J Cardiol 2001; 38: 93 – 97

Arrhythmogenic Right Ventricular
Dysplasia Presenting as Regression ofLeft Ventricular Dysfunction: A Case
ReportTaku INOUE, MD
Masahiro

TAMASHIRO, MD

Mitsuteru MATSUOKA, MD
Takafumi

MIYARA, MD

Koichiro

OKUMURA, MD

TakashiTOUMA, MD

Osahiko

SUNAGAWA, MD

Koshiro

FUKIYAMA, MD

Abstract ─────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────
Arrhythmogenic right ventricular dysplasia is considered to be a slowly progressive disease in which left
ventricular dysfunction and congestive heart failure usually appear at the end stage. The initial clinical presentation of this 56-year-old Japanese woman was left-sided heart failure, and the diagnosis was dilated
cardiomyopathy, but her left ventricular size and ejection fraction regressed during 10 years of treatment,
whereas her right ventricular parameters showed no change.
─────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────J Cardiol 2001 ; 38（2）: 93−97

Key Words■ Cardiomyopathies, other（arrhythmogenic right ventricular dysplasia）■ Heart failure■ Quality improvement（regression）

INTRODUCTION
Congestive heart failure（predominantly rightsided）and sustained ventricular tachycardia arecommon clinical manifestations associated witharrhythmogenic right ventricular dysplasia（ARVD）1）. Left-sided heart failure could alsooccur in the end-stage of the disease. We report acase of ARVD in a Japanese woman who was firsttreated under a diagnosis of congestive heart failure
with dilated cardiomyopathy.
CASE REPORTA 56-year-old Japanese woman who had beentreated for hypertension was admitted to a hospital

due to cerebral infarction in August 1989. Coronary
angiography and right and left ventriculography
were performed. The diagnosis was dilated cardiomyopathy. She was referred to our hospital complaining of exertional dyspnea and systemic edema
in May 1990. There was no increase in serum levelof creatinine kinase. Electrocardiography showednormal sinus rhythm with low voltage in all leads
and Q waves in leads Ⅲ and aⅤF. Epsilon waves
could be seen in leads Ⅴ1 and Ⅴ2 as at the previous. Left and right ventriculogadmission（Fig. 1−left）
raphy revealed marked dilation of both ventricles
with depressed ventricular ejection fraction（Fig. 2 :Right ventricular volume was calculated by themethods based on Simpson s rule2））. There were──────────────────────────────────────────────
琉球大学医学部 第三内科 : 〒 903−0215 沖縄県中頭郡西原町字上原 207
The Third Department of Internal Medicine, Faculty of Medicine, University of the Ryukyus, Okinawa
Address for correspondence: INOUE T, MD, The Third Department of Internal Medicine, Faculty of Medicine, University of theRyukyus, Uehara 207, Nishihara-cho, Nakagami-gun, Okinawa 903−0215
Manuscript received March 15, 2001 ; revised April 23, 2001 ; accepted April 25, 2001

93

94

Inoue, Tamashiro, Matsuoka et alFig. 1 Electrocardiograms recorded in 1990 and 1999
Left : In 1990, epsilon waves could be seen in leads Ⅴ1 and Ⅴ2.
Right : In 1999, epsilon waves became apparent in leads Ⅴ1 to Ⅴ4.

no coronary lesions or congenital defects.
Therefore, the diagnosis of dilated cardiomyopathy
was confirmed. Treatment with drugs such as
digoxin, diuretics, and angiotensin converting
enzyme inhibitor were continued. She was readmitted to our hospital in July 1999 with sustained ventricular tachycardia and left bundle branch block
configuration. After ventricular tachycardia wasconverted to sinus rhythm, an epsilon wave wasapparently detected in leads Ⅴ1 to Ⅴ（Fig.
1−4right）. Right ventriculography indicated marked
dilation of the right ventricle and depressed rightventricular ejection fraction that was almost thesame as that in 1990. Biopsy findings of the rightventricular septum showed fibrofatty replacementand degenerative changes or moderate fibrosis ofmyocytes（Fig. 3）. The diagnosis of ARVD wasbased on the diagnostic criteria suggested by

McKenna et al.3） During the 10 years since her
original treatment, the dilated left ventricle had
shrunk, and both cardiac index and left ventricularejection fraction had improved. However, the rightventricular size and ejection fraction wereunchanged（Table 1）.
DISCUSSION
Congestive heart failure（predominantly rightsided）and sustained ventricular tachycardia arecommon clinical conditions in patients withARVD 4）. About 18% of reported ARVD cases
include left ventricular involvement 4−13）, butpatients presenting with left-sided heart failure arerare. ARVD is considered a slowly progressive disease6,14）, with a natural course that can be schematically distinguished into four phases15）: Concealed
phase, overt electrical disorder, right ventricularJ Cardiol 2001; 38: 93 – 97

ARVD With Left Ventricular Regression

95

Fig. 2 Right and left ventriculograms in 1990
Left : Right ventriculogram（RVG）showing marked right ventricular dilation with decreased ejection fraction（right ventricular end-diastolic volume index 145 ml/m2, right ventricular end-systolic volume index
127 ml/m2, right ventricular ejection fraction 21% ; 30 °right anterior oblique view, end-diastolic phase）.
Right : Left ventriculogram（LVG）demonstrating marked left ventricular dilation with decreased ejection
fraction and cardiac index（left ventricular end-diastolic volume index 161 ml/m2, left ventricular end-systolic volume index 111 ml/m2, cardiac index 1.54 l/min/m2, left ventricular ejection fraction 31% ; 30 °rightanterior oblique view, end-diastolic phase）.

Fig. 3 Photomicrograph of the endomyocardial biopsy specimen from the rightventricular septum
Fibrofatty replacement and degeneration of
myocytes are present（hematoxylin-eosinstain, original magnification × 100）.

failure, and congestive heart failure. Congestive
heart failure usually appears during the end stage ofthe disease. Many studies have focused on the roleof ventricular arrhythmias, so there is little data
about the incidence and prognostic significance ofheart failure in ARVD. Five of six patients with iniJ Cardiol 2001; 38: 93 – 97

tial congestive heart failure subsequently died4）.
The cumulative survival rate of ARVD patients withcongestive heart failure was significantly lower
than that in patients with arrhythmias or no symptoms4）. Therefore, the association of congestive
heart failure with ARVD would be an adverse prog-

96

Inoue, Tamashiro, Matsuoka et al

Table 1 Serial changes of cardiac parameters detected by cardiac pool scintigraphy, echocardiography and cardiac catheterization
1990

1996

1998

1999

RVEF
（%）

11（−）

1313

LVEF（%）

12（−）

2741

RVDd（mm）

5048

4651

LVDd（mm）

51443840

LVDs（mm）

4638

4530

LVEF（%）

2738

48

51145

（−）

（−）

155

Cardiac pool scintigraphy

Echocardiography

Cardiac catheterization
RVEDVI（ml/m2）
RVESVI（ml/m ）

127

（−）

（−）

121

LVEDVI（ml/m2）

161

（−）

（−）

（−）

LVESVI（ml/m2）

111

（−）

（−）

（−）

CI（l/min/m2）

1.54（−）

（−）

1.84

2

RV＝right ventricular ; LV＝left ventricular ; EF＝ejection fraction ; Dd＝diastolic dimension ; Ds＝systolicdimension ; EDVI＝end-diastolic volume index ; ESVI＝end-systolic volume index ; CI＝cardiac index.

nostic sign.
Our patient had a unique clinical picture. The initial clinical presentation was left-sided heart failureand she was treated for dilated cardiomyopathy.
During the 10-year treatment period, the left ventricular size reduced, and both cardiac index andleft ventricular ejection fraction had improved
without concomitant right ventricular change（Table 1）. Hypertensive heart disease or myocarditis should also be considered as the cause of left
ventricular dysfunction and heart failure in thiscase. However, we have no evidence of apparent
left ventricular hypertrophy or elevation of cardiac
enzyme levels. In general, drugs such as diuretics
要or angiotensin converting enzyme inhibitor are useful for promoting preload and/or afterload reduction as part of the treatment for congestive heart
failure. In our patient, the left ventricular size could
be reduced with such treatment. However, patientswith ARVD who present with left-sided heart failure involving dilation of both ventricles anddepressed cardiac function do not usually benefit
from drug therapy.
A few cases have mimicked dilated cardiomyopathy like our case16,17）. All patients underwent
heart transplantation or died. Our patient is uniquein demonstrating regression of the dilated anddepressed left ventricle.
約

左心機能が改善した不整脈源性右室異形成症の 1 例
井 上卓

玉城正弘

松岡満照

宮良高史

奥村耕一郎

當 間隆

砂川長彦

柊山幸志郎

症例は 56 歳，女性．不整脈源性右室異形成症は緩徐進行性の心筋疾患で，右室の拡大と右室心筋の脂肪変性，心室頻拍を特徴とするが，終末像として左心室機能低下とうっ血性心不全をきたすこともある．本症例は左心不全で発症し，心機能や形態から拡張型心筋症と診断されて経過観察されていた．しかし，その後の 10 年の治療経過で，左心室のサイズは縮小し左室駆出率は改善したが，右心系には変化がなかった．

J Cardiol 2001; 38（2）: 93−97
J Cardiol 2001; 38: 93 – 97

ARVD With Left Ventricular Regression

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