CARDIOVASCULAR
Supramitral Ring: Good Prognosis in a Subset of
Patients With Congenital Mitral Stenosis
Sathiakar Paul Collison, MS, Sunil Kumar Kaushal, MCh,
Kulbushan Singh Dagar, MCh, Parvathi Unninayar Iyer, MD, Sumir Girotra, MD,
Sitaraman Radhakrishnan, DM, Savitri Shrivastava, DM, and
Krishna Subramony Iyer, MCh
Escorts Heart Institute and Research Centre, New Delhi, India
Background. Management of congenital stenotic mitral
valvular abnormalities remains an important therapeutic
challenge. Supramitral ring constitutes a small but inad-
equately described subset that has a relatively good
outcome with appropriate management.
Methods. Between 1996 and 2004, 15 patients with
supramitral ring were managed in this institution. The
demographic and clinical features, diagnostic modalities,
morphology of the rings, and the surgical management
were studied retrospectively.
Results. Accurate preoperative diagnosis was possible
by transthoracic echocardiography in 11 patients (73%).
The associated anomalies were ventricular septal defects
in 8 patients (53%) and abnormalities of the left ventric-
ular outflow tract in 7 patients (47%). A circumferential
supramitral ring, separate from the mitral valve, was
present in 8 patients (53%). In the remaining, the ring
was attached circumferentially to the anterior and the
C ongenital mitral valvular abnormalities remain an
important therapeutic challenge [1–3]. The malfor-
mation is both diverse and rare, being identified in 0.6%
of autopsied hearts with congenital heart disease, and
constituting 0.21% to 0.42% of clinical series [1, 2, 4]. In
view of the rarity of the lesion, the frequency of associ-
ated defects and the relatively limited experience in each
institution, congenital mitral stenosis remains inade-
quately understood and continues to remain a surgical
challenge [3, 5–7]. These factors, when combined, have
contributed to the perception that prognosis is poor for
all patients with congenital mitral stenosis [8, 9].
In Carpentier’s classification of congenital mitral ste-
nosis [2], supramitral ring is categorized under congeni-
tal mitral stenosis associated with normal papillary mus-
cles. It is a rare malformation—fewer than 100 cases
having been reported in literature according to a recent
review [10]. Another recent study indicates that suprami-
tral ring may be present in upto 8% of all children with
congenital mitral valve disease [11]. Increasing aware-
Accepted for publication June 24, 2005.
Address correspondence to Dr K. S. Iyer, Department of Pediatric and
Congenital Heart Surgery, Escorts Heart Institute and Research Centre,
New Delhi 110025, India; e-mail address: iyerks_ehirc@yahoo.com.
© 2006 by The Society of Thoracic Surgeons
Published by Elsevier Inc
posterior mitral leaflets and was most densely adherent
at the posteroinferior commissure in 4 of these 7 patients
(57%). Complete excision of ring was possible in all
cases, without damage to the mitral valve. There was 1
in-hospital death (6%). At a mean follow-up of 30
months, 14 survivors continue to do well, with no signif-
icant recurrence of mitral stenosis.
Conclusions. Patients with supramitral ring constitute
a subset of patients with congenital mitral stenosis who
have a relatively good prognosis. In many cases, the
supramitral ring is entirely separate from the mitral
valve, and when attached, it is usually most prominent at
the posteroinferior commissure. In both cases, complete
resection is surgically feasible and usually provides
lasting relief.
(Ann Thorac Surg 2006;81:997–1001)
© 2006 by The Society of Thoracic Surgeons
ness of this condition as well as refinements in diagnostic
techniques will probably allow more frequent diagnosis
of this condition [21]. The identification of this subset of
patients is important, however, because of the relatively
better prognosis with appropriate management. There is
paucity of information in the literature about the pathol-
ogy, diagnosis, and surgical management of this entity,
and this retrospective analysis of a series of 15 patients
managed in our institution attempts to address some of
these issues.
Material and Methods
Between January 1996 and December 2004, 15 patients
were operated upon in our institution for supramitral
ring causing significant mitral stenosis, either in isolation
or in association with other cardiac anomalies. The mean
age of the patients was 26.5 months (range, 2 months to 6
years): 1 (6%) was younger than 3 months, 5 (34%) were
between 3 months and 12 months, and the remaining 9
(60%) were between 1 and 5 years of age. The weight of
the patients ranged from 4 kg to 14 kg (mean, 9 kg): 4
(26%) were below the weight of 5 kg, 5 (34%) between 5
and 10 kg, and 6 (40%) were between 10 and 15 kg. The
ratio of males to females was 1.1 to 1.
0003-4975/06/$32.00
doi:10.1016/j.athoracsur.2005.06.079
 998 COLLISON ET AL
CARDIOVASCULAR
SUPRAMITRAL RING
Clinical Presentation
Two children (13%) were asymptomatic, and were diag-
nosed on incidental postnatal echocardiography. Twelve
patients (81%) presented with failure to thrive or fre-
quent respiratory tract infections. One child (6%) pre-
sented with pulmonary edema requiring preoperative
ventilatory and inotropic support.
Cardiac morphology was delineated by transthoracic
echocardiography in all cases. Cardiac catheterization
and angiocardiography was performed when associated
anomalies were not clearly defined on echocardiography
(n ⫽ 4), or when assessment of pulmonary vascular
disease was required.
Preoperative Diagnosis
Preoperative diagnosis was possible by transthoracic
echocardiography (Fig 1), at the first examination, in 11
patients (73%). In 1 patient (7%), the diagnosis was made
at a follow-up examination (see below) whereas in 3
patients (20%), the diagnosis was made at the time of
surgery during examination of the mitral valve for sus-
pected mitral valve abnormalities. Among these, 1 pa-
tient had had a repair of coarctation of aorta and pulmo-
nary artery banding for multiple muscular ventricular
septal defects (VSDs) at the age of 4 months. When she
presented for definitive closure of VSDs, the transtho-
racic echocardiogram revealed turbulence across the
mitral valve prompting inspection of the valve at surgery,
when a supramitral ring was discovered.
The second patient was diagnosed with coarctation of
the aorta and severe congenital mitral stenosis in the
neonatal period. In view of the severity of the mitral
stenosis, the child’s family was counseled regarding the
poor prognosis of the disease and they opted for balloon
dilatation of the coarctation as a palliative procedure. She
then presented at 3 years of age (weight 12 kg) with a
history of recurrent respiratory infections but was other-
wise well preserved. Transthoracic echocardiography re-
vealed the presence of the supramitral ring (transmitral
Fig 1. Preoperative transthoracic echocardiography showing the su-
pramitral ring (thick arrow) in relation to the mitral valve (thin
arrow).
Ann Thorac Surg
2006;81:997–1001
Table 1. Associated Anomalies
Anomaly Number (%)
Ventricular septal defect 8 (53)
Subaortic membrane 4 (28)
Double-outlet right verticle 2 (14)
Coarctation aorta 6 (43)
Shone’s complex 2 (14)
gradient 8 mm Hg). At operation, a levoatriocardinal vein
was found in addition to the supramitral ring, which was
decompressing the left atrium and thereby reducing the
severity of pulmonary venous hypertension. The mitral
valve was entirely normal.
The third patient was found to have a supramitral ring
at the time of mitral valve repair for mitral regurgitation
due to elongated anterior chordae. The fourth patient
had a VSD along with a gradient and turbulence across
the mitral valve. On exposure of the mitral valve, the
supramitral ring was detected and excised. Two patients
(13%) had the constellation of findings associated with
Shone’s complex. The additional cardiac anomalies are
described in Table 1. Only 1 patient had an isolated
supramitral ring.
Mitral Valve Assessment
The mitral valve was systematically assessed in all the
patients using transthoracic echocardiography. The leaf-
lets, the chordae, and the papillary muscles were viewed
and the size of the mitral annulus was measured, and the
Z score calculated. Color Doppler was used to quantify
the peak and mean transmitral gradients. All the patients
had significant preoperative transmitral gradients (Fig 2).
The mean transmitral gradients ranged from 7 mm Hg
to 30 mm Hg (mean, 12 mm Hg). The patient with the
isolated supramitral ring had the largest gradient of 30
mm Hg. The Z scores were found to be within the normal
range in all the patients except for 2. These were the
children with Shone’s complex: the Z scores were –2.4
and –3.0.
The morphology of the supramitral rings in our pa-
tients is detailed in Figure 3. In all patients, the suprami-
tral ring was circumferential. In 7 patients (47%), the
supramitral ring was attached to the left atrium away
from the mitral annulus (supra-annular supramitral
ring). In this subgroup, the mitral valve was found to be
normal in 5 patients (63%). In the remaining 2 patients,
the mitral valvular anomalies found were elongated
anterior chordae causing regurgitation in 1 patient and
hypoplastic annulus with all chordae being attached to a
single papillary muscle in the other: both were amenable
to adequate repair.
In 8 patients (53%), supramitral ring was found to arise
from the left atrial aspect of the mitral valve leaflet. In
this subgroup, the attachment was found to be maximal
at the posteroinferior commissure in 5 (63%). In 1 patient,
the attachment was only to the posterior leaflet of the
mitral valve, and the ring was supra annular along the
Ann Thorac Surg
2006;81:997–1001
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
were found attached to dysplastic mitral valves with
thickened leaflets and chordae. In the remaining 5 pa-
tients, the mitral valve apparatus was normal.
Altogether, in 11 of the 15 patients (73%) the mitral
valve was found to be normal after the excision of the
supramitral ring, and did not require any surgical
intervention.
Surgery
All the intracardiac anomalies were corrected in a single-
stage procedure. After institution of standard cardiopul-
monary bypass, topical cooling, antegrade cold blood
cardioplegia, and systemic hypothermia to 30oC, the
mitral valve was approached in all cases through the
interatrial septum. Morphologic abnormalities of the
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
Fig 3. Morphology of the supramitral rings.
COLLISON ET AL 999
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SUPRAMITRAL RING
incisions (multiple arrows in Fig 4D) were made in the
posterior part of the ring. These incisions included the
full thickness of the ring and extended into the media of
the posterior leaflet. The segments of the ring between
these incisions were then resected in this plane. In the
patients with abnormal mitral valves, appropriate repair
was carried out. For the patient with the associated
elongated anterior chordae, chordal shortening and com-
missuroplasty was performed. The child with fused chor-
dae and papillary muscles was managed by splitting of
the chordae and papillary muscles. The 2 patients with
Shone’s complex had severely dysplastic leaflets with
fused subvalvular apparatus, and in these patients mitral
valve repair was not feasible. In the rest of the patients,
the mitral valve leaflets were then thoroughly inspected
for any perforations, and competency of the valve was
Fig 4. Technique of surgical excision of supramitral ring. (A, B) Af-
ter discerning the anatomy clearly, radial incisions (arrows) were
made in the supramitral ring at the level of both commissures (1
and 2) up to the mitral annulus. (C) Using pointed scissors, the seg-
ment of the ring adjacent to the anterior leaflet was excised. (D)
Next, several radial incisions (arrows) were made in the posterior
part of the ring. See text for full description. (Illustrations by
Krishna Subramony Iyer, MCh)
 1000 COLLISON ET AL
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SUPRAMITRAL RING
Fig 5. Postoperative transthoracic echocardiogram showing laminar
flow across the mitral valve.
assessed by instillation saline into the left ventricular
cavity. The mitral annulus was also sized with appropri-
ately sized dilators. The associated cardiac anomalies
were then corrected. All patients had intraoperative
epicardial or transesophageal echocardiography to assess
the adequacy of surgery.
Results
Early Results
There was 1 early death (6%) of a patient with suprami-
tral ring as a part of Shone’s complex who was operated
on at 2 months of age. He had persistent pulmonary
venous hypertension in the postoperative period related
to residual subaortic stenosis from the dysplastic mitral
valve and left ventricular dysfunction. He died on the
54th postoperative day after prolonged ventilation, sep-
ticemia, and multiorgan failure.
The remaining 14 patients had relatively uncompli-
cated postoperative courses. Mean duration of ventila-
tion was 26 hours, and mean intensive care stay was 6.6
days (range 3 to 10 days). The postoperative gradients are
detailed in Figure 2.
Long-Term Results
All 14 survivors are on regular follow-up and are being
reviewed by our pediatric cardiologist. Mean follow-up
has been 30 months (range, 3 months to 5.5 years).
There were no late deaths, and there were no patients
who required reoperation. All patients are off cardiac
medications. Postoperative transthoracic echocardio-
gram revealed no significant mitral stenosis in any pa-
tient (Fig 5). One patient with Shone’s complex has
moderate left ventricular outflow obstruction. The trans-
mitral gradients by echocardiography at follow-up is
shown in Figure 2.
Comment
Fisher [12] was the first to report membranous supraval-
vular mitral stenosis, the supramitral ring, in 1902. Since
then, there have been many case reports and a few short
Ann Thorac Surg
2006;81:997–1001
series [13–17]. A recent review highlights the rarity of this
condition [10]. It is commonly associated with various
other anomalies of the heart. However, even more rare is
the occurrence of an isolated supramitral ring, first de-
scribed by Chung and associates [18] in 1974, similar to
the case described in this article.
Supramitral ring must be differentiated from the more
common cor triatriatum [19]. Cor triatriatum develops as
a result of failure of embryological development of the
left pulmonary vein during the fifth week of gestation.
Hence, the left atrium gets divided into distinct proximal
and distal chambers. Usually an atrial septal defect is
associated. Additionally, in cor triatriatum, the mem-
brane is generally well separated from the mitral valve, is
proximal to the left atrial appendage, and consists of
fibromuscular tissue. In contrast, supramitral ring is
thought to be derived from a failure of the endocardial
cushions to divide completely. It is usually located in
close proximity to the mitral valve, and the opening of the
left atrial appendage is usually above a supramitral ring.
The ring is fibrous.
The physiologic consequence of the supramitral ring is
similar to that of other obstructive anomalies that can
occur in the left atrium, namely, pulmonary vein stenosis,
cor triatriatum, and mitral valvular stenosis, and includes
elevated pulmonary venous pressure as well as pulmo-
nary arterial hypertension. Hence, children present with
congestive heart failure and failure to thrive. Preopera-
tive diagnosis of supramitral ring is difficult. Using trans-
thoracic echocardiography, the diagnosis has generally
been made in only approximately 50% of cases. In a study
by Sullivan and coworkers [20], postoperative retrospec-
tive analysis of echocardiographic tapes utilizing a de-
tailed frame-by-frame examination yielded the diagnosis
in 91%; however, the preoperative diagnosis was made in
this study only in 45% of the cases. In our series, too,
preoperative diagnosis was made in only 73% of cases.
That could possibly reflect difficulties in delineating a
membrane in the atrium in our patients, most of whom
had associated ventricular septal defects with left to right
shunts and hence increased blood flow in the left atrium.
In our patients in whom diagnosis was made intraoper-
atively, the preoperative echocardiogram revealed only
turbulence across the mitral valve; and upon exposure of
the mitral valve, the valve itself was found to be normal,
the only anomaly being the supramitral ring, which was
excised with good outcome. Although the supramitral
ring can occasionally be diagnosed by angiocardiography
[17], it is fairly insensitive in the diagnosis of the condi-
tion. Transesophageal echocardiography is perhaps the
best tool to diagnose supramitral ring [21]. It can differ-
entiate supramitral ring from cor triatriatum, define the
attachment of the obstructing membrane, and quantify
the gradient.
The cardiac anomalies associated with supramitral ring
have not been well described. From this study, we
propose that the associated cardiac anomalies can be
grouped broadly into two categories: supramitral ring
associated with VSD and supramitral ring associated
with left-sided obstructive pathologies, especially sub-
Ann Thorac Surg
2006;81:997–1001
aortic membrane, bicuspid aortic valve, and coarctation
of the aorta. In the first scenario, this is important in the
context of patients with VSD and turbulence across the
mitral valve in which a supramitral ring needs to be ruled
out. In the second scenario, in patients with multilevel
left heart obstructions, a supramitral ring should be
excluded to prevent residual defects, as has been re-
ported previously [22, 23].
The levoatriocardinal vein that was associated with the
supramitral ring in one of our patients is the first descrip-
tion of the association of these two anomalies. In this
patient, the levoatriocardinal vein acted as a “pop-off”
valve and allowed decompression of the left atrium,
reducing the severity of pulmonary venous hypertension
and delaying the onset of debilitating symptoms.
This study also gives a comprehensive description of
the spectrum of morphologic variations of the suprami-
tral ring. We have seen that it may be a complete
circumferential ring or a partial ring, and that it may
adhere to and interfere with the posterior leaflet of the
mitral valve, a finding observed by others too [10].
We emphasis that the transseptal approach can be
planned in most cases. This allows for adequate surgical
exposure for excision of the ring as well as for optimal
assessment of mitral valve anatomy. Most of the com-
monly associated anomalies can be managed through
this incision such as VSD closure, subaortic membrane
excision through the VSD, and correction of double
outlet right ventricle, facilitating a single stage total
correction for all patients.
The first surgical correction of supramitral ring was
described by Lynch and colleagues [13] in 1962 in a study
of 14 patients operated on at the Hospital for Sick
Children in London. The study showed that surgical
correction of this anomaly can be performed successfully,
leading to excellent late clinical results. This finding has
been confirmed by our study. Our only death was an
infant with Shone’s complex, which itself is a complex
disease process with a guarded prognosis. All the rest of
our patients have done well as demonstrated by fol-
low-up echocardiograms.
In conclusion, supramitral ring is an entity whose
morphology and optimal management is still being elu-
cidated. A high index of suspicion is required when
performing transthoracic echocardiography in the clini-
cal settings discussed above, and transesophageal echo-
cardiogram is recommended whenever transmitral gra-
dients are obtained. A transseptal approach allows
optimal exposure of the ring and the mitral valve, allow-
ing ring excision without compromising mitral valve
function, while also facilitating simultaneous correction
of associated cardiac anomalies. Appropriate evaluation
and management of this rare anomaly results in a grati-
fying long-term outcome.
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