Catheterization and Cardiovascular Interventions 85:166–169 (2015)
Transcatheter Closure of Ruptured Sinus of Valsalva Aneurysm into the Right Ventricle With an Amplatzer Vascular Plug II Harald Rittger,1* MD, Ulrike Gundlach,1 MD, and Andreas Koch,2 MD Objective: We report a case of a 52-year-old female patient with perforated sinus of Valsalva (SVA) aneurysm presenting with severe left-to-right shunt from the aorta into the right ventricle. Background: Aneuryms of the aortic sinus, better known as SVA are rare. Until interventional devices were introduced, the only treatment option for ruptured SVA aneurysms was surgery. Methods and Results: The case was discussed in the heart team and decision was made to implant an Amplatzer Vascular Plug (AVP) II. Initially, closing attempts using AVP 16 mm and 14 mm devices were performed. Presumably as a consequence of inadequate sizing, the discs were not well formed in the connecting tunnel, leaving a severe rest shunt after both procedures. Finally, after implantation of a 12 mm AVP-device, only a minimal rest shunt remained detectable. Absence of aortic regurgitation was confirmed by transesophageal echocardiography, and the occluder was released. Mean pulmonary artery pressure immediately decreased from 39 to 15 mm Hg. Medication with aspirin (100 mg qid) and clopidogrel (75 mg qid) was initiated. Follow-up examinations at 4 weeks and 3 months confirmed the minimal rest shunt and a continuous decrease of systolic pulmonary artery pressure to 35 mm Hg, as assessed with transthoracal echocardiography. Conclusion: In conclusion, in cases of SVA rupture, closure with an AVP II represents an alternative to surgical treatment and other devices used for transcatheter treatment of SVA. VC 2014 Wiley Periodicals, Inc.
Key words: sinus Valsalva aneurysm; transcatheter closure; Amplatzer Vascular Plug
INTRODUCTION
Aneuryms of the aortic sinus, better known as sinus of Valsalva (SVA) are rare. Etiology is either congenital or acquired due to trauma, infections, or postsurgery. The prevalence is reported to be higher in Asians and in men [1]. The prevalence of congenital defects in patients undergoing cardiac surgery is less than 1% [2]. According to Edwards and Burchell, it is caused by a congenital defect in the aortic media and an incomplete fusion of the distal bulbar septum and truncal ridges combined with malfusion of the aortic media and annulus fibrosis of the aortic valve [3]. When present, it appears most commonly in the right sinus rather than in the non-coronary or left sinus [2]. It rarely leads to symptoms, if it is not ruptured, but in case of rupture, it can cause worsening of heart failure symptoms or cause shunt with substantial effects on hemodynamics. Rupture in patients not undergoing surgery is estimated to be between 40% and 76% [4,5]. Sakakabara et al. have proposed a nomenclature for SVA aneuryms based on their site of origin [6]. According to this nomenclature, the lesion treated in C 2014 Wiley Periodicals, Inc. V
the present case is the most common, arising from the left part of the right sinus.
CASE REPORT
A 52-year-old female patient, who originated from the Philippines, was admitted to our clinic due to pro-
1
€tsklinikum Erlangen, ErlanMedizinische, Klinik 2, Universita gen, Germany 2 €tsklinikum Erlangen, €r Kinderkardiologie, Universita Klinik fu Erlangen, Germany Conflict of interest: Nothing to report. *Correspondence to: Harald Rittger, MD, Universit€atsklinikum Erlangen, Medizinische Klinik 2, Ulmenweg 18, 91054 Erlangen, Germany. E-mail: [email protected] Received 11 November 2013; Revision accepted 26 December 2013 DOI: 10.1002/ccd.25382 Published online 9 January 2014 in Wiley Online Library (wileyonlinelibrary.com)
Interventional Closure of Ruptured Sinus of Valsalva Aneurysm
167
Fig. 1. TEE demonstrating a severe shunt from the right aortic cusp into the right ventricle. [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.]
gressively increasing dyspnoea (New York Heart Association Class III) for several months. The patient had previously seen a general practitioner, who diagnosed a systolic heart murmur at initial examination and transferred the patient for echocardiography to our hospital. The patient reported no history of heart disease. Echocardiography revealed a shunt from the aortic root into the right ventricular outflow tract, moderate dilatation of the right ventricle with severely reduced function, and pulmonary hypertension with a systolic pulmonary artery pressure (PAP) of 60 mm Hg. There was no aortic regurgitation or ventricular septal defect. Lab values at admission were all in a normal range. Transesophageal echocardiography (TEE) confirmed the shunt into the right ventricular outflow tract, and detected its origin in the right aortic sinus (Fig. 1a and b). CT-angiography confirmed the rupture of an aneurysm of the right coronary sinus and the connection into the right ventricle with a defect size of 7 mm. The main stem of the pulmonary artery was dilated to a diameter 42 mm. Right heart catheterization showed a left-to-right shunt of 67% (Qp:Qs ¼ 3:1) and pulmonary hypertension with a mean PAP of 39 mm Hg. The case was discussed in the heart team. There is no device specifically designed for implantation in ruptured SVAs. As one margin of the device would be located within the right aortic cusp, there were concerns to cause aortic regurgitation with a larger disc, occluding the channel from the aortic margin. Therefore, initial plans to implant an Amplatzer DuctOccluder [1] or an Amplatzer-Septal-Occluder [7] were
discarded and decision was made to implant an Amplatzer Vascular Plug II (AVP II). The AVP (St. Jude Medical, St. Paul, MN) is a self-expanding, multi-layered Nitinol mesh occlusion device, which is available in different forms, shapes, and sizes. It is manufactured from 3 to 22 mm (Fig. 2). The procedure was performed under TEE guidance. After aortic root angiogram, which confirmed the leftto-right shunt, the ruptured aneurysm was crossed from the aorta with a 0.035 angled Terumo guide wire and a 5 Fr multipurpose catheter (MP 1). The right ventricle was passed, and the main stem of the pulmonary artery was engaged. Thereafter the initial guide wire was exchanged for a super-stiff guide wire (Amplatzer Super Stiff, Boston Scientific, Natick, MA) and a flexible 6 Fr sheath (Cook Medical, Bloomington, IN) was introduced into the pulmonary artery. Initially, closing attempts using AVP 16 mm and 14 mm devices were performed. Presumably as a consequence of inadequate sizing, the discs were not well formed in the connecting tunnel, leaving a severe rest shunt after both procedures. Finally after implantation of a 12 mm AVP-device, only a minimal rest shunt remained detectable. Absence of aortic regurgitation was confirmed by TEE, and the occluder was released (Fig. 3a and b). Mean pulmonary artery pressure immediately decreased from 39 to 15 mm Hg. Medication with aspirin (100 mg qid) and clopidogrel (75 mg qid) was initiated. Follow-up examinations at 4 weeks and 3 months confirmed the minimal rest shunt and a continuous decrease of systolic PAP to 35 mm Hg, as assessed with transthoracal echocardiography.
Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).
168
Rittger et al.
DISCUSSION
Until interventional devices were introduced, the only treatment option for ruptured SVA aneurysms was surgery. As various devices for transcatheter closure of vascular connections have become available, this technique appears to be a viable alternative to the surgical approach. It was first described by Cullen et al. in 1994, who performed SVA closure with a Rushkind umbrella device—originally designed for arterial duct
closure—in a 34-year-old man. Since then, there have been several historical reports about SVA-closure with coils and other devices [1,7–9]. To our knowledge, this is the first case of closure of a perforated SVA aneurysm with an AVP. The AVP is somewhat different to the more often used duct occluder. Both connections of the two discs at the proximal and distal margins of the device with the central part of the occluder are flexible. Consequently, the occluding and the anchoring part of this device can individually adjust to the given anatomy and an angulated final position can be achieved. In contrast to the AVP II, the Amplatzer duct occluder is much less adjustable to a tortuous anatomy, because all parts of the duct occluder tend to realign in a straight position after implantation. The new Amplatzer duct occluder II and the “Amplatzer duct occluder II-additional sizes” have the same flexibility as the AVP II, and therefore potentially would have been alternative devices. Unfortunately, both devices are available only in relatively small sizes (maximum waist diameter 6 and 5 mm, respectively). Therefore, in the present case, the AVP II was the only possible alternative for interventional closure of the shunt.
CONCLUSION
Fig. 2. Design and shape of the Amplatzer AVP II Occluder (courtesy of St. Jude Medical). [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.]
In conclusion, in cases of SVA rupture, closure with an AVP II represents an alternative to surgical treatment and other devices used for transcatheter treatment of SVA.
Fig. 3. (a) Aortogram with ruptured aneurysm of the right coronary cusp with shunt into the right ventricle. (b) Aortogram with the defect after closure with an Amplatzer-AVP-II-Occluder. [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.] Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).
Interventional Closure of Ruptured Sinus of Valsalva Aneurysm
REFERENCES 1. Chang CW, Chiu SN, Wu ET, Tsai SK, Wu MH, Wang JK. Transcatheter closure of a ruptured sinus of Valsalva aneurysm. Circ J 2006;70:1043–1047. 2. Takach T, Reul G, Cuncan M, Cooley D, Livesay J, Ott D, Frazier O. Sinus of vaslsalva aneurysm or fistula: Management and outcome. Ann Thorac Surg 1999;68:1573–1577. 3. Edwards E, Burchell HB. The pathological anatomy of deficiencies between the aortic root and the heart, including aortic sinus aneurysms. Thorax 1957;12:dd125–dd139. 4. Goldberg N, Krasnow N. Sinus of vasalva aneurysms. Clin Cardiol 1990;13:831–836.
169
5. 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;70:1466–1471. 6. Sakakabara S, Konno S. Congenital aneurysm of the sinus of Valsalva anatomy and classification. Am Heart J 1962;63:405–424. 7. Abidin N, Clarke B, Khattar RS. Percutaneous closure of ruptured sinus of Valsalva aneurysm using an Amplatzer occluder device. Heart 2005;91:244. 8. Chu SH, Hung CR, 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;99:288–298. 9. Naka Y, Kadoba K, Ohtake S, Sawa Y, Hirata N, Nishimura M, Matuda H. The long-term outcome of a surgical repair of SVA aneurysm. Ann Thorac Surg 2000;70:727–729
Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).
Transcatheter Closure of Ruptured Sinus of Valsalva Aneurysm into the Right Ventricle With an Amplatzer Vascular Plug II Harald Rittger,1* MD, Ulrike Gundlach,1 MD, and Andreas Koch,2 MD Objective: We report a case of a 52-year-old female patient with perforated sinus of Valsalva (SVA) aneurysm presenting with severe left-to-right shunt from the aorta into the right ventricle. Background: Aneuryms of the aortic sinus, better known as SVA are rare. Until interventional devices were introduced, the only treatment option for ruptured SVA aneurysms was surgery. Methods and Results: The case was discussed in the heart team and decision was made to implant an Amplatzer Vascular Plug (AVP) II. Initially, closing attempts using AVP 16 mm and 14 mm devices were performed. Presumably as a consequence of inadequate sizing, the discs were not well formed in the connecting tunnel, leaving a severe rest shunt after both procedures. Finally, after implantation of a 12 mm AVP-device, only a minimal rest shunt remained detectable. Absence of aortic regurgitation was confirmed by transesophageal echocardiography, and the occluder was released. Mean pulmonary artery pressure immediately decreased from 39 to 15 mm Hg. Medication with aspirin (100 mg qid) and clopidogrel (75 mg qid) was initiated. Follow-up examinations at 4 weeks and 3 months confirmed the minimal rest shunt and a continuous decrease of systolic pulmonary artery pressure to 35 mm Hg, as assessed with transthoracal echocardiography. Conclusion: In conclusion, in cases of SVA rupture, closure with an AVP II represents an alternative to surgical treatment and other devices used for transcatheter treatment of SVA. VC 2014 Wiley Periodicals, Inc.
Key words: sinus Valsalva aneurysm; transcatheter closure; Amplatzer Vascular Plug
INTRODUCTION
Aneuryms of the aortic sinus, better known as sinus of Valsalva (SVA) are rare. Etiology is either congenital or acquired due to trauma, infections, or postsurgery. The prevalence is reported to be higher in Asians and in men [1]. The prevalence of congenital defects in patients undergoing cardiac surgery is less than 1% [2]. According to Edwards and Burchell, it is caused by a congenital defect in the aortic media and an incomplete fusion of the distal bulbar septum and truncal ridges combined with malfusion of the aortic media and annulus fibrosis of the aortic valve [3]. When present, it appears most commonly in the right sinus rather than in the non-coronary or left sinus [2]. It rarely leads to symptoms, if it is not ruptured, but in case of rupture, it can cause worsening of heart failure symptoms or cause shunt with substantial effects on hemodynamics. Rupture in patients not undergoing surgery is estimated to be between 40% and 76% [4,5]. Sakakabara et al. have proposed a nomenclature for SVA aneuryms based on their site of origin [6]. According to this nomenclature, the lesion treated in C 2014 Wiley Periodicals, Inc. V
the present case is the most common, arising from the left part of the right sinus.
CASE REPORT
A 52-year-old female patient, who originated from the Philippines, was admitted to our clinic due to pro-
1
€tsklinikum Erlangen, ErlanMedizinische, Klinik 2, Universita gen, Germany 2 €tsklinikum Erlangen, €r Kinderkardiologie, Universita Klinik fu Erlangen, Germany Conflict of interest: Nothing to report. *Correspondence to: Harald Rittger, MD, Universit€atsklinikum Erlangen, Medizinische Klinik 2, Ulmenweg 18, 91054 Erlangen, Germany. E-mail: [email protected] Received 11 November 2013; Revision accepted 26 December 2013 DOI: 10.1002/ccd.25382 Published online 9 January 2014 in Wiley Online Library (wileyonlinelibrary.com)
Interventional Closure of Ruptured Sinus of Valsalva Aneurysm
167
Fig. 1. TEE demonstrating a severe shunt from the right aortic cusp into the right ventricle. [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.]
gressively increasing dyspnoea (New York Heart Association Class III) for several months. The patient had previously seen a general practitioner, who diagnosed a systolic heart murmur at initial examination and transferred the patient for echocardiography to our hospital. The patient reported no history of heart disease. Echocardiography revealed a shunt from the aortic root into the right ventricular outflow tract, moderate dilatation of the right ventricle with severely reduced function, and pulmonary hypertension with a systolic pulmonary artery pressure (PAP) of 60 mm Hg. There was no aortic regurgitation or ventricular septal defect. Lab values at admission were all in a normal range. Transesophageal echocardiography (TEE) confirmed the shunt into the right ventricular outflow tract, and detected its origin in the right aortic sinus (Fig. 1a and b). CT-angiography confirmed the rupture of an aneurysm of the right coronary sinus and the connection into the right ventricle with a defect size of 7 mm. The main stem of the pulmonary artery was dilated to a diameter 42 mm. Right heart catheterization showed a left-to-right shunt of 67% (Qp:Qs ¼ 3:1) and pulmonary hypertension with a mean PAP of 39 mm Hg. The case was discussed in the heart team. There is no device specifically designed for implantation in ruptured SVAs. As one margin of the device would be located within the right aortic cusp, there were concerns to cause aortic regurgitation with a larger disc, occluding the channel from the aortic margin. Therefore, initial plans to implant an Amplatzer DuctOccluder [1] or an Amplatzer-Septal-Occluder [7] were
discarded and decision was made to implant an Amplatzer Vascular Plug II (AVP II). The AVP (St. Jude Medical, St. Paul, MN) is a self-expanding, multi-layered Nitinol mesh occlusion device, which is available in different forms, shapes, and sizes. It is manufactured from 3 to 22 mm (Fig. 2). The procedure was performed under TEE guidance. After aortic root angiogram, which confirmed the leftto-right shunt, the ruptured aneurysm was crossed from the aorta with a 0.035 angled Terumo guide wire and a 5 Fr multipurpose catheter (MP 1). The right ventricle was passed, and the main stem of the pulmonary artery was engaged. Thereafter the initial guide wire was exchanged for a super-stiff guide wire (Amplatzer Super Stiff, Boston Scientific, Natick, MA) and a flexible 6 Fr sheath (Cook Medical, Bloomington, IN) was introduced into the pulmonary artery. Initially, closing attempts using AVP 16 mm and 14 mm devices were performed. Presumably as a consequence of inadequate sizing, the discs were not well formed in the connecting tunnel, leaving a severe rest shunt after both procedures. Finally after implantation of a 12 mm AVP-device, only a minimal rest shunt remained detectable. Absence of aortic regurgitation was confirmed by TEE, and the occluder was released (Fig. 3a and b). Mean pulmonary artery pressure immediately decreased from 39 to 15 mm Hg. Medication with aspirin (100 mg qid) and clopidogrel (75 mg qid) was initiated. Follow-up examinations at 4 weeks and 3 months confirmed the minimal rest shunt and a continuous decrease of systolic PAP to 35 mm Hg, as assessed with transthoracal echocardiography.
Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).
168
Rittger et al.
DISCUSSION
Until interventional devices were introduced, the only treatment option for ruptured SVA aneurysms was surgery. As various devices for transcatheter closure of vascular connections have become available, this technique appears to be a viable alternative to the surgical approach. It was first described by Cullen et al. in 1994, who performed SVA closure with a Rushkind umbrella device—originally designed for arterial duct
closure—in a 34-year-old man. Since then, there have been several historical reports about SVA-closure with coils and other devices [1,7–9]. To our knowledge, this is the first case of closure of a perforated SVA aneurysm with an AVP. The AVP is somewhat different to the more often used duct occluder. Both connections of the two discs at the proximal and distal margins of the device with the central part of the occluder are flexible. Consequently, the occluding and the anchoring part of this device can individually adjust to the given anatomy and an angulated final position can be achieved. In contrast to the AVP II, the Amplatzer duct occluder is much less adjustable to a tortuous anatomy, because all parts of the duct occluder tend to realign in a straight position after implantation. The new Amplatzer duct occluder II and the “Amplatzer duct occluder II-additional sizes” have the same flexibility as the AVP II, and therefore potentially would have been alternative devices. Unfortunately, both devices are available only in relatively small sizes (maximum waist diameter 6 and 5 mm, respectively). Therefore, in the present case, the AVP II was the only possible alternative for interventional closure of the shunt.
CONCLUSION
Fig. 2. Design and shape of the Amplatzer AVP II Occluder (courtesy of St. Jude Medical). [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.]
In conclusion, in cases of SVA rupture, closure with an AVP II represents an alternative to surgical treatment and other devices used for transcatheter treatment of SVA.
Fig. 3. (a) Aortogram with ruptured aneurysm of the right coronary cusp with shunt into the right ventricle. (b) Aortogram with the defect after closure with an Amplatzer-AVP-II-Occluder. [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.] Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).
Interventional Closure of Ruptured Sinus of Valsalva Aneurysm
REFERENCES 1. Chang CW, Chiu SN, Wu ET, Tsai SK, Wu MH, Wang JK. Transcatheter closure of a ruptured sinus of Valsalva aneurysm. Circ J 2006;70:1043–1047. 2. Takach T, Reul G, Cuncan M, Cooley D, Livesay J, Ott D, Frazier O. Sinus of vaslsalva aneurysm or fistula: Management and outcome. Ann Thorac Surg 1999;68:1573–1577. 3. Edwards E, Burchell HB. The pathological anatomy of deficiencies between the aortic root and the heart, including aortic sinus aneurysms. Thorax 1957;12:dd125–dd139. 4. Goldberg N, Krasnow N. Sinus of vasalva aneurysms. Clin Cardiol 1990;13:831–836.
169
5. 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;70:1466–1471. 6. Sakakabara S, Konno S. Congenital aneurysm of the sinus of Valsalva anatomy and classification. Am Heart J 1962;63:405–424. 7. Abidin N, Clarke B, Khattar RS. Percutaneous closure of ruptured sinus of Valsalva aneurysm using an Amplatzer occluder device. Heart 2005;91:244. 8. Chu SH, Hung CR, 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;99:288–298. 9. Naka Y, Kadoba K, Ohtake S, Sawa Y, Hirata N, Nishimura M, Matuda H. The long-term outcome of a surgical repair of SVA aneurysm. Ann Thorac Surg 2000;70:727–729
Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).
