather than sequentially until release of the devices for fear of dislodging the pre-placed ADO across the ruptured SOVA, especially since we contemplated the need for a large sizing balloon pressing on the interatrial septum from the RA side during the balloon assist technique used to close ASDs with a deficient aortic rim. Given the “wind-sock” morphology of the ruptured SOVA, ADO is the best-suited device for closure. In the present case, we used a 12 x 14 mm ADO since the aortic end measured 10 mm. In our experience, selecting an ADO approximately 2–4 mm larger than the aortic end of the SOVA (as measured on echocardiography or angiography) yields satisfactory results. ² At times there is a need to upsize the device to accommodate for flimsy margins of the defect and allow for complete closure. Moreover, the ADO has gained popularity as the preferred device for closure of a ruptured SOVA, as it is user-friendly, easily retrievable and repositionable. However coils as well as other Amplatzer devices, have been used by others. ² To our knowledge, only one case has reported transcatheter closure of a SOVA with an ASD.6 Unlike this report, where the patient had a ruptured right coronary SOVA draining into the right atrium with a deficient inferior margin ASD, our patient had noncoronary SOVA rupturing into the right atrium with a coexisting deficient aortic margin ASD. Although both these reports describe a two-staged repair, the rationale for this type of repair was different for these patients. Unlike us, Cui et al⁶ undertook ASD closure 9 months following ruptured SOVA closure to allow for resolution of the pulmonary hypertension. In conclusion, although there has been limited literature on repair of isolated ruptured SOVA, ^3–5 ruptured SOVA with co-existing ASD is rarer still and thus far, only one case report has described percutaneous closure of this combination. ⁶ Albeit technically challenging, our experience suggests that percutaneous closure of a ruptured SOVA and an asociated ASD is a safe and effective way to avoid sternotomy and cardiopulmonary bypass in the hemodynamically unstable patient.

References

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__________________________________________________________________- From the Department of Medicine, University of Massachusetts Medical School, Worcester, Massachusetts, and the *Department of Cardiology, Seth G.S. Medical College and KEM Hospital, Mumbai, India. The authors report no conflicts of interest regarding the content herein. Manuscript submitted September 9, 2009, provisional acceptance given September 29, 2009, final version accepted October 6, 2009. Address for correspondence: Prafulla Kerkar MD, DM, FACC, FSCAI, Professor and Chief of Cardiology, Department of Cardiology, CVTC Building, KEM Hospital, E. Borges Road, Parel, Mumbai 400012, India. E-mail: prafullakerkar@rediffmail.com