Cardiovascular Revascularization Medicine 15 (2014) 165–170
Contents lists available at ScienceDirect
Cardiovascular Revascularization Medicine
Perforated Sinus of Valsalva (PSOV) aneurysm closure with a muscular VSD occluder Giuseppe Gioia ⁎, Jingsheng Zheng, Amit Ray, Mario Gioia AtlantiCare Regional Medical Center, Pomona NJ, USA Cardiac Cath Lab, AtlantiCare Regional Medical Center, Pomona NJ 08205, USA
a r t i c l e
i n f o
Article history: Received 17 July 2013 Received in revised form 26 August 2013 Accepted 5 September 2013
a b s t r a c t We report a case of a Perforated Sinus of Valsalva Aneurysm (PSOV) closure using an Amplatzer muscular ventricular septal defect occluder (mVSD) device and describe a novel and potentially safer way for defect sizing. A literature review of the endovascular treatment of this disease is presented. © 2014 Elsevier Inc. All rights reserved.
Keywords: Sinus of Valsalva Perforation Endovascular Closure
1. Introduction
2.1. Procedure
Closure of a perforated sinus of Valsalva aneurysm has been traditionally performed by surgical approach and cardiopulmonary bypass. Recently, few reports with intermediate term follow up indicate that a less invasive percutaneous approach can be offered to these patients with satisfactory results. The type of device appropriate for this purpose has not been established and sizing of the defect poses some challenges due to the intrinsic weakness of the perforated aortic wall.
The procedure was carried out under general anesthesia and endotracheal intubation with TEE guidance. A 6 Fr sheath was placed into the left common femoral artery and an 8 Fr long sheath into the left common femoral vein. Following the administration of 7000 UI of unfractionated heparin, the perforation was crossed from the aortic side with a 6 Fr multipurpose (MP) catheter with the help of an angled stiff Terumo guide wire (Terumo, Elkton, MD). After looping the guidewire into RV apex it was redirected retrograde back into the right atrium and down to the IVC followed by the MP catheter. In order to overcome difficulties in advancing the MP catheter through the right atrium and down into the IVC a 5 Fr angled tip glide catheter was inserted coaxially into the MP catheter. The Terumo wire was then exchanged with a Bentson 0.035 inch guidewire successfully snared out from the left common femoral vein using a 10 mm goose neck snare (Cook incorporated, Bloomington, IN) to form an arterial venous loop. Over this wire, from the venous side, a 24 mm Amplatzer sizing balloon was advanced and positioned with the distal half into the ascending aorta and the proximal half into the RA. Because of the differential pressure between the aorta and RA, we gently (1–2 atm) inflated the balloon just enough to cause its expansion into the RA; by a more gradual and steady inflation we inflated the segment of the balloon inside the perforation tract enough to create occlusion of the fistula confirmed by TEE color flow and repeat angiogram. At that point the segment of the balloon inside the fistula would take an isosceles triangular shape with its vertex toward the aorta and the base at the exit of the perforation into the RA. We measured the base of this balloon formed triangle as the diameter of the defect at the atrial side and the height of the triangle as the length of the
2. Case report The patient is a 43-year-old Asian man who presented with a heart murmur, decreased exercise tolerance and palpitation over the past 6 months. His physical exam revealed a continuous murmur best heard on the left lower sternal border with normal P2 intensity, no precordial hyperactivity and normal pulse pressure. Echocardiography and TEE revealed the presence of a perforated non coronary (NC) cusp sinus of Valsalva aneurysm (wind sock appearance) into the right atrium (Fig. 1) with significant left to right shunt. The distance from the septal leaflet of the tricuspid valve from the defect was measured to be 1.3 cm. Right and left heart catheterization revealed QP/QS ratio of 2.4 and pulmonary systolic pressure of 32 mmHg. An aortogram showed the left to right shunt from the non-coronary cusp of the aorta into the right atrium; the RCA ostium was 1.5 cm distant from the aneurysm (Fig. 2).
⁎ Corresponding author. Tel.: +1 609 748 7552. E-mail address: [email protected] (G. Gioia). 1553-8389/$ – see front matter © 2014 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.carrev.2013.09.002
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G. Gioia et al. / Cardiovascular Revascularization Medicine 15 (2014) 165–170
Fig. 1. Preclosure two dimensional and color flow TEE images of the perforated sinus of Valsalva.
perforation (Figs. 3 and 4). We intentionally kept the distal balloon within the aorta collapsed to prevent overstretching. The defect diameter, as obtained by balloon sizing, was 6.5 mm, slightly larger than the TEE (6.0 mm) and angiographically deter-
mined diameter (5.5 mm) at the same location. Its length was measured to be 9 mm. After removal of the sizing balloon, an Amplatzer 7 Fr delivery sheath was advanced into the ascending aorta from the venous side.
Fig. 2. Aortogram from the LAO projection showing the shunt from the non-coronary cusp of the aorta into the right atrium.
G. Gioia et al. / Cardiovascular Revascularization Medicine 15 (2014) 165–170
167
Fig. 3. Sizing balloon wedging into the perforation.
An Amplatzer 8 mm mVSD Occluder (AGA Medical Corp, Golden Valley, MN) was initially selected and deployed. While the device was still attached to the delivery cable a repeat aortic angiogram and TEE color flow imaging were obtained confirming the presence of significant high jet residual shunt. The 8 mm mVSD device was then recaptured by advancing the delivery sheath into the aorta. A 10 mm mVSD occluder was selected, brought in position and deployed. Post implant repeat aortogram revealed a non-significant foamylike residual shunt into the RA (Fig. 5) without any high velocity jet confirmed by TEE (Fig. 6). No interference with the aortic and tricuspid valve function was documented by TEE. At 6-month follow up transthoracic echocardiography revealed only trivial residual shunt with no tricuspid or aortic valve dysfunction or sign of perforation. The patient symptoms completely resolved. 3. Discussion This case shows that, in selected patients with perforated sinus of Valsalva aneurysm, a double disk device such as mVSD occluder can be used and, likely, will provide adequate sealing with good stability lessening the chances for device embolization. To our knowledge this is the first report to document the feasibility of using an mVSD occluder for a congenital perforated sinus of Valsalva aneurysm and we showed very good midterm results with nearly complete occlusion at 6-month follow up. No evidence of erosion, endocarditis or valve dysfunction was documented. A similar device was indeed implanted but for a post-surgical recurrent sinus of Valsalva perforation [1]. Also, we described a modified sizing balloon technique useful, in our opinion, in situation where overstretching must be avoided such as in PSOV. In fact the aortic wall of these patients is intrinsically weak and susceptible to tearing from a fully inflated balloon. Tearing the perforation edges and potentially enlarging the defect are real risks
[2]. The use of a balloon tipped catheter could be used instead [2] but it will eventually size the aortic entry side of the perforation giving no information about its length and distal exit diameter. Knowing the length of the perforation could help in device selection (dashed line in Fig. 4). Overstretching in our case was likely prevented by keeping the balloon inside the aortic collapsed at all times, while the atrial and transition segment was gently inflated until stop color flow was documented by TEE. Our initial choice to use an 8 mm device was however not optimal and motivated by the proximity of the septal leaflet of the tricuspid valve to the exit of the defect. Retrospectively we could have safely used a 10 mm to start with considering that at least 2 mm difference between defect size and device selected should have been in place (only 1.5 mm in our case). Other noninvasive modalities can be used for defect sizing prior to closure. Contrast computed tomography and cardiac magnetic resonance are the most promising modalities in this regard [1]. However, the role of these non invasive imaging modalities in this setting has not been clearly established and remains an open field of investigation. TEE is a valid alternative in defect sizing with balloon but occasionally the exit site of the defect is not clearly seen. Intracardiac ultrasound (ICE) could be used for sizing allowing the procedure to be performed without general anesthesia; data about this particular application are lacking. The use of a double disk device in our case was made possible by an adequate distance between the distal exit of the perforation (RA side) and the septal leaflet of the tricuspid valve. A shorter distance would have made this device unsuitable for closure without compromising tricuspid valve function. For the same talk, sitting the left disk at the aortic entrance side of the perforation is dependent on the distance of the RCA ostium to the mouth of the aneurysm; in our case the distance was sufficient to allow deployment of a 10 mm mVSD device. There are very few reports in the literature of endovascular PSOV closure. Traditional treatment has been surgical, and most of the data
168
G. Gioia et al. / Cardiovascular Revascularization Medicine 15 (2014) 165–170
Fig. 4. Sizing balloon with drawing showing the isosceles triangle shape of the balloon wedging into the perforation.
with long term outcomes are from surgical reports. PSOV being a rare congenital defect has a natural history that is not well defined, but if left untreated, appears to have a poor prognosis [3,4]. Pathology shows a thinning of the aortic wall with incomplete fusion of the bulbar septum and truncal ridges leading to malfusion of the aortic media and annulus resulting in aneurysm formation [5,6]. Sinus of Valsalva aneurysm may involve all three sinuses, but they arise more frequently from the right coronary sinus (80%–85%) than from the NC sinus (5%–15%) [7].Perforation most often occurs into either the right atrium or right ventricle, but occasionally into the left ventricle, pulmonary artery, superior vena cava or mediastinum. Conventional surgical repair under cardiopulmonary bypass carries low morbidity and mortality [8,9], but development of post operative infective endocarditis may be fatal [9]. A more recent review of 100 surgically treated cases [10] with a mean length of stay in hospital of 8 days, showed an early surgical mortality of 3% but of 5% at two years. The long term survival was shown to be 90% at 15 years. However, in this surgical series aortic valve surgery was required in 14% of their patient for subsequent severe aortic regurgitation. The risk of post operative severe aortic insufficiency requiring surgery after surgical PSOV repair has been reported to be from 0% to 30% [11,12]. Surgical recurrence depends on the modality of surgical closure, patching being the preferred method nowadays as opposed to
direct suturing, and has been reported to be less than 10% long term [12,13]. Endovascular closure has potential advantage over surgical repair not only for treatment of recurrence. The need for sternotomy and cardiopulmonary bypass can be avoided, which is especially important when hemodynamic instability is present following an acute rupture. The length of stay in hospital can be potentially significantly shortened. Obviously, there is no role for transcatheter PSOV closure in the presence of concomitant ventricular septal defect (VSD) or significant aortic regurgitation requiring surgery; however, concomitant PSOV and ASD closure has been previously reported [14,15]. Along with a few case reports [1,14–18] there are 3 small case series [2,20,21] of consecutive patients with PSOV treated percutaneously over the last 10 years. Six patients were single case report and 35 patients were enrolled in the three small series with a total of 41 treated patients (Table 1). The majority of the patients were treated with ADO devices (36 out of 41, 88%) whereas two patients received an ASO and 1 an mVSD occluder. Two early patients from the Arora [2] series were treated with the old Rashkind umbrella device. Procedural success rate was high (38 out of 41, 93%) and midterm severe adverse events were low (5%) with only one death and one subsequent surgical closure reported as major adverse events [2]. Residual shunts are frequently seen post
G. Gioia et al. / Cardiovascular Revascularization Medicine 15 (2014) 165–170
169
Fig. 5. TEE two dimensional and color flow post implant.
device implantation but most were mild and they improved over time [20]. In our case a mild residual shunt was seen immediately post implant but became trivial at 6-month follow-up. No cases of significant aortic regurgitation, endocarditis or erosion were
reported. It seems from these data that a percutaneous approach for PSOV closure would be preferable in some subset of patients, for example those presenting with decompensated heart failure and hemodynamic instability; if anatomic futures are favorable these
Fig. 6. Final aortogram after deployment of the 10 mm mVSD occluder.
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G. Gioia et al. / Cardiovascular Revascularization Medicine 15 (2014) 165–170
Table 1 Summary of percutaneous PSOV closure in the literature. Residual Adverse Patients Procedural F/U (months) Outcomes shunts at (n) success follow up (n, %) (n, %) (n, %) Arora [2]
8
Sivadanpillai 7 [23] Kerkar [22] 20 Case reports [14–19] Total
6
41
7 (88)
7 (100) 18(90) 6 (100)
38 (93)
44
1 death; 1 surgery
9
0
24
0 0
2 (5)
1 (emolysis)
Device
ADO: 5 ASO: 1 RU: 2 ADO: 7
4 (mild) ADO: 20 1 moderate 2(mild) ADO: 4 ASO: 1 mVSD: 1 8 (21) ADO: 36 ASO: 2 mVSD: 1
RU: Rashkind Umbrella; ADO: Amplatzer Duct Occluder; ASO: Amplatzer Septal Occluder.
young patients could be offered a less invasive percutaneous alternative to surgery. The low number of patients treated percutaneously and the relative short term follow up speak against any conclusion about the preferred method to treat a perforated sinus of Valsalva. To shed light on this argument, a multicenter registry would be very helpful. Reporting bias has to be considered: often small series or successful case report in literature is more likely to be reported than failed or complicated ones, potentially skewing the data toward more optimistic conclusions. In this regard again a multicenter registry would be important. Finally, the choice of the device still is an open question. The majority of patients treated by transcatheter means so far were with ADO devices. We think that the selection would depend on the anatomy of the fistula and in such regard the use of imaging technique such as CT scan [1] needs to be further defined. We think that if the penetration is away enough from aortic and tricuspid valve and coronary ostium, a double disk device (mVSD or ADO2) would be preferable because it would offer more stability and less chance of device embolization. Should any interference be anticipated with the aortic or tricuspid valve functioning or the right disk be of impediment to the right ventricular outflow tract, a non-disk device (AVP 2) or ADO (single left disk) would be preferable. 4. Conclusion We closed a perforated sinus of Valsalva aneurysm using a muscular VSD. We think that an mVSD occluder may offer more
stability and lessen the potential for device embolization as compared with single disk device. We describe a modification of sizing this defect with the balloon in order to prevent overstretching of this already naturally weak aortic wall. References [1] 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;22:336–8. [2] Arora R, Trehan V, Rangasetty UM, Mukhopadhyay S, Thakur AK, Kaira GS. Transcatheter closure of ruptured sinus of Valsalva aneurysm. J Interv Cardiol 2004;17:53–8. [3] Sawyer JL, Adam JE, Scott Jr H. Surgical treatment for aneurysms of the aortic sinus with aortic atria fistula. Surgery 1957;41:46–56. [4] Sakakabara S, Konno. Congenital aneurysm of the sinus of Valsalva anatomy and classification. Am Heart J 1962;63:405–24. [5] Tanabe T, Yokota A, Sugie S. Surgical treatment of aneurysm of the sinus of Valsalva. Ann Thorac Surg 1979;27:133–6. [6] Edwards JE, Burchell HB. The pathological anatomy of deficiencies between aortic root and the heart including aortic sinus aneurysm. Thorax 1957;12:125–39. [7] Chen TO. About sinus of Valsalva aneurysm. J Thorac Cardiovasc Surg 2000;41:647. [8] Hamid IA, Jothi M, Rajan S. Transaortic repair of ruptured aneurysm of sinus of Valsalva—15 years experience. J Thorac Cardiovasc Surg 1994;107:1464–8. [9] Au WK, Chiu SW, Mok CK, Lee WT, Cheung D, He GW. Repair of ruptured sinus of Valsalva aneurysm. Determinant of long term survival. Ann Thorac Surg 1998;66: 1604–10. [10] Yan F, Huo Q, Qiao J, Murat V, Ma S-F. Surgery for sinus of Valsalva aneurysm: 27 years experience with 100 patients. Asian Cardiovasc Thorac Ann 2008;16: 361–5. [11] 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: 1466–71. [12] 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;90:20–9. [13] Sarikaya S, Adedemir T, Elibol A, Buyukbayrak F, Onk A, Kirali K. Surgery for ruptured sinus of Valsalva aneurysm: 25 years experience with 55 cases. Eur J Cardiothorac Surgery 2012;10:1093. [14] 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;25:208–13. [15] Metha NK, Mishra N, Kerkar P. Percutaneous closure of ruptured sinus of valsalva aneurysm and atrial septal defect. J Invasive Cardiol 2010;22:82–5. [16] 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;66:657–60. [17] Fedson S, Jolly N, Lang RM, Hijaz ZM. Percutaneous closure of a ruptured sinus of Valsalva aneurysm using the Amplatzer duct occluder. Catheter Cardiovasc Interv 2003;58:406–11. [18] Abidin N, Clarke B, Khattar RS. Percutaneous closure of ruptured sinus of Valsalva aneurysm using an Amplatzer occluder device. Heart 2005;91:244. [19] Kerkar PG, Lanjewar P, 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;10:1093. [20] Sivadasanpillai H, Valaparambil A, Sivasubramonian S, Mahadevan KK, Sasidheran B, Namboodiri N, et al. Percutaneous closure of ruptured sinus of Valvalva aneurysm: intermediate term follow-up results. Eurointervention 2010;6: 214–9.
Contents lists available at ScienceDirect
Cardiovascular Revascularization Medicine
Perforated Sinus of Valsalva (PSOV) aneurysm closure with a muscular VSD occluder Giuseppe Gioia ⁎, Jingsheng Zheng, Amit Ray, Mario Gioia AtlantiCare Regional Medical Center, Pomona NJ, USA Cardiac Cath Lab, AtlantiCare Regional Medical Center, Pomona NJ 08205, USA
a r t i c l e
i n f o
Article history: Received 17 July 2013 Received in revised form 26 August 2013 Accepted 5 September 2013
a b s t r a c t We report a case of a Perforated Sinus of Valsalva Aneurysm (PSOV) closure using an Amplatzer muscular ventricular septal defect occluder (mVSD) device and describe a novel and potentially safer way for defect sizing. A literature review of the endovascular treatment of this disease is presented. © 2014 Elsevier Inc. All rights reserved.
Keywords: Sinus of Valsalva Perforation Endovascular Closure
1. Introduction
2.1. Procedure
Closure of a perforated sinus of Valsalva aneurysm has been traditionally performed by surgical approach and cardiopulmonary bypass. Recently, few reports with intermediate term follow up indicate that a less invasive percutaneous approach can be offered to these patients with satisfactory results. The type of device appropriate for this purpose has not been established and sizing of the defect poses some challenges due to the intrinsic weakness of the perforated aortic wall.
The procedure was carried out under general anesthesia and endotracheal intubation with TEE guidance. A 6 Fr sheath was placed into the left common femoral artery and an 8 Fr long sheath into the left common femoral vein. Following the administration of 7000 UI of unfractionated heparin, the perforation was crossed from the aortic side with a 6 Fr multipurpose (MP) catheter with the help of an angled stiff Terumo guide wire (Terumo, Elkton, MD). After looping the guidewire into RV apex it was redirected retrograde back into the right atrium and down to the IVC followed by the MP catheter. In order to overcome difficulties in advancing the MP catheter through the right atrium and down into the IVC a 5 Fr angled tip glide catheter was inserted coaxially into the MP catheter. The Terumo wire was then exchanged with a Bentson 0.035 inch guidewire successfully snared out from the left common femoral vein using a 10 mm goose neck snare (Cook incorporated, Bloomington, IN) to form an arterial venous loop. Over this wire, from the venous side, a 24 mm Amplatzer sizing balloon was advanced and positioned with the distal half into the ascending aorta and the proximal half into the RA. Because of the differential pressure between the aorta and RA, we gently (1–2 atm) inflated the balloon just enough to cause its expansion into the RA; by a more gradual and steady inflation we inflated the segment of the balloon inside the perforation tract enough to create occlusion of the fistula confirmed by TEE color flow and repeat angiogram. At that point the segment of the balloon inside the fistula would take an isosceles triangular shape with its vertex toward the aorta and the base at the exit of the perforation into the RA. We measured the base of this balloon formed triangle as the diameter of the defect at the atrial side and the height of the triangle as the length of the
2. Case report The patient is a 43-year-old Asian man who presented with a heart murmur, decreased exercise tolerance and palpitation over the past 6 months. His physical exam revealed a continuous murmur best heard on the left lower sternal border with normal P2 intensity, no precordial hyperactivity and normal pulse pressure. Echocardiography and TEE revealed the presence of a perforated non coronary (NC) cusp sinus of Valsalva aneurysm (wind sock appearance) into the right atrium (Fig. 1) with significant left to right shunt. The distance from the septal leaflet of the tricuspid valve from the defect was measured to be 1.3 cm. Right and left heart catheterization revealed QP/QS ratio of 2.4 and pulmonary systolic pressure of 32 mmHg. An aortogram showed the left to right shunt from the non-coronary cusp of the aorta into the right atrium; the RCA ostium was 1.5 cm distant from the aneurysm (Fig. 2).
⁎ Corresponding author. Tel.: +1 609 748 7552. E-mail address: [email protected] (G. Gioia). 1553-8389/$ – see front matter © 2014 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.carrev.2013.09.002
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G. Gioia et al. / Cardiovascular Revascularization Medicine 15 (2014) 165–170
Fig. 1. Preclosure two dimensional and color flow TEE images of the perforated sinus of Valsalva.
perforation (Figs. 3 and 4). We intentionally kept the distal balloon within the aorta collapsed to prevent overstretching. The defect diameter, as obtained by balloon sizing, was 6.5 mm, slightly larger than the TEE (6.0 mm) and angiographically deter-
mined diameter (5.5 mm) at the same location. Its length was measured to be 9 mm. After removal of the sizing balloon, an Amplatzer 7 Fr delivery sheath was advanced into the ascending aorta from the venous side.
Fig. 2. Aortogram from the LAO projection showing the shunt from the non-coronary cusp of the aorta into the right atrium.
G. Gioia et al. / Cardiovascular Revascularization Medicine 15 (2014) 165–170
167
Fig. 3. Sizing balloon wedging into the perforation.
An Amplatzer 8 mm mVSD Occluder (AGA Medical Corp, Golden Valley, MN) was initially selected and deployed. While the device was still attached to the delivery cable a repeat aortic angiogram and TEE color flow imaging were obtained confirming the presence of significant high jet residual shunt. The 8 mm mVSD device was then recaptured by advancing the delivery sheath into the aorta. A 10 mm mVSD occluder was selected, brought in position and deployed. Post implant repeat aortogram revealed a non-significant foamylike residual shunt into the RA (Fig. 5) without any high velocity jet confirmed by TEE (Fig. 6). No interference with the aortic and tricuspid valve function was documented by TEE. At 6-month follow up transthoracic echocardiography revealed only trivial residual shunt with no tricuspid or aortic valve dysfunction or sign of perforation. The patient symptoms completely resolved. 3. Discussion This case shows that, in selected patients with perforated sinus of Valsalva aneurysm, a double disk device such as mVSD occluder can be used and, likely, will provide adequate sealing with good stability lessening the chances for device embolization. To our knowledge this is the first report to document the feasibility of using an mVSD occluder for a congenital perforated sinus of Valsalva aneurysm and we showed very good midterm results with nearly complete occlusion at 6-month follow up. No evidence of erosion, endocarditis or valve dysfunction was documented. A similar device was indeed implanted but for a post-surgical recurrent sinus of Valsalva perforation [1]. Also, we described a modified sizing balloon technique useful, in our opinion, in situation where overstretching must be avoided such as in PSOV. In fact the aortic wall of these patients is intrinsically weak and susceptible to tearing from a fully inflated balloon. Tearing the perforation edges and potentially enlarging the defect are real risks
[2]. The use of a balloon tipped catheter could be used instead [2] but it will eventually size the aortic entry side of the perforation giving no information about its length and distal exit diameter. Knowing the length of the perforation could help in device selection (dashed line in Fig. 4). Overstretching in our case was likely prevented by keeping the balloon inside the aortic collapsed at all times, while the atrial and transition segment was gently inflated until stop color flow was documented by TEE. Our initial choice to use an 8 mm device was however not optimal and motivated by the proximity of the septal leaflet of the tricuspid valve to the exit of the defect. Retrospectively we could have safely used a 10 mm to start with considering that at least 2 mm difference between defect size and device selected should have been in place (only 1.5 mm in our case). Other noninvasive modalities can be used for defect sizing prior to closure. Contrast computed tomography and cardiac magnetic resonance are the most promising modalities in this regard [1]. However, the role of these non invasive imaging modalities in this setting has not been clearly established and remains an open field of investigation. TEE is a valid alternative in defect sizing with balloon but occasionally the exit site of the defect is not clearly seen. Intracardiac ultrasound (ICE) could be used for sizing allowing the procedure to be performed without general anesthesia; data about this particular application are lacking. The use of a double disk device in our case was made possible by an adequate distance between the distal exit of the perforation (RA side) and the septal leaflet of the tricuspid valve. A shorter distance would have made this device unsuitable for closure without compromising tricuspid valve function. For the same talk, sitting the left disk at the aortic entrance side of the perforation is dependent on the distance of the RCA ostium to the mouth of the aneurysm; in our case the distance was sufficient to allow deployment of a 10 mm mVSD device. There are very few reports in the literature of endovascular PSOV closure. Traditional treatment has been surgical, and most of the data
168
G. Gioia et al. / Cardiovascular Revascularization Medicine 15 (2014) 165–170
Fig. 4. Sizing balloon with drawing showing the isosceles triangle shape of the balloon wedging into the perforation.
with long term outcomes are from surgical reports. PSOV being a rare congenital defect has a natural history that is not well defined, but if left untreated, appears to have a poor prognosis [3,4]. Pathology shows a thinning of the aortic wall with incomplete fusion of the bulbar septum and truncal ridges leading to malfusion of the aortic media and annulus resulting in aneurysm formation [5,6]. Sinus of Valsalva aneurysm may involve all three sinuses, but they arise more frequently from the right coronary sinus (80%–85%) than from the NC sinus (5%–15%) [7].Perforation most often occurs into either the right atrium or right ventricle, but occasionally into the left ventricle, pulmonary artery, superior vena cava or mediastinum. Conventional surgical repair under cardiopulmonary bypass carries low morbidity and mortality [8,9], but development of post operative infective endocarditis may be fatal [9]. A more recent review of 100 surgically treated cases [10] with a mean length of stay in hospital of 8 days, showed an early surgical mortality of 3% but of 5% at two years. The long term survival was shown to be 90% at 15 years. However, in this surgical series aortic valve surgery was required in 14% of their patient for subsequent severe aortic regurgitation. The risk of post operative severe aortic insufficiency requiring surgery after surgical PSOV repair has been reported to be from 0% to 30% [11,12]. Surgical recurrence depends on the modality of surgical closure, patching being the preferred method nowadays as opposed to
direct suturing, and has been reported to be less than 10% long term [12,13]. Endovascular closure has potential advantage over surgical repair not only for treatment of recurrence. The need for sternotomy and cardiopulmonary bypass can be avoided, which is especially important when hemodynamic instability is present following an acute rupture. The length of stay in hospital can be potentially significantly shortened. Obviously, there is no role for transcatheter PSOV closure in the presence of concomitant ventricular septal defect (VSD) or significant aortic regurgitation requiring surgery; however, concomitant PSOV and ASD closure has been previously reported [14,15]. Along with a few case reports [1,14–18] there are 3 small case series [2,20,21] of consecutive patients with PSOV treated percutaneously over the last 10 years. Six patients were single case report and 35 patients were enrolled in the three small series with a total of 41 treated patients (Table 1). The majority of the patients were treated with ADO devices (36 out of 41, 88%) whereas two patients received an ASO and 1 an mVSD occluder. Two early patients from the Arora [2] series were treated with the old Rashkind umbrella device. Procedural success rate was high (38 out of 41, 93%) and midterm severe adverse events were low (5%) with only one death and one subsequent surgical closure reported as major adverse events [2]. Residual shunts are frequently seen post
G. Gioia et al. / Cardiovascular Revascularization Medicine 15 (2014) 165–170
169
Fig. 5. TEE two dimensional and color flow post implant.
device implantation but most were mild and they improved over time [20]. In our case a mild residual shunt was seen immediately post implant but became trivial at 6-month follow-up. No cases of significant aortic regurgitation, endocarditis or erosion were
reported. It seems from these data that a percutaneous approach for PSOV closure would be preferable in some subset of patients, for example those presenting with decompensated heart failure and hemodynamic instability; if anatomic futures are favorable these
Fig. 6. Final aortogram after deployment of the 10 mm mVSD occluder.
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G. Gioia et al. / Cardiovascular Revascularization Medicine 15 (2014) 165–170
Table 1 Summary of percutaneous PSOV closure in the literature. Residual Adverse Patients Procedural F/U (months) Outcomes shunts at (n) success follow up (n, %) (n, %) (n, %) Arora [2]
8
Sivadanpillai 7 [23] Kerkar [22] 20 Case reports [14–19] Total
6
41
7 (88)
7 (100) 18(90) 6 (100)
38 (93)
44
1 death; 1 surgery
9
0
24
0 0
2 (5)
1 (emolysis)
Device
ADO: 5 ASO: 1 RU: 2 ADO: 7
4 (mild) ADO: 20 1 moderate 2(mild) ADO: 4 ASO: 1 mVSD: 1 8 (21) ADO: 36 ASO: 2 mVSD: 1
RU: Rashkind Umbrella; ADO: Amplatzer Duct Occluder; ASO: Amplatzer Septal Occluder.
young patients could be offered a less invasive percutaneous alternative to surgery. The low number of patients treated percutaneously and the relative short term follow up speak against any conclusion about the preferred method to treat a perforated sinus of Valsalva. To shed light on this argument, a multicenter registry would be very helpful. Reporting bias has to be considered: often small series or successful case report in literature is more likely to be reported than failed or complicated ones, potentially skewing the data toward more optimistic conclusions. In this regard again a multicenter registry would be important. Finally, the choice of the device still is an open question. The majority of patients treated by transcatheter means so far were with ADO devices. We think that the selection would depend on the anatomy of the fistula and in such regard the use of imaging technique such as CT scan [1] needs to be further defined. We think that if the penetration is away enough from aortic and tricuspid valve and coronary ostium, a double disk device (mVSD or ADO2) would be preferable because it would offer more stability and less chance of device embolization. Should any interference be anticipated with the aortic or tricuspid valve functioning or the right disk be of impediment to the right ventricular outflow tract, a non-disk device (AVP 2) or ADO (single left disk) would be preferable. 4. Conclusion We closed a perforated sinus of Valsalva aneurysm using a muscular VSD. We think that an mVSD occluder may offer more
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