Catheterization and Cardiovascular Interventions 86:1136–1140 (2015)
Transcatheter Repair of Aortic Leaflet Perforation in a Patient With Prior Surgical Bicuspid Aortic Valve Repair Wayland Lim, MD, Thomas Smith, MD, and Jason H. Rogers,* MD A 47-year-old male underwent surgery for functional bicuspid aortic valve severe regurgitation with a fused right and left coronary cusp. The patient presents nine years after surgical bicuspid aortic valve repair with symptomatic severe aortic regurgitation, diagnosed by TEE and MRI, caused primarily from a perforation located at the base of the surgically fused coronary cusps. The patient had a minimally dilated aortic root that did not yet necessitate surgical intervention. We present a novel percutaneous bicuspid aortic valve perforation repair that potentially decreases the number of surgical operations the patient must undergo during his lifetime. VC 2015 Wiley Periodicals, Inc. Key words: Amplatzer septal occluder; aortic regurgitation; percutaneous repair
INTRODUCTION
Aortic regurgitation can occur from multiple causes including congenital abnormalities, infection, aortic dissection or aneurysm, iatrogenic from percutaneous intervention, or blunt trauma [1]. Medical therapy is the primary course prior to symptoms or specific structural adaptive changes that are identified. In this case report, we present a patient with bicuspid aortic regurgitation who underwent surgical repair. Many years later the patient presented with recurrent symptomatic aortic regurgitation. We present a novel case of transcatheter repair for bicuspid aortic valve perforation with regurgitation. CASE DESCRIPTION
A 47-year-old male presented to the cardiology clinic with increasing lethargy and dyspnea on exertion. Nine years prior the patient had similar symptoms when he was diagnosed with severe aortic regurgitation caused by a fused and calcified right and left coronary cusp raphe limiting aortic valve closure. The ascending aorta was not aneurysmal. The patient did not want a mechanical valve and underwent surgical valve repair involving excision of the calcified raphe followed by surgical fusion using pledgeted 5.0 Ethibond (Ethicon, Bridgewater, NJ), and a running double layer of 5.0 to rejoin the right and left valve leaflets recreating a functioning bicuspid aortic valve. Next, a commissuroplasty of the bicuspid commissures was performed with interrupted 4.0 pledgeted prolene sutures (Fig. 1). Post-procedure, the patient had trace to 1þ aortic regurgitation. The postoperative course was uncompliC 2015 Wiley Periodicals, Inc. V
cated and the patient had improvement in dyspnea and lethargy. Transthoracic echocardiogram performed prior to the current presentation demonstrated a mildly dilated left ventricle with normal function, mildly dilated aortic root at 4.0 cm, bicuspid aortic valve with fused left and right coronary cusps, and (severe) 3–4þ aortic regurgitation. The aortic regurgitation was characterized as having a vena contracta greater than 0.6 cm, central jet occupying more than 65% of the left ventricular outflow tract, and 570 msec pressure half time. To better characterize the regurgitant jet transesophageal echocardiogram was performed. The regurgitant flow was comprised of two jets; one (1þ) central and the other (3þ) at the base of the previously surgically fused right and left coronary cusps. The larger jet appeared to travel through a 0.5 cm perforation versus surgical line dehiscence (Fig. 2). The aortic root was dilated and measured to be 4.2 cm with a normal
Division of Cardiovascular Medicine, Department of Medicine, University of California, Davis, California Conflict of interest: Dr. Rogers is a proctor for St Jude Medical. *Correspondence to: Jason H. Rogers, Division of Cardiovascular Medicine, Department of Medicine, University of California, Davis, CA. E-mail:[email protected] Received 18 November 2014; Revision accepted 23 March 2015 DOI: 10.1002/ccd.25958 Published online 24 April 2015 in Wiley Online Library (wileyonlinelibrary.com)
Transcatheter Repair for Bicuspid Aortic Valve Perforation With Regurgitation
diameter ascending aorta. There was no angiographic significant coronary artery disease seen on cardiac catheterization. To further characterize the regurgitant flow through the valvular defect and commissure an MRI was performed. Moderate severe aortic valve regurgitation was estimated with a 36% regurgitant fraction and a mildly ectatic aortic root at 4.3 cm with normal ascending aorta diameter [2]. Cardiothoracic surgery was consulted to evaluate for surgical intervention. While the patient could undergo surgery, the decision was made to elect for percutaneous repair. The rationale included several factors. First,
1137
the patient had a mildly dilated aortic root that did not warrant urgent surgical intervention at this time. Second, the patient did not want a mechanical valve because of the life-long anticoagulation and strongly preferred aortic valve repair or bioprosthetic aortic valve replacement. Based on his age, he would likely require a minimum of two redo surgical interventions, one to replace the current valve and a future surgery if his aorta dilated further and/or he required another bioprosthetic aortic valve. Each reoperation would have a higher mortality risk associated with the surgery. Transcatheter repair of the perforation with an
Fig. 1. Schematic demonstrating aortic valve before and after percutaneous repair. A: The left and right leaflets are labeled. The dotted line represents the surgical leaflet fusion after calcified raphe excision. The asterisk denotes the leaflet perforation. B: The leaflet perforation has now been closed using an Amplatzer septal occluder demarcated by the circle with hash marks.
Fig. 2. TEE images before (A–D) and after (E–G) percutaneous aortic valvular regurgitation repair. A: Long axis of the bicuspid aortic valve demonstrating a valvular defect in the fused left and right coronary cusp resulting in severe regurgitant flow with and without Doppler. B: Short axis of the bicuspid aortic valve during systole. The left and right coronary cusps are fused. C: Short axis image of the bicuspid aortic valve during diastole. D: Short axis of the bicuspid aortic valve during diastole with and without Doppler flow demonstrating a valvular defect in the fused left and right coronary
cusp. E: Long axis of the bicuspid aortic valve after percutaneous closure of the aortic valvular defect. The valvular regurgitant jet is greatly improved. F: Short axis of the aortic valve during systole after 4 mm Amplazter septal occluder. Leaflet movement does not appear to be limited by the device. G: Short axis of the aortic valve during diastole after 4 mm Amplazter septal occluder. Leaflet closure is not limited by the device. [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).
1138
Lim et al.
Fig. 3. Fluoroscopic and intracardiac echocardiogram images during the procedure. (A) and (C) are paired images with the 0.035 glidewire directed through the valvular defect. B: Demonstrates ICE imaging of the regurgitant jet through the defect after device deployment. D: This fluoroscopic diastolic image of
the 4 mm Amplazter septal occluder device during ascending aortic angiography through a 7 Fr angled pigtail. E, F: ICE images of the aortic valve during systole and diastole after device deployment. [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.]
Amplatzer device was considered to be feasible, was felt to be an effective less invasive option, and the heart team elected for this approach.
the left ventricle. A 4-Fr angled glide catheter (Terumo) was directed over the glidewire into the left ventricle. The AL2 guide catheter was advanced over the glide catheter and guidewire into the left ventricle. Although a 7-Fr system could be used an 8-Fr catheter provides added support preventing the rigid delivery cable from straightening the catheter. A 4 mm Amplazter septal occluder (St Jude Medical, St. Paul, MN) was then advanced through the AL2 guide catheter. The septal occluder was positioned and deployed across the aortic valve, with the left atrial disc on the ventricular side of the leaflet, and the right atrial disc on the aortic side (Fig. 3). Transthoracic echocardiogram the following day demonstrated 1þ central aortic regurgitation with some
DESCRIPTION OF PROCEDURE
The procedure was performed with conscious sedation using fluoroscopic and intracardiac echocardiographic guidance (ICE). The aortic valve is well seen with the ICE catheter and it offers patient comfort by not intubating the patient. Using femoral arterial access an 8 Fr AL2 guide catheter was directed to the left sinus of valsalva. A 0.03500 glidewire (Terumo, Somerset, NJ) was directed through the valvular defect into
Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).
Transcatheter Repair for Bicuspid Aortic Valve Perforation With Regurgitation
Fig. 4. Hemoglobin chart after percutaneous closure. The vertical axis is hemoglobin and the horizontal axis is days after closure. [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.]
trivial residual regurgitation around the septal occluder. The patient was discharged home the following day with aspirin 81 mg and clopidogrel 75 mg daily. CASE FOLLOW-UP
In clinic follow-up one week later, the patient complained about dark colored urine. Laboratory testing demonstrated normocytic anemia with Hgb (hemoglobin) of 8.9 g/l. An elevated LDH and decreased haptoglobin with urine analysis revealing amber color urine that was occult blood largely positive with only 3–6 red blood cells per high-powered field confirmed hemolysis. Prior to procedure, the patient’s Hgb was 13 g/l and the following day discharge Hgb was 12.4 g/l consistent with procedural blood loss and did not raise concerns for hemolysis. The patient was treated conservatively with iron supplementation. Clopidogrel was discontinued. No blood transfusions were required and the patient’s Hgb normalized over time. The most recent lab value 5 1=2 months later was 12.4 g/l (Fig. 4). Echocardiography in follow-up showed complete resolution of the trace regurgitation around the Amplatzer device. DISCUSSION
We herein report the first successful transcatheter repair of an aortic leaflet defect using an Amplatzer septal occluder. Although the mechanism of late perforation is not known, possibilities include late suture dehiscence, prior endocarditis, or native valvular tissue degeneration. While surgical aortic repair was a consideration, this would be a second attempt at open repair. The patient’s first repair, surgical excision of
1139
the calcified raphe followed by ligation without using bioprosthetic material, has been reported to provide the longest durability with 60% surviving 10 years without adverse events in a pediatric study [3]. Our patient had this same surgery described and has survived 9 years. Although not well studied, repeat surgical repair is not likely to have the same durability, as the defect would require leaflet reconstruction and possible patch repair. In the pediatric study, 50% of the patients that required the use of patches during primary repair had a significant event within 7 years compared to 14 years for those without patches. After a multi-disciplinary heart team meeting, the decision for attempted percutaneous aortic regurgitation intervention was planned as a method for avoiding surgical intervention and potentially reducing the number of future surgical re-interventions. Percutaneous valve repair has been described in two previous mitral valve cases, but never in the aortic position. The first case utilized an Amplatzer vascular plug to close a native mitral anterior leaflet puncture that likely occurred during a previous aortic valve replacement [4]. The second reported case by our group involved a mitral valve anterior leaflet perforation many years after combined coronary artery bypass grafting and mitral annuloplasty [5]. The etiology of the perforation in this case was suspected to be surgical technique with excessive suture tension or previous endocarditis. After serial negative blood cultures the perforation was closed successfully using an Amplatzer septal occluder. The current aortic regurgitation case is a unique scenario because the fused left and right coronary cusps created a large leaflet whose movement was not restricted by the smallest Amplatzer septal occluder that we used. This device could potentially be too large for a standard tricuspid aortic valve leaflet. Although not an issue in this scenario, placing an occluder device on the left or non-coronary aortic leaflets could also alter the motion of the mitral valve anterior leaflet resulting in either mitral stenosis or regurgitation. Valvular regurgitation can be seen after surgical mitral or aortic replacement. Surgical prosthetic aortic valves have reported 2–10% paravalvular leak rates, and mitral valves have slightly higher 7–17% paravalvular leak rates [6–8]. Paravalvular leaks resulting in aortic insufficiency occur more frequently after transcatheter aortic valve placement, up to 12% in the PARTNER trial [9]. A small fraction of these patients will develop symptomatic valvular regurgitation, heart failure, or hemolysis. The patients presenting with symptomatic valvular regurgitation within one year of surgical therapy have primarily congestive heart failure; hemolysis is seen in 13–47% of these patients [8,10]. Hemolysis is an important complication that
Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).
1140
Lim et al.
can result from residual high-velocity regurgitant jets after paravalvular leak closure, and this was seen in our patient. Symptomatic hemolysis can be treated with bone marrow stimulating agents (such as erythropoietin), intermittent transfusions, iron supplementation, repeat surgery, or repeat percutaneous paravalvular leak closure [11]. In a large published series, 36% of patients developed transfusion-dependent hemolytic anemia after transcatheter paravalvular leak closure with half of those persistent at up to 35 months follow-up. There was no correlation with regurgitation size and hemolysis. In another study, 35% of subjects had acute worsening symptoms or hemolysis after closure that resolved over 6 months [12]. After fivemonths our patient has demonstrated dramatic improvement of his anemia without the need for transfusion or bone marrow stimulators. The mechanism of his hemolytic anemia appeared to be related to the small amount of residual regurgitation around the Amplatzer device, which resolved over time as the device endothelialized. Surgical bicuspid aortic valve repair is a reasonable primary intervention with a 10 year freedom from reoperation at 78% at the Cleveland Clinc [13]. Of those undergoing reoperation, only 8% had redo-repair with the remaining undergoing replacement. As previously mentioned, this patient would likely require surgical reintervention for aortic dilation and/or valve failure. At this time, his aortic regurgitation has been successfully managed with the percutaneous placement of the Amplatzer septal occluder. In summary, percutaneous aortic valve repair using an Amplatzer device may be a safe and feasible technique in appropriately selected patients. This has delayed an additional reoperation for a patient who will likely require surgical intervention at a later date for another indication.
REFERENCES 1. Nishimura RA, Otto CM, Bonow RO, et al. 2014 AHA/ACC guideline for the management of patients with valvular heart disease: A report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. J Am Coll Cardiol 2014;63:2438–2488. 2. Myerson SG. Heart valve disease: Investigation by cardiovascular magnetic resonance. J Cardiovasc Magn Reson 2012;14:7. 3. Siddiqui J, Brizard CP, Konstantinov IE, et al. Outcomes after operations for bicuspid aortic valve disease in the pediatric population. Ann Thorac Surg 2013;96:2175–2183. 4. Velasco S, Larman M, Eneriz M. Percutaneous closure of a native mitral valve perforation. Rev Esp Cardiol 2010;63:597. 5. Javed U, Smith TW, Rogers JH. Percutaneous repair of anterior mitral leaflet perforation. J Invasive Cardiol 2012;24:134–137. 6. Hammermeister K, Sethi GK, Henderson WG, Grover FL, Oprian C, Rahimtoola SH. Outcomes 15 years after valve replacement with a mechanical versus a bioprosthetic valve: Final report of the veterans affairs randomized trial. J Am Coll Cardiol 2000;36:1152–1158. 7. Ionescu A, Fraser AG, Butchart EG. Prevalence and clinical significance of incidental paraprosthetic valvar regurgitation: A prospective study using transoesophageal echocardiography. Heart 2003;89:1316–1321. 8. Genoni M, Franzen D, Vogt P, et al. Paravalvular leakage after mitral valve replacement: Improved long-term survival with aggressive surgery? Eur J Cardiothorac Surg 2000;17:14–19. 9. Kodali SK, Williams MR, Smith CR, et al. Two-year outcomes after transcatheter or surgical aortic-valve replacement. N Engl J Med 2012;366:1686–1695. 10. De Cicco G, Russo C, Moreo A, et al. Mitral valve periprosthetic leakage: Anatomical observations in 135 patients from a multicentre study. Eur J Cardiothorac Surg 2006;30:887–891. 11. Sorajja P, Cabalka AK, Hagler DJ, Rihal CS. Long-term followup of percutaneous repair of paravalvular prosthetic regurgitation. J Am Coll Cardiol 2011;58:2218–2224. 12. Ruiz CE, Jelnin V, Kronzon I, et al. Clinical outcomes in patients undergoing percutaneous closure of periprosthetic paravalvular leaks. J Am Coll Cardiol 2011;58:2210–2217. 13. Svensson LG, Al Kindi AH, Vivacqua A, et al. Long-term durability of bicuspid aortic valve repair. Ann Thorac Surg 2014;97:1539–1548.
Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).
Transcatheter Repair of Aortic Leaflet Perforation in a Patient With Prior Surgical Bicuspid Aortic Valve Repair Wayland Lim, MD, Thomas Smith, MD, and Jason H. Rogers,* MD A 47-year-old male underwent surgery for functional bicuspid aortic valve severe regurgitation with a fused right and left coronary cusp. The patient presents nine years after surgical bicuspid aortic valve repair with symptomatic severe aortic regurgitation, diagnosed by TEE and MRI, caused primarily from a perforation located at the base of the surgically fused coronary cusps. The patient had a minimally dilated aortic root that did not yet necessitate surgical intervention. We present a novel percutaneous bicuspid aortic valve perforation repair that potentially decreases the number of surgical operations the patient must undergo during his lifetime. VC 2015 Wiley Periodicals, Inc. Key words: Amplatzer septal occluder; aortic regurgitation; percutaneous repair
INTRODUCTION
Aortic regurgitation can occur from multiple causes including congenital abnormalities, infection, aortic dissection or aneurysm, iatrogenic from percutaneous intervention, or blunt trauma [1]. Medical therapy is the primary course prior to symptoms or specific structural adaptive changes that are identified. In this case report, we present a patient with bicuspid aortic regurgitation who underwent surgical repair. Many years later the patient presented with recurrent symptomatic aortic regurgitation. We present a novel case of transcatheter repair for bicuspid aortic valve perforation with regurgitation. CASE DESCRIPTION
A 47-year-old male presented to the cardiology clinic with increasing lethargy and dyspnea on exertion. Nine years prior the patient had similar symptoms when he was diagnosed with severe aortic regurgitation caused by a fused and calcified right and left coronary cusp raphe limiting aortic valve closure. The ascending aorta was not aneurysmal. The patient did not want a mechanical valve and underwent surgical valve repair involving excision of the calcified raphe followed by surgical fusion using pledgeted 5.0 Ethibond (Ethicon, Bridgewater, NJ), and a running double layer of 5.0 to rejoin the right and left valve leaflets recreating a functioning bicuspid aortic valve. Next, a commissuroplasty of the bicuspid commissures was performed with interrupted 4.0 pledgeted prolene sutures (Fig. 1). Post-procedure, the patient had trace to 1þ aortic regurgitation. The postoperative course was uncompliC 2015 Wiley Periodicals, Inc. V
cated and the patient had improvement in dyspnea and lethargy. Transthoracic echocardiogram performed prior to the current presentation demonstrated a mildly dilated left ventricle with normal function, mildly dilated aortic root at 4.0 cm, bicuspid aortic valve with fused left and right coronary cusps, and (severe) 3–4þ aortic regurgitation. The aortic regurgitation was characterized as having a vena contracta greater than 0.6 cm, central jet occupying more than 65% of the left ventricular outflow tract, and 570 msec pressure half time. To better characterize the regurgitant jet transesophageal echocardiogram was performed. The regurgitant flow was comprised of two jets; one (1þ) central and the other (3þ) at the base of the previously surgically fused right and left coronary cusps. The larger jet appeared to travel through a 0.5 cm perforation versus surgical line dehiscence (Fig. 2). The aortic root was dilated and measured to be 4.2 cm with a normal
Division of Cardiovascular Medicine, Department of Medicine, University of California, Davis, California Conflict of interest: Dr. Rogers is a proctor for St Jude Medical. *Correspondence to: Jason H. Rogers, Division of Cardiovascular Medicine, Department of Medicine, University of California, Davis, CA. E-mail:[email protected] Received 18 November 2014; Revision accepted 23 March 2015 DOI: 10.1002/ccd.25958 Published online 24 April 2015 in Wiley Online Library (wileyonlinelibrary.com)
Transcatheter Repair for Bicuspid Aortic Valve Perforation With Regurgitation
diameter ascending aorta. There was no angiographic significant coronary artery disease seen on cardiac catheterization. To further characterize the regurgitant flow through the valvular defect and commissure an MRI was performed. Moderate severe aortic valve regurgitation was estimated with a 36% regurgitant fraction and a mildly ectatic aortic root at 4.3 cm with normal ascending aorta diameter [2]. Cardiothoracic surgery was consulted to evaluate for surgical intervention. While the patient could undergo surgery, the decision was made to elect for percutaneous repair. The rationale included several factors. First,
1137
the patient had a mildly dilated aortic root that did not warrant urgent surgical intervention at this time. Second, the patient did not want a mechanical valve because of the life-long anticoagulation and strongly preferred aortic valve repair or bioprosthetic aortic valve replacement. Based on his age, he would likely require a minimum of two redo surgical interventions, one to replace the current valve and a future surgery if his aorta dilated further and/or he required another bioprosthetic aortic valve. Each reoperation would have a higher mortality risk associated with the surgery. Transcatheter repair of the perforation with an
Fig. 1. Schematic demonstrating aortic valve before and after percutaneous repair. A: The left and right leaflets are labeled. The dotted line represents the surgical leaflet fusion after calcified raphe excision. The asterisk denotes the leaflet perforation. B: The leaflet perforation has now been closed using an Amplatzer septal occluder demarcated by the circle with hash marks.
Fig. 2. TEE images before (A–D) and after (E–G) percutaneous aortic valvular regurgitation repair. A: Long axis of the bicuspid aortic valve demonstrating a valvular defect in the fused left and right coronary cusp resulting in severe regurgitant flow with and without Doppler. B: Short axis of the bicuspid aortic valve during systole. The left and right coronary cusps are fused. C: Short axis image of the bicuspid aortic valve during diastole. D: Short axis of the bicuspid aortic valve during diastole with and without Doppler flow demonstrating a valvular defect in the fused left and right coronary
cusp. E: Long axis of the bicuspid aortic valve after percutaneous closure of the aortic valvular defect. The valvular regurgitant jet is greatly improved. F: Short axis of the aortic valve during systole after 4 mm Amplazter septal occluder. Leaflet movement does not appear to be limited by the device. G: Short axis of the aortic valve during diastole after 4 mm Amplazter septal occluder. Leaflet closure is not limited by the device. [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).
1138
Lim et al.
Fig. 3. Fluoroscopic and intracardiac echocardiogram images during the procedure. (A) and (C) are paired images with the 0.035 glidewire directed through the valvular defect. B: Demonstrates ICE imaging of the regurgitant jet through the defect after device deployment. D: This fluoroscopic diastolic image of
the 4 mm Amplazter septal occluder device during ascending aortic angiography through a 7 Fr angled pigtail. E, F: ICE images of the aortic valve during systole and diastole after device deployment. [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.]
Amplatzer device was considered to be feasible, was felt to be an effective less invasive option, and the heart team elected for this approach.
the left ventricle. A 4-Fr angled glide catheter (Terumo) was directed over the glidewire into the left ventricle. The AL2 guide catheter was advanced over the glide catheter and guidewire into the left ventricle. Although a 7-Fr system could be used an 8-Fr catheter provides added support preventing the rigid delivery cable from straightening the catheter. A 4 mm Amplazter septal occluder (St Jude Medical, St. Paul, MN) was then advanced through the AL2 guide catheter. The septal occluder was positioned and deployed across the aortic valve, with the left atrial disc on the ventricular side of the leaflet, and the right atrial disc on the aortic side (Fig. 3). Transthoracic echocardiogram the following day demonstrated 1þ central aortic regurgitation with some
DESCRIPTION OF PROCEDURE
The procedure was performed with conscious sedation using fluoroscopic and intracardiac echocardiographic guidance (ICE). The aortic valve is well seen with the ICE catheter and it offers patient comfort by not intubating the patient. Using femoral arterial access an 8 Fr AL2 guide catheter was directed to the left sinus of valsalva. A 0.03500 glidewire (Terumo, Somerset, NJ) was directed through the valvular defect into
Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).
Transcatheter Repair for Bicuspid Aortic Valve Perforation With Regurgitation
Fig. 4. Hemoglobin chart after percutaneous closure. The vertical axis is hemoglobin and the horizontal axis is days after closure. [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.]
trivial residual regurgitation around the septal occluder. The patient was discharged home the following day with aspirin 81 mg and clopidogrel 75 mg daily. CASE FOLLOW-UP
In clinic follow-up one week later, the patient complained about dark colored urine. Laboratory testing demonstrated normocytic anemia with Hgb (hemoglobin) of 8.9 g/l. An elevated LDH and decreased haptoglobin with urine analysis revealing amber color urine that was occult blood largely positive with only 3–6 red blood cells per high-powered field confirmed hemolysis. Prior to procedure, the patient’s Hgb was 13 g/l and the following day discharge Hgb was 12.4 g/l consistent with procedural blood loss and did not raise concerns for hemolysis. The patient was treated conservatively with iron supplementation. Clopidogrel was discontinued. No blood transfusions were required and the patient’s Hgb normalized over time. The most recent lab value 5 1=2 months later was 12.4 g/l (Fig. 4). Echocardiography in follow-up showed complete resolution of the trace regurgitation around the Amplatzer device. DISCUSSION
We herein report the first successful transcatheter repair of an aortic leaflet defect using an Amplatzer septal occluder. Although the mechanism of late perforation is not known, possibilities include late suture dehiscence, prior endocarditis, or native valvular tissue degeneration. While surgical aortic repair was a consideration, this would be a second attempt at open repair. The patient’s first repair, surgical excision of
1139
the calcified raphe followed by ligation without using bioprosthetic material, has been reported to provide the longest durability with 60% surviving 10 years without adverse events in a pediatric study [3]. Our patient had this same surgery described and has survived 9 years. Although not well studied, repeat surgical repair is not likely to have the same durability, as the defect would require leaflet reconstruction and possible patch repair. In the pediatric study, 50% of the patients that required the use of patches during primary repair had a significant event within 7 years compared to 14 years for those without patches. After a multi-disciplinary heart team meeting, the decision for attempted percutaneous aortic regurgitation intervention was planned as a method for avoiding surgical intervention and potentially reducing the number of future surgical re-interventions. Percutaneous valve repair has been described in two previous mitral valve cases, but never in the aortic position. The first case utilized an Amplatzer vascular plug to close a native mitral anterior leaflet puncture that likely occurred during a previous aortic valve replacement [4]. The second reported case by our group involved a mitral valve anterior leaflet perforation many years after combined coronary artery bypass grafting and mitral annuloplasty [5]. The etiology of the perforation in this case was suspected to be surgical technique with excessive suture tension or previous endocarditis. After serial negative blood cultures the perforation was closed successfully using an Amplatzer septal occluder. The current aortic regurgitation case is a unique scenario because the fused left and right coronary cusps created a large leaflet whose movement was not restricted by the smallest Amplatzer septal occluder that we used. This device could potentially be too large for a standard tricuspid aortic valve leaflet. Although not an issue in this scenario, placing an occluder device on the left or non-coronary aortic leaflets could also alter the motion of the mitral valve anterior leaflet resulting in either mitral stenosis or regurgitation. Valvular regurgitation can be seen after surgical mitral or aortic replacement. Surgical prosthetic aortic valves have reported 2–10% paravalvular leak rates, and mitral valves have slightly higher 7–17% paravalvular leak rates [6–8]. Paravalvular leaks resulting in aortic insufficiency occur more frequently after transcatheter aortic valve placement, up to 12% in the PARTNER trial [9]. A small fraction of these patients will develop symptomatic valvular regurgitation, heart failure, or hemolysis. The patients presenting with symptomatic valvular regurgitation within one year of surgical therapy have primarily congestive heart failure; hemolysis is seen in 13–47% of these patients [8,10]. Hemolysis is an important complication that
Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).
1140
Lim et al.
can result from residual high-velocity regurgitant jets after paravalvular leak closure, and this was seen in our patient. Symptomatic hemolysis can be treated with bone marrow stimulating agents (such as erythropoietin), intermittent transfusions, iron supplementation, repeat surgery, or repeat percutaneous paravalvular leak closure [11]. In a large published series, 36% of patients developed transfusion-dependent hemolytic anemia after transcatheter paravalvular leak closure with half of those persistent at up to 35 months follow-up. There was no correlation with regurgitation size and hemolysis. In another study, 35% of subjects had acute worsening symptoms or hemolysis after closure that resolved over 6 months [12]. After fivemonths our patient has demonstrated dramatic improvement of his anemia without the need for transfusion or bone marrow stimulators. The mechanism of his hemolytic anemia appeared to be related to the small amount of residual regurgitation around the Amplatzer device, which resolved over time as the device endothelialized. Surgical bicuspid aortic valve repair is a reasonable primary intervention with a 10 year freedom from reoperation at 78% at the Cleveland Clinc [13]. Of those undergoing reoperation, only 8% had redo-repair with the remaining undergoing replacement. As previously mentioned, this patient would likely require surgical reintervention for aortic dilation and/or valve failure. At this time, his aortic regurgitation has been successfully managed with the percutaneous placement of the Amplatzer septal occluder. In summary, percutaneous aortic valve repair using an Amplatzer device may be a safe and feasible technique in appropriately selected patients. This has delayed an additional reoperation for a patient who will likely require surgical intervention at a later date for another indication.
REFERENCES 1. Nishimura RA, Otto CM, Bonow RO, et al. 2014 AHA/ACC guideline for the management of patients with valvular heart disease: A report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. J Am Coll Cardiol 2014;63:2438–2488. 2. Myerson SG. Heart valve disease: Investigation by cardiovascular magnetic resonance. J Cardiovasc Magn Reson 2012;14:7. 3. Siddiqui J, Brizard CP, Konstantinov IE, et al. Outcomes after operations for bicuspid aortic valve disease in the pediatric population. Ann Thorac Surg 2013;96:2175–2183. 4. Velasco S, Larman M, Eneriz M. Percutaneous closure of a native mitral valve perforation. Rev Esp Cardiol 2010;63:597. 5. Javed U, Smith TW, Rogers JH. Percutaneous repair of anterior mitral leaflet perforation. J Invasive Cardiol 2012;24:134–137. 6. Hammermeister K, Sethi GK, Henderson WG, Grover FL, Oprian C, Rahimtoola SH. Outcomes 15 years after valve replacement with a mechanical versus a bioprosthetic valve: Final report of the veterans affairs randomized trial. J Am Coll Cardiol 2000;36:1152–1158. 7. Ionescu A, Fraser AG, Butchart EG. Prevalence and clinical significance of incidental paraprosthetic valvar regurgitation: A prospective study using transoesophageal echocardiography. Heart 2003;89:1316–1321. 8. Genoni M, Franzen D, Vogt P, et al. Paravalvular leakage after mitral valve replacement: Improved long-term survival with aggressive surgery? Eur J Cardiothorac Surg 2000;17:14–19. 9. Kodali SK, Williams MR, Smith CR, et al. Two-year outcomes after transcatheter or surgical aortic-valve replacement. N Engl J Med 2012;366:1686–1695. 10. De Cicco G, Russo C, Moreo A, et al. Mitral valve periprosthetic leakage: Anatomical observations in 135 patients from a multicentre study. Eur J Cardiothorac Surg 2006;30:887–891. 11. Sorajja P, Cabalka AK, Hagler DJ, Rihal CS. Long-term followup of percutaneous repair of paravalvular prosthetic regurgitation. J Am Coll Cardiol 2011;58:2218–2224. 12. Ruiz CE, Jelnin V, Kronzon I, et al. Clinical outcomes in patients undergoing percutaneous closure of periprosthetic paravalvular leaks. J Am Coll Cardiol 2011;58:2210–2217. 13. Svensson LG, Al Kindi AH, Vivacqua A, et al. Long-term durability of bicuspid aortic valve repair. Ann Thorac Surg 2014;97:1539–1548.
Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).
