Download right ventricular myxoma infiltrating the tricuspid valve and

Case Report
RIG H T VENTRICULAR M Y X O M A INFILTRATING THE
TRICUSPID VALVE A N D OBSTRUCTING THE RIGHT
VENTRICULAR INFLOW A N D O U TFLO W TRACTS
Hakan Tezcan, M . D.* / Oğuz Caymaz, M . D.* / Ahmet Toprak, M . D.*
A li Serdar Fak, M.D.* / Cevat Yakut, M . D.** / Ahmet O ktay, M.D.*
* D e pa rtm e nt o f Cardiology, School o f M edicine, Marmara University, Istanbul, Turkey.
* Ko§uyolu, Heart Training and Research Hospital, Istanbul, Turkey.
ABSTRACT
CASE REPORT
Myxomas originating in the right ventricle are
extremely rare tumors. Herein, a case of right
ventricular myxoma, infiltrating the tricuspid valve and
causing right ventricular inflow and outflow tract
obstruction,
is
described.
Two-dimentional
echocardiography diagnosed the mass easily and
cardiac Doppler with color-flow examinations defined
precisely its hemodynamic consequences to the heart.
Magnetic resonance imaging study helped In
preoperatively defining its intracardiac extension and
the differential diagnosis by its very specific tissue
characterization. The patient was successfully treated
surgically and the tricuspid valve was replaced with a
bioprosthesis because of tumor invasion of the
valvular tissue.
A 20-year-old woman was admitted because of
palpitations and dyspnea on exertion with a duration of
one year. She was known to have had two episodes of
syncope related to exertion within the last year. On
physical examination; the heart rate was 1 1 0 beats per
minute with a regular rhythm. There was no jugular
venous distension, but a prominent "a" wave was
noted. Cardiac examination revealed a remarkable left
parasternal lift (tumor shock); and a systolic thrill at the
second and third left intercostal spaces. The first heart
sound was normal, while the second heart sound was
widely split (tumor plop). A late diastolic sound,
presumably a fourth heart sound originating from the
right heart, was also heard. There was a grade 3/6
harsh systolic ejection murmur at the second and third
left intercostal spaces. No diastolic murmur was
audible. The chest radiograph showed a mildly
enlarged right heart silhouette and a prominent
pulmonary conus with normal pulmonary vascularity.
The electrocardiogram showed sinus tachycardia,
right axis deviation, P-pulmonale, RV hypertrophy and
incomplete right bundle branch block. A 2-D
echocardiogram disclosed a huge mass in the RV
cavity, which prolapsed into the pulmonary artery
during systole (Fig. 1), and was also attached to the
TV obstructing the inflow tract (Fig. 2). Right atrium
(RA) and RV were markedly dilated, while left ventricle
was rather small with a paradoxical septal motion.
Color-flow and Doppler examinations revealed mild
tricuspid and pulmonary regurgitations, a systolic
gradient of 23 mm Hg (Vmax: 2.4 m/sec) at the RV
outflow and mean diastolic gradient of 3 mm Hg
K e y W o rd s: Myxoma, right heart ventricle,
tricuspid valve, ventricular outflow obstruction,
ventricular inflow obstruction.
INTRODUCTION
Right venticular (RV) myxomas are rare tumors, which
usually cause RV outflow tract (RVOT) and/or inflow
tract obstruction (1, 2). Herein, we describe another
such case demonstrated by two-dimentional (2-D)
echocardiography and magnetic resonance imaging
(MRI) of the heart. The patient was successfully
treated surgically, but replacement of the tricuspid
valve (TV) was inevitable due to tumor Invasion as
rarely reported previously (2 ).
( A c c e p t e d 3 0 O c to b e r , 1 9 9 9 )
M a r m a r a M e d ic a l J o u r n a l 2 0 0 0 ; 1 3 ( 1 ) : 3 3 - 3 5
33
H akan Tezcan, et al
F ig . 3
F ig . l
Transthoracic 2-D échocardiographie dem onstration
of the right ventricular (RV) mass, which prolapses
into the pulm onary artery (PA) during systole.
Parasternal short axis view. RA: right atrium , Ao:
aorta, RVOT: right ventricular outflow tract, PV:
pulm onary valve. TM: tum or mass
F ig . 4
F ig . 2
Transthoracic 2-D echocardiography of the tum or
m ass (TM) obstructing the inflow tract of the right
ventricular (RV) cavity and invading tricuspid valve
(TV). P arasternal long axis view. RA: right atrium .
(Vmax: 1.4 m/sec) at the TV. MRI study of the heart
was done to define the extent of the cardiac
involvement and to reveal any possible pulmonary
embolism. The tumor appeared to be attached to the
RV free wall and also to the tricuspid leaflets
obstructing the inflow and prolapsing into the
pulmonary artery (Figs.3 and 4). The lungs were clear
with no signs of infarction.
The patient underwent surgical exploration with a
presumptive diagnosis of a RV tumor. A mass, 16x8x6
cm in size and adherent to the anterior RV wall,
interventricular septum, papillary muscles and TV, was
resected (Fig. 5). The lobulated and friable tumor
34
T 1 -w eighted m agnetic resonance im age in axial plane
sh o w s rig h t v e n tric u la r c a v ity o b stru c tio n and
tricuspid valve infiltration of the tum or.
T1-w eighted m agnetic resonance im age in coronal
plane dem onstrates right ve n tricu la r tum or prolapse
into the pulm onary artery.
mass, which extended from the base of the TV to the
apex of the right ventricle, had a pedicle of 5x8 cm on
the anterior RV wall. Anterior and septal leaflets of the
TV was infiltrated by the tumor and it was not possible
to preserve them during the resection. The valve was
excised and replaced with a valve bioprosthesis
(Biocor, No. 31). The pathological diagnosis was
myxoma. The patient had an uneventful recovery and
had no problems throughout the 3 months of follow-up.
DISCUSSION
Myxomas constitute from 35 to 50 percent of all
cardiac neoplasms, of which 75 percent arise in the left
atrium and nearly all of the rest in the right atrium.
Myxoma of the right ventricle is extremely rare as is a
R ig h t v e n t r ic u la r m y x o m a
RV myxomas uncommonly originate in the TV (10) and
valve involvement of a myxoma requiring TV
replacement is extremely rare. To the best of our
knowledge, there are only three such cases reported
previously in medical literature (2, 11). Interestingly, in
a case report with an organized RV trombus attached
to the RV wall and the TV mimicking a RV myxoma,
the valve had to be replaced during surgical resection
(9).
The cardiac surgeon exploring a RV myxoma should
keep in mind that it might be necessary to replace the
TV during the resection of the tumor. 2-D
echocardiography and MRI may indicate this
possibility preoperatively.
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