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An uncommon source of mitral valve regurgitation
DIAGNOSTIC DILEMMAS Steven Konstadt, MD Jack S. Shanewise, MD Section Editors
An Uncommon Source of Mitral Valve Regurgitation Marty L. Ericksen, MD, and James A. DiNardo, MD
A
13-YEAR-OLD BOY was in his normal state of health when he presented with a cardiac murmur noted on routine physical examination. He had been completely asymptomatic without syncope, chest pain, palpitations, dyspnea, and had good exercise tolerance. His only ongoing medical issue was attention deficit disorder for which he was treated with methylphenidate hydrochloride (Ritalin; Ciba-Geigy, Basel, Switzerland). The patient’s transthoracic echocardiography, completed at an outside institution, showed normal segmental anatomy of the left ventricle. The
Fig 3.
Fig 1. TEE, midesophageal 4-chamber view.
Fig 2.
TEE, midesophageal 2-chamber view.
TEE, midesophageal LAX.
transthoracic echocardiography examination also showed an ostium primum atrial septal defect (ASD), with an additional small ostium secundum ASD, and moderate mitral regurgitation (MR). There was right ventricular volume overload, and the right ventricular systolic pressure was estimated to be less than half of the systemic pressure by septal position. The left atrium was dilated. The patient was taken to the operating room on the day of his admission for ASD closure and mitral valve repair. Intraoperative transesophageal echocardiography (TEE) was performed before cardiopulmonary bypass, and the following images were obtained. Figure 1 is a midesophageal (ME) 4-chamber view, Figure 2 is an ME 2-chamber view, and Figure 3 is an ME long-axis view (LAX). What is the etiology of the MR?
From the Department of Anesthesiology, Harvard Medical School, Children’s Hospital Boston, Boston, MA. Address reprint requests to James A. DiNardo, MD, Division of Cardiac Anesthesiology, Department of Anesthesiology, Harvard Medical School, Children’s Hospital Boston, 300 Longwood Avenue, Boston, MA 02115.E-mail: [email protected] © 2005 Elsevier Inc. All rights reserved. 1053-0770/05/1901-0024$30.00/0 doi:10.1053/j.jvca.2004.11.022 Key words: transesophageal echocardiography, mitral valve regurgitation, cleft mitral valve, atrial septal defect, atrioventricular canal defects
Journal of Cardiothoracic and Vascular Anesthesia, Vol 19, No 1 (February), 2005: pp 123-125
123
ERICKSEN AND DINARDO
124
Fig 4.
TEE, transgastric basal SAX.
DIAGNOSIS: CLEFT MITRAL VALVE
In this case, the cleft mitral valve is associated with an ostium primum ASD. This patient represents the mildest form of an atrioventricular (AV) canal defect with 2 separate AV valve orifices, a small ostium primum ASD, and no defect at the level of the ventricles. The first echocardiogram (Fig 1) is an ME 4-chamber view, which shows a defect in the anterior
Fig 5.
leaflet of the mitral valve (MV). In this particular patient, there are 2 separate AV valve annuli in association with an ostium primum ASD. In addition, there is no apical displacement of the tricuspid valve relative to the MV and thus both valves appear to insert at the same level at the crux of the heart. Figure 2 is an ME 2-chamber view, which exhibits a central MR jet, and Figure 3 is an ME LAX view of the MV, which shows a broad-based jet, with possible multiple defects in the anterior leaflet of the MV. Figure 4 is a transgastric basal short-axis view (TG basal SAX), which clearly delineates a cleft MV, in diastole, not associated with a common AV defect or a common AV valve orifice. The cleft is largest at its origin in the A2 portion of the MV at the leaflet tip. It then extends anteriolaterally toward the A1 region of the leaflet base. The anterior leaflet is supported by an intact MV annulus. The posterior leaflet is uninvolved and of normal size. The cleft points toward the left ventricular outflow rather than toward the interventricular septum as would be seen with a common AV valve defect. From the ME position, the LAX views of the MV can be used to quantitate MR by the regurgitant jet area,1 pulmonary vein flow pattern,2 proximal isovelocity surface area (PISA),3 2-dimensional/Doppler quantitative calculation of regurgitant fraction and volume,4 and proximal regurgitant jet width.5 However, a comprehensive, systematic echocardiography evaluation of the MV is necessary to accurately delineate mitral valve anatomy and the mechanism of stenosis or regurgitation.
Line drawing of the short-axis view of the mitral valve depicting lines where LAXs were cut across the mitral valve.
CLEFT MITRAL VALVE
125
Multiplane TEE is indispensable in this regard.6 It has been suggested that a comprehensive TEE MV evaluation can be accomplished using the ME 4-chamber, the ME 2-chamber, and the TG basal SAX views with some modifications.7 Others have suggested that single-plane TEE when used properly can provide a comparable amount of information.8 It is clear in this instance that only an en-face view of the anterior leaflet of the MV, such as that obtainable with a TG basal SAX view, provided clear delineation of the lesion. The other images presented here suggest that a defect exists in the anterior leaflet
producing a broad-based jet of mitral regurgitation in some views. However, clear delineation of the defect and the mechanism of MR is not possible with these views alone. The same is true of the TG LAX and deep TG LAX views of the MV. Figure 5 clearly shows that no one ME view is capable of reliably imaging the cleft along its entire length in such a manner as to clearly delineate the anatomy. The TG basal SAX, as shown in this case, proves invaluable when evaluating the location of the MR. As with any TEE study, multiple image planes are needed to make the correct diagnosis.
REFERENCES 1. Castello R, Lenzen P, Aguirre F, et al: Quantitation of mitral regurgitation by transesophageal echocardiography with Doppler colorflow mapping: Correlation with cardiac catheterization. J Am Coll Cardiol 19:1516-1521, 1992 2. Klein AL, Stewart WJ, Bartlett J, et al: Effects of mitral regurgitation on pulmonary venous flow and left atrial pressure: An intraoperative transesophageal echocardiographic study. J Am Coll Cardiol 20:1345-1352, 1992 3. Enriquez-Sarano M, Miller FA Jr, Hayes SN, et al: Effective mitral regurgitant orifice area: Clinical use and pitfalls of the proximal isovelocity surface area method. J Am Coll Cardiol 25:703-709, 1995 4. Enriquez-Sarano M, Seward JB, Bailey KR, et al: Effective regurgitant orifice area: A noninvasive Doppler development of an old hemodynamic concept. J Am Coll Cardiol 23:443-451, 1994
5. Tribouilloy C, Shen WF, QuereJ-P, et al: Assesment of severity of mitral regurgitant jet width at its origin with transesophageal Doppler color-flow imaging. Circulation 85:1248-1253, 1992 6. Garwood S: Con: Single-plane echocardiography does not provide an accurate and adequate examination of the native mitral valve. J Cardiothorac Vasc Anesth 16:515-520, 2002 7. Lambert AS, Miller JP, Merrick SH, et al: Improved evaluation of the location and mechanism of mitral valve regurgitation with a systematic transesophageal echocardiography examination. Anesth Analg 88:1205-1212, 1999 8. Maslow A, Schwartz C, Bert A: Pro: Single plane echocardiography provides an accurate and adequate examination of the native mitral valve. J Cardiothorac Vasc Anesth 16:508-514, 2002
An Uncommon Source of Mitral Valve Regurgitation Marty L. Ericksen, MD, and James A. DiNardo, MD
A
13-YEAR-OLD BOY was in his normal state of health when he presented with a cardiac murmur noted on routine physical examination. He had been completely asymptomatic without syncope, chest pain, palpitations, dyspnea, and had good exercise tolerance. His only ongoing medical issue was attention deficit disorder for which he was treated with methylphenidate hydrochloride (Ritalin; Ciba-Geigy, Basel, Switzerland). The patient’s transthoracic echocardiography, completed at an outside institution, showed normal segmental anatomy of the left ventricle. The
Fig 3.
Fig 1. TEE, midesophageal 4-chamber view.
Fig 2.
TEE, midesophageal 2-chamber view.
TEE, midesophageal LAX.
transthoracic echocardiography examination also showed an ostium primum atrial septal defect (ASD), with an additional small ostium secundum ASD, and moderate mitral regurgitation (MR). There was right ventricular volume overload, and the right ventricular systolic pressure was estimated to be less than half of the systemic pressure by septal position. The left atrium was dilated. The patient was taken to the operating room on the day of his admission for ASD closure and mitral valve repair. Intraoperative transesophageal echocardiography (TEE) was performed before cardiopulmonary bypass, and the following images were obtained. Figure 1 is a midesophageal (ME) 4-chamber view, Figure 2 is an ME 2-chamber view, and Figure 3 is an ME long-axis view (LAX). What is the etiology of the MR?
From the Department of Anesthesiology, Harvard Medical School, Children’s Hospital Boston, Boston, MA. Address reprint requests to James A. DiNardo, MD, Division of Cardiac Anesthesiology, Department of Anesthesiology, Harvard Medical School, Children’s Hospital Boston, 300 Longwood Avenue, Boston, MA 02115.E-mail: [email protected] © 2005 Elsevier Inc. All rights reserved. 1053-0770/05/1901-0024$30.00/0 doi:10.1053/j.jvca.2004.11.022 Key words: transesophageal echocardiography, mitral valve regurgitation, cleft mitral valve, atrial septal defect, atrioventricular canal defects
Journal of Cardiothoracic and Vascular Anesthesia, Vol 19, No 1 (February), 2005: pp 123-125
123
ERICKSEN AND DINARDO
124
Fig 4.
TEE, transgastric basal SAX.
DIAGNOSIS: CLEFT MITRAL VALVE
In this case, the cleft mitral valve is associated with an ostium primum ASD. This patient represents the mildest form of an atrioventricular (AV) canal defect with 2 separate AV valve orifices, a small ostium primum ASD, and no defect at the level of the ventricles. The first echocardiogram (Fig 1) is an ME 4-chamber view, which shows a defect in the anterior
Fig 5.
leaflet of the mitral valve (MV). In this particular patient, there are 2 separate AV valve annuli in association with an ostium primum ASD. In addition, there is no apical displacement of the tricuspid valve relative to the MV and thus both valves appear to insert at the same level at the crux of the heart. Figure 2 is an ME 2-chamber view, which exhibits a central MR jet, and Figure 3 is an ME LAX view of the MV, which shows a broad-based jet, with possible multiple defects in the anterior leaflet of the MV. Figure 4 is a transgastric basal short-axis view (TG basal SAX), which clearly delineates a cleft MV, in diastole, not associated with a common AV defect or a common AV valve orifice. The cleft is largest at its origin in the A2 portion of the MV at the leaflet tip. It then extends anteriolaterally toward the A1 region of the leaflet base. The anterior leaflet is supported by an intact MV annulus. The posterior leaflet is uninvolved and of normal size. The cleft points toward the left ventricular outflow rather than toward the interventricular septum as would be seen with a common AV valve defect. From the ME position, the LAX views of the MV can be used to quantitate MR by the regurgitant jet area,1 pulmonary vein flow pattern,2 proximal isovelocity surface area (PISA),3 2-dimensional/Doppler quantitative calculation of regurgitant fraction and volume,4 and proximal regurgitant jet width.5 However, a comprehensive, systematic echocardiography evaluation of the MV is necessary to accurately delineate mitral valve anatomy and the mechanism of stenosis or regurgitation.
Line drawing of the short-axis view of the mitral valve depicting lines where LAXs were cut across the mitral valve.
CLEFT MITRAL VALVE
125
Multiplane TEE is indispensable in this regard.6 It has been suggested that a comprehensive TEE MV evaluation can be accomplished using the ME 4-chamber, the ME 2-chamber, and the TG basal SAX views with some modifications.7 Others have suggested that single-plane TEE when used properly can provide a comparable amount of information.8 It is clear in this instance that only an en-face view of the anterior leaflet of the MV, such as that obtainable with a TG basal SAX view, provided clear delineation of the lesion. The other images presented here suggest that a defect exists in the anterior leaflet
producing a broad-based jet of mitral regurgitation in some views. However, clear delineation of the defect and the mechanism of MR is not possible with these views alone. The same is true of the TG LAX and deep TG LAX views of the MV. Figure 5 clearly shows that no one ME view is capable of reliably imaging the cleft along its entire length in such a manner as to clearly delineate the anatomy. The TG basal SAX, as shown in this case, proves invaluable when evaluating the location of the MR. As with any TEE study, multiple image planes are needed to make the correct diagnosis.
REFERENCES 1. Castello R, Lenzen P, Aguirre F, et al: Quantitation of mitral regurgitation by transesophageal echocardiography with Doppler colorflow mapping: Correlation with cardiac catheterization. J Am Coll Cardiol 19:1516-1521, 1992 2. Klein AL, Stewart WJ, Bartlett J, et al: Effects of mitral regurgitation on pulmonary venous flow and left atrial pressure: An intraoperative transesophageal echocardiographic study. J Am Coll Cardiol 20:1345-1352, 1992 3. Enriquez-Sarano M, Miller FA Jr, Hayes SN, et al: Effective mitral regurgitant orifice area: Clinical use and pitfalls of the proximal isovelocity surface area method. J Am Coll Cardiol 25:703-709, 1995 4. Enriquez-Sarano M, Seward JB, Bailey KR, et al: Effective regurgitant orifice area: A noninvasive Doppler development of an old hemodynamic concept. J Am Coll Cardiol 23:443-451, 1994
5. Tribouilloy C, Shen WF, QuereJ-P, et al: Assesment of severity of mitral regurgitant jet width at its origin with transesophageal Doppler color-flow imaging. Circulation 85:1248-1253, 1992 6. Garwood S: Con: Single-plane echocardiography does not provide an accurate and adequate examination of the native mitral valve. J Cardiothorac Vasc Anesth 16:515-520, 2002 7. Lambert AS, Miller JP, Merrick SH, et al: Improved evaluation of the location and mechanism of mitral valve regurgitation with a systematic transesophageal echocardiography examination. Anesth Analg 88:1205-1212, 1999 8. Maslow A, Schwartz C, Bert A: Pro: Single plane echocardiography provides an accurate and adequate examination of the native mitral valve. J Cardiothorac Vasc Anesth 16:508-514, 2002