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Subaortic stenosis due to accessory tissue of the mitral valve associated with Ebstein's anomaly in an adult
International
Journal
of
cardiology ELSEVIER
International Journal of Cardiology 57 (1996) 286-288
Subaortic stenosis due to accessory tissue of the mitral valve associated with Ebstein’s anomaly in an adult Mitswki
Isobe*, Masao Tanaka, Morie Sekiguchi
The First Department of Internal Medicine, Shinshu University School of Medicine, 3-l-l Asahi, Matsumoto 390, Japan Received 21 May 1996; revised 23 July 1996; accepted 3 September 1996
Abstract A 26-year-old man with a history of effort-induced syncopal attacks was found to have Ebstein’s anomaly. A cardiac catheterization revealed a pressure gradient of 75 mmHg between the left ventricle and the left ventricular outtlow tract which was caused by mitral accessorytissue. The accessorytissue was resected,the aortic valve was replaced, and the Ebstein’s anomaly was corrected.The degreeof mitral regurgitation was seento increasefollowing surgery. This is the first reported case of an accessorymitral valve complicated with Ebstein’s anomaly. Keywords: Subaortic stenosis; Mitral regurgitation; Ebstein’s anomaly; Accessory mitral valve
1. Introduction Accessory mitral valve is a rare congenital cause of subaortic stenosis. Thirty-one cases have been noted to date [l-4]. This anomaly is sometimes accompanied by other congenital anomalies. We report an adult case of Ebstein’s anomaly with subaortic stenosis due to the presence of an accessory mitral valve. This is the first case report of the combination of these anomalies.
2. Case report The patient was a 26-year-old man referred to our hospital for investigation of a history of syncopal *Corresponding author. Tel.: +81 263 372630; fax: +81 263 363722; e-mail: [email protected].
attacks. He had experienced exertional syncope three times over the past 2 years. He showed typical features of Ebstein’s anomaly: increased third and fourth sounds and tricuspid regurgitant murmur at auscultation of the chest. An electrocardiogram showed complete right bundle branch block and first degree atrioventricular block. A chest rentogenogram showed an enlarged right atrium and right ventricle. Ebstein’s anomaly was confirmed by identification of atrialized right ventricle. There was a 24% left-toright shunt at the atria1 level across the atrial septal defect. In addition to these findings, aortic stenosis and regurgitation were noted. An echocardiogram revealed the existence of parachute-shaped accessory mitral tissue which obstructed the left ventricular outflow tract (Fig. 1). The systolic pressure in the left ventricle was 210 mmHg, and that of the ascending aorta was 13.5 mmHg, giving a pressure
0167-5273/96/$15.00 Copyright 0 1996 Elsevier Science Ireland Ltd. All rights reserved PII SO167-5273(96)02825-Z
M. Isobe et al. / Internutional
Journal of Cardiology 57 (1996) 286-288
Fig. 1. Cross sectional echocardiography in short axis view (A) and long axis view (B). Accessory mitral valve (arrow) seen moving into left ventricular outflow tract during systole and moving back into the left ventricle during diastole. Dilated right atrium is also noted (A). Ao, aortic root; LA, left atrium; LV, left ventricle; RV, right ventricle.
gradient of 75 mmHg. Mild mitral regurgitation due to prolapse of the anterior mitral leaflet was also found. The accessory tissue, including the fibrous membrane and chordae was excised during open heart surgery. The accessory mitral valve had a parachute configuration, convex toward the aortic valve, with three independent chordae causing marked obstruction to the left ventricular outflow tract in systole. The membrane contained connective tissue with the appearance of mitral valve tissue. The aortic valve was replaced to correct aortic regurgitation caused by the prolapse of the noncoronary cusp. Complete correction of Ebstein’s anomaly was also performed, including closure of atria1 septal defect and reconstruction of the right ventricle with replacement of the tricuspid valve. The postoperative course was good except for an episode of pulmonary embolism. However, a significant exacerbation of mitral reg-
urgitation was confirmed by echocardiography (Fig. 2), which is being treated with medication.
3. Discussion Anomalies of the mitral valve causing left ventricular outflow tract obstruction are extremely rare. MacLean et al. reported the first case of this anomaly in a 29-year-old man [l]. Since the development of echocardiography, the majority of cases are detected at infancy. This is the second oldest patient reporting a case of this anomaly after the first report by MacLean et al. To our knowledge, 31 cases have been reported so far [2-41. Among them, 10 cases revealed that accessory mitral tissue was an isolated anomaly. However, this anomaly is often accompanied by other congenital anomalies. The common causes are ventricular septal defect (five cases) and
M. Isobe et al. I International
Journal of Cardiology 57 (1996) 286-288
Fig. 2. Color flow images during systole in long axis view before (A) and after (B) operation. Panel A shows the obstruction of left ventricular ejection flow by an accessorymitral valve. Mild mitral regurgitation (blue) is noted before operation. In contrast,the left atrium is filled with severe mitral regurgitant flow after surgery. Ao, aortic root; LA, left atrium; LV, left ventricle; RV, right ventricle.
transposition of the great arteries or corrected transposition of the great arteries (four cases), subaortic ring or membrane (four cases), and dextrocardia (four cases).However, there have been no reports of the coexistence of Ebstein’s anomaly and accessory mitral valve, or any reports of atrial septal defect. In the present case,replacementof the aortic valve was necessary. A significant increase in mitral regurgitation was noted early after surgery, although the normal tissues connected to the normal mitral valve, including papillary muscle and chordae, were not damaged. We speculate that resection of the chordae of the accessorytissue which was connected to the normal anterior papillary muscle caused an alteration in the functional integrity of the mitral complex, subsequently increasing the possibility of mitral regurgitation. The occurrence of mitral reg-
urgitation after surgery to correct this anomaly should be given special attention. References [l] MacLean LD, Culligan JA, Kane DJ. Subaortic stenosisdue to accessorytissue on the mitral valve. J Thorac Cardiovasc Surg 1963; 45: 382-388. [2] Szedo F, Thomka I, Arvay A. Accessory mitral valve tissue causing left ventricular outflow tract obstruction. J Cardiovasc Surg 1987; 28: 388-390. [3] Sono J, McKay R, Arnold RM. Accessory mitral valve leaflet causing aortic regurgitation and left ventricular outflow tract obstruction. Case report and review of published reports [review]. Br Heart J 1988; 59: 491-497. [4] Yasui H, Kado H, Tokunaga S et al. Tram-ventricular septal defect approach for resection of accessory mitral valve tissue. Ann Thorac Surg 1993; 55: 950-953.
Journal
of
cardiology ELSEVIER
International Journal of Cardiology 57 (1996) 286-288
Subaortic stenosis due to accessory tissue of the mitral valve associated with Ebstein’s anomaly in an adult Mitswki
Isobe*, Masao Tanaka, Morie Sekiguchi
The First Department of Internal Medicine, Shinshu University School of Medicine, 3-l-l Asahi, Matsumoto 390, Japan Received 21 May 1996; revised 23 July 1996; accepted 3 September 1996
Abstract A 26-year-old man with a history of effort-induced syncopal attacks was found to have Ebstein’s anomaly. A cardiac catheterization revealed a pressure gradient of 75 mmHg between the left ventricle and the left ventricular outtlow tract which was caused by mitral accessorytissue. The accessorytissue was resected,the aortic valve was replaced, and the Ebstein’s anomaly was corrected.The degreeof mitral regurgitation was seento increasefollowing surgery. This is the first reported case of an accessorymitral valve complicated with Ebstein’s anomaly. Keywords: Subaortic stenosis; Mitral regurgitation; Ebstein’s anomaly; Accessory mitral valve
1. Introduction Accessory mitral valve is a rare congenital cause of subaortic stenosis. Thirty-one cases have been noted to date [l-4]. This anomaly is sometimes accompanied by other congenital anomalies. We report an adult case of Ebstein’s anomaly with subaortic stenosis due to the presence of an accessory mitral valve. This is the first case report of the combination of these anomalies.
2. Case report The patient was a 26-year-old man referred to our hospital for investigation of a history of syncopal *Corresponding author. Tel.: +81 263 372630; fax: +81 263 363722; e-mail: [email protected].
attacks. He had experienced exertional syncope three times over the past 2 years. He showed typical features of Ebstein’s anomaly: increased third and fourth sounds and tricuspid regurgitant murmur at auscultation of the chest. An electrocardiogram showed complete right bundle branch block and first degree atrioventricular block. A chest rentogenogram showed an enlarged right atrium and right ventricle. Ebstein’s anomaly was confirmed by identification of atrialized right ventricle. There was a 24% left-toright shunt at the atria1 level across the atrial septal defect. In addition to these findings, aortic stenosis and regurgitation were noted. An echocardiogram revealed the existence of parachute-shaped accessory mitral tissue which obstructed the left ventricular outflow tract (Fig. 1). The systolic pressure in the left ventricle was 210 mmHg, and that of the ascending aorta was 13.5 mmHg, giving a pressure
0167-5273/96/$15.00 Copyright 0 1996 Elsevier Science Ireland Ltd. All rights reserved PII SO167-5273(96)02825-Z
M. Isobe et al. / Internutional
Journal of Cardiology 57 (1996) 286-288
Fig. 1. Cross sectional echocardiography in short axis view (A) and long axis view (B). Accessory mitral valve (arrow) seen moving into left ventricular outflow tract during systole and moving back into the left ventricle during diastole. Dilated right atrium is also noted (A). Ao, aortic root; LA, left atrium; LV, left ventricle; RV, right ventricle.
gradient of 75 mmHg. Mild mitral regurgitation due to prolapse of the anterior mitral leaflet was also found. The accessory tissue, including the fibrous membrane and chordae was excised during open heart surgery. The accessory mitral valve had a parachute configuration, convex toward the aortic valve, with three independent chordae causing marked obstruction to the left ventricular outflow tract in systole. The membrane contained connective tissue with the appearance of mitral valve tissue. The aortic valve was replaced to correct aortic regurgitation caused by the prolapse of the noncoronary cusp. Complete correction of Ebstein’s anomaly was also performed, including closure of atria1 septal defect and reconstruction of the right ventricle with replacement of the tricuspid valve. The postoperative course was good except for an episode of pulmonary embolism. However, a significant exacerbation of mitral reg-
urgitation was confirmed by echocardiography (Fig. 2), which is being treated with medication.
3. Discussion Anomalies of the mitral valve causing left ventricular outflow tract obstruction are extremely rare. MacLean et al. reported the first case of this anomaly in a 29-year-old man [l]. Since the development of echocardiography, the majority of cases are detected at infancy. This is the second oldest patient reporting a case of this anomaly after the first report by MacLean et al. To our knowledge, 31 cases have been reported so far [2-41. Among them, 10 cases revealed that accessory mitral tissue was an isolated anomaly. However, this anomaly is often accompanied by other congenital anomalies. The common causes are ventricular septal defect (five cases) and
M. Isobe et al. I International
Journal of Cardiology 57 (1996) 286-288
Fig. 2. Color flow images during systole in long axis view before (A) and after (B) operation. Panel A shows the obstruction of left ventricular ejection flow by an accessorymitral valve. Mild mitral regurgitation (blue) is noted before operation. In contrast,the left atrium is filled with severe mitral regurgitant flow after surgery. Ao, aortic root; LA, left atrium; LV, left ventricle; RV, right ventricle.
transposition of the great arteries or corrected transposition of the great arteries (four cases), subaortic ring or membrane (four cases), and dextrocardia (four cases).However, there have been no reports of the coexistence of Ebstein’s anomaly and accessory mitral valve, or any reports of atrial septal defect. In the present case,replacementof the aortic valve was necessary. A significant increase in mitral regurgitation was noted early after surgery, although the normal tissues connected to the normal mitral valve, including papillary muscle and chordae, were not damaged. We speculate that resection of the chordae of the accessorytissue which was connected to the normal anterior papillary muscle caused an alteration in the functional integrity of the mitral complex, subsequently increasing the possibility of mitral regurgitation. The occurrence of mitral reg-
urgitation after surgery to correct this anomaly should be given special attention. References [l] MacLean LD, Culligan JA, Kane DJ. Subaortic stenosisdue to accessorytissue on the mitral valve. J Thorac Cardiovasc Surg 1963; 45: 382-388. [2] Szedo F, Thomka I, Arvay A. Accessory mitral valve tissue causing left ventricular outflow tract obstruction. J Cardiovasc Surg 1987; 28: 388-390. [3] Sono J, McKay R, Arnold RM. Accessory mitral valve leaflet causing aortic regurgitation and left ventricular outflow tract obstruction. Case report and review of published reports [review]. Br Heart J 1988; 59: 491-497. [4] Yasui H, Kado H, Tokunaga S et al. Tram-ventricular septal defect approach for resection of accessory mitral valve tissue. Ann Thorac Surg 1993; 55: 950-953.