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(AAN)
[1–3] [PREPRINTER stage]
Case Study
Double-orifice mitral valve treated by percutaneous balloon valvuloplasty
Asian Cardiovascular & Thoracic Annals 0(0) 1–3 ß The Author(s) 2015 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492315589195 aan.sagepub.com
Thomas George Varghese, Vinod Raghunath Revankar, Monica Papanna and Harshini Srinivasan
Abstract Double-orifice mitral valve is an rare anomaly characterized by a mitral valve with a single fibrous annulus and 2 orifices that open into the left ventricle. It is often associated with other congenital anomalies, most commonly atrioventricular canal defects, and rarely associated with a stenotic or regurgitant mitral valve. A patient who was diagnosed with congenital double-orifice mitral valve with severe mitral stenosis was treated successfully by percutaneous balloon mitral valvotomy rather than the conventional open surgical approach, demonstrating the utility of percutaneous correction of this anomaly.
Keywords Heart defects, congenital, mitral valve annuloplasty, mitral valve insufficiency, mitral valve stenosis
Introduction
Case report
Although double-orifice mitral valve (DOMV) can occur as an isolated anomaly, it is often associated with other congenital anomalies, most commonly atrioventricular canal defects.1,2 DOMV is characterized by a mitral valve with a single fibrous annulus with 2 orifices that open into the left ventricle. Trowitzsch and colleagues3 proposed a classification based on echocardiographic findings and divided DOMV into 3 types. The eccentric or hole type is the most common, accounting for 85%. It is characterized by a larger main orifice and a smaller accessory orifice situated at either the anterolateral or posteromedial commissure. In approximately 15% of patients with DOMV, a central bridge of fibrous or abnormal leaflet tissue connects the 2 valve leaflets, dividing the orifice into medial and lateral parts (central or bridge type). The incomplete bridging type is characterized by a small strand of fibrous tissue that connects only the tips of the anterior and posterior leaflets. While surgical correction of the anomaly and mitral valve replacement are the preferred modalities of treatment, some cases have been treated by percutaneous balloon mitral valvotomy (BMV).4–6 Our patient presented with a central or bridge type of DOMV with severe mitral stenosis, which was treated successfully by BMV.
A 38-year-old man presented with a 6-month history of dyspnea on exertion in New York Heart Association functional class III. He had been diagnosed with mitral stenosis a few years perviously, for which he was advised medical treatment with low-dose diuretics. General examination was unremarkable, a detailed cardiac evaluation showed a loud first heart sound with an opening snap and a grade III mid-diastolic murmur best heard in the mitral area. Further evaluation by 2-dimensional transthoracic echocardiography revealed a central type of DOMV with severe mitral stenosis of one of the orifices (Figure 1). The peak and mean pressure gradients across the stenotic mitral valve were 19 and 10 mm Hg, respectively, the orifice area of the stenotic valve was 0.80 cm2 with mild mitral regurgitation (MR). Color-flow examination showed mild mitral and tricuspid regurgitation with mild resting pulmonary hypertension. No other
Department of Cardiology, Sapthagiri Institute of Medical Sciences Bangalore, India Corresponding author: Thomas George Varghese, Department of Cardiology, Sapthagiri Institute of Medical Sciences, Bangalore 560090, India. Email: [email protected]
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(AAN)
[1–3] [PREPRINTER stage]
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Asian Cardiovascular & Thoracic Annals 0(0)
Figure 1. Echocardiography showing the two stenotic orifices of the double-orifice mitral valve: MVO1 and MVO2. MVO1 was the more severely stenotic orifice with a valve area of only 0.8 cm2.
abnormality was detected on echocardiography. Based on these findings, the patient was advised BMV. Selection of the balloon catheter size was not according on the usual height-based formula, rather, the diameter of the stenotic orifice area was considered, with a smaller size being taken for initial dilation. A 23-mm balloon was used in the Inoue technique of BMV. Left ventricular angiography was carried out to assess the degree of MR and pulmonary arterial pressures. Tran’s septal catheterization by the atrial septal puncture technique was performed, and the balloon catheter was placed in the left atrium under echocardiographic guidance. The stenotic orifice was dilated in a stepwise manner, increasing the balloon size by 1 mm per step. The absence of worsening MR or leaflet tears was confirmed at the end of every dilation, to prevent complications. The dilation was stopped once echocardiography confirmed separation of the valve leaflets and a single mitral valve orifice (Figure 2). After the procedure, pressure gradient measurements were favorable with no worsening of MR (Table 1). The patient showed significant symptomatic improvement post-BMV. He was discharged on the 3rd day with a low-dose diuretic rather than an open restrictive interatrial communication because of his significant functional improvement, and echocardiography revealed a near normal deceleration slope and a normalized pressure half-time. He was subsequently followed up on a monthly basis for 3 months, and then every 2 months for a year, and remained asymptomatic during the entire follow-up period.
Discussion DOMV is a very rare congenital anomaly. It is usually misdiagnosed as rheumatic mitral stenosis and treated accordingly with long-term penicillin prophylaxis. Chronologic history and proper echocardiography can
Figure 2. Echocardiography showing only one orifice after dilatation of the stenotic orifice by balloon mitral valvuloplasty.
Table 1. Pressure gradients and orifice area measurements before and after balloon mitral valvuloplasty. Variable
Before BMV
After BMV
Aortic pressure (mm Hg) Left atrial pressure (mm Hg) Left ventricular pressure (mm Hg) Peak pressure gradient (mm Hg) Mean pressure gradient (mm Hg) Orifice area (cm2)
93/55 (68) 31/20 (26) 96/0 (4) 19 10 0.8
115/75 (75) 11/24 (7) 114/6 (13) 6 3 1.9
BMV: balloon mitral valvuloplasty.
correctly diagnose this disorder.7,8 Both surgical and balloon dilatation are feasible options for treatment of DOMV, however, surgery is preferred in view of the complex anatomy. Because of its low morbidity and mortality rates, BMV must be considered as a treatment option in suitable patients. Unlike conventional BMV performed in rheumatic mitral stenosis where the balloon size is chosen on the basis of the patient’s height,6 in DOMV, the orifices are very small and hence a slightly smaller balloon based on the area of the stenotic orifice needs to be considered. The central type of DOMV has anatomy favorable for BMV, but the risk of leaflet rupture and severe MR is higher in the eccentric type of DOMV due to the unfavorable anatomy that hinders balloon dilatation of the stenotic orifice. Treatment for patients with DOMV must be considered when they are symptomatic, as in our patient who has severe dyspnea and functional impairment. To prevent severe post-procedure MR, proper patient selection is important; patients with the central type of DOMV with minimal or no adhesions and those without significant subvalvular apparatus fusion are ideal candidates for BMV. We think that this case may promote discussion on this mode of treatment for DOMV.
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(AAN)
[1–3] [PREPRINTER stage]
Varghese et al.
3
Conflict of interest statement None declared.
Funding This research received no specific grant from any funding agency in the public, commerical, or not-for-profit sectors.
References 1. Tandon R, Takkar S, Kumbhkarni S, et al. A rare case of double orifice mitral valve with perimembranous ventricular septal defect: application of three-dimensional echocardiography for clinical decision making. Ann Pediatr Cardiol 2010; 3: 87–89. 2. Taksande A, Goutami V and Thomas E. Double orifice mitral valve associated with ventricular septal defect in an infant: case report. Images Paediatr Cardiol 2011; 13: 6–9. 3. Ban˜o-Rodrigo A, Van Praagh S, Trowitzsch E and Van Praagh R. Double-orifice mitral valve: a study of 27 postmortem cases with developmental, diagnostic and surgical considerations. Am J Cardiol 1988; 61: 152–160. 4. Bonow RO, Carabello BA, Chatterjee K, et al. 2008 Focused update incorporated into the ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of
5.
6.
7.
8.
Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease): endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. Circulation 2008; 118: e523–e661. Tomita Y, Yasui H and Tominaga R. Mitral valve repair for isolated double-orifice mitral valve with torn chordae. Ann Thorac Surg 1997; 64: 1831–1834. Lau KW, Gao W, Ding ZP and Hung JS. Immediate and long-term results of percutaneous Inoue balloon mitral commissurotomy with use of a simple height-derived balloon sizing method for the stepwise dilation technique. Mayo Clin Proc 1996; 71: 556–563. Zalzstein E, Hamilton R, Zucker N, Levitas A and Gross GJ. Presentation, natural history, and outcome in children and adolescents with double orifice mitral valve. Am J Cardiol 2004; 93: 1067–1069. Das BB, Pauliks LB, Knudson OA, et al. Double-orifice mitral valve with intact atrioventricular septum: an echocardiographic study with anatomic and functional considerations. J Am Soc Echocardiogr 2005; 18: 231–236.
Downloaded from aan.sagepub.com at MICHIGAN STATE UNIV LIBRARIES on June 14, 2015
(AAN)
[1–3] [PREPRINTER stage]
Case Study
Double-orifice mitral valve treated by percutaneous balloon valvuloplasty
Asian Cardiovascular & Thoracic Annals 0(0) 1–3 ß The Author(s) 2015 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492315589195 aan.sagepub.com
Thomas George Varghese, Vinod Raghunath Revankar, Monica Papanna and Harshini Srinivasan
Abstract Double-orifice mitral valve is an rare anomaly characterized by a mitral valve with a single fibrous annulus and 2 orifices that open into the left ventricle. It is often associated with other congenital anomalies, most commonly atrioventricular canal defects, and rarely associated with a stenotic or regurgitant mitral valve. A patient who was diagnosed with congenital double-orifice mitral valve with severe mitral stenosis was treated successfully by percutaneous balloon mitral valvotomy rather than the conventional open surgical approach, demonstrating the utility of percutaneous correction of this anomaly.
Keywords Heart defects, congenital, mitral valve annuloplasty, mitral valve insufficiency, mitral valve stenosis
Introduction
Case report
Although double-orifice mitral valve (DOMV) can occur as an isolated anomaly, it is often associated with other congenital anomalies, most commonly atrioventricular canal defects.1,2 DOMV is characterized by a mitral valve with a single fibrous annulus with 2 orifices that open into the left ventricle. Trowitzsch and colleagues3 proposed a classification based on echocardiographic findings and divided DOMV into 3 types. The eccentric or hole type is the most common, accounting for 85%. It is characterized by a larger main orifice and a smaller accessory orifice situated at either the anterolateral or posteromedial commissure. In approximately 15% of patients with DOMV, a central bridge of fibrous or abnormal leaflet tissue connects the 2 valve leaflets, dividing the orifice into medial and lateral parts (central or bridge type). The incomplete bridging type is characterized by a small strand of fibrous tissue that connects only the tips of the anterior and posterior leaflets. While surgical correction of the anomaly and mitral valve replacement are the preferred modalities of treatment, some cases have been treated by percutaneous balloon mitral valvotomy (BMV).4–6 Our patient presented with a central or bridge type of DOMV with severe mitral stenosis, which was treated successfully by BMV.
A 38-year-old man presented with a 6-month history of dyspnea on exertion in New York Heart Association functional class III. He had been diagnosed with mitral stenosis a few years perviously, for which he was advised medical treatment with low-dose diuretics. General examination was unremarkable, a detailed cardiac evaluation showed a loud first heart sound with an opening snap and a grade III mid-diastolic murmur best heard in the mitral area. Further evaluation by 2-dimensional transthoracic echocardiography revealed a central type of DOMV with severe mitral stenosis of one of the orifices (Figure 1). The peak and mean pressure gradients across the stenotic mitral valve were 19 and 10 mm Hg, respectively, the orifice area of the stenotic valve was 0.80 cm2 with mild mitral regurgitation (MR). Color-flow examination showed mild mitral and tricuspid regurgitation with mild resting pulmonary hypertension. No other
Department of Cardiology, Sapthagiri Institute of Medical Sciences Bangalore, India Corresponding author: Thomas George Varghese, Department of Cardiology, Sapthagiri Institute of Medical Sciences, Bangalore 560090, India. Email: [email protected]
Downloaded from aan.sagepub.com at MICHIGAN STATE UNIV LIBRARIES on June 14, 2015
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(AAN)
[1–3] [PREPRINTER stage]
2
Asian Cardiovascular & Thoracic Annals 0(0)
Figure 1. Echocardiography showing the two stenotic orifices of the double-orifice mitral valve: MVO1 and MVO2. MVO1 was the more severely stenotic orifice with a valve area of only 0.8 cm2.
abnormality was detected on echocardiography. Based on these findings, the patient was advised BMV. Selection of the balloon catheter size was not according on the usual height-based formula, rather, the diameter of the stenotic orifice area was considered, with a smaller size being taken for initial dilation. A 23-mm balloon was used in the Inoue technique of BMV. Left ventricular angiography was carried out to assess the degree of MR and pulmonary arterial pressures. Tran’s septal catheterization by the atrial septal puncture technique was performed, and the balloon catheter was placed in the left atrium under echocardiographic guidance. The stenotic orifice was dilated in a stepwise manner, increasing the balloon size by 1 mm per step. The absence of worsening MR or leaflet tears was confirmed at the end of every dilation, to prevent complications. The dilation was stopped once echocardiography confirmed separation of the valve leaflets and a single mitral valve orifice (Figure 2). After the procedure, pressure gradient measurements were favorable with no worsening of MR (Table 1). The patient showed significant symptomatic improvement post-BMV. He was discharged on the 3rd day with a low-dose diuretic rather than an open restrictive interatrial communication because of his significant functional improvement, and echocardiography revealed a near normal deceleration slope and a normalized pressure half-time. He was subsequently followed up on a monthly basis for 3 months, and then every 2 months for a year, and remained asymptomatic during the entire follow-up period.
Discussion DOMV is a very rare congenital anomaly. It is usually misdiagnosed as rheumatic mitral stenosis and treated accordingly with long-term penicillin prophylaxis. Chronologic history and proper echocardiography can
Figure 2. Echocardiography showing only one orifice after dilatation of the stenotic orifice by balloon mitral valvuloplasty.
Table 1. Pressure gradients and orifice area measurements before and after balloon mitral valvuloplasty. Variable
Before BMV
After BMV
Aortic pressure (mm Hg) Left atrial pressure (mm Hg) Left ventricular pressure (mm Hg) Peak pressure gradient (mm Hg) Mean pressure gradient (mm Hg) Orifice area (cm2)
93/55 (68) 31/20 (26) 96/0 (4) 19 10 0.8
115/75 (75) 11/24 (7) 114/6 (13) 6 3 1.9
BMV: balloon mitral valvuloplasty.
correctly diagnose this disorder.7,8 Both surgical and balloon dilatation are feasible options for treatment of DOMV, however, surgery is preferred in view of the complex anatomy. Because of its low morbidity and mortality rates, BMV must be considered as a treatment option in suitable patients. Unlike conventional BMV performed in rheumatic mitral stenosis where the balloon size is chosen on the basis of the patient’s height,6 in DOMV, the orifices are very small and hence a slightly smaller balloon based on the area of the stenotic orifice needs to be considered. The central type of DOMV has anatomy favorable for BMV, but the risk of leaflet rupture and severe MR is higher in the eccentric type of DOMV due to the unfavorable anatomy that hinders balloon dilatation of the stenotic orifice. Treatment for patients with DOMV must be considered when they are symptomatic, as in our patient who has severe dyspnea and functional impairment. To prevent severe post-procedure MR, proper patient selection is important; patients with the central type of DOMV with minimal or no adhesions and those without significant subvalvular apparatus fusion are ideal candidates for BMV. We think that this case may promote discussion on this mode of treatment for DOMV.
Downloaded from aan.sagepub.com at MICHIGAN STATE UNIV LIBRARIES on June 14, 2015
XML Template (2015) [21.5.2015–9:50am] //blrnas3.glyph.com/cenpro/ApplicationFiles/Journals/SAGE/3B2/AANJ/Vol00000/150081/APPFile/SG-AANJ150081.3d
(AAN)
[1–3] [PREPRINTER stage]
Varghese et al.
3
Conflict of interest statement None declared.
Funding This research received no specific grant from any funding agency in the public, commerical, or not-for-profit sectors.
References 1. Tandon R, Takkar S, Kumbhkarni S, et al. A rare case of double orifice mitral valve with perimembranous ventricular septal defect: application of three-dimensional echocardiography for clinical decision making. Ann Pediatr Cardiol 2010; 3: 87–89. 2. Taksande A, Goutami V and Thomas E. Double orifice mitral valve associated with ventricular septal defect in an infant: case report. Images Paediatr Cardiol 2011; 13: 6–9. 3. Ban˜o-Rodrigo A, Van Praagh S, Trowitzsch E and Van Praagh R. Double-orifice mitral valve: a study of 27 postmortem cases with developmental, diagnostic and surgical considerations. Am J Cardiol 1988; 61: 152–160. 4. Bonow RO, Carabello BA, Chatterjee K, et al. 2008 Focused update incorporated into the ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of
5.
6.
7.
8.
Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease): endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. Circulation 2008; 118: e523–e661. Tomita Y, Yasui H and Tominaga R. Mitral valve repair for isolated double-orifice mitral valve with torn chordae. Ann Thorac Surg 1997; 64: 1831–1834. Lau KW, Gao W, Ding ZP and Hung JS. Immediate and long-term results of percutaneous Inoue balloon mitral commissurotomy with use of a simple height-derived balloon sizing method for the stepwise dilation technique. Mayo Clin Proc 1996; 71: 556–563. Zalzstein E, Hamilton R, Zucker N, Levitas A and Gross GJ. Presentation, natural history, and outcome in children and adolescents with double orifice mitral valve. Am J Cardiol 2004; 93: 1067–1069. Das BB, Pauliks LB, Knudson OA, et al. Double-orifice mitral valve with intact atrioventricular septum: an echocardiographic study with anatomic and functional considerations. J Am Soc Echocardiogr 2005; 18: 231–236.
Downloaded from aan.sagepub.com at MICHIGAN STATE UNIV LIBRARIES on June 14, 2015
![[Percutaneous balloon valvuloplasty in mitral stenosis].](/assets/img/hold.png)
