Pi Is 0003497505012002
Pi Is 0003497505012002
Pi Is 0003497505012002
Background. Management of congenital stenotic mitral posterior mitral leaflets and was most densely adherent
valvular abnormalities remains an important therapeutic at the posteroinferior commissure in 4 of these 7 patients
challenge. Supramitral ring constitutes a small but inad- (57%). Complete excision of ring was possible in all
equately described subset that has a relatively good cases, without damage to the mitral valve. There was 1
outcome with appropriate management. in-hospital death (6%). At a mean follow-up of 30
Methods. Between 1996 and 2004, 15 patients with months, 14 survivors continue to do well, with no signif-
supramitral ring were managed in this institution. The icant recurrence of mitral stenosis.
demographic and clinical features, diagnostic modalities, Conclusions. Patients with supramitral ring constitute
morphology of the rings, and the surgical management a subset of patients with congenital mitral stenosis who
were studied retrospectively. have a relatively good prognosis. In many cases, the
Results. Accurate preoperative diagnosis was possible supramitral ring is entirely separate from the mitral
by transthoracic echocardiography in 11 patients (73%). valve, and when attached, it is usually most prominent at
The associated anomalies were ventricular septal defects the posteroinferior commissure. In both cases, complete
in 8 patients (53%) and abnormalities of the left ventric- resection is surgically feasible and usually provides
ular outflow tract in 7 patients (47%). A circumferential lasting relief.
supramitral ring, separate from the mitral valve, was
present in 8 patients (53%). In the remaining, the ring (Ann Thorac Surg 2006;81:997–1001)
was attached circumferentially to the anterior and the © 2006 by The Society of Thoracic Surgeons
CARDIOVASCULAR
2006;81:997–1001 SUPRAMITRAL RING
Fig 2. Transmitral gradients. The boxes represent 95% confidence intervals. The vertical lines represent the range of the gradients interval. The
patient with an extreme gradient (30 mm Hg) has been excluded. (POST OP ⫽ postoperative; Preop ⫽ preoperative.)
anterior leaflet. In 2 patients (25%), the supramitral rings incisions (multiple arrows in Fig 4D) were made in the
were found attached to dysplastic mitral valves with posterior part of the ring. These incisions included the
thickened leaflets and chordae. In the remaining 5 pa- full thickness of the ring and extended into the media of
tients, the mitral valve apparatus was normal. the posterior leaflet. The segments of the ring between
Altogether, in 11 of the 15 patients (73%) the mitral these incisions were then resected in this plane. In the
valve was found to be normal after the excision of the patients with abnormal mitral valves, appropriate repair
supramitral ring, and did not require any surgical was carried out. For the patient with the associated
intervention. elongated anterior chordae, chordal shortening and com-
missuroplasty was performed. The child with fused chor-
Surgery dae and papillary muscles was managed by splitting of
All the intracardiac anomalies were corrected in a single- the chordae and papillary muscles. The 2 patients with
stage procedure. After institution of standard cardiopul- Shone’s complex had severely dysplastic leaflets with
monary bypass, topical cooling, antegrade cold blood fused subvalvular apparatus, and in these patients mitral
cardioplegia, and systemic hypothermia to 30oC, the valve repair was not feasible. In the rest of the patients,
mitral valve was approached in all cases through the the mitral valve leaflets were then thoroughly inspected
interatrial septum. Morphologic abnormalities of the for any perforations, and competency of the valve was
mitral valve were systematically assessed. The full extent
of the supramitral ring was delineated. After discerning
the anatomy clearly, radial incisions were made in the
supramitral ring at the level of both the commissures (1
and 2 in Fig 4A and B) up to the mitral annulus. Using
pointed scissors, the segment of the ring adjacent to the
anterior leaflet was excised (Fig 4C). Next, several radial
CARDIOVASCULAR
2006;81:997–1001 SUPRAMITRAL RING
aortic membrane, bicuspid aortic valve, and coarctation 2. Carpentier A, Branchini B, Cour JC, et al. Congenital mal-
of the aorta. In the first scenario, this is important in the formations of the mitral valve in children. Pathology and
context of patients with VSD and turbulence across the surgical treatment. J Thorac Cardiovasc Surg 1976;72:854 – 66.
3. Prifti E, Vanine V, Bonacchi M, et al. Repair of congenital
mitral valve in which a supramitral ring needs to be ruled malformations of the mitral valve: early and midterm results.
out. In the second scenario, in patients with multilevel Ann Thorac Surg 2002;73:614 –21.
left heart obstructions, a supramitral ring should be 4. Davachi F, Moller JH, Edwards JE. Disease of the mitral
excluded to prevent residual defects, as has been re- valve in infancy. Circulation 1971;63:565–79.
ported previously [22, 23]. 5. Kirklin JW, Barratt-Boyes BG. Cardiac surgery. 2nd ed. New
York: Churchill Livingstone, 1993;21– 6.
The levoatriocardinal vein that was associated with the
6. Chauvaud S, Fuzellier JF, Houel R, et al. Reconstructive
supramitral ring in one of our patients is the first descrip- surgery in congenital mitral valve insufficiency (Carpentier’s
tion of the association of these two anomalies. In this techniques): long-term results. J Thorac Cardiovasc Surg
patient, the levoatriocardinal vein acted as a “pop-off” 1998;115:84 –92.
valve and allowed decompression of the left atrium, 7. Coles JG, Williams WG, Watanabe T, et al. Surgical experi-
reducing the severity of pulmonary venous hypertension ence with reparative techniques in patients with congenital
mitral valvular anomalies. Circulation 1987;76(Suppl 3):117–
and delaying the onset of debilitating symptoms.
22.
This study also gives a comprehensive description of 8. Stellin G, Bortolotti U, Mazzucco A, et al. Repair of congen-
the spectrum of morphologic variations of the suprami- itally malformed mitral valve in children. J Thorac Cardio-
tral ring. We have seen that it may be a complete vasc Surg 1988;95:480 –5.
circumferential ring or a partial ring, and that it may 9. Zweng TN, Bluett MK, Mosca R, et al. Mitral valve replace-
adhere to and interfere with the posterior leaflet of the ment in the first 5 years of life. Ann Thorac Surg 1989;47:
720 –724.
mitral valve, a finding observed by others too [10]. 10. Mychaskiw G II, Sachdev V, Braden DA, et al. Supramitral
We emphasis that the transseptal approach can be ring: an unusual cause of congenital mitral stenosis. Case
planned in most cases. This allows for adequate surgical series and review. J Cardiovasc Surg 2002;43:199 –202.
exposure for excision of the ring as well as for optimal 11. Agarwal S, Airan B, Chowdhury UK, et al. Ventricular septal
assessment of mitral valve anatomy. Most of the com- defect with congenital mitral vale disease: long-term results
of corrective surgery. Indian Heart J 2002;54:67–73.
monly associated anomalies can be managed through
12. Fisher T. Two cases of congenital disease of the left side of
this incision such as VSD closure, subaortic membrane the heart. Br Med J 1902;1:639 – 41.
excision through the VSD, and correction of double 13. Lynch MF, Ryan NJ, Williams CR, et al. Preoperative diag-
outlet right ventricle, facilitating a single stage total nosis and surgical correction of supravalvular mitral stenosis
correction for all patients. and ventricular septal defect. Circulation 1962;25:854 – 61.
The first surgical correction of supramitral ring was 14. Shone JD, Sellers RD, Andeerson RC, Adams P Jr, Lillehei
CW, Edwards JE. The developmental complex of “parachute
described by Lynch and colleagues [13] in 1962 in a study
mitral valve”, supravalvular ring of the left atrium, subaortic
of 14 patients operated on at the Hospital for Sick stenosis, and coarctation of the aorta. Am J Cardiol 1963;11:
Children in London. The study showed that surgical 714 –25.
correction of this anomaly can be performed successfully, 15. Anabtawi IN, Ellison RG. Congenital stenosing ring of the
leading to excellent late clinical results. This finding has left atrioventricular canal (supravalvular mitral stenosis).
been confirmed by our study. Our only death was an J Thorac Cardiovasc Surg 1965;45:994 –1005.
16. Macartney FJ, Scott O, Ionescu MI, Deverall PB. Diagnosis
infant with Shone’s complex, which itself is a complex and management of parachute mitral valve and supravalvu-
disease process with a guarded prognosis. All the rest of lar mitral ring. Br Heart J 1974;36:641–52.
our patients have done well as demonstrated by fol- 17. Oglietti J, Rejl GJ, Leachman RD, Cooley DA. Supravalvular
low-up echocardiograms. stenosing ring of the left atrium. Ann Thorac Surg 1976;21:
In conclusion, supramitral ring is an entity whose 421– 6.
18. Chung KJ, Manning JA, Lipchik EO, Gramiak R, Mahoney
morphology and optimal management is still being elu-
EB. Isolated supravalvular stenosing ring of left atrium:
cidated. A high index of suspicion is required when diagnosis before operation and successful surgical treat-
performing transthoracic echocardiography in the clini- ment. Chest 1974;65:25– 8.
cal settings discussed above, and transesophageal echo- 19. Jacobstein MD, Hirschfield SS. Concealed left atrial mem-
cardiogram is recommended whenever transmitral gra- brane: pitfalls in the diagnosis of cor triatriatum and supra-
dients are obtained. A transseptal approach allows valve mitral ring. Am J Cardiol 1982;49:780 – 6.
20. Sullivan ID, Robinson PJ, DeLeval M, Graham TP. Membra-
optimal exposure of the ring and the mitral valve, allow-
nous supravalvular mitral stenosis: a treatable form of con-
ing ring excision without compromising mitral valve genital heart disease. J Am Coll Cardiol 1986;8:159 – 64.
function, while also facilitating simultaneous correction 21. Muhiudeen Russel IA, Miller Hance WC, Silverman NH.
of associated cardiac anomalies. Appropriate evaluation Intraoperative transesophageal echocardiography for pedi-
and management of this rare anomaly results in a grati- atric patients with congenital heart disease. Anest Analg
fying long-term outcome. 1998;87:1058 –76.
22. Manganas C, Iliopoulos J, Chard RB, et al. Reoperation and
coarctation of the aorta: the need for lifelong surveillance.
References Ann Thorac Surg 2001;72:1222– 4.
23. Levine JC, Sanders SP, Colen SD, et al. The risk of having
1. Collins-Nakai RL, Rosenthal A, Castaneda AR, et al. Con- additional obstructive lesions in neonatal coarctation of the
genital mitral stenosis. Circulation 1977;56:1039 – 47. aorta. Cardiol Young 2001;11:44.