Catheterization and Cardiovascular Interventions 85:615–619 (2015)
Transcatheter Neoaortic Valve Replacement Utilizing the Melody Valve in Hypoplastic Left Heart Syndrome Mary Hunt Martin,1* MD, Peter J. Gruber,2 MD, and Robert G. Gray,1 MD Percutaneous transcatheter pulmonary valve replacement with the Melody Valve is fast becoming an important adjunct in the treatment of older children and adults with failing right ventricular outflow tract conduits. Recently, the Melody Valve has also been successfully implanted in the tricuspid, mitral, and aortic positions, typically within a failing bioprosthetic valve. We present a patient who underwent Fontan palliation for hypoplastic left heart syndrome variant and subsequently developed severe neoaortic regurgitation, which was successfully treated with a transcatheter neoaortic valve replacement. To our knowledge, this is the first successful use of the Melody Valve in the neoaortic position in a patient with single-ventricle physiology. Successful relief of neoaortic valve regurgitation using replacement with a transcatheter valve may allow avoidance of additional surgery, increase functional longevity of single-ventricle palliation, and postpone the need for orthotopic heart transplantation. VC 2014 Wiley Periodicals, Inc. Key words: congenital heart disease; pediatric intervention; valve disease
INTRODUCTION
Percutaneous pulmonary valve replacement has become an important treatment option for patients with congenital heart disease and failing right ventricular outflow tract conduits [1,2]. Prior to the advent of transcatheter valve implantation, these patients faced a future of multiple surgical pulmonary valve replacements utilizing cardiopulmonary bypass and repeat sternotomies that may increase patient morbidity. The Melody Valve, (Medtronic, Minneapolis, MN,) is currently the only percutaneous valve-stent that is approved for use in the pulmonary position in the United States, albeit on a Humanitarian Device Exemption status. Since the introduction of the Melody Valve in 2000, its use has been reported within the native right ventricular outflow tract, as well as within surgically placed bioprosthetic valves in the pulmonic, tricuspid, mitral, and even aortic positions [3–5]. To date, the performance of the Melody Valve in high-pressure hemodynamic environments has exceeded expectations [6]. There have been no reports of Melody Valve implantation within a native semilunar valve neither in the systemic position, nor in a patient with palliated hypoplastic left heart syndrome (HLHS). We report the successful implantation of a Medtronic Melody Valve C 2014 Wiley Periodicals, Inc. V
in the neoaortic position in a patient with HLHS variant and severe neoaortic insufficiency. CASE HISTORY
This 8-year-old, 23-kg girl initially presented in the newborn period with severe aortic valve stenosis, mild left ventricular hypoplasia, and poor left ventricular function. Because her mitral valve measurements were within normal limits and her left ventricle only mildly 1
Department of Pediatric Cardiology, University of Utah at Primary Children’s Medical Center, Salt Lake City, Utah 2 Department of Pediatric Cardiothoracic Surgery, University of Utah at Primary Children’s Medical Center, Salt Lake City, Utah Conflict of interest: Nothing to report *Correspondence to: Mary Hunt Martin, MD; Primary Children’s Medical Center, 100 North Mario Capecchi Drive, Suite 1500, Salt Lake City, UT, 84113. E-mail: [email protected] Received 17 July 2013; Revision accepted 2 March 2014 DOI: 10.1002/ccd.25472 Published online 11 March 2014 in Wiley Online Library (wileyonlinelibrary.com)
616
Martin et al.
Fig. 1. Echocardiographic images preintervention showing severe neoaortic insufficiency in two dimensions (A) and with color Doppler (B).
hypoplastic, she initially underwent balloon aortic valvuloplasty on day of life 2. Despite relief of the aortic stenosis, her left ventricle failed to support her systemic circulation. She underwent a stage I palliation with a Damus–Kaye Stansel procedure, patch augmentation of her aortic arch, and a right ventricle to pulmonary artery conduit. She developed progressive neoaortic and native aortic insufficiency, and at the time of her stage II surgery at 4 months of age, she also underwent oversewing of the native aortic valve and repair of the neoaortic valve. This involved commissuroplasty at each commissure. The fenestrated Fontan completion utilizing an extracardiac 18-mm Gore-Tex tube was performed at 2.5 years of age and included tricuspid annuloplasty for progressive tricuspid regurgitation. Over the following year, she developed increasing cyanosis and underwent percutaneous closure of her Fontan fenestration with a 4-mm Amplatzer Septal Occluder device at 4 years of age. Her condition deteriorated over the next 4 years as her neoaortic valve insufficiency progressed and she became symptomatic with fatigue and frequent dizzy spells. Her echocardiogram and magnetic resonance imaging (MRI) demonstrated normal right ventricular systolic function, unobstructed Fontan pathway, mild neoaortic stenosis, and severe insufficiency, (Fig. 1), with holodiastolic flow reversal in the abdominal aorta, and a neoaortic regurgitant fraction of 43%. Because she had already undergone neoaortic valve repair, she was likely to require valve replacement to eliminate the regurgitation. Given the potential benefits of eliminating her neoaortic insufficiency without the use of repeat sternotomy, cardiopulmonary bypass, and expo-
sure to multiple blood products, we explored the option of percutaneous neoaortic valve replacement. INTERVENTION
Both MRI and echocardiogram estimated the neoaortic valve annulus to be approximately 23 mm in diameter, and given her previous surgical valvotomy and valvar stenosis and calcification, we anticipated that balloon sizing would yield a Melody “landing zone” closer to 20 mm. Additionally, given the recent series published showing excellent Melody Valve function at a diameter of 24 mm, we considered it a possibility to implant the valve at 24 mm if necessary to stabilize the valve [7]. In planning the procedure, we discussed several approaches including a hybrid procedure utilizing surgical access via a transapical or transaortic approach; however, we ultimately decided to attempt a transvenous approach via her right internal jugular vein utilizing a transeptal needle to cross the Fontan conduit to access the right atrium. The internal jugular approach was chosen over the femoral approach due to the presence of the Ampatzer Septal Occluder, previously used to close her fenestration, in the inferior portion of the Fontan baffle, leaving little space for access to the heart from below without removing the device. We planned to balloon size the neoaortic valve, and to attempt a Melody Valve implant only if there seemed to be a reasonable waist in the balloon at the stenotic valve in which to seat the new valve. Consent for offlabel use of a humanitarian device was obtained. The procedure was performed under general anesthesia. A 9-French sheath was placed in the left femoral
Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).
Transcatheter Neoaortic Valve Replacement
617
Fig. 2. Angiography performed in the aortic root showing patent DKS anastamosis and severe neoaortic insufficiency, (A) absence of coronary artery compression during balloon inflation across the neoaortic valve, and a discrete waist in the compliant balloon, (B) Melody Valve in place in the neoaortic position. There is trace neoaortic insufficiency and no coronary artery compression, (C).
artery and a 6-French sheath was placed in the right femoral vein. The gradient across the neoaortic valve was 14 mm Hg and the pulse pressure in the descending aorta was 69 mm Hg. Angiography in the aortic root showed severe regurgitation of the neoaortic valve and significant dilation of her reconstructed ascending aorta, (Fig. 2) A 22 mm 5 cm Tyshak II balloon, (NuMed, Hopkinton, NY), was advanced over an 0.035” Amplatz superstiff J-wire, (Cook, Bloomington, IN), positioned in the right ventricular (RV) apex, and gently inflated across the neoaortic valve annulus, demonstrating a 18-mm discrete waist. At this point, a 7-French long Mullins sheath, (Cook), was placed in the right internal jugular vein and advanced over a wire into the Fontan baffle. Angiography was performed in the Fontan baffle to visualize the baffle and its relationship to the right atrium, which was seen in levophase. A Brockenbrough transeptal needle was bent to increase its angle and was advanced through the sheath and dilator. Under fluoroscopic guidance, we performed a “transseptal” puncture across the Fontan baffle into the right atrium. A 6-French wedge catheter was then advanced through the sheath and across the tricuspid and neoaortic valves into the descending aorta. An exchange-length Rosen wire was subsequently advanced through the wedge catheter and snared in the descending aorta, creating a rail system for guidewire support. To assess the risk of potential coronary artery compression by the valvestent, a 22 mm 4 cm Tyshak II balloon was advanced across the neoaortic valve and we performed simultaneous balloon inflation and ascending aortic angiography. This demonstrated favorable coronary artery anatomy with unobstructed flow arising from the hypoplastic
native aortic root. The Fontan baffle perforation was then dilated with an 8-mm cutting balloon, (Boston Scientific, Miami, FL), which enlarged the fenestration sufficiently to allow the Melody delivery system to pass. We hand-crimped the Melody Valve onto a 22-mm Ensemble delivery system and exchanged the 0.035” Rosen wire for a 0.035” Lunderquist wire, bent to a shape imitating the prior path of the Rosen wire, again snaring the distal guidewire in the descending aorta. The delivery system (22 French) was then advanced through the right internal jugular vein, Fontan baffle, tricuspid valve, and across the neoaortic valve. The Melody Valve was then deployed across the neoaortic annulus. Despite the stiff wire and delivery system across the tricuspid and neoaortic valves, the patient remained hemodynamically stable as the valve was advanced through the heart. During and immediately following valve implantation, the patient developed complete heart block that quickly resolved without treatment or recurrence. Postimplantation angiography and transthoracic echocardiography confirmed appropriate positioning of the Melody Valve, without regurgitation through the valve itself and 2 small 1-mm perivalvar leaks (Fig. 3). No other echo modalities were used during the procedure. The guidewire was removed and postimplantation hemodynamics showed no pressure gradient across the valve and a pulse pressure that had narrowed to 45 mm Hg. Finally, her recently created Fontan fenestration was occluded utilizing a 6-mm Amplatzer Septal Occluder delivered using a 7-French Amplatzer system via an internal jugular venous approach. The procedure time was 3 hr
Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).
618
Martin et al.
Fig. 3. Echocardiographic images postintervention showing Melody Valve in neoaortic position in two dimensions (A), with unobstructed outflow by color Doppler (B), and with only trace parivalvar leak, and no insufficiency of the valve (C).
and 55 min, and the fluoroscopy time was 49 min. She was discharged the following day after echocardiogram demonstrated unchanged position and function of the Melody Valve. At her 2-month follow-up appointment, her echocardiogram demonstrates stable Melody Valve position and function with trace perivalvar leaks. She reports markedly improved energy, and resolution of her dizzy spells. DISCUSSION
We describe a successful, novel, and minimally invasive approach to palliation of the failed neoaortic valve in a high-risk patient with HLHS. Studies regarding the longevity of the pulmonary valve in the systemic circulation of patients with an arterial switch, Ross procedure, and HLHS show that it typically performs well over time [8]. However, in the event that the neoaortic valve does fail, particularly in the patient where surgical valve replacement is particularly high risk, percutaneous valve replacement may be considered in patients with favorable anatomy. This case demonstrates the technical feasibility of Melody Valve placement within a native semilunar valve in the systemic circulation. The patient’s anatomy was optimal, with mild stenosis of the valve providing a “landing zone.” Unlike implantation in the native aortic position, this patient’s coronary artery origins were not at risk for being directly covered by the stent as they arise off of her native ascending aorta. Nonetheless, we carefully considered the anatomical
issues that might be present including the potential for compression of the native aortic root or coronary branches with valve implantation in the neoaortic position. Despite the complexity of the procedure, there were no significant complications. At the most recent follow-up, this patient has had an excellent clinical outcome; however, we anticipate that the function of the Melody Valve will decline over time, and have planned for close observation with serial echocardiograms. Use of the Melody Valve as an intermediate solution in this child leaves her with several options when future intervention is needed. These include placement of a second Melody Valve within the first, surgical valve replacement, and orthotopic heart transplantation. In the meantime, her quality of life has improved by using this minimally invasive approach. CONCLUSIONS
To our knowledge, this is the first reported successful transcatheter implantation of a Melody Valve in the neoaortic position in a patient with HLHS. This case demonstrates technical feasibility of the procedure in a patient with favorable anatomy, as well as short-term clinical success using a novel approach to palliation of the failed neoaortic valve. REFERENCES 1. Zahn EM, Hellenbrand WE, Lock JE, McElhinney DB. Implantation of the melody transcatheter pulmonary valve in patients with a dysfunctional right ventricular outflow tract conduit early
Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).
Transcatheter Neoaortic Valve Replacement results from the U.S. Clinical trial. J Am Coll Cardiol 2009;54: 1722–1729. 2. McElhinney DB, Hellenbrand WE, Zahn EM, et al. Short- and medium-term outcomes after transcatheter pulmonary valve placement in the expanded multicenter US melody valve trial. Circulation 2010;122:507–516. 3. Cullen MW, Cabalka AK, Alli OO, et al. Transvenous, antegrade melody valve-in-valve implantation for bioprosthetic mitral and tricuspid valve dysfunction: A case series in children and adults. JACC Cardiovasc Interv 2013;6:598–605. 4. Roberts PA, Boudjemline Y, Cheatham JP, et al. Percutaneous tricuspid valve replacement in congenital and acquired heart disease. J Am Coll Cardiol 2011;58:117–122.
619
5. Boshoff DE, Cools BL, Heying R, et al. Off-label use of percutaneous pulmonary valved stents in the right ventricular outflow tract: Time to rewrite the label? Catheter Cardiovasc Interv 2013;81:987–995. 6. Hasan BS, McElhinney DB, Brown DW, et al. Short-term performance of the transcatheter Melody valve in high-pressure hemodynamic environments in the pulmonary and systemic circulations. Circ Cardiovasc Interv 2011;4:615–620. 7. Cheatham SL, Holzer RJ, Chisolm JL, Cheatham JP. The medtronic melody(R) transcatheter pulmonary valve implanted AT 24 mm Diameter. IT Works. Catheter Cardiovasc Interv 2013. 8. Schmid FX, Hilker M, Kampmann C, Mayer E, Oelert H. Clinical performance of the native pulmonary valve in the systemic circulation. J Heart Valve Dis 1998;7:620–625.
Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).
Transcatheter Neoaortic Valve Replacement Utilizing the Melody Valve in Hypoplastic Left Heart Syndrome Mary Hunt Martin,1* MD, Peter J. Gruber,2 MD, and Robert G. Gray,1 MD Percutaneous transcatheter pulmonary valve replacement with the Melody Valve is fast becoming an important adjunct in the treatment of older children and adults with failing right ventricular outflow tract conduits. Recently, the Melody Valve has also been successfully implanted in the tricuspid, mitral, and aortic positions, typically within a failing bioprosthetic valve. We present a patient who underwent Fontan palliation for hypoplastic left heart syndrome variant and subsequently developed severe neoaortic regurgitation, which was successfully treated with a transcatheter neoaortic valve replacement. To our knowledge, this is the first successful use of the Melody Valve in the neoaortic position in a patient with single-ventricle physiology. Successful relief of neoaortic valve regurgitation using replacement with a transcatheter valve may allow avoidance of additional surgery, increase functional longevity of single-ventricle palliation, and postpone the need for orthotopic heart transplantation. VC 2014 Wiley Periodicals, Inc. Key words: congenital heart disease; pediatric intervention; valve disease
INTRODUCTION
Percutaneous pulmonary valve replacement has become an important treatment option for patients with congenital heart disease and failing right ventricular outflow tract conduits [1,2]. Prior to the advent of transcatheter valve implantation, these patients faced a future of multiple surgical pulmonary valve replacements utilizing cardiopulmonary bypass and repeat sternotomies that may increase patient morbidity. The Melody Valve, (Medtronic, Minneapolis, MN,) is currently the only percutaneous valve-stent that is approved for use in the pulmonary position in the United States, albeit on a Humanitarian Device Exemption status. Since the introduction of the Melody Valve in 2000, its use has been reported within the native right ventricular outflow tract, as well as within surgically placed bioprosthetic valves in the pulmonic, tricuspid, mitral, and even aortic positions [3–5]. To date, the performance of the Melody Valve in high-pressure hemodynamic environments has exceeded expectations [6]. There have been no reports of Melody Valve implantation within a native semilunar valve neither in the systemic position, nor in a patient with palliated hypoplastic left heart syndrome (HLHS). We report the successful implantation of a Medtronic Melody Valve C 2014 Wiley Periodicals, Inc. V
in the neoaortic position in a patient with HLHS variant and severe neoaortic insufficiency. CASE HISTORY
This 8-year-old, 23-kg girl initially presented in the newborn period with severe aortic valve stenosis, mild left ventricular hypoplasia, and poor left ventricular function. Because her mitral valve measurements were within normal limits and her left ventricle only mildly 1
Department of Pediatric Cardiology, University of Utah at Primary Children’s Medical Center, Salt Lake City, Utah 2 Department of Pediatric Cardiothoracic Surgery, University of Utah at Primary Children’s Medical Center, Salt Lake City, Utah Conflict of interest: Nothing to report *Correspondence to: Mary Hunt Martin, MD; Primary Children’s Medical Center, 100 North Mario Capecchi Drive, Suite 1500, Salt Lake City, UT, 84113. E-mail: [email protected] Received 17 July 2013; Revision accepted 2 March 2014 DOI: 10.1002/ccd.25472 Published online 11 March 2014 in Wiley Online Library (wileyonlinelibrary.com)
616
Martin et al.
Fig. 1. Echocardiographic images preintervention showing severe neoaortic insufficiency in two dimensions (A) and with color Doppler (B).
hypoplastic, she initially underwent balloon aortic valvuloplasty on day of life 2. Despite relief of the aortic stenosis, her left ventricle failed to support her systemic circulation. She underwent a stage I palliation with a Damus–Kaye Stansel procedure, patch augmentation of her aortic arch, and a right ventricle to pulmonary artery conduit. She developed progressive neoaortic and native aortic insufficiency, and at the time of her stage II surgery at 4 months of age, she also underwent oversewing of the native aortic valve and repair of the neoaortic valve. This involved commissuroplasty at each commissure. The fenestrated Fontan completion utilizing an extracardiac 18-mm Gore-Tex tube was performed at 2.5 years of age and included tricuspid annuloplasty for progressive tricuspid regurgitation. Over the following year, she developed increasing cyanosis and underwent percutaneous closure of her Fontan fenestration with a 4-mm Amplatzer Septal Occluder device at 4 years of age. Her condition deteriorated over the next 4 years as her neoaortic valve insufficiency progressed and she became symptomatic with fatigue and frequent dizzy spells. Her echocardiogram and magnetic resonance imaging (MRI) demonstrated normal right ventricular systolic function, unobstructed Fontan pathway, mild neoaortic stenosis, and severe insufficiency, (Fig. 1), with holodiastolic flow reversal in the abdominal aorta, and a neoaortic regurgitant fraction of 43%. Because she had already undergone neoaortic valve repair, she was likely to require valve replacement to eliminate the regurgitation. Given the potential benefits of eliminating her neoaortic insufficiency without the use of repeat sternotomy, cardiopulmonary bypass, and expo-
sure to multiple blood products, we explored the option of percutaneous neoaortic valve replacement. INTERVENTION
Both MRI and echocardiogram estimated the neoaortic valve annulus to be approximately 23 mm in diameter, and given her previous surgical valvotomy and valvar stenosis and calcification, we anticipated that balloon sizing would yield a Melody “landing zone” closer to 20 mm. Additionally, given the recent series published showing excellent Melody Valve function at a diameter of 24 mm, we considered it a possibility to implant the valve at 24 mm if necessary to stabilize the valve [7]. In planning the procedure, we discussed several approaches including a hybrid procedure utilizing surgical access via a transapical or transaortic approach; however, we ultimately decided to attempt a transvenous approach via her right internal jugular vein utilizing a transeptal needle to cross the Fontan conduit to access the right atrium. The internal jugular approach was chosen over the femoral approach due to the presence of the Ampatzer Septal Occluder, previously used to close her fenestration, in the inferior portion of the Fontan baffle, leaving little space for access to the heart from below without removing the device. We planned to balloon size the neoaortic valve, and to attempt a Melody Valve implant only if there seemed to be a reasonable waist in the balloon at the stenotic valve in which to seat the new valve. Consent for offlabel use of a humanitarian device was obtained. The procedure was performed under general anesthesia. A 9-French sheath was placed in the left femoral
Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).
Transcatheter Neoaortic Valve Replacement
617
Fig. 2. Angiography performed in the aortic root showing patent DKS anastamosis and severe neoaortic insufficiency, (A) absence of coronary artery compression during balloon inflation across the neoaortic valve, and a discrete waist in the compliant balloon, (B) Melody Valve in place in the neoaortic position. There is trace neoaortic insufficiency and no coronary artery compression, (C).
artery and a 6-French sheath was placed in the right femoral vein. The gradient across the neoaortic valve was 14 mm Hg and the pulse pressure in the descending aorta was 69 mm Hg. Angiography in the aortic root showed severe regurgitation of the neoaortic valve and significant dilation of her reconstructed ascending aorta, (Fig. 2) A 22 mm 5 cm Tyshak II balloon, (NuMed, Hopkinton, NY), was advanced over an 0.035” Amplatz superstiff J-wire, (Cook, Bloomington, IN), positioned in the right ventricular (RV) apex, and gently inflated across the neoaortic valve annulus, demonstrating a 18-mm discrete waist. At this point, a 7-French long Mullins sheath, (Cook), was placed in the right internal jugular vein and advanced over a wire into the Fontan baffle. Angiography was performed in the Fontan baffle to visualize the baffle and its relationship to the right atrium, which was seen in levophase. A Brockenbrough transeptal needle was bent to increase its angle and was advanced through the sheath and dilator. Under fluoroscopic guidance, we performed a “transseptal” puncture across the Fontan baffle into the right atrium. A 6-French wedge catheter was then advanced through the sheath and across the tricuspid and neoaortic valves into the descending aorta. An exchange-length Rosen wire was subsequently advanced through the wedge catheter and snared in the descending aorta, creating a rail system for guidewire support. To assess the risk of potential coronary artery compression by the valvestent, a 22 mm 4 cm Tyshak II balloon was advanced across the neoaortic valve and we performed simultaneous balloon inflation and ascending aortic angiography. This demonstrated favorable coronary artery anatomy with unobstructed flow arising from the hypoplastic
native aortic root. The Fontan baffle perforation was then dilated with an 8-mm cutting balloon, (Boston Scientific, Miami, FL), which enlarged the fenestration sufficiently to allow the Melody delivery system to pass. We hand-crimped the Melody Valve onto a 22-mm Ensemble delivery system and exchanged the 0.035” Rosen wire for a 0.035” Lunderquist wire, bent to a shape imitating the prior path of the Rosen wire, again snaring the distal guidewire in the descending aorta. The delivery system (22 French) was then advanced through the right internal jugular vein, Fontan baffle, tricuspid valve, and across the neoaortic valve. The Melody Valve was then deployed across the neoaortic annulus. Despite the stiff wire and delivery system across the tricuspid and neoaortic valves, the patient remained hemodynamically stable as the valve was advanced through the heart. During and immediately following valve implantation, the patient developed complete heart block that quickly resolved without treatment or recurrence. Postimplantation angiography and transthoracic echocardiography confirmed appropriate positioning of the Melody Valve, without regurgitation through the valve itself and 2 small 1-mm perivalvar leaks (Fig. 3). No other echo modalities were used during the procedure. The guidewire was removed and postimplantation hemodynamics showed no pressure gradient across the valve and a pulse pressure that had narrowed to 45 mm Hg. Finally, her recently created Fontan fenestration was occluded utilizing a 6-mm Amplatzer Septal Occluder delivered using a 7-French Amplatzer system via an internal jugular venous approach. The procedure time was 3 hr
Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).
618
Martin et al.
Fig. 3. Echocardiographic images postintervention showing Melody Valve in neoaortic position in two dimensions (A), with unobstructed outflow by color Doppler (B), and with only trace parivalvar leak, and no insufficiency of the valve (C).
and 55 min, and the fluoroscopy time was 49 min. She was discharged the following day after echocardiogram demonstrated unchanged position and function of the Melody Valve. At her 2-month follow-up appointment, her echocardiogram demonstrates stable Melody Valve position and function with trace perivalvar leaks. She reports markedly improved energy, and resolution of her dizzy spells. DISCUSSION
We describe a successful, novel, and minimally invasive approach to palliation of the failed neoaortic valve in a high-risk patient with HLHS. Studies regarding the longevity of the pulmonary valve in the systemic circulation of patients with an arterial switch, Ross procedure, and HLHS show that it typically performs well over time [8]. However, in the event that the neoaortic valve does fail, particularly in the patient where surgical valve replacement is particularly high risk, percutaneous valve replacement may be considered in patients with favorable anatomy. This case demonstrates the technical feasibility of Melody Valve placement within a native semilunar valve in the systemic circulation. The patient’s anatomy was optimal, with mild stenosis of the valve providing a “landing zone.” Unlike implantation in the native aortic position, this patient’s coronary artery origins were not at risk for being directly covered by the stent as they arise off of her native ascending aorta. Nonetheless, we carefully considered the anatomical
issues that might be present including the potential for compression of the native aortic root or coronary branches with valve implantation in the neoaortic position. Despite the complexity of the procedure, there were no significant complications. At the most recent follow-up, this patient has had an excellent clinical outcome; however, we anticipate that the function of the Melody Valve will decline over time, and have planned for close observation with serial echocardiograms. Use of the Melody Valve as an intermediate solution in this child leaves her with several options when future intervention is needed. These include placement of a second Melody Valve within the first, surgical valve replacement, and orthotopic heart transplantation. In the meantime, her quality of life has improved by using this minimally invasive approach. CONCLUSIONS
To our knowledge, this is the first reported successful transcatheter implantation of a Melody Valve in the neoaortic position in a patient with HLHS. This case demonstrates technical feasibility of the procedure in a patient with favorable anatomy, as well as short-term clinical success using a novel approach to palliation of the failed neoaortic valve. REFERENCES 1. Zahn EM, Hellenbrand WE, Lock JE, McElhinney DB. Implantation of the melody transcatheter pulmonary valve in patients with a dysfunctional right ventricular outflow tract conduit early
Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).
Transcatheter Neoaortic Valve Replacement results from the U.S. Clinical trial. J Am Coll Cardiol 2009;54: 1722–1729. 2. McElhinney DB, Hellenbrand WE, Zahn EM, et al. Short- and medium-term outcomes after transcatheter pulmonary valve placement in the expanded multicenter US melody valve trial. Circulation 2010;122:507–516. 3. Cullen MW, Cabalka AK, Alli OO, et al. Transvenous, antegrade melody valve-in-valve implantation for bioprosthetic mitral and tricuspid valve dysfunction: A case series in children and adults. JACC Cardiovasc Interv 2013;6:598–605. 4. Roberts PA, Boudjemline Y, Cheatham JP, et al. Percutaneous tricuspid valve replacement in congenital and acquired heart disease. J Am Coll Cardiol 2011;58:117–122.
619
5. Boshoff DE, Cools BL, Heying R, et al. Off-label use of percutaneous pulmonary valved stents in the right ventricular outflow tract: Time to rewrite the label? Catheter Cardiovasc Interv 2013;81:987–995. 6. Hasan BS, McElhinney DB, Brown DW, et al. Short-term performance of the transcatheter Melody valve in high-pressure hemodynamic environments in the pulmonary and systemic circulations. Circ Cardiovasc Interv 2011;4:615–620. 7. Cheatham SL, Holzer RJ, Chisolm JL, Cheatham JP. The medtronic melody(R) transcatheter pulmonary valve implanted AT 24 mm Diameter. IT Works. Catheter Cardiovasc Interv 2013. 8. Schmid FX, Hilker M, Kampmann C, Mayer E, Oelert H. Clinical performance of the native pulmonary valve in the systemic circulation. J Heart Valve Dis 1998;7:620–625.
Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).
