Hostname: page-component-8448b6f56d-jr42d Total loading time: 0 Render date: 2024-04-25T03:57:25.442Z Has data issue: false hasContentIssue false
  • English
  • Français

An analysis of 24 autopsied cases with supramitral rings

Published online by Cambridge University Press:  11 December 2008

Pradeep Vaideeswar ,
Milind M. Baldi  and
Sandeep Warghade
Pradeep Vaideeswar*
Affiliation:
Department of Pathology (Cardiovascular & Thoracic Division), Seth G. S. Medical College, Mumbai, India
Milind M. Baldi
Affiliation:
Department of Pathology (Cardiovascular & Thoracic Division), Seth G. S. Medical College, Mumbai, India
Sandeep Warghade
Affiliation:
Department of Pathology (Cardiovascular & Thoracic Division), Seth G. S. Medical College, Mumbai, India
*
Correspondence to: Dr Pradeep Vaideeswar, Department of Pathology (Cardiovascular & Thoracic Division), Seth G. S. Medical College, Parel, Mumbai 400 012, India. Tel: 91-22-24136951, ext 2550; Residence – 91-22-32501327; Fax: 91-22-22-24143435; E-mail: shreeprajai@yahoo.co.in
Get access Rights & Permissions [Opens in a new window]

Abstract

The supramitral ring is a rare congenital malformation formed by presence of a ridge of connective tissue, usually attached at or above the mitral annulus. The incidence and clinical presentation is highly variable due to difficulty in diagnosis. A review of autopsied congenital heart diseases at our institute over a 17-year-period revealed 24 cases of supramitral ring. These were classified with respect to the morphology of the ridge and the presence of associated cardiac lesions. The ring was found in 1.5% of the autopsied specimens of congenitally malformed hearts, and in 37.5% of those with obstructed left-sided inflow tracts. The majority of the specimens came from children (79.2%). A clinical diagnosis had been made in only two. In one-third of the cases, the ring was associated with incomplete Shone’s complex. Varied anomalies were seen in others, chiefly ventricular septal defects. An interesting association was the presence of rheumatic mitral valvar disease, found in 3 cases. There was no difference in the completeness or width of the supramitral ridge in the hearts from those with or without Shone’s complex. Circumferential rings were fleshy and stenosing, while incomplete rings had variable locations and stenosis. The presence of a supramitral ring may be underestimated due to association with other cardiac anomalies, both congenital and acquired. Since the ridge need not always produce stenosis, the correct designation would be simply a supramitral ring.

Keywords

Congenital heart diseasecongenital mitral stenosissupravalvar mitral stenosis
Type
Original Article
Information
Cardiology in the Young , Volume 19 , Issue 1 , February 2009 , pp. 70 - 75
Copyright
Copyright © Cambridge University Press 2008

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1. Deshpande, J, Vaideeswar, P, Amonkar, G, Vasandani, S. Rheumatic heart disease in the past decade: An autopsy analysis. Indian Heart J 2002; 54: 676–680.Google ScholarPubMed
2. Ruckman, RN, van Praagh, R. Anatomic types of congenital mitral stenosis: Report of 49 autopsy cases with consideration of diagnosis and surgical implications. Am J Cardiol 1978; 42: 592–601.Google Scholar
3. Shone, JD, Sellers, RD, Anderson, RC, Adams, P Jr, Lillehei, CW, Edwards, JE. The developmental complex of ‘parachute mitral valve’, supravalvular ridge of left atrium, subaortic stenosis and coarctation of aorta. Am J Cardiol 1963; 11: 714–725.CrossRefGoogle Scholar
4. Fisher, T. Two cases of congenital disease of the left side of the heart. Br Heart J 1902; 1: 639–641.Google Scholar
5. Jacobstein, MD, Hirschfield, SS. Concealed left atrial membrane: pitfalls in the diagnosis of cor triatriatum and supravalvar mitral ring. Am J Cardiol 1982; 49: 780–786.Google Scholar
6. 2ndMychaskiw, G, Sachdev, V, Braden, DA, Health, BJ. Supramitral ring: an unusual cause of congenital mitral stenosis. Case series and review. J Cardiovasc Surg (Torino) 2002; 43: 199–202.Google Scholar
7. Coles, JG, Williams, WG, Watanabe, T, et al. Surgical experience with reparative techniques in patients with congenital mitral valvular anomalies. Circulation 1987; 76: SIII 117–122.Google Scholar
8. Uva, MS, Galletti, L, Gayet, FL, et al. Surgery for congenital mitral valve disease in the first year of life. J Thorac Cardiovasc Surg 1995; 109: 164–176.Google Scholar
9. Banerjee, A, Kohl, T, Silverman, NH. Echocardiographic evaluation of congenital mitral value anomalies in children. Am J Cardiol 1995; 76: 1284–1291.Google Scholar
10. Moraes, F, Lapa, C, Ventura, C, et al. Supravalvular congenital mitral stenosis. Arq Bras Cardiol 2002; 79: 82–84.Google Scholar
11. Collison, SP, Kaushal, SK, Dagar, KS, et al. Supramitral ring: Good prognosis in a subset of patients with congenital mitral stenosis. Ann Thorac Surg 2006; 81: 997–1001.Google Scholar
12. Muhiudeen Russel, IA, Miller Hanee, WC, Silverman, NH. Intraoperative transesophageal echocardiography for pediatric patents with congenital heart disease. Anest Analg 1998; 87: 1058–1076.Google Scholar
13. Chung, KJ, Manning, JA, Lipchik, EO, Gramiak, R, Mahoney, EB. Isolated supravalvular stenosing ring of left atrium: diagnosis before operation and successful surgical treatment. Chest 1974; 65: 25–28.Google Scholar
14. Coto, EO, Judez, VM, Juffe, A, et al. Supravalvular stenotic mitral ring. A new case with surgical correction. J Thorac Cardiovasc Surg 1976; 71: 537–539.Google Scholar
15. Bokhandi, SS, Tullu, MS, Shatrarao, VB, Bavdekar, SB, Kamat, JR. Congenital heart disease with rheumatic fever and rheumatic heart disease: a coincidence or an association. J Postgrad Med 2002; 48: 238.Google Scholar
16. Lynch, MF, Ryan, NJ, Williams, CR, et al. Preoperative diagnosis and surgical correction of supravalvular mitral stenosis and ventricular septal defect. Circulation 1962; 25: 854–861.Google Scholar
17. Spevak, PJ, Bass, JL, Ben-Shachar, G, et al. Balloon angioplasty for congenital mitral stenosis. Am J Cardiol 1990; 66: 472–476.Google Scholar
18. Tulloh, RMR, Bull, C, Elliot, MJ, Sullivan, SD. Supravalvar mitral stenosis: risk factors for recurrence or death after resection. Br Heart J 1995; 73: 164–168.Google Scholar