Repair of a Duplicate Mitral Valve in a Patient With Ascending Aortic Aneurysm and Bicuspid Aortic Valve Guy Jensen, MD, Thomas Smith, MD, and Mona Flores, MD Departments of Cardiothoracic Surgery and Cardiovascular Medicine, University of California Davis Medical Center, Sacramento, California
We present the case of a 48-year-old man presenting to the emergency department with acute onset of chest pain radiating to the back in the setting of a known ascending aortic aneurysm. Preoperative echocardiography revealed an incompetent duplicate mitral valve, with a medially directed eccentric 3D jet of mitral regurgitation, and a bicuspid aortic valve. The patient was scheduled for aortic root and valve replacement with concurrent mitral valve repair. Intraoperative examination revealed an incompetent duplicate mitral valve separated from the physiologic mitral valve by a band of interatrial tissue. Successful repair was undertaken by oversewing the mitral valve. Postoperative echocardiography allowed for evaluation of this unique repair, which revealed no evidence of further mitral regurgitation and confirmed a successful repair of the mitral regurgitation without any need for an implantable device. Our goal is that this case and its positive treatment outcome, along with our threedimensional echocardiographic images correlated with the intraoperative findings and photographs, will further the understanding of the diagnosis and treatment of this rare condition. (Ann Thorac Surg 2014;97:e67–9) Ó 2014 by The Society of Thoracic Surgeons
A
lthough the discovery and subsequent repair of double-orifice mitral valves (DOMV) has been reported in the past, these patients remain rare [1, 2]. DOMV consists of 2 anatomically distinct mitral orifices Accepted for publication Sept 13, 2013. Address correspondence to Dr Flores, UC Davis Medical Center, Cypress Bldg, 2221 Stockton Blvd, Ste 2116, Sacramento, CA 95817; e-mail: mona. fl[email protected].
separated by a band of fibrous tissue. Less common are duplicate mitral valves, such as the one described here, that are distinguished by the presence of separate, complete subvalvular apparatuses [3]. We present the case of a 48-year-old man presenting to the emergency department with acute onset of chest pain radiating to the back in the setting of a known ascending aortic aneurysm. Computed tomography angiography of the chest revealed a 4.7-cm aneurysm of the ascending aorta extending from the aortic root to a point just proximal to the take-off of the innominate artery. As part of a standard preoperative workup, the patient underwent transesophageal echocardiography (Fig 1). This revealed an incompetent duplicate mitral valve, with a medially directed eccentric 3þ jet of mitral regurgitation (MR). Posteromedially to the duplicate valve, a physiologically normal mitral valve was present. This valve demonstrated 1þ centrally located MR. A transthoracic echocardiogram was read as having the appearance of a double-orifice valve with 2þ to 3þ MR with a 1þ regurgitant jet from posterior commissure area and 2þ to 3þ regurgitant jet due to prolapse from the anterior commissure area. Additional findings included a bicuspid aortic valve. Bicuspid aortic valves are seen with DOMV with relative frequency, occurring between 18% and 33% of the time [4, 5]. Taking into account the patient’s bicuspid aortic valve, the diameter of his ascending aneurysm, his symptoms, and his severe MR, he was scheduled for aortic root and valve replacement with concurrent mitral valve repair. Intraoperatively, the mitral valve was approached through a transeptal incision and was examined for evidence of the incompetence seen on echocardiography. The mitral valve appeared normal on the initial examination. Closer inspection revealed a separate, smaller mitral valve with unique chordae and leaflets located lateral and anterior to the mitral valve and separated by a bridge of normal atrial tissue (Fig 2). The second mitral valve was nearly totally incompetent, despite the existence of a subvalvular apparatus (Fig 3). The decision was made to oversew the duplicate mitral valve. This treatment was chosen because the large band of separating atrial tissue and the large size of the functioning valve would serve to reduce the risk of causing iatrogenic mitral stenosis or worsening the MR (Fig 4). The remaining Fig 1. Transesophageal echocardiography. (A) The asterisk (*) marks the normal anatomical mitral valve in the open position. The arrow marks the smaller duplicate mitral valve in the open position. (B) The asterisk (*) marks the normal anatomical mitral valve in the closed position. The arrow marks the smaller duplicate mitral valve in the closed position. (AV ¼ aortic valve; LAA ¼ left atrial appendage.)
Ó 2014 by The Society of Thoracic Surgeons Published by Elsevier Inc
0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2013.09.116
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CASE REPORT JENSEN ET AL REPAIR OF DUPLICATE MITRAL VALVE
Fig 2. A separate, smaller mitral valve with unique chordae and leaflets was located lateral and anterior to the mitral valve and separated by a bridge of normal atrial tissue.
mitral valve was tested with saline, and no evidence of MR was seen. The interatrial septum and atrium were closed, and the aortic root replacement proceeded without complication. An intraoperative transesophageal echocardiography at the conclusion of the procedure revealed a competent mitral valve with trace to no MR. A repeat transthoracic echocardiogram at 1 month postoperatively found no regurgitant jet from the duplicate valve. The 1þ MR from the functional mitral valve was unchanged from the preoperative study (Fig 5).
Fig 3. The second mitral valve was found to be nearly totally incompetent, despite the existence of a subvalvular apparatus.
Ann Thorac Surg 2014;97:e67–9
Fig 4. Oversewing the duplicate mitral valve.
Comment Oversewing the duplicate valve to repair the regurgitation does carry the risk of causing iatrogenic incompetence or stenosis in the functional mitral valve [1]. In this patient, however, the large area of normal atrial tissue bridging the 2 valves lowered the likelihood of this occurring. Literature to guide care in this case is scarce and mostly anecdotal. Ando and colleagues [3] report a repair of an incompetent duplicate valve in a similar manner with good results. This group placed an autologous pericardial patch in addition to oversewing the valve. Special care in that patient and in ours was taken to ensure that the remaining valve was competent before the septotomy was closed. In addition, intraoperative transesophageal echocardiography demonstrated no MR in either patient. This was consistent with preoperative imaging, as was the stable 1þ MR seen on transthoracic
Fig 5. Repeat transthoracic echocardiography obtained 1 month postoperatively. The asterisk (*) marks the jet of mitral regurgitation. The arrow marks the repaired duplicate mitral valve. (LV ¼ left ventricle).
Ann Thorac Surg 2014;97:e67–9
echocardiogram at 1 month postoperatively. We achieved a safe, functional repair of the MR by simply oversewing the nonfunctional mitral valve, thus avoiding the increased cardiopulmonary bypass pump time, risk, and cost of replacing the mitral valve. Historically, abnormalities such as these were diagnosed intraoperatively or postmortem. We describe a patient in whom the anomaly was diagnosed before operative intervention. The preoperative diagnosis of rare cardiac abnormalities is becoming more common due to advancements in echocardiographic technology [6, 7]. Advances in echocardiography will continue to better define these rare anatomic relationships as we are no longer confined to intraoperative or postmortem studies for the evaluation of unique cases of cardiac anatomy. There are multiple case reports of DOMV echocardiography without correlation with intraoperative findings [2, 7]. It is our goal that this case and its positive treatment outcome, along with our 3D echocardiographic images correlated with the intraoperative findings and photographs, will further the understanding of the diagnosis and treatment of this rare condition.
CASE REPORT JENSEN ET AL REPAIR OF DUPLICATE MITRAL VALVE
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References 1. Sharma V, Burkhart HM, Schaff HV, et al. Double-orifice left atrioventricular valve in patients with atrioventricular septal defects, surgical strategies and outcome. Ann Thorac Surg 2012;93:2017–21. 2. Rozo JC, Medina D, Guerrero C, Calderon AM, Mesa A. Accessory mitral valve without left ventricular outflow tract obstruction in an adult. Texas Heart Institute J 2008;35:324–6. 3. Ando K, Tomita Y, Masuda M, et al. Repair for a duplicate mitral valve with torn chordae. J Thorac Cardiovasc Surg 2007;134:1062–3. 4. Ba~ no-Rodrigo A, Van Praagh S, Trowitzsch E, Van Praagh R. Double-orifice mitral valve: a study of 27 postmortem cases with developmental, diagnostic and surgical considerations. Am J Cardiol 1988;61:152–60. 5. Das BB, Pauliks LB, Knudson OA Jr, et al. Double-orifice mitral valve with intact atrioventricular septum: an echocardiographic study with anatomic and functional considerations. J Am Soc Echo 2005;18:231–6. 6. Tani T, Kim K, Fujii Y, et al. Mitral valve repair for doubleorifice mitral valve with flail leaflet: the usefulness of realtime three-dimensional transesophageal echocardiography. Ann Thorac Surg 2012;93:e97–8. 7. Lee GY, Chang SA, Park SW. Double orifice mitral valve with bicuspid aortic valve in real time three-dimensional transesophageal echocardiographic examination. Echo 2012;29:E253–4.
We present the case of a 48-year-old man presenting to the emergency department with acute onset of chest pain radiating to the back in the setting of a known ascending aortic aneurysm. Preoperative echocardiography revealed an incompetent duplicate mitral valve, with a medially directed eccentric 3D jet of mitral regurgitation, and a bicuspid aortic valve. The patient was scheduled for aortic root and valve replacement with concurrent mitral valve repair. Intraoperative examination revealed an incompetent duplicate mitral valve separated from the physiologic mitral valve by a band of interatrial tissue. Successful repair was undertaken by oversewing the mitral valve. Postoperative echocardiography allowed for evaluation of this unique repair, which revealed no evidence of further mitral regurgitation and confirmed a successful repair of the mitral regurgitation without any need for an implantable device. Our goal is that this case and its positive treatment outcome, along with our threedimensional echocardiographic images correlated with the intraoperative findings and photographs, will further the understanding of the diagnosis and treatment of this rare condition. (Ann Thorac Surg 2014;97:e67–9) Ó 2014 by The Society of Thoracic Surgeons
A
lthough the discovery and subsequent repair of double-orifice mitral valves (DOMV) has been reported in the past, these patients remain rare [1, 2]. DOMV consists of 2 anatomically distinct mitral orifices Accepted for publication Sept 13, 2013. Address correspondence to Dr Flores, UC Davis Medical Center, Cypress Bldg, 2221 Stockton Blvd, Ste 2116, Sacramento, CA 95817; e-mail: mona. fl[email protected].
separated by a band of fibrous tissue. Less common are duplicate mitral valves, such as the one described here, that are distinguished by the presence of separate, complete subvalvular apparatuses [3]. We present the case of a 48-year-old man presenting to the emergency department with acute onset of chest pain radiating to the back in the setting of a known ascending aortic aneurysm. Computed tomography angiography of the chest revealed a 4.7-cm aneurysm of the ascending aorta extending from the aortic root to a point just proximal to the take-off of the innominate artery. As part of a standard preoperative workup, the patient underwent transesophageal echocardiography (Fig 1). This revealed an incompetent duplicate mitral valve, with a medially directed eccentric 3þ jet of mitral regurgitation (MR). Posteromedially to the duplicate valve, a physiologically normal mitral valve was present. This valve demonstrated 1þ centrally located MR. A transthoracic echocardiogram was read as having the appearance of a double-orifice valve with 2þ to 3þ MR with a 1þ regurgitant jet from posterior commissure area and 2þ to 3þ regurgitant jet due to prolapse from the anterior commissure area. Additional findings included a bicuspid aortic valve. Bicuspid aortic valves are seen with DOMV with relative frequency, occurring between 18% and 33% of the time [4, 5]. Taking into account the patient’s bicuspid aortic valve, the diameter of his ascending aneurysm, his symptoms, and his severe MR, he was scheduled for aortic root and valve replacement with concurrent mitral valve repair. Intraoperatively, the mitral valve was approached through a transeptal incision and was examined for evidence of the incompetence seen on echocardiography. The mitral valve appeared normal on the initial examination. Closer inspection revealed a separate, smaller mitral valve with unique chordae and leaflets located lateral and anterior to the mitral valve and separated by a bridge of normal atrial tissue (Fig 2). The second mitral valve was nearly totally incompetent, despite the existence of a subvalvular apparatus (Fig 3). The decision was made to oversew the duplicate mitral valve. This treatment was chosen because the large band of separating atrial tissue and the large size of the functioning valve would serve to reduce the risk of causing iatrogenic mitral stenosis or worsening the MR (Fig 4). The remaining Fig 1. Transesophageal echocardiography. (A) The asterisk (*) marks the normal anatomical mitral valve in the open position. The arrow marks the smaller duplicate mitral valve in the open position. (B) The asterisk (*) marks the normal anatomical mitral valve in the closed position. The arrow marks the smaller duplicate mitral valve in the closed position. (AV ¼ aortic valve; LAA ¼ left atrial appendage.)
Ó 2014 by The Society of Thoracic Surgeons Published by Elsevier Inc
0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2013.09.116
e68
CASE REPORT JENSEN ET AL REPAIR OF DUPLICATE MITRAL VALVE
Fig 2. A separate, smaller mitral valve with unique chordae and leaflets was located lateral and anterior to the mitral valve and separated by a bridge of normal atrial tissue.
mitral valve was tested with saline, and no evidence of MR was seen. The interatrial septum and atrium were closed, and the aortic root replacement proceeded without complication. An intraoperative transesophageal echocardiography at the conclusion of the procedure revealed a competent mitral valve with trace to no MR. A repeat transthoracic echocardiogram at 1 month postoperatively found no regurgitant jet from the duplicate valve. The 1þ MR from the functional mitral valve was unchanged from the preoperative study (Fig 5).
Fig 3. The second mitral valve was found to be nearly totally incompetent, despite the existence of a subvalvular apparatus.
Ann Thorac Surg 2014;97:e67–9
Fig 4. Oversewing the duplicate mitral valve.
Comment Oversewing the duplicate valve to repair the regurgitation does carry the risk of causing iatrogenic incompetence or stenosis in the functional mitral valve [1]. In this patient, however, the large area of normal atrial tissue bridging the 2 valves lowered the likelihood of this occurring. Literature to guide care in this case is scarce and mostly anecdotal. Ando and colleagues [3] report a repair of an incompetent duplicate valve in a similar manner with good results. This group placed an autologous pericardial patch in addition to oversewing the valve. Special care in that patient and in ours was taken to ensure that the remaining valve was competent before the septotomy was closed. In addition, intraoperative transesophageal echocardiography demonstrated no MR in either patient. This was consistent with preoperative imaging, as was the stable 1þ MR seen on transthoracic
Fig 5. Repeat transthoracic echocardiography obtained 1 month postoperatively. The asterisk (*) marks the jet of mitral regurgitation. The arrow marks the repaired duplicate mitral valve. (LV ¼ left ventricle).
Ann Thorac Surg 2014;97:e67–9
echocardiogram at 1 month postoperatively. We achieved a safe, functional repair of the MR by simply oversewing the nonfunctional mitral valve, thus avoiding the increased cardiopulmonary bypass pump time, risk, and cost of replacing the mitral valve. Historically, abnormalities such as these were diagnosed intraoperatively or postmortem. We describe a patient in whom the anomaly was diagnosed before operative intervention. The preoperative diagnosis of rare cardiac abnormalities is becoming more common due to advancements in echocardiographic technology [6, 7]. Advances in echocardiography will continue to better define these rare anatomic relationships as we are no longer confined to intraoperative or postmortem studies for the evaluation of unique cases of cardiac anatomy. There are multiple case reports of DOMV echocardiography without correlation with intraoperative findings [2, 7]. It is our goal that this case and its positive treatment outcome, along with our 3D echocardiographic images correlated with the intraoperative findings and photographs, will further the understanding of the diagnosis and treatment of this rare condition.
CASE REPORT JENSEN ET AL REPAIR OF DUPLICATE MITRAL VALVE
e69
References 1. Sharma V, Burkhart HM, Schaff HV, et al. Double-orifice left atrioventricular valve in patients with atrioventricular septal defects, surgical strategies and outcome. Ann Thorac Surg 2012;93:2017–21. 2. Rozo JC, Medina D, Guerrero C, Calderon AM, Mesa A. Accessory mitral valve without left ventricular outflow tract obstruction in an adult. Texas Heart Institute J 2008;35:324–6. 3. Ando K, Tomita Y, Masuda M, et al. Repair for a duplicate mitral valve with torn chordae. J Thorac Cardiovasc Surg 2007;134:1062–3. 4. Ba~ no-Rodrigo A, Van Praagh S, Trowitzsch E, Van Praagh R. Double-orifice mitral valve: a study of 27 postmortem cases with developmental, diagnostic and surgical considerations. Am J Cardiol 1988;61:152–60. 5. Das BB, Pauliks LB, Knudson OA Jr, et al. Double-orifice mitral valve with intact atrioventricular septum: an echocardiographic study with anatomic and functional considerations. J Am Soc Echo 2005;18:231–6. 6. Tani T, Kim K, Fujii Y, et al. Mitral valve repair for doubleorifice mitral valve with flail leaflet: the usefulness of realtime three-dimensional transesophageal echocardiography. Ann Thorac Surg 2012;93:e97–8. 7. Lee GY, Chang SA, Park SW. Double orifice mitral valve with bicuspid aortic valve in real time three-dimensional transesophageal echocardiographic examination. Echo 2012;29:E253–4.
