Baylor University Medical Center Proceedings October 2017 - 435
Combined mitral and aortic valve stenosis caused by two
different etiologies, rheumatic and congenital
William C. Roberts, MD, and Joshua K. Dodderer, BS
Table 1. Pertinent findings in the five patients with stenotic congenitally bicuspid aortic valves and stenotic rheumatic mitral valves
Pressures (mm Hg)
Case
number
Age at VR
BMI
(years) Sex (kg/m2)
PAW
(mean)
RV
(s/d)
RA
(mean)
LV
(s/d)
Ao
(s/d)
LV-Ao
(psg)
PAW-LV
(mdg)
AV area MV area AV weight MV weight
(cm2)
(cm2)
(g)
(g)
1
44
M
26
21
86/19
16
126/14 108/66
18
16
-
1.54
0.9
3.8
2
46
M
26
26
48/18
13
139/17 133/88
28
15
0.46
0.95
1.37
3.44
3
50
M
27
39
56/18
13
173/40 146/88
27
25
1.0
1.28
-
-
4
53
F
34
-
-
-
-
-
0.9
1.8
-
-
5
74
F
21
33
79/17
54
31
-
0.53
0.95
-
-
-
-
226/19 195/85
Ao indicates aorta; AV, aortic valve; BMI, body mass index; LV, left ventricular; mdg, mean diastolic gradient; MV, mitral valve; PAW, pulmonary artery wedge; psg, peak systolic
gradient; RA, right atrium; RV, right ventricular; s/d, peak systole/end diastole; VR, valve replacement.
Described herein are five patients who had double left-sided cardiac
valve replacement for mitral and aortic valve stenosis resulting from
two different etiologies: rheumatic heart disease, the cause of the mitral
stenosis, and congenital heart disease (bicuspid valve), the underlying
cause of the aortic stenosis.
C
ombined mitral and aortic valve stenosis in the same
patient today in the Western world is relatively uncommon. Worldwide, its most common cause is rheumatic heart disease. Today in native Europeans and in
European Americans, the most common cause of this combination appears to be massive mitral annular calcification (probably
the consequence of atherosclerosis) as the cause of the mitral stenosis and either congenital (unicuspid or bicuspid aortic valve)
or nonrheumatic causes (tricuspid aortic valve) as the cause of
the aortic stenosis (atherosclerotic etiology) (1). On very rare
occasions, the mitral stenosis may be the result of rheumatic
heart disease and the aortic stenosis, the result of an underlying
congenital heart disease (unicuspid or bicuspid aortic valve).
Such was the case in the five patients described herein.
METHODS
Shown in Table 1 are pertinent findings in the five patients
with combined rheumatic mitral stenosis and aortic stenosis superimposed on a congenitally bicuspid aortic valve. Photographs
Proc (Bayl Univ Med Cent) 2017;30(4):435-436
of the operatively excised valves are shown in Figures 1 and 2.
Only Case 1 is still alive. Cases 2 to 5 survived from 6.9 to
14.4 years (mean 11.2) after the double valve replacement.
DISCUSSION
The patients described here had two different causes for
the two stenotic valves on the left side of the heart: rheumatic
heart disease for the mitral stenosis and congenital heart disease
(bicuspid aortic valve) for the aortic stenosis. Relatively few
such combinations have been reported previously. McReynolds
and associates (2) in 1976 described an 18-year-old man with
a purely regurgitant congenitally bicuspid aortic valve (without commissioned fusion) and a diffusely fibrotic nonstenotic
mitral valve proven to be of rheumatic etiology by the presence
of Aschoff nodules in the left atrial wall. Tejada and associates (3) in 2001 described a 60-year-old woman who had a
noncalcified congenitally bicuspid aortic valve and percutaneous valvuloplasty for a typical rheumatic stenotic mitral valve.
Muthiah (4) in India in 2016 described by echocardiogram a
From the Baylor Heart and Vascular Institute and the Departments of Internal
Medicine (Division of Cardiology) and Pathology, Baylor University Medical Center,
Dallas, Texas (Roberts); and Texas Tech University Health Sciences Center Paul
L. Foster School of Medicine, El Paso, Texas (Dodderer).
Corresponding author: William C. Roberts, MD, Baylor Heart and Vascular
Institute, Baylor University Medical Center at Dallas, 621 North Hall Street, Dallas,
TX 75226 (e-mail: william.roberts1@bswhealth.org).
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