Heart, Lung and Circulation (2014) 23, e267–e269 1443-9506/04/$36.00 http://dx.doi.org/10.1016/j.hlc.2014.08.002
CLINICAL SPOTLIGHT
Mitral Valve Repair for Double-orifice Mitral Valve Qun-Jun Duan, M.D. a, Zhan Gao, M.D. b* a
Second Affiliated Hospital of Zhejiang University, School of Medicine, Hangzhou, China, Department of Cardiovascular Surgery Children’s Hospital of Zhejiang University, School of Medicine, Hangzhou, China, Department of Thoracic and Cardiovascular Surgery
b
Received 6 May 2014; received in revised form 4 August 2014; accepted 6 August 2014; online published-ahead-of-print 19 August 2014
We present an eight year-old girl who required an operation for moderate mitral insufficiency associated with partial atrioventricular septal defect. Echocardiography disclosed an ostium primum atrial septal defect and double-orifice mitral valve with moderate mitral regurgitation secondary to a cleft in the anterior leaflet and prolapse of the anterior leaflet. Intraoperative inspection revealed that the chordae from each orifice were attached to a single papillary muscle which resulted in a unique double-orifice mitral valve. Mitral valve repair using chordal shortening and cleft closure was successfully performed. Postoperative echocardiography observed trivial MR and no mitral stenosis. Keywords
CHD Valve Mitral regurgitation Mitral valve repair Atrial septal defect ostium primum
Congenital mitral valve (MV) malformation is a rare disease, which consists mainly of double orifice mitral valve (DOMV), simple anterior leaves cleft of MV, and parachute mitral valve. DOMV is characterised by a single fibrous annulus and two valve orifices of varying sizes with two separate subvalvular structures. It has been reported to be associated with a variety of other congenital malformations such as partial atrioventricular septal defect (AVSD), bicuspid aortic valve and aortic coarctation [1,2]. We present an unusual case of a double-orifice MV leaflet with moderate mitral insufficiency associated with partial AVSD. This malformation of MV is usually considered not suitable for repair and should be replaced with a prosthetic heart valve. Here, we report a successful MV repair for a prolapsed double-orifice MV. An eight year-old girl was referred to our institution for further cardiac examination. She had been asymptomatic with a heart murmur found incidentally. Physical examination upon admission revealed a grade 2/6 holosystolic murmur along the left sternal border. Transthoracic echocardiography disclosed an ostium primum atrial septal defect and moderate mitral regurgitation secondary to a cleft in the anterior leaflet and prolapse of the anterior leaflet (A1). A conventional parasternal short-axis view demonstrated DOMV at the level of
MV during diastole (Fig. 1). Each orifice was nearly circular and of approximately equal size. The lateral orifice was located anteriorly in respect to the medial which was placed posteriorly. Two papillary muscles were demonstrated at the level of the papillary muscles. No other congenital cardiac abnormality was detected. The operation was performed through a median sternotomy. The MV was found to have two orifices that were separated by a fibrous bridge at the annular level (Fig. 2). This central bridge of fibrous, which was across the middle scallop of the posterior leaflet (P2) and middle segment of the anterior leaflet (A2), divided the mitral orifice into two equal orifices, located posteromedially and anterolaterally respectively. Both orifices were complete with competent subvalvular apparatuses. The chordae from each orifice were attached to a single papillary muscle which resulted in a unique double-orifice MV. The anterolateral and posterior papillary muscles were formed by two separate muscles. There was no additional papillary muscle in the middle portion of the left ventricle. A cleft of the anterior MV leaflet was noted. One chord, connected to the anterior leaflet of the anterolaterally orifice, was thickened and elongated. Regurgitant jets were demonstrated in this segment when the left
*Corresponding author at: Department of Thoracic and Cardiovascular Surgery, Children’s Hospital of Zhejiang University, School of Medicine, #57 Zhugan Xiang, Hangzhou 310003, China. Tel.: +86-0571-87061007x60611; Fax: +86-0571-87033296., Email: [email protected] © 2014 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier Inc. All rights reserved.
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interrupted sutures. The repair was tested with cold saline injection and no regurgitation existed. Atrial septum primum was closed conventionally. Postoperative transthoracic echocardiography observed trivial MR and no mitral stenosis. The patient was discharged one week after the operation and was in NYHA functional class I at two-year follow-up and was scheduled for longterm follow-up.
Discussion
Figure 1 A transthoracic echocardiography parasternal short-axis view shows the anterolateral and posteromedial orifices of the mitral valve, which are of the equal size, and the central fibrous bridge.
Figure 2 An intraoperative surgical view from right atrium shows the anterolateral mitral orifice and posteromedial mitral orifice with a central fibrous bridge. Cleft on the anterior leaflet was closed with interrupted sutures. Also seen was the primum ASD. AMO: anterolateral mitral orifice. CFB: central fibrous bridge. IS: interrupted sutures. PMO: posteromedial mitral orifice.
ventricle was filled with cold saline solution. The prolapse of anterior leaflet of anterolaterally orifice was repaired with chordal shortening. A 5-0 nonabsorbable running suture was fashioned to the prolapsed anterior leaflet. The suture involved the chordal-cusp junction of the prolapsing portion and plicated the rough zone on the ventricular aspect, thus strengthening the chordae and remodelling the leaflet’s free edge. The chordae were included in the suture for a length of about 3 mm. And the cleft of anterior MV was closed with
DOMV is a rare congenital malformation. It was found in 0.05% of 13,400 patients undergoing echocardiographic evaluation [1]. The clinical spectrum of DOMV varies from Shone’s complex, which has been defined as the presence of four left-sided defects such as supravalvular mitral membrane, DOMV, subaortic stenosis, and aortic coarctation, to isolated DOMV with normal valve function. The haemodynamic effects of a DOMV are variable, ranging from virtual competence to severe regurgitation. There are no specific clinical signs to suggest DOMV. Echocardiography represents the diagnostic method of choice to detect the malformation. The most common associated anomaly reported in the literature is atrioventricular septal defect [2,5], as was seen in this case. DOMV has reasonable function in about 50% of patients and significant stenosis or regurgitation is clinically presented in the other 50% [2]. Patients with an isolated DOMV usually have no functional abnormalities or clinical findings of mitral regurgitation or stenosis [3]. Classification of DOMV was based on the size and location of two orifices [4]. Eccentric or hole type is the most common variety of DOMV which accounts for about 85% of cases. It is characterised by a small accessory orifice situated at either the anterolateral or posteromedial commissure. In about 15% of patients, a central bridge of fibrous or abnormal leaflet tissue connects the two leaflets of the mitral valve, dividing the orifice into medial and lateral parts; this is the central or bridge type. These two openings may be equal or unequal. The papillary muscles are usually normal, with chordae surrounding each orifice inserting into one papillary muscle as was observed in our case. The management strategy depends on the type and severity of MV dysfunction and also the associated congenital defects. The treatment includes leaving the valve uncorrected, valve repair, or valve replacement. MV repair and reconstruction procedures are preferred, especially in young patients, just as in our case. There are a few basic principles that should be followed when valve repair for DOMV is considered [4]. An isolated DOMV with normal haemodynamics needs no active intervention. Division of the bridging tissue results in severe regurgitation that is not amenable for repair [4,5]. Therefore, no attempt is made to convert the dual orifice into a single one. Valve cleft should be closed unless the overall valve area appears small, in which case partial or no cleft closure should be applied. If the accessory orifice is competent, it should be left untouched. Standard MV repair
e269
Double-orifice mitral valve
techniques that are used for acquired diseases, such as artificial chordae, chordal shortening and annuloplasty bands, can be applied as needed, and valve replacement is considered as a last resort. In our case, cleft closure and chordal shortening were applied with successful valve repair. We accordingly suggest that current MV repair techniques remained a valuable option even on DOMV regurgitation.
Acknowledgments This study was supported by a grant from the National Natural Science Foundation of China (No. 81200129) and a grant from the Health and Family Planning Commission of Zhejiang Province (No. 2013RCB007).
References Conclusion We reported an unusual case of DOMV with moderate mitral insufficiency associated with leaflet cleft and partial AVSD. Following the general principles of mitral-valve repair, the regurgitation of DOMV can be successfully repaired.
Disclosures None.
[1] Banerjee A, Kohl T, Silverman NH. Echocardiographic evaluation of congenital mitral valve anomalies in children. Am J Cardiol 1995;76:1284–91. [2] Kim SJ, Kwon SH. Incidentally found double-orifice mitral valve in an elderly patient. Arch Cardiovasc Dis 2011;104:552–3. [3] Zalzstein E, Hamilton R, Zucker N, Levitas A, Gross GJ. Presentation, natural history, and outcome in children and adolescents with double orifice mitral valve. Am J Cardiol 2004;93:1067–9. [4] Sharma V, Burkhart HM, Schaff HV, Cabalka AK, Grogan MA, Dearani JA. Double-orifice left atrioventricular valve in patients with atrioventricular septal defects: surgical strategies and outcome. Ann Thorac Surg 2012;93:2017–20. discussion 2020-1. [5] Lee CN, Danielson GK, Schaff HV, Puga FJ, Mair DD. Surgical treatment of double-orifice mitral valve in atrioventricular canal defects. Experience in 25 patients. J Thorac Cardiovasc Surg 1985;90:700–5.
CLINICAL SPOTLIGHT
Mitral Valve Repair for Double-orifice Mitral Valve Qun-Jun Duan, M.D. a, Zhan Gao, M.D. b* a
Second Affiliated Hospital of Zhejiang University, School of Medicine, Hangzhou, China, Department of Cardiovascular Surgery Children’s Hospital of Zhejiang University, School of Medicine, Hangzhou, China, Department of Thoracic and Cardiovascular Surgery
b
Received 6 May 2014; received in revised form 4 August 2014; accepted 6 August 2014; online published-ahead-of-print 19 August 2014
We present an eight year-old girl who required an operation for moderate mitral insufficiency associated with partial atrioventricular septal defect. Echocardiography disclosed an ostium primum atrial septal defect and double-orifice mitral valve with moderate mitral regurgitation secondary to a cleft in the anterior leaflet and prolapse of the anterior leaflet. Intraoperative inspection revealed that the chordae from each orifice were attached to a single papillary muscle which resulted in a unique double-orifice mitral valve. Mitral valve repair using chordal shortening and cleft closure was successfully performed. Postoperative echocardiography observed trivial MR and no mitral stenosis. Keywords
CHD Valve Mitral regurgitation Mitral valve repair Atrial septal defect ostium primum
Congenital mitral valve (MV) malformation is a rare disease, which consists mainly of double orifice mitral valve (DOMV), simple anterior leaves cleft of MV, and parachute mitral valve. DOMV is characterised by a single fibrous annulus and two valve orifices of varying sizes with two separate subvalvular structures. It has been reported to be associated with a variety of other congenital malformations such as partial atrioventricular septal defect (AVSD), bicuspid aortic valve and aortic coarctation [1,2]. We present an unusual case of a double-orifice MV leaflet with moderate mitral insufficiency associated with partial AVSD. This malformation of MV is usually considered not suitable for repair and should be replaced with a prosthetic heart valve. Here, we report a successful MV repair for a prolapsed double-orifice MV. An eight year-old girl was referred to our institution for further cardiac examination. She had been asymptomatic with a heart murmur found incidentally. Physical examination upon admission revealed a grade 2/6 holosystolic murmur along the left sternal border. Transthoracic echocardiography disclosed an ostium primum atrial septal defect and moderate mitral regurgitation secondary to a cleft in the anterior leaflet and prolapse of the anterior leaflet (A1). A conventional parasternal short-axis view demonstrated DOMV at the level of
MV during diastole (Fig. 1). Each orifice was nearly circular and of approximately equal size. The lateral orifice was located anteriorly in respect to the medial which was placed posteriorly. Two papillary muscles were demonstrated at the level of the papillary muscles. No other congenital cardiac abnormality was detected. The operation was performed through a median sternotomy. The MV was found to have two orifices that were separated by a fibrous bridge at the annular level (Fig. 2). This central bridge of fibrous, which was across the middle scallop of the posterior leaflet (P2) and middle segment of the anterior leaflet (A2), divided the mitral orifice into two equal orifices, located posteromedially and anterolaterally respectively. Both orifices were complete with competent subvalvular apparatuses. The chordae from each orifice were attached to a single papillary muscle which resulted in a unique double-orifice MV. The anterolateral and posterior papillary muscles were formed by two separate muscles. There was no additional papillary muscle in the middle portion of the left ventricle. A cleft of the anterior MV leaflet was noted. One chord, connected to the anterior leaflet of the anterolaterally orifice, was thickened and elongated. Regurgitant jets were demonstrated in this segment when the left
*Corresponding author at: Department of Thoracic and Cardiovascular Surgery, Children’s Hospital of Zhejiang University, School of Medicine, #57 Zhugan Xiang, Hangzhou 310003, China. Tel.: +86-0571-87061007x60611; Fax: +86-0571-87033296., Email: [email protected] © 2014 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier Inc. All rights reserved.
e268
Q.-J. Duan, Z. Gao
interrupted sutures. The repair was tested with cold saline injection and no regurgitation existed. Atrial septum primum was closed conventionally. Postoperative transthoracic echocardiography observed trivial MR and no mitral stenosis. The patient was discharged one week after the operation and was in NYHA functional class I at two-year follow-up and was scheduled for longterm follow-up.
Discussion
Figure 1 A transthoracic echocardiography parasternal short-axis view shows the anterolateral and posteromedial orifices of the mitral valve, which are of the equal size, and the central fibrous bridge.
Figure 2 An intraoperative surgical view from right atrium shows the anterolateral mitral orifice and posteromedial mitral orifice with a central fibrous bridge. Cleft on the anterior leaflet was closed with interrupted sutures. Also seen was the primum ASD. AMO: anterolateral mitral orifice. CFB: central fibrous bridge. IS: interrupted sutures. PMO: posteromedial mitral orifice.
ventricle was filled with cold saline solution. The prolapse of anterior leaflet of anterolaterally orifice was repaired with chordal shortening. A 5-0 nonabsorbable running suture was fashioned to the prolapsed anterior leaflet. The suture involved the chordal-cusp junction of the prolapsing portion and plicated the rough zone on the ventricular aspect, thus strengthening the chordae and remodelling the leaflet’s free edge. The chordae were included in the suture for a length of about 3 mm. And the cleft of anterior MV was closed with
DOMV is a rare congenital malformation. It was found in 0.05% of 13,400 patients undergoing echocardiographic evaluation [1]. The clinical spectrum of DOMV varies from Shone’s complex, which has been defined as the presence of four left-sided defects such as supravalvular mitral membrane, DOMV, subaortic stenosis, and aortic coarctation, to isolated DOMV with normal valve function. The haemodynamic effects of a DOMV are variable, ranging from virtual competence to severe regurgitation. There are no specific clinical signs to suggest DOMV. Echocardiography represents the diagnostic method of choice to detect the malformation. The most common associated anomaly reported in the literature is atrioventricular septal defect [2,5], as was seen in this case. DOMV has reasonable function in about 50% of patients and significant stenosis or regurgitation is clinically presented in the other 50% [2]. Patients with an isolated DOMV usually have no functional abnormalities or clinical findings of mitral regurgitation or stenosis [3]. Classification of DOMV was based on the size and location of two orifices [4]. Eccentric or hole type is the most common variety of DOMV which accounts for about 85% of cases. It is characterised by a small accessory orifice situated at either the anterolateral or posteromedial commissure. In about 15% of patients, a central bridge of fibrous or abnormal leaflet tissue connects the two leaflets of the mitral valve, dividing the orifice into medial and lateral parts; this is the central or bridge type. These two openings may be equal or unequal. The papillary muscles are usually normal, with chordae surrounding each orifice inserting into one papillary muscle as was observed in our case. The management strategy depends on the type and severity of MV dysfunction and also the associated congenital defects. The treatment includes leaving the valve uncorrected, valve repair, or valve replacement. MV repair and reconstruction procedures are preferred, especially in young patients, just as in our case. There are a few basic principles that should be followed when valve repair for DOMV is considered [4]. An isolated DOMV with normal haemodynamics needs no active intervention. Division of the bridging tissue results in severe regurgitation that is not amenable for repair [4,5]. Therefore, no attempt is made to convert the dual orifice into a single one. Valve cleft should be closed unless the overall valve area appears small, in which case partial or no cleft closure should be applied. If the accessory orifice is competent, it should be left untouched. Standard MV repair
e269
Double-orifice mitral valve
techniques that are used for acquired diseases, such as artificial chordae, chordal shortening and annuloplasty bands, can be applied as needed, and valve replacement is considered as a last resort. In our case, cleft closure and chordal shortening were applied with successful valve repair. We accordingly suggest that current MV repair techniques remained a valuable option even on DOMV regurgitation.
Acknowledgments This study was supported by a grant from the National Natural Science Foundation of China (No. 81200129) and a grant from the Health and Family Planning Commission of Zhejiang Province (No. 2013RCB007).
References Conclusion We reported an unusual case of DOMV with moderate mitral insufficiency associated with leaflet cleft and partial AVSD. Following the general principles of mitral-valve repair, the regurgitation of DOMV can be successfully repaired.
Disclosures None.
[1] Banerjee A, Kohl T, Silverman NH. Echocardiographic evaluation of congenital mitral valve anomalies in children. Am J Cardiol 1995;76:1284–91. [2] Kim SJ, Kwon SH. Incidentally found double-orifice mitral valve in an elderly patient. Arch Cardiovasc Dis 2011;104:552–3. [3] Zalzstein E, Hamilton R, Zucker N, Levitas A, Gross GJ. Presentation, natural history, and outcome in children and adolescents with double orifice mitral valve. Am J Cardiol 2004;93:1067–9. [4] Sharma V, Burkhart HM, Schaff HV, Cabalka AK, Grogan MA, Dearani JA. Double-orifice left atrioventricular valve in patients with atrioventricular septal defects: surgical strategies and outcome. Ann Thorac Surg 2012;93:2017–20. discussion 2020-1. [5] Lee CN, Danielson GK, Schaff HV, Puga FJ, Mair DD. Surgical treatment of double-orifice mitral valve in atrioventricular canal defects. Experience in 25 patients. J Thorac Cardiovasc Surg 1985;90:700–5.
![[Mitral valve repair versus mitral valve replacement].](/assets/img/hold.png)
