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235 Vol.-3, No.-2, January 2011 Cardiovas Journal
Introduction:
Tricuspid valve stenosis is a valvular heart disease
which results in the narrowing of the orifice of the
tricuspid valve of the heart. It’s relatively a rare
condition. The stenosis causes increased resistance
to blood flow through the valve. It is almost always
caused by rheumatic fever and is generally
accompanied by mitral stenosis.Rare other causes
include carcinoid syndrome, endocarditis,
endomyocardial fibrosis, lupus erythematosus,
right atrial myxoma and congenital tricuspid
atresia.1-9
The mortality associated with tricuspid stenosis
depends on the precipitating cause. The general
mortality rate is approximately 5%.1
Tricuspid stenosis is found in approximately 3% of
the international population. It is more prevalent
in areas with a high incidence of rheumatic fever.
The congenital form of the disease is rare and true
incidence is not available.1,2
Tricuspid stenosis can present as a congenital
lesion or later in life when it is due to some other
condition. The congenital form accounts for
approximately 0.3% of all congenital heart disease
cases. The frequency of tricuspid stenosis in the
older population, due to secondary causes, ranges
from 0.3-3.2%.2
Tricuspid stenosis is observed more commonly in
women than in men, similar to mitral stenosis of
rheumatic origin. The congenital form of the
disease has a slightly higher male predominance.2
Tricuspid valve stenosis itself usually doesn’t
require treatment. However, if there is damage
to other valves in the heart as well, then surgical
repair or replacement must be considered.The
treatment is usually by surgery (tricuspid valve
replacement) or percutaneous balloon
valvuloplasty. The resultant tricuspid regurgitation
from percutaneous treatment is better tolerated
than insufficiency occurring during mitral
valvuloplasty.1-3
Here we describe a patient with history of prior
Closed Mitral Commissurotomy (CMC) presented
with severe Tricuspid Stenosis with Tricuspid
Regurgitation (Grade-IV), Mitral Restenosis
(Severe), Mitral Regurgitation (Grade-1+), Aortic
Stenosis (Mild) and Aortic Regurgitation (Grade-2).
Case History:
Mrs. X , a 34 years old housewife, admitted into
NICVD, with the complaints of exertional
shortness of breath for last 11 years and shortness
of breath on lying flat for last 2 years. She also
complained of recurrent swelling of both lower
limbs and distension of abdomen that was
occasionally relieved by intravenous diuretics. She
was diagnosed as a case of chronic rheumatic heart
disease with severe mitral stenosis and underwent
CMC in 1993 when he was only 15 years old. After
A Case of Severe Tricuspid Stenosis of
Rheumatic Origin
MT Rahman, M Das, M Ullah, Z Rahman, A Hossain, LA Sayami, SS Roy, AAS Majumder
Department of Cardiology, National Institute of Cardiovascular Disease, Dhaka, Bangladesh.
Abstract:
Tricuspid valve stenosis is a valvular heart disease which results in the narrowing of the orifice of
the tricuspid valve of the heart. It’s relatively a rare condition. It is almost always caused by
rheumatic fever and is generally accompanied by mitral stenosis.Other rare causes include carcinoid
syndrome, endocarditis, endomyocardial fibrosis, lupus erythematosus, right atrial myxoma and
congenital tricuspid atresia. Here we describe a patient with history of prior CMC presented with
severe Tricuspid Stenosis with Tricuspid Regurgitation (Grade-IV), Mitral Restenosis (Severe),
Mitral Regurgitation (grade-1+), Aortic Stenosis (Mild) and Aortic Regurgitation (Grade-2).
(Cardiovasc. j. 2011; 3(2): 235-238)
Address of Correspondence- Dr. Md. Toufiqur Rahman , Associate Professor, Department of Cardiology , NICVD,
Dhaka, Bangladesh.
Keywords:
Tricuspid
stenosis;
Rheumatic heart
disease; Valvular
heart disease.
236 Vol.-3, No.-2, January 2011 Cardiovas Journal
doing CMC she was reasonably well for the next 8
years.
On examination, patient was ill looking,
orthopnoeic, mildly anaemic, edematous, mildly
icteric, not cyanosed. Her neck vein was engorged.
Pulse rate was 80 b/min, irregularly irregular with
pulsus deficit of 16 beats/ min. B.P. was 80/60
mmHg. Apex is shifted 2 cm lateral to left
midclavicular line and left parasternal heave was
present. First heart sound was variable, P2 was
palpable. There was a mid diastolic murmur ,
grade-3/6 over apical area and a ejection systolic
murmur over right second intercostal space and
a diastolic murmur over left lower sternal border
having grade of 3/6. Breath sound was vesicular
and there was bilateral basal crepitations. There
was tense ascites and tender hepatomegaly. On
careful Echo Doppler examination, patient had
Tricuspid Stenosis (Severe) with Tricuspid
Regurgitation (Grade-IV), Mitral Stenosis (Severe),
Mitral Regurgitation (grade-1+), Aortic Stenosis
(Mild) and Aortic Regurgitation (Grade-2) (Fig-
1,2,3). She was treated conservatively.
Discussion:
Tricuspid valve dysfunction can result from
morphological alterations in the valve or from
functional aberrations of the myocardium.
Tricuspid stenosis is almost always rheumatic in
origin and is generally accompanied by mitral and
aortic valve involvement.1
Most stenotic tricuspid valves are associated with
clinical evidence of regurgitation that can be
documented by performing a physical examination
(murmur), echocardiography, or angiography.
Stenotic tricuspid valves are always anatomically
abnormal, and the cause is limited to a few
conditions. With the exceptions of congenital
causes or active infective endocarditis, tricuspid
stenosis takes years to develop.2,3
Sato M et al described a case of tricuspid stenosis
found intraoperatively during aortic valve
replacement and open mitral commissurotomy
with congestive heart failure due to mitral
regurgitation and tricuspid regurgitation and so,
commissurotomy and modified Kay’s annuloplasty
was tried but failed to decrease the central
regurgitation. A CarboMedics 23 mm prosthesis
was implanted in the mitral position and a
Capentier-Edwards 29 mm in the tricuspid
position.10
Tsutomu Saito 11 et al reported the case of a 42-
year-old man in whom dyspnea on exertion was
found to be caused by isolated tricuspid stenosis.
Two-dimensional echocardiogram showed
thickening of the tricuspid valve with a markedly
enlarged right atrium. A color-flow Doppler
examination-revealed severe tricuspid stenosis
without regurgitation and a Doppler-derived
Fig.-1: Mitral stenosis
Fig.-2: Aortic regurgitation
Fig.-3: Tricuspid Stenosis
A Case of Severe Tricuspid Stenosis of Rheumatic Origin MT Rahman et al.
236
237 Vol.-3, No.-2, January 2011 Cardiovas Journal
tricuspid diastolic pressure gradient of 23 mmHg.
At the time of surgery, the patient was noted to
have a stenotic tricuspid valve with thickened
leaflets, fused commissures, and almost normal
chorda tendineae. The valve leaflets were teased
apart to the scattered specimen, and tricuspid
valve replacement was successfully performed.
Microscopic examination of the specimen
demonstrated infective endocarditis. Isolated
acquired tricuspid stenosis is extremely rare and
that was the first case of infective endocarditis
being involved as the primary cause.
Yash Y et al reported a patient with congenital
tricuspid stenosis successfully treated by
percutaneous balloon valvotomy that appears to
be an alternative to surgery when the anatomy,
as demonstrated by echocardiography, is
suitable.12
Taira Kayano et al13 reported Tricuspid Valve
Stenosis Related to Subvalvular Adhesion of
Pacemaker Lead An excessive loop of a ventricular
lead, especially a subvalvular loop, can cause
opening limitation of the tricuspid valve, and the
entangling of the lead in the subvalvular structures
can easily induce reactive fibrosis and adhesions.
Hussain et al described a case tricuspid stenosis
in a young woman 11 years after endocardial
ventricular lead implantation. The cause of the
stenosis was perforation of the septal leaflet by
the lead at the time of insertion and then successful
management with percutaneous balloon
valvuloplasty without the need for lead removal.14
Ashraf et al reported a case of concurrent
successful percutaneous valvuloplasty of combined
mitral and tricuspid value stenosis employing the
Inoue technique.15 D J Heaven et al16 described
two cases of pacemaker lead related tricuspid
stenosis. Sivert Svane reported six autopsied cases
of Congenital Tricuspid Stenosis17 .M E Lee et al
reported a case of unusual complications of
endocardial pacing like simultaneous thrombosis
of the superior and inferior venae cavae and
supravalvular, valvular, and infravalvular stenosis
of the tricuspid valve.18 L G Van der Hauwaert et
al 19 reported a case of fibroma of the right
ventricle producing severe tricuspid stenosis.
Roguin et al20 reported Long-term follow-up of
patients with severe rheumatic tricuspid stenosis.
13 patients (11 women and 2 men) with severe
tricuspid stenosis who were followed closely for 6
to 31 years. The mean tricuspid pressure gradient
ranged from 3 to 9 mm Hg. Twelve patients
underwent surgery for mitral and/or aortic valve
lesions without complications. Concomitant
tricuspid valve surgery was performed on six
patients. After successful repair of the mitral or
aortic valve, and regardless of the type of tricuspid
valve surgery, severe tricuspid stenosis was found
to be very well tolerated by all the patients over
many years of follow-up. L V Basso et al reported
a case of Tricuspid valve obstruction due to
intravenous leiomyomatosis.21
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Cardiovascular Journal Volume 3, No. 2, 2011
237
238 Vol.-3, No.-2, January 2011 Cardiovas Journal
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A Case of Severe Tricuspid Stenosis of Rheumatic Origin MT Rahman et al.
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