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Melody valve in aortic position to facilitate mechanical circulatory support as a bridge to cardiac transplantation
Journal Pre-proof Melody valve in aortic position to facilitate mechanical circulatory support as a bridge to cardiac transplantation Sachin Khambadkone, MD, Michael Burch, MD, Matthew Fenton, MD, Graham Derrick, MRCP, Tim Thiruchelvam, MBCHB, Ann Karimova, MD, Lyubomyr Bohuta, MD, T.Y. Hsia, MD PII:
S0003-4975(19)31434-1
DOI:
https://doi.org/10.1016/j.athoracsur.2019.08.044
Reference:
ATS 33067
To appear in:
The Annals of Thoracic Surgery
Received Date: 13 July 2019 Revised Date:
1 August 2019
Accepted Date: 12 August 2019
Please cite this article as: Khambadkone S, Burch M, Fenton M, Derrick G, Thiruchelvam T, Karimova A, Bohuta L, Hsia TY, Melody valve in aortic position to facilitate mechanical circulatory support as a bridge to cardiac transplantation, The Annals of Thoracic Surgery (2019), doi: https://doi.org/10.1016/ j.athoracsur.2019.08.044. This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. © 2019 by The Society of Thoracic Surgeons
Melody valve in aortic position to facilitate mechanical circulatory support as a bridge to cardiac transplantation Running Head: Melody valve in aortic position
Sachin Khambadkone1 MD, Michael Burch2 MD, Matthew Fenton2MD, Graham Derrick1 MRCP, Tim Thiruchelvam3 MBCHB, Ann Karimova3 MD, Lyubomyr Bohuta4 MD, T Y Hsia4 MD
Divisions of Cardiology1, Transplantation2, Cardiac Intensive Care3 and Cardiac Surgery4 Great Ormond Street Hospital London, UK
Correspondence to Dr Sachin Khambadkone Great Ormond Street Hospital NHS Foundation Trust Great Ormond Street London, UNITED KINGDOM [email protected]
Key words: Melody valve, ventricular assist device, aortic regurgitation
Abstract We report a patient with congenital heart disease and end stage heart failure after interventions for congenital heart disease resulting in aortic regurgitation that could not be treated with mechanical support to bridge to transplantation. We used Melody valve in aortic position to achieve aortic competence to allow use of a left ventricular assist device to bridge them successfully to cardiac transplantation.
Patients with congenital heart disease and end stage heart failure are a complex group to bridge to transplantation, particularly when their weight, size and the residual lesions are limiting factors to consider effective extracorporeal support. (1) A competent aortic valve is important for effective use of Extracorporeal membrane oxygenation (ECMO) and left ventricular assist devices (LVAD). (2) We describe a patient with aortic regurgitation complicating their clinical course for standard risk transplantation.
A 3 year old girl with bicuspid aortic valve stenosis had balloon aortic valvotomy at another institution, and was referred to us with severe left ventricular dysfunction, mixed aortic valve disease with severe aortic regurgitation, left ventricular endocardial fibroelastosis (EFE) and pulmonary hypertension for transplant assessment. A haemodynamic assessment with hybrid cardiac catheterization and MRI flow measurements showed a mean pulmonary artery pressure (mPAP) of 39 mm Hg, pulmonary capillary wedge pressure (PCWP) of 23 mm Hg ,transpulmonary gradient of 16 and pulmonary vascular resistance at baseline of 5 Wood units.m2 dropping to 3 with pulmonary vasodilator testing. She was listed for cardiac transplantation. Soon after the investigation, she sepsis and acute haemodynamic deterioration, and veno-arterial ECMO was commenced with atrial septectomy and repair of aortic valve (approximation of defect in anterior cusp) to reduce aortic regurgitation. Unfortunately, left ventricle continued to dilate with progressive worsening of aortic regurgitation. The aortic valve measured 11.3 mm at the annulus (z 2.1) and replacement with mechanical or bio-prosthetic valve was not an option. Hence, we decided to use Melody TPVR (Medtronic Inc.) as an off-label device in aortic position after seeking approval from the competent regulatory authority (Medicines and Healthcare Product Regulatory Agency MHRA) and the Hospital authorities at our Institution. The Melody valve was modified by creating
fenestrations to maintain coronary flow, whilst meticulous attention was paid to avoiding the commissures and leaflets. (Figure 1) It was delivered on a 18 mm EnsembleR delivery system. Under ECMO support, a hybrid trans-apical approach failed due to severe restriction by the sutured aortic valve and it was implanted by hybrid open approach on cardiopulmonary bypass through aortotomy, mounted on a 18 mm balloon catheter. The Melody valve was seen to be functioning well on transesophageal echocardiogram, with preserved atrioventricular conduction and no further deterioration of the mitral valve function.(Figure 2) Flow could be identified into the coronary arteries. Mild “paravalvar” leak was seen through the fenestrations created within the venous wall of the Melody valve, but this was assessed as mild regurgitation and a cardiac index (CI) of 1.5 to 1.6 L/min/m2 was achieved after recommencing ECMO. The early post –procedure course was complicated with severe bleeding needing blood products and chest exploration. After stabilization, a CI of 2.6 L/min/m2 could be achieved with no significant LV dilatation. The patient was then converted to a LVAD with Levitronix CentriMag pump connected to a 9 mm LV Berlin Heart EXCOR cannula at the apex, and 6 mm outflow cannula into the ascending aorta, with ECMO decannulation and closure of atrial septectomy. The heart ejected more effectively with an arterial pulse pressure of between30 to 40 mm of Hg, diastolic blood pressure between 40 and 60 mm Hg and preserved right ventricular function. Anticoagulation was maintained with heparin as per out institutional protocol with anti-Xa levels between 0.3 to 0.5, and Aspirin and Dipyridamole was added.
The left ventricular internal diameter in diastole (LVIDd) decreased from 45.6 mm to 38.6 mm. After a couple of weeks, she was converted to Berlin Heart EXCOR LVAD with a 25 ml chamber. The BH pump worked very well with CI of 2.55 L/min/m2 and good systemic perfusion on clinical and laboratory assessment. Unfortunately, there was a long waiting period of 324 days from the commencement of ECMO and 317 days after Melody valve implant, until her successful orthotopic cardiac transplant. During this course, the Melody valve worked well with maximum velocity 2 m/s
on ventricular assist and mild paravalvar leak, but only trivial leak from the leaflets. At the time of explant, the Melody valve was nicely endothelialised in the aortic root, and the stent crown could be identified at both ends. (Figure 3) The leaflets showed slight fibrous changes to their free edges, but appeared intact. 10 months after her transplant, the patient is doing very well and has started school. She has normal effort tolerance and no neurological deficits.
Comment Aortic regurgitation associated with long term use of continuous flow left ventricular assist devices (CF-LVAD) has emphasized the importance of aortic valve competence in maintaining good decompression of the left ventricle and sufficient coronary perfusion.(3) In end stage heart failure following treatment of congenital valvar aortic stenosis, it is not unusual to encounter residual haemodynamic lesions of ventricular outflows. Our patient had two catheter interventions that caused significant aortic regurgitation. Conventional strategies such as Ross operation or mechanical valve insertion were both considered but rejected due to concerns of severe ventricular function impairment and potential need for ventricular assist respectively. Aortic root replacement with a homograft would have sensitized the patient. Allternatives such as transcatheter aortic valve implants or percutaneous occluder devices have been considered to treat aortic regurgitation for adults on VAD, but reported mortality is quite high (31% peri-procedural, additional 25% at 1 year).(4) In 10 patients treated with occlider devices, Retzer and colleagues reported 70% in-hospital mortality attributed to right ventricular failure. (5) We have used Melody valve in high pressure circulations in the context of pulmonary valve implantation in pulmonary hypertension with competence maintained over a median of 20 months. (6) There are other reports of Melody valve implantation in the aortic position with good results.(7) We hence considered this approach to
establish a competent aortic valve to maintain eligibility for a ventricular assist as a bridge to transplantation. The trans-apical approach would have offered a less invasive implantation however, the previous surgical attempt to repair the valve had restricted the manipulation of the delivery system. The significant early reduction of left ventricular size clearly showed the benefit of the Melody valve. Meticulous attention to anticoagulation and presence of some ejection probably maintained “washing” jets within the valve to prevent thrombosis. To the best of our knowledge, this is the first report of Melody valve in aortic position to facilitate use of mechanical support as a bridge to transplantation for end stage heart failure in a child with congenital heart disease. We believe that Melody valve provides a good bio-prosthetic alternative for establishing competence of valvar function when mechanical circulatory assist is considered necessary.
References: 1. Blume ED, Naftel DC, Bastardi HJ, Duncan BW, Kirklin JK, Webber SA. Outcomes of children bridged to heart transplantation with ventricular assist devices: a multi-institutional study. Circulation 2006 ;19:2313-2319 2. Padalino M, Bortolussi G, Maschietto N, Guariento A, Stellin G. Surgery for Semilunar VALVE Regurgitation During Ventricular Assis Device Support in Children, Ann Thorac Surg 2015;100:e135-137 3. Aggarwal A, Raghuvir R, Eryazici P et al. The development of aortic insufficiency in continuous flow left ventricular assist device-supported patients. Ann Thorac Surg 2013;95:493-498 4. Phan K, Haswell JM, Xu J et al Percutaneous Trans-catheter Interventions for Aortic Insufficiency on Continuous Flow Left ventricular assist device patients: A systematic review and meta-analysis. ASAIO 2017;63:117-122 5. Retzer EM, Sayer GT, Fedson SE et al . Predictors of survival following transcatheter aortic valve closure for left ventricular assist device associated aortic insufficiency. Cathet Cardiovasc Interv 2016; 87:971-979 6. Lurz PM Nordmeyer J, Coats L et al. Immediate clinical and haemodynamic benefits of restoration of pulmonary valvar competence in patients with pulmonary hypertnsion. Heart. 2009:95:646-650. 7. 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;1:615-620
Figure Legends Figure 1. Melody valve with fenestrations to facilitate coronary flow. Figure 2. Trans-esophageal echocardiogram showing unobstructed outflow through the Melody valve and preserved flow in the left coronary artery. Figure 3. Explanted heart showing Melody valve through the aorta * and in the LVOT ^
S0003-4975(19)31434-1
DOI:
https://doi.org/10.1016/j.athoracsur.2019.08.044
Reference:
ATS 33067
To appear in:
The Annals of Thoracic Surgery
Received Date: 13 July 2019 Revised Date:
1 August 2019
Accepted Date: 12 August 2019
Please cite this article as: Khambadkone S, Burch M, Fenton M, Derrick G, Thiruchelvam T, Karimova A, Bohuta L, Hsia TY, Melody valve in aortic position to facilitate mechanical circulatory support as a bridge to cardiac transplantation, The Annals of Thoracic Surgery (2019), doi: https://doi.org/10.1016/ j.athoracsur.2019.08.044. This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. © 2019 by The Society of Thoracic Surgeons
Melody valve in aortic position to facilitate mechanical circulatory support as a bridge to cardiac transplantation Running Head: Melody valve in aortic position
Sachin Khambadkone1 MD, Michael Burch2 MD, Matthew Fenton2MD, Graham Derrick1 MRCP, Tim Thiruchelvam3 MBCHB, Ann Karimova3 MD, Lyubomyr Bohuta4 MD, T Y Hsia4 MD
Divisions of Cardiology1, Transplantation2, Cardiac Intensive Care3 and Cardiac Surgery4 Great Ormond Street Hospital London, UK
Correspondence to Dr Sachin Khambadkone Great Ormond Street Hospital NHS Foundation Trust Great Ormond Street London, UNITED KINGDOM [email protected]
Key words: Melody valve, ventricular assist device, aortic regurgitation
Abstract We report a patient with congenital heart disease and end stage heart failure after interventions for congenital heart disease resulting in aortic regurgitation that could not be treated with mechanical support to bridge to transplantation. We used Melody valve in aortic position to achieve aortic competence to allow use of a left ventricular assist device to bridge them successfully to cardiac transplantation.
Patients with congenital heart disease and end stage heart failure are a complex group to bridge to transplantation, particularly when their weight, size and the residual lesions are limiting factors to consider effective extracorporeal support. (1) A competent aortic valve is important for effective use of Extracorporeal membrane oxygenation (ECMO) and left ventricular assist devices (LVAD). (2) We describe a patient with aortic regurgitation complicating their clinical course for standard risk transplantation.
A 3 year old girl with bicuspid aortic valve stenosis had balloon aortic valvotomy at another institution, and was referred to us with severe left ventricular dysfunction, mixed aortic valve disease with severe aortic regurgitation, left ventricular endocardial fibroelastosis (EFE) and pulmonary hypertension for transplant assessment. A haemodynamic assessment with hybrid cardiac catheterization and MRI flow measurements showed a mean pulmonary artery pressure (mPAP) of 39 mm Hg, pulmonary capillary wedge pressure (PCWP) of 23 mm Hg ,transpulmonary gradient of 16 and pulmonary vascular resistance at baseline of 5 Wood units.m2 dropping to 3 with pulmonary vasodilator testing. She was listed for cardiac transplantation. Soon after the investigation, she sepsis and acute haemodynamic deterioration, and veno-arterial ECMO was commenced with atrial septectomy and repair of aortic valve (approximation of defect in anterior cusp) to reduce aortic regurgitation. Unfortunately, left ventricle continued to dilate with progressive worsening of aortic regurgitation. The aortic valve measured 11.3 mm at the annulus (z 2.1) and replacement with mechanical or bio-prosthetic valve was not an option. Hence, we decided to use Melody TPVR (Medtronic Inc.) as an off-label device in aortic position after seeking approval from the competent regulatory authority (Medicines and Healthcare Product Regulatory Agency MHRA) and the Hospital authorities at our Institution. The Melody valve was modified by creating
fenestrations to maintain coronary flow, whilst meticulous attention was paid to avoiding the commissures and leaflets. (Figure 1) It was delivered on a 18 mm EnsembleR delivery system. Under ECMO support, a hybrid trans-apical approach failed due to severe restriction by the sutured aortic valve and it was implanted by hybrid open approach on cardiopulmonary bypass through aortotomy, mounted on a 18 mm balloon catheter. The Melody valve was seen to be functioning well on transesophageal echocardiogram, with preserved atrioventricular conduction and no further deterioration of the mitral valve function.(Figure 2) Flow could be identified into the coronary arteries. Mild “paravalvar” leak was seen through the fenestrations created within the venous wall of the Melody valve, but this was assessed as mild regurgitation and a cardiac index (CI) of 1.5 to 1.6 L/min/m2 was achieved after recommencing ECMO. The early post –procedure course was complicated with severe bleeding needing blood products and chest exploration. After stabilization, a CI of 2.6 L/min/m2 could be achieved with no significant LV dilatation. The patient was then converted to a LVAD with Levitronix CentriMag pump connected to a 9 mm LV Berlin Heart EXCOR cannula at the apex, and 6 mm outflow cannula into the ascending aorta, with ECMO decannulation and closure of atrial septectomy. The heart ejected more effectively with an arterial pulse pressure of between30 to 40 mm of Hg, diastolic blood pressure between 40 and 60 mm Hg and preserved right ventricular function. Anticoagulation was maintained with heparin as per out institutional protocol with anti-Xa levels between 0.3 to 0.5, and Aspirin and Dipyridamole was added.
The left ventricular internal diameter in diastole (LVIDd) decreased from 45.6 mm to 38.6 mm. After a couple of weeks, she was converted to Berlin Heart EXCOR LVAD with a 25 ml chamber. The BH pump worked very well with CI of 2.55 L/min/m2 and good systemic perfusion on clinical and laboratory assessment. Unfortunately, there was a long waiting period of 324 days from the commencement of ECMO and 317 days after Melody valve implant, until her successful orthotopic cardiac transplant. During this course, the Melody valve worked well with maximum velocity 2 m/s
on ventricular assist and mild paravalvar leak, but only trivial leak from the leaflets. At the time of explant, the Melody valve was nicely endothelialised in the aortic root, and the stent crown could be identified at both ends. (Figure 3) The leaflets showed slight fibrous changes to their free edges, but appeared intact. 10 months after her transplant, the patient is doing very well and has started school. She has normal effort tolerance and no neurological deficits.
Comment Aortic regurgitation associated with long term use of continuous flow left ventricular assist devices (CF-LVAD) has emphasized the importance of aortic valve competence in maintaining good decompression of the left ventricle and sufficient coronary perfusion.(3) In end stage heart failure following treatment of congenital valvar aortic stenosis, it is not unusual to encounter residual haemodynamic lesions of ventricular outflows. Our patient had two catheter interventions that caused significant aortic regurgitation. Conventional strategies such as Ross operation or mechanical valve insertion were both considered but rejected due to concerns of severe ventricular function impairment and potential need for ventricular assist respectively. Aortic root replacement with a homograft would have sensitized the patient. Allternatives such as transcatheter aortic valve implants or percutaneous occluder devices have been considered to treat aortic regurgitation for adults on VAD, but reported mortality is quite high (31% peri-procedural, additional 25% at 1 year).(4) In 10 patients treated with occlider devices, Retzer and colleagues reported 70% in-hospital mortality attributed to right ventricular failure. (5) We have used Melody valve in high pressure circulations in the context of pulmonary valve implantation in pulmonary hypertension with competence maintained over a median of 20 months. (6) There are other reports of Melody valve implantation in the aortic position with good results.(7) We hence considered this approach to
establish a competent aortic valve to maintain eligibility for a ventricular assist as a bridge to transplantation. The trans-apical approach would have offered a less invasive implantation however, the previous surgical attempt to repair the valve had restricted the manipulation of the delivery system. The significant early reduction of left ventricular size clearly showed the benefit of the Melody valve. Meticulous attention to anticoagulation and presence of some ejection probably maintained “washing” jets within the valve to prevent thrombosis. To the best of our knowledge, this is the first report of Melody valve in aortic position to facilitate use of mechanical support as a bridge to transplantation for end stage heart failure in a child with congenital heart disease. We believe that Melody valve provides a good bio-prosthetic alternative for establishing competence of valvar function when mechanical circulatory assist is considered necessary.
References: 1. Blume ED, Naftel DC, Bastardi HJ, Duncan BW, Kirklin JK, Webber SA. Outcomes of children bridged to heart transplantation with ventricular assist devices: a multi-institutional study. Circulation 2006 ;19:2313-2319 2. Padalino M, Bortolussi G, Maschietto N, Guariento A, Stellin G. Surgery for Semilunar VALVE Regurgitation During Ventricular Assis Device Support in Children, Ann Thorac Surg 2015;100:e135-137 3. Aggarwal A, Raghuvir R, Eryazici P et al. The development of aortic insufficiency in continuous flow left ventricular assist device-supported patients. Ann Thorac Surg 2013;95:493-498 4. Phan K, Haswell JM, Xu J et al Percutaneous Trans-catheter Interventions for Aortic Insufficiency on Continuous Flow Left ventricular assist device patients: A systematic review and meta-analysis. ASAIO 2017;63:117-122 5. Retzer EM, Sayer GT, Fedson SE et al . Predictors of survival following transcatheter aortic valve closure for left ventricular assist device associated aortic insufficiency. Cathet Cardiovasc Interv 2016; 87:971-979 6. Lurz PM Nordmeyer J, Coats L et al. Immediate clinical and haemodynamic benefits of restoration of pulmonary valvar competence in patients with pulmonary hypertnsion. Heart. 2009:95:646-650. 7. 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;1:615-620
Figure Legends Figure 1. Melody valve with fenestrations to facilitate coronary flow. Figure 2. Trans-esophageal echocardiogram showing unobstructed outflow through the Melody valve and preserved flow in the left coronary artery. Figure 3. Explanted heart showing Melody valve through the aorta * and in the LVOT ^