Percutaneous Device Closure of Ruptured Sinus of Valsalva Aneurysm: A Preliminary Experience

Abstract: Objective. To evaluate the immediate and mid-term outcomes of percutaneous device closure using patent ductus arteriosus (PDA) or ventricular septal defect (VSD) occluders in patients with ruptured sinus of Valsalva aneurysm (RSVA). Methods. Ten patients (6 males and 4 females; age range, 19-63 years) were selected for the percutaneous device closure of RSVA between January 2005 and September 2011. In 2 patients, RSVA originated from the right coronary sinus (RCS) and ruptured into the right atrium (RA); in 6, from the RCS into the right ventricle, including 2 with very small VSDs; and in 2, from non-coronary sinus into the RA. Results. The defects were angiographically found to be 7-15 mm at their narrowest end. All the defects were successfully occluded using either a PDA occluder 2-4 mm larger than the narrowest end or a VSD occluder 3-5 mm larger than the narrowest end. No coronary arteries were checked for patency after closure. Immediately postoperative or follow-up echocardiography showed neither significant residual shunt nor severe aortic regurgitation (AR). Clinically, the patients developed no device embolism, infective endocarditis, or hemolysis. One patient was found to have newly-developed AR at the first follow-up at 1 month. The RSVA had been closed with a PDA occluder. One case suffered from periprocedural myocardial infarction without any symptoms. Conclusion. It is feasible and effective to use percutaneous device closure of RSVA with a PDA or VSD occluder.

J INVASIVE CARDIOL 2013;25(10):492-496

Key words: percutaneous closure, ruptured aneurysm, sinus of Valsalva

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Ruptured sinus of Valsalva aneurysm (RSVA) occurs rarely, with an incidence higher in Asian versus Western populations.¹ The most common cause of sinus of Valsalva aneurysm (SOVA) is the congenital deficiency of elastic and muscular tissues in the middle layer. Under continuous aortic pressure, the wind-sock like aneurysm ultimately ruptures into a heart chamber. Other acquired causes include infection, operation, and degeneration. RSVA originates mostly from the right coronary sinus (RCS), followed by the non-coronary sinus (NCS), but rarely from the left coronary sinus. The patients often remain asymptomatic before the SOVA ruptures. When it ruptures, however, nearly 80% of the patients develop exertional dyspnea, tachycardia, or heart failure at different degrees of severity.²

The traditional treatment involves surgical repair with a patch closure at both ends under cardiopulmonary bypass. Percutaneous device closure of RSVA is an alternative with the merits of smaller trauma. Since Cullen reported the first case of transcatheter closure of RSVA,³ different kinds of closure devices have been applied.^3-11 However, most of them were reported in the form of anecdotal cases. In the current study, we investigated our preliminary experiences with a consecutive series of patients with percutaneous device closure using a patent ductus arteriosus (PDA) or ventricular septal defect (VSD) Amplatzer-type occluder.

Methods

Between January 2005 and September 2011, a total of 10 patients (6 males and 4 females; age range, 19-63 years) were selected for percutaneous device closure of RSVA. The 10 patients were classified into New York Heart Association (NYHA) class 1 (n = 2), class 2 (n = 2), class 3 (n = 4), and class 4 (n = 2). The condition was congenital in 9 patients and due to the complication of previous infective endocarditis in 1 patient. All patients underwent a clinical examination, electrocardiography, chest x-ray, transthoracic echocardiography (TTE), and transesophageal echocardiography (TEE) if necessary. Only those without associated defects requiring surgical correction or concurrent infective endocarditis were selected for percutaneous device closure. Written informed consent was obtained from every patient.

The procedure was performed under local anesthesia with the guidance of fluoroscopic and TTE. Unfractionated heparin (100 U/kg body weight) was intravenously administered. Seldinger technique was used to achieve percutaneous access to the right femoral artery and vein so that two hemostatic sheaths were inserted. Routine right and left heart catheterization was performed, respectively, and Qp/Qs was calculated. A 5 Fr Pigtail catheter was then introduced to perform left ventricle angiography to screen for VSD and supravalvular aortography to evaluate aortic regurgitation (AR). An aortogram in the left anterior oblique and right anterior oblique views delineated the opening, dimension, and fistular connection to the cardiac chamber. The narrowest ruptured site was measured. A 6 Fr right coronary artery catheter was advanced into the ascending aorta, over a 0.035˝ angled-tip glidewire (Terumo) to cross the ruptured site. An Amplatz gooseneck snare (Microvena) was attached to the glidewire, which was exteriorated via the femoral vein. Thus, a stable arteriovenous guidewire loop was established. 

According to the measurement in the aortogram, a PDA occluder (Shanghai Memory Alloy Material Co, Ltd; Figure 7) 2-4 mm larger than the narrowest diameter of the ruptured site was chosen to close most of the lesions except for 2 cases whose right ventricular outflow was relatively narrow; a VSD occluder (Shanghai Memory Alloy Material Co, Ltd; Figures 3 and 4) 1-3 mm larger than the narrowest diameter was chosen. The appropriately sized occluder was then introduced to the ruptured site through the delivery sheath placed over the arteriovenous guidewire loop. The entire assembly was pulled back until the disk blocked the aortic end of the ruptured site without slipping into the aneurysm. 

TTE and aortography were performed to make sure that there was no significant residual shunting, severe AR, tricuspid regurgitation or encroachment on coronary artery ostia. Only then was the occluder released from the delivery cable. After the procedure, prophylactic antibiotics were administered for 2 days. Each patient underwent TTE before discharge, and received acetylsalicylic acid in the dose of 3-5 mg/kg on a daily basis for 6 months. The patients underwent chest x-ray, electrocardiogram, and TTE at the follow-up intervals of 1, 3, 6, and 12 months.

Results

Table 1 shows that by echocardiography, RCS ruptured into the RA in 2 patients, into the RV inflow in 1, RCS drained into RV outflow in 5, and NCS into RA in 2. Of the 6 patients with RCS draining into the RV, 2 were accompanied by very small subaortic VSDs, and another 2 by mild AR. Measured by aortogram, the mean diameter of the narrowest ruptured site was 10.1 ± 2.7 mm. Qp/Qs ranged from 1.4 to 3.1. Seven patients revealed mild-to-moderate pulmonary hypertension, while the other 4 were within normal range. It was confirmed by aortogram that cases #2 and #7 (with relatively narrow RV outflow) were closed with the VSD occluders implanted (Figures 1, 2, 3, and 4). Their waist diameters were 10 mm and 14 mm, respectively. The other 8 cases were closed with PDA occluders (Figures 5, 6, and 7), with waist diameters ranging from 9-18 mm. RSVA occlusion was successful in all patients. The mean procedure time was 60 ± 42 minutes and mean fluoroscopy time was 24 ± 8 minutes.

Slight residual shunt was found in 1 patient, but disappeared the next day, as revealed by TTE. There were no cases of infective endocarditis, thromboembolism, hemolysis, or other complications. The mean hospitalization duration was 6.3 ± 2.4 days. All patients were discharged 72 hours after the procedure except for 1 patient (case #6), whose echocardiography revealed decreased LV function on the first day post procedure. The patient was free of symptoms, but electrocardiogram revealed inversion of T waves in lead V1-V2 and there was a moderate elevation of cTnT from 0.022 ng/mL to 0.084 ng/mL, with NT-proBNP decreasing from 6236 ng/mL to 1804 ng/mL. At 6 months, the patient’s echocardiography showed decreased movement of the inferoposterior wall with a left ventricular ejection fraction (LVEF) of 50% (Table 1). 

The follow-up period of all the subjects ranged from 13-48 months. The clinical symptoms of 8 patients with NYHA 2 through 4 improved significantly, to class 2 in 3 cases and to class 1 in the other 5 cases. TTE showed that LA and LV end-diastolic diameters decreased significantly compared to before the procedure (P<.05), and that the systolic pulmonary artery pressure was below 40 mm Hg in the 10 patients. One patient developed new-onset AR, and 2 maintained mild AR. In addition, TTE indicated that the RV outflow tract slightly narrowed in 1 patient, but without hemodynamic impairment (Table 2).

Discussion

SOVA, a congenital abnormality, occurs rarely. When it ruptures, mostly to the right heart resulting in left-to-right shunt, the patients may experience severe heart failure. Although conventional surgical correction under cardiopulmonary bypass carries low mortality, postoperative septicemia, infective endocarditis, and prolonged recovery time make percutaneous device closure an attractive alternative. Cullen et al attempted the first percutaneous closure of RSVA using Rashkind umbrella in 1994.³ Since then, Gianturco coil, Amplatzer PDA occluders (ADO), and Amplatzer VSD occluders have been applied in clinical practice.^4-11 Because of the potential arterial damage using that route, Rashkind umbrella and Gianturco coil failed to be widely used. ADO by venous route have been recommended for ruptured SOVA with diameters <12 mm. The largest waist of Chinese-made PDA occluders for closure of RSVA with diameters ≥12 mm is 30 mm.

In 4 patients, 12-15 mm shunts were successfully closed with Chinese-made PDA occluders with waists 14-18 mm in diameter. Hence, it is feasible and safe to occlude larger RSVAs with domestically manufactured PDA occluders. Case #5 resulted in some AR at 1 month. The RSVA had been closed with a PDA occluder and immediate aortography after closure showed that the PDA occluder appeared somewhat big. 

In the current study, VSD occluders, made in China similar to Amplatzer occluders, were applied to 2 patients with relatively narrow RV outflow tract without hemodynamic significance. Its waist is only 2 mm in length to decrease the incidence of obstruction of the RV outflow tract and related hemolysis.⁵ The current attempts were found to be successful, without causing any complications. Follow-up echocardiogram showed that the diameter of RV outflow was 16 mm in 1 patient — narrower than before, but with only insignificant hemodynamic impact. There was no diameter change in the other patient. Thus, VSD occluders were demonstrated to be safe and effective in the 2 patients of RSVA with relatively narrow RV outflow. 

Small leaks in the sinus of Valsalva can easily be closed through a retrograde purely arterial access using a modern PDA occluder or a vascular plug (Amplatzer; St. Jude Medical). In the current study, an arteriovenous loop was applied to all patients, whose RSVA diameters were 7-15 mm, so that relatively large occluders and transport sheaths had to be used. It appeared advantageous to deliver the transport sheaths through the vein to avoid injuries to the arteries.

The coronary ostia are usually quite high in relation to SOVA. Device impingement occurs rarely. Regular selective coronary angiography was not performed in the current study. In case #6, however, the T-wave inversion in leads V1-V2 and slightly elevated cTnT on the second day after the procedure, as well as decreased movement of inferoposterior wall with LVEF 50% in TTE at 6 months, indicated possible impairment of the right coronary arteries. This case indicates that selective coronary angiography should be performed to exclude coexisting coronary artery disease or encroachment of the occluder on the coronary ostia. The respective patient refused to clear up the situation with coronary artery computed tomography angiography or selective right coronary arteriography. Another explanation could be air embolism. However, this is unlikely to cause ventricular damage without significant and protracted initial symptoms.

RSVA and VSD coexist. Two patients had subaortic VSD, 2-3 mm in diameter as indicated by ventriculography. The dynamic change caused by such a small VSD can be neglected; therefore, they were left untreated. 

The 6-day hospital stay is explained by the hospital policy to admit patients several days before the intervention for work-up and to observe them afterward for complications.

Conclusion

Our preliminary investigation suggested that percutaneous device closure of RSVA, as indicated by the encouraging short- and mid-term outcomes, can be feasible and effective in treating selected patients.

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