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Asian Cardiovasc Thorac Ann 2001;9:325-326
© 2001 Asia Publishing EXchange Pte Ltd

CASE STUDY

Accessory Mitral Valve Tissue Obstructing Left Ventricular Outflow Tract

Lokeswara Rao Sajja, MCh, Gopi Chand Mannam, FRCS, Sriramulu Sompalli, MD1, Satish Kumar Missula, DA1

Division of Cardiothoracic Surgery
1 Division of Cardiac Anesthesiology Medwin Hospital Hyderabad, Andhra Pradesh, India
For reprint information contact: Gopi Chand Mannam, FRCS Tel: 91 40 666 1935 Fax: 91 40 662 5003 email: gmannam{at}yahoo.com Department of Cardiothoracic Surgery, CARE Hospital, Road No. 1, Banjara Hills, Hyderabad, Andhra Pradesh 500034, India.

   ABSTRACT
TOP
 ABSTRACT
INTRODUCTION
 CASE REPORT
 DISCUSSION
 REFERENCES
 
A 6-year-old girl underwent surgical excision of accessory mitral valve tissue causing significant subaortic stenosis. Preoperative 2-dimensional echocardio-graphy gave the necessary information on this isolated anomaly. The approach through an aortotomy provided adequate exposure. Postoperatively, there was no residual gradient across the left ventricular outflow tract, or mitral regurgitation.


   INTRODUCTION
TOP
 ABSTRACT
 INTRODUCTION
CASE REPORT
 DISCUSSION
 REFERENCES
 
Accessory valve tissue is a rare congenital cardiac malformation and most reported cases have involved obstruction of the left ventricular outflow tract (LVOT) by accessory mitral valve tissue. Patients with isolated accessory mitral valve tissue usually present with symptoms during the first decade of life, and the severity depends on the gradient across the LVOT.1 The lesion may be associated with other malformations such as ventricular septal defect, patent ductus arteriosus, or transposition of the great arteries. Symptoms tend to be modified by coexisting cardiac defects.2,3


   CASE REPORT
TOP
 ABSTRACT
 INTRODUCTION
 CASE REPORT
DISCUSSION
 REFERENCES
 
A 6-year-old girl was investigated for failure to thrive. A grade 3/6 systolic murmur was detected in the aortic area, which was not conducted to the carotid arteries. An electrocardiogram showed voltage criteria for left ventricular hypertrophy. Echocardiography revealed a heterogenous mobile mass in the subaortic area, which was attached to the anterior mitral leaflet (FigurelGo). A pressure gradient of 40 mm Hg across the LVOT and grade 1+ aortic regurgitation were recorded. There was no other cardiac abnormality noted, but she was not subjected to cardiac catheterization or left ventricu-lography. Excision of the accessory mitral valve tissue was carried out via a median sternotomy using standard cardiopulmonary bypass (CPB), moderate hypothermia (28°C), and cold hyperkalemic blood cardioplegia. An oblique aortotomy revealed no abnormality of the aortic valve. The accessory mitral valve tissue was attached by chordae to the anterior mitral leaflet and the anterior papillary muscle. It was excised by severing the attachment of the chordae, with preservation of the anterior papillary muscle. The aortotomy was closed and the child was weaned easily from CPB. Perioperative echocardiography revealed a competent mitral valve and no pressure gradient across the LVOT. Postoperative recovery was uncompli-cated. Echocardiography during follow-up showed no residual gradient across the LVOT, or mitral regurgitation. Mild aortic regurgitation that had been present pre-operatively persisted (Figure 2Go). The gross appearance of the resected accessory mitral valve tissue was similar to that of true valve tissue. It consisted of 2 leaflet-like structures supported by 2 chordae attached to the anterior mitral leaflet and another 2 chordae attached to the anterior papillary muscle (Figure 3Go). Histology revealed normal valve tissue with myxoid degeneration.



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  		Figure 1. Preoperative echocardiogram showing accessory mitral valve tissue in continuity with the anterior mitral leaflet and bulging into the left ventricular outflow tract. AML = anterior mitral leaflet, LA = left atrium, LV = left ventricle, PML = posterior mitral leaflet, RA = right atrium, RV = right ventricle.

 


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  		Figure 2. Postoperative echocardiogram showing no residual accessory mitral valve tissue. LA = left atrium, LV = left ventricle, RA = right atrium.

 


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  		Figure 3. Photograph of the resected accessory mitral valve tissue. White arrows indicate the severed chordae.

 

   DISCUSSION
TOP
 ABSTRACT
 INTRODUCTION
 CASE REPORT
 DISCUSSION
REFERENCES
 
More than 40 cases of LVOT obstruction caused by accessory mitral valve tissue have been reported.1 In the majority of these patients, accessory valve tissue was found in association with other cardiac malformations.4 Two-dimensional echocardiography can provide adequate information on the accessory valve tissue and other coexisting cardiac malformations.5 When associated with other intracardiac defects, failure to recognize the presence of accessory mitral valve tissue causing LVOT obstruction results in persistence of obstruction or even death. Yasui and colleagues6 described a patient who could not be weaned from CPB because of LVOT obstruction caused by accessory mitral valve tissue that had not been recognized. It has also been reported that patients with intracardiac malformations and accessory mitral valve tissue that was not removed, had high incidences of early mortality and reoperation.

Excision of accessory tissue is carried out through an aortotomy in most cases.1 A combined aortotomy and atriotomy approach was reported to provide greater safety and complete removal of accessory tissue without damaging the mitral valve, especially in small infants.1 A left ventriculotomy used by some authors is unnecessary and should be avoided because of the risks associated with ventriculotomy.7,8 It is important to identify intra-operatively the anatomical relationship between the accessory tissue and the mitral valve, to avoid damaging the valve during the procedure. It is difficult sometimes to identity the chordae of the accessory tissue if it is attached to the anterior mitral leaflet or papillary muscle. In children over one year of age, an approach through an aortotomy gives adequate access to the subaortic area and excision of the accessory tissue can be carried out safely without damaging the true mitral valve.


   REFERENCES
TOP
 ABSTRACT
 INTRODUCTION
 CASE REPORT
 DISCUSSION
 REFERENCES
 

  1. Meyer-Hetling K, Alexi-Meskishvili VV, Dahnert I. Critical subaortic stenosis in a newborn caused by accessory mitral valve tissue. Ann Thorac Surg 2000;69:1934–7.[Abstract/Free Full Text]

  2. Ow EP, DeLeon SY, Freeman JE, Quinones JA, Bell TJ, Sullivan HJ, et al. Recognition and management of accessory mitral tissue causing severe subaortic stenosis. Ann Thorac Surg 1994;57:952–5.[Abstract]

  3. Hartyanszky IL, Kadar K, Bojeldein S, Bodor G. Mitral valve anomalies obstructing left ventricular outflow. Eur J Cardio-thorac Surg 1997;12:504–6.[Abstract]

  4. Yoshimura N, Yamaguchi M, Oshima Y, Oka S, Ootaki Y, Tei T, et al. Clinical and pathological features of accessory valve tissue. Ann Thorac Surg 2000;69:1205–8.[Abstract/Free Full Text]

  5. Ascuitto RJ, Ross-Ascuitto NT, Kopf GS, Kleinmen CS, Talner NS. Accessory mitral valve tissue causing left ventricular outflow obstruction (two-dimensional echocardiographic diagnosis and surgical approach). Ann Thorac Surg 1986;42:581–4.[Abstract]

  6. Yasui H, Kado H, Tokunaga S, Kanegae Y, Fukae K, Masuda M, et al. Trans-ventricular septal defect approach for resection of accessory mitral valve tissue. Ann Thorac Surg 1993;55:950–3.[Abstract]

  7. Kelly DT, Wulfsberg E, Rowe RD. Discrete subaortic stenosis. Circulation 1972;46:309–22.[Abstract/Free Full Text]

  8. Mathewson JW, Riemenschneider TA, McGough EC, Condor VR. Left ventricular outflow tract obstruction produced by redundant mitral valve tissue in a neonate. Clinical, angiographic and operative findings. Circulation 1976;53:196–9.[Abstract/Free Full Text]





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