© 2015, Wiley Periodicals, Inc. DOI: 10.1111/echo.12938
Echocardiography
Trileaflet Mitral Valve with Three Papillary Muscles Associated with Hypertrophic Cardiomyopathy: A Novel Case Salvatore Rosanio, M.D., Ph.D., F.A.C.C., F.E.S.C.,* Cameron J. Simonsen, M.S.,* John Starwalt, R.D.C.S., R.V.T., R.V.S., R.C.S.,* Abdul M. Keylani, M.D., F.A.C.C.,* and Antonio Vitarelli, M.D., F.A.C.C.† *Department of Internal Medicine, Division of Cardiology, University of North Texas Health Science Center, Fort Worth, Texas; and †Echocardiology and Adult Congenital Heart Disease Units, La Sapienza University, Rome, Italy
Congenital mitral valve (MV) malformations are uncommon, except for MV prolapse. Despite their infrequency, most of them are well-known and defined entities, such as congenital MV stenosis with two papillary muscles, parachute MV, supravalvular mitral ring, hypoplastic MV, isolated cleft in the anterior and/or posterior leaflets, and double-orifice MV. A trileaflet MV with three separate papillary muscles with concordant atrioventricular and ventricle–arterial connections is exceptionally rare. To the best of the authors’ knowledge, it has been reported only once in association with subaortic valvular stenosis. We hereby describe a novel case associated with hypertrophic cardiomyopathy. (Echocardiography 2015;32:1435–1437) Key words: mitral valve, hypertrophic cardiomyopathy, congenital heart disease, papillary muscle Congenital mitral valve (MV) malformations are rare and often complex, except for MV prolapse. They may affect the valvar leaflets, tensor apparatus, and papillary muscles.1,2 In a transthoracic 2D echocardiographic color Doppler study compared with data obtained by cineangiography, cardiac surgery, and autopsy, congenitally malformed MVs were detected in 65 (0.5%) subjects of the 13 400 cases examined over a 7.5-year period.3 Most of them have been well described and defined, such as congenital MV stenosis with two papillary muscles, parachute MV (a single papillary muscle variant), supravalvular mitral ring, hypoplastic MV, isolated cleft in the anterior and/or posterior leaflets, and double-orifice MV.1–7 A trileaflet MV with three separate papillary muscles with concordant atrioventricular and ventricle–arterial connections is exceptionally rare. To the best of the authors’ knowledge, it has been reported only once in association with subaortic valvular stenosis.8
Address for correspondence and reprint requests: Salvatore Rosanio, M.D., Ph.D., Department of Internal Medicine, Division of Cardiology, 855 Montgomery Street, PCC Room 315, Fort Worth, TX 76107. Fax: (817) 735-2748; E-mail: [email protected]
We hereby describe a novel case associated with hypertrophic cardiomyopathy. Case Presentation: A 56-year-old African American female with no major clinical complaint and cardiovascular risk factors was referred to our institution for transthoracic 2D color Doppler echocardiography to evaluate a grade III/VI ejection systolic murmur. The study unveiled asymmetrical septal thickening (septal/posterior wall thickness ratio of 1.6), systolic anterior motion of the mitral apparatus (Fig. 1A), no significant left ventricular outflow tract obstruction at rest (baseline peak systolic and mean pressure gradients of 18 mmHg and 9 mmHg, respectively, and of 25 mmHg and 12 mmHg, respectively, during Valsalva maneuver), normal cardiac dimensions and systolic function with a mild-to-moderate mitral regurgitation. Surprisingly, the study did not reveal the common “fish mouth” morphology of a bileaflet MV but the unexpected “shamrock,” “Mercedes-star” appearance of a trileaflet MV (Fig. 1B,C; movie clip S1).8,9 The three leaflets were coupled to three separate evenly spaced and roughly equal in size papillary muscles (Fig. 1C; movie clip S2). The three leaflets were circa similar in length (medial, 21 mm; lateral,
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20 mm; and posterior, 20 mm, respectively) with a central coaptation of 8 mm in length (Fig. 1D). There was normal mitral-aortic continuity and no evidence for obvious deformities involving the subvalvular apparatus (Fig. 1F). Real time 3D transesophageal echocardiography (3DTEE) corroborated the diagnosis and ruled out the presence of clefts bisecting the leaflets from their free edges to the MV annulus engendering the characteristic “V” shape cleft morphology (Fig. 2; movie clip S3).6 Comment: Our case of trileaflet MV with three separate papillary muscles can be considered as a unique phenotypic association with hypertrophic cardiomyopathy with the other only one case reported in combination with subaortic valvular stenosis (mentioned in earlier text).8 Bearing in mind the association with hypertrophic cardiomyopathy, another solo case of trileaf-
let MV with a direct papillary muscle insertion into the “anterior-septal” mitral leaflet in absence of three distinct papillary muscles has been described in a symptomatic female octogenarian with no prior cardiac history.9
Figure 2. A. and B. Real time 3DTEE imaging oriented from a ventricular aspect in diastole and systole, respectively, showing no clefts bisecting the leaflets from their free edges to the mitral valve annulus.
Figure 1. A. Mono-dimensional mode across the mitral valve leaflets depicting the systolic anterior motion of the anterior leaflet (SAM, arrow). B. and C. Parasternal 2D short-axis views showing the “shamrock,” “Mercedes-star” appearance of the trileaflet (medial, lateral, and posterior) mitral valve during diastole and systole, respectively. D. Parasternal 2D short-axis view showing three separate papillary muscles (lateral [LPM], medial [MPM], and posterior [PPM]). E. Four-chamber 2D apical view showing a central mitral valve coaptation of 8 mm in length (arrow), which is defined as the length between the coaptation point of the leaflets and the leaflets tips. F. Parasternal 2D long-axis view depicting concordant atrioventricular and ventricle–arterial connections with no gross deformities involving the subvalvular apparatus. Ao = aorta; LA = left atrium; LV = left ventricle; RA = right atrium; RV = right ventricle.
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Trileaflet Mitral Valve with Three Papillary Muscles
Of note, more than two papillary muscles have been reported primarily in cases associated with double-orifice MV.7 The clinical significance of our case report is yet unknown. However, we want to emphasize with the present case the invaluable diagnostic role of 3DTEE which allowed us to rule out the presence of the distinctive “V” shape leaflet cleft morphology and as well the T-shaped MV closure line during systole resembling a trileaflet MV.6,10 Lastly, as reported by Irwin et al.9 and consistent with our findings, a trileaflet MV may be also differentiated from isolated leaflet cleft by three equally spaced commissures, central leaflet coaptation, and the nonexistence of a characteristically located anterolateral commissure. References
gu 1. Se ela PE, Houyel L, Acar P: Congenital malformations of the mitral valve. Arch Cardiovasc Dis 2011;104:465– 479. 2. Remenyi B, Gentles TL: Congenital mitral valve lesions: correlation between morphology and imaging. Ann Pediatr Card 2012;5:3–12. 3. Banerjee A, Kohl T, Silverman NH: Echocardiographic evaluation of congenital mitral valve anomalies in children. Am J Cardiol 1995;76:1284–1291. 4. Marino BS, Kruge LE, Cho L, et al: Parachute mitral valve: morphologic descriptors, associated lesions, and out-
5.
6.
7. 8.
9. 10.
comes after biventricular repair. J Thorac Cardiovasc Surg 2009;137:385–393. Mohammadi S, Bergeron S, Voisine P, et al: Mitral valve cleft in both anterior and posterior leaflet: an extremely rare anomaly. Ann Thorac Surg 2006;82: 2287–2289. Seguela PE, Brosset P, Acar P: Isolated cleft of the posterior mitral valve leaflet assessed by real-time 3D echocardiography. Arch Cardiovasc Dis 2011;104:365– 366. Hashimoto H: Double-orifice mitral valve with three papillary muscles. Chest 1993;104:1616–1617. Kozak MF, Sivanandam S, De Marchi CH, et al: A trileaflet “mitral valve” with three papillary muscles: brand new echocardiographic finding. Congenit Heart Dis 2011;6:70–73. Irwin RB, Macnab A, Schmitt M: Tri-leaflet mitral valve in combination with hypertrophic cardiomyopathy. Eur Heart J 2011;32:534. Vignalou JB, Ewe SH, Schalij MJ, et al: Isolated posterior mitral leaflet cleft resembling trileaflet valve. Eur Heart J Cardiovasc Imaging 2012;13:535.
Supporting Information Additional Supporting Information may be found in the online version of this article: Movie clip S1. Figure 1B,C. Movie clip S2. Figure 1D. Movie clip S3. Figure 2.
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Echocardiography
Trileaflet Mitral Valve with Three Papillary Muscles Associated with Hypertrophic Cardiomyopathy: A Novel Case Salvatore Rosanio, M.D., Ph.D., F.A.C.C., F.E.S.C.,* Cameron J. Simonsen, M.S.,* John Starwalt, R.D.C.S., R.V.T., R.V.S., R.C.S.,* Abdul M. Keylani, M.D., F.A.C.C.,* and Antonio Vitarelli, M.D., F.A.C.C.† *Department of Internal Medicine, Division of Cardiology, University of North Texas Health Science Center, Fort Worth, Texas; and †Echocardiology and Adult Congenital Heart Disease Units, La Sapienza University, Rome, Italy
Congenital mitral valve (MV) malformations are uncommon, except for MV prolapse. Despite their infrequency, most of them are well-known and defined entities, such as congenital MV stenosis with two papillary muscles, parachute MV, supravalvular mitral ring, hypoplastic MV, isolated cleft in the anterior and/or posterior leaflets, and double-orifice MV. A trileaflet MV with three separate papillary muscles with concordant atrioventricular and ventricle–arterial connections is exceptionally rare. To the best of the authors’ knowledge, it has been reported only once in association with subaortic valvular stenosis. We hereby describe a novel case associated with hypertrophic cardiomyopathy. (Echocardiography 2015;32:1435–1437) Key words: mitral valve, hypertrophic cardiomyopathy, congenital heart disease, papillary muscle Congenital mitral valve (MV) malformations are rare and often complex, except for MV prolapse. They may affect the valvar leaflets, tensor apparatus, and papillary muscles.1,2 In a transthoracic 2D echocardiographic color Doppler study compared with data obtained by cineangiography, cardiac surgery, and autopsy, congenitally malformed MVs were detected in 65 (0.5%) subjects of the 13 400 cases examined over a 7.5-year period.3 Most of them have been well described and defined, such as congenital MV stenosis with two papillary muscles, parachute MV (a single papillary muscle variant), supravalvular mitral ring, hypoplastic MV, isolated cleft in the anterior and/or posterior leaflets, and double-orifice MV.1–7 A trileaflet MV with three separate papillary muscles with concordant atrioventricular and ventricle–arterial connections is exceptionally rare. To the best of the authors’ knowledge, it has been reported only once in association with subaortic valvular stenosis.8
Address for correspondence and reprint requests: Salvatore Rosanio, M.D., Ph.D., Department of Internal Medicine, Division of Cardiology, 855 Montgomery Street, PCC Room 315, Fort Worth, TX 76107. Fax: (817) 735-2748; E-mail: [email protected]
We hereby describe a novel case associated with hypertrophic cardiomyopathy. Case Presentation: A 56-year-old African American female with no major clinical complaint and cardiovascular risk factors was referred to our institution for transthoracic 2D color Doppler echocardiography to evaluate a grade III/VI ejection systolic murmur. The study unveiled asymmetrical septal thickening (septal/posterior wall thickness ratio of 1.6), systolic anterior motion of the mitral apparatus (Fig. 1A), no significant left ventricular outflow tract obstruction at rest (baseline peak systolic and mean pressure gradients of 18 mmHg and 9 mmHg, respectively, and of 25 mmHg and 12 mmHg, respectively, during Valsalva maneuver), normal cardiac dimensions and systolic function with a mild-to-moderate mitral regurgitation. Surprisingly, the study did not reveal the common “fish mouth” morphology of a bileaflet MV but the unexpected “shamrock,” “Mercedes-star” appearance of a trileaflet MV (Fig. 1B,C; movie clip S1).8,9 The three leaflets were coupled to three separate evenly spaced and roughly equal in size papillary muscles (Fig. 1C; movie clip S2). The three leaflets were circa similar in length (medial, 21 mm; lateral,
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20 mm; and posterior, 20 mm, respectively) with a central coaptation of 8 mm in length (Fig. 1D). There was normal mitral-aortic continuity and no evidence for obvious deformities involving the subvalvular apparatus (Fig. 1F). Real time 3D transesophageal echocardiography (3DTEE) corroborated the diagnosis and ruled out the presence of clefts bisecting the leaflets from their free edges to the MV annulus engendering the characteristic “V” shape cleft morphology (Fig. 2; movie clip S3).6 Comment: Our case of trileaflet MV with three separate papillary muscles can be considered as a unique phenotypic association with hypertrophic cardiomyopathy with the other only one case reported in combination with subaortic valvular stenosis (mentioned in earlier text).8 Bearing in mind the association with hypertrophic cardiomyopathy, another solo case of trileaf-
let MV with a direct papillary muscle insertion into the “anterior-septal” mitral leaflet in absence of three distinct papillary muscles has been described in a symptomatic female octogenarian with no prior cardiac history.9
Figure 2. A. and B. Real time 3DTEE imaging oriented from a ventricular aspect in diastole and systole, respectively, showing no clefts bisecting the leaflets from their free edges to the mitral valve annulus.
Figure 1. A. Mono-dimensional mode across the mitral valve leaflets depicting the systolic anterior motion of the anterior leaflet (SAM, arrow). B. and C. Parasternal 2D short-axis views showing the “shamrock,” “Mercedes-star” appearance of the trileaflet (medial, lateral, and posterior) mitral valve during diastole and systole, respectively. D. Parasternal 2D short-axis view showing three separate papillary muscles (lateral [LPM], medial [MPM], and posterior [PPM]). E. Four-chamber 2D apical view showing a central mitral valve coaptation of 8 mm in length (arrow), which is defined as the length between the coaptation point of the leaflets and the leaflets tips. F. Parasternal 2D long-axis view depicting concordant atrioventricular and ventricle–arterial connections with no gross deformities involving the subvalvular apparatus. Ao = aorta; LA = left atrium; LV = left ventricle; RA = right atrium; RV = right ventricle.
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Trileaflet Mitral Valve with Three Papillary Muscles
Of note, more than two papillary muscles have been reported primarily in cases associated with double-orifice MV.7 The clinical significance of our case report is yet unknown. However, we want to emphasize with the present case the invaluable diagnostic role of 3DTEE which allowed us to rule out the presence of the distinctive “V” shape leaflet cleft morphology and as well the T-shaped MV closure line during systole resembling a trileaflet MV.6,10 Lastly, as reported by Irwin et al.9 and consistent with our findings, a trileaflet MV may be also differentiated from isolated leaflet cleft by three equally spaced commissures, central leaflet coaptation, and the nonexistence of a characteristically located anterolateral commissure. References
gu 1. Se ela PE, Houyel L, Acar P: Congenital malformations of the mitral valve. Arch Cardiovasc Dis 2011;104:465– 479. 2. Remenyi B, Gentles TL: Congenital mitral valve lesions: correlation between morphology and imaging. Ann Pediatr Card 2012;5:3–12. 3. Banerjee A, Kohl T, Silverman NH: Echocardiographic evaluation of congenital mitral valve anomalies in children. Am J Cardiol 1995;76:1284–1291. 4. Marino BS, Kruge LE, Cho L, et al: Parachute mitral valve: morphologic descriptors, associated lesions, and out-
5.
6.
7. 8.
9. 10.
comes after biventricular repair. J Thorac Cardiovasc Surg 2009;137:385–393. Mohammadi S, Bergeron S, Voisine P, et al: Mitral valve cleft in both anterior and posterior leaflet: an extremely rare anomaly. Ann Thorac Surg 2006;82: 2287–2289. Seguela PE, Brosset P, Acar P: Isolated cleft of the posterior mitral valve leaflet assessed by real-time 3D echocardiography. Arch Cardiovasc Dis 2011;104:365– 366. Hashimoto H: Double-orifice mitral valve with three papillary muscles. Chest 1993;104:1616–1617. Kozak MF, Sivanandam S, De Marchi CH, et al: A trileaflet “mitral valve” with three papillary muscles: brand new echocardiographic finding. Congenit Heart Dis 2011;6:70–73. Irwin RB, Macnab A, Schmitt M: Tri-leaflet mitral valve in combination with hypertrophic cardiomyopathy. Eur Heart J 2011;32:534. Vignalou JB, Ewe SH, Schalij MJ, et al: Isolated posterior mitral leaflet cleft resembling trileaflet valve. Eur Heart J Cardiovasc Imaging 2012;13:535.
Supporting Information Additional Supporting Information may be found in the online version of this article: Movie clip S1. Figure 1B,C. Movie clip S2. Figure 1D. Movie clip S3. Figure 2.
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