Left ventricular dysplasia

involvement

in right ventricular

Right ventricular dysplasia, a heart muscle disease of unknown cause, anatomically characterized by variable replacement of myocardial muscle with adipose or fibroadlpose tissue, is usually considered a selective disorder of the right ventricle. However, concomitant left ventricular involvement has been noted in a few cases. The aim of this study was to evaluate the prevalence and characteristics of left ventricular Involvement In right ventricular dysplasia, as well as possible progression of the disease. Thirty-nine patients with right ventricular dysplasia,were studied by M-mode and two-dimensional echocardiography; 28 of them also underwent cardiac catheterization, and in 25 endomyocardial biopsy was performed. On first examination the left ventricle was normal in 25 patients, whereas in the remaining 14 right ventricular abhormalitles were associated with left ventricular involvement, characterized by asynergic areas (12 patients) or diffuse mild hypokinesis (two patients). During follow-up (27 patients, 84.1 + 66.1 months) 10 patients showed worsening of right ventricular function; in nine the appearance or worsening of left ventricular abnormalities was observed. Five patients died (four in congestive heart failure and one suddenly). Results of postmortem examination (available in two patients) showed atrophy of myocells and a massive fatty and fibrous infiltration of the right ventricular hall, associated with degenerative changes and fibrosis of the left ventricle. In conclusion, right ventricular dysplasia may be associated with left ventricular Involvement and the disorder appears to be progressive in some instances. (AM HEART J 1992;123:711.)

Bruno Pinamonti, MD, Gianfranco Sinagra, MD, Alessandro Salvi, MD, Andrea Di Lenarda, MD, Tullio Morgera, MD, Furio Silvestri, MD, Rossana Bussani, MD, and Fulvio Camerini, MD. Trieste, Italy

Right ventricular dysplasia is considered a heart muscle disease of unknown cause, which is characterized anatomically by a partial or extensive adipose or fibroadipose substitution of the right ventricular myocardium. 1*2 Although this condition is believed to be a selective disorder involving the right ventricle, there have been occasional reports of concomitant “minor” abnormalities of the left ventricle.3-7 Moreover, there are few data on the progression and natural history of this disease.8-10 The aim of this study was to evaluate the prevalence and characteristics of left ventricular involvement in right ventricular dysplasia, as well as possible progression of the disease. METHODS Patient selection. Right ventricular dysplasia was diagnosed when two-dimensional echocardiography and/or anFrom the Departments Maggiore and University.

of Cardiology

Received

for publication

Feb. 19, 1991;

Reprint Ospedale

requests: Maggiore,

4/I/34348

Bruno Piazza

Pinamonti, Ospedale

and

Morbid

accepted

Aug.

Anatomy,

Ospedale

14, 1991.

MD, Department of 1 34100, Trieste, Italy.

Cardiology,

giography demonstrated localized or diffuse structural and dynamic abnormalities involving exclusively or mainly the right ventricle in the absence of any other identifiable structural heart or pulmonary disease.‘O Right ventricular abnormalities included akinetic or dyskinetic areas, discrete wall bulges that persisted in systole and diastole, and/or global dilatation and hypokinesis.lO-l3 Moreover, patients with minor changes in left ventricular function (ejection fraction 45 % to 51% ) in the presence of akinetic or dyskinetic right ventricular bulges were also included. These changes in the right ventricle have been considered specific for right ventricular dysplasia.l’, 13-15All patients were evaluated clinically by means of standard ECG, chest x-ray examination, Holter monitoring, and M-mode and two-dimensional echocardiography. Twenty-eight patients underwent cardiac catheterization, and endomyocardial biopsy was performed in 25 of them (right ventricle in 19, left ventricle in three, and both ventricles in three). Echocardiography. All echocardiographic examinations were performed and interpreted by one of us (B. P.), who has had extensive training and experience in echocardiography. During the M-mode study standard measurements were performed. l6 The two-dimensional examination was performed from all standard approaches and sections.17 Additional nonstandard views were also obtained for better visualization of the right ventric1e.n’ The two 711

7 12

Pinamonti

et al.

Table

I. Clinical

and follow-up

Patient Age (yr) Sex Group

American

March 1992 Heart Journal

data

Clinical presentation

Physical findings

ECG

Arrhythmia morphology

Heart

size

N1-V4

LVBBB

N

2

O-ray)

-

Follow-up duration

Clinical course

1

l/39 F

SVT

N

T -

yr, 9 mo

2145

M

NSVT

N

T - N1-V4

LVBBB

N

13

yr, 3 mo

3136

F

NSVT

N

N

LVBBB

N

4 yr

4117

F

VEB

N

N

LVBBB

N

7

yr, 2 mo

5125

F

VEB

N

N

LVBBB

N

6

yr, 9 mo

6149 M

SVT

N

T - N1-Vs

LVBBB

N

13 yr,

7 mo

RN1 7140

M

SVT

N

T - /Ds-aVr

LVBBB

N

7

8136

F

SVT

N

N

LVBBB

N

10 yr,

NSVT

N

N

LVBBB

N

4 yr, 5 mo

SVT

N

T -

LVBBB

+

14 yr,

RAE QRSAF, RBBB, LAH cW, AF

LVBBB

+

1 mo

POLYM

+

23 yr

LVBBB

+

9 yr

0 POLYM

N N

5 5

LVBBB LVBBB LVBBB 0

r: N N

9112 M 10143

F

VI

D3, Vn,

avF,

yr, 5 mo 3

mo

9 mo

R + VI,

QVacW 11/5 mo F

HF

12137

M

HF

13/51

F

HF

Liver+

IVP MR, TR, OE IVP OE, Liver+ 2O SP,

ASYM VEB (PROP) ASYM-NSVT (AM10 + B) ASYM VEB (DISO) ASYM VEB ASYM VEB ASYM VEB (AMIO) ASYM VEB (AMIO) ASYM (AMIO) ASYM VEB HF, TR, (AMIO) PM Death (HF) PM Death (HF) SVT, PM Death (HF)

LIFT 14146 M 15/12 F

ABN ECG NSVT

N N

CW

16/33 17146 18/33 19/10

SVT VEB NSVT ABN ECG

40 40

T - /VI-V3 N N T - N1-V4 ST + N1-V4

M F F M

MR, LIFT N

T - N1-V4

yr, 10 mo yr, 9 mo

10 yr 2 yr, 9 mo

ASYM VF, HF death (sudden) VEB VEB

1 yr, 4 mo

ASYM

ABN, Abnormal; AF, atria1 fibrillation; AMIO, amiodarone; ASP REP, aspecific repolarization abnormalities; ASYM, asymptomatic; p, beta blocker; DISO, disopyramide; eW, epsilon waves in anterior precordial leads; HF, heart failure; IVP, increased venous pressure; LAH, left anterior hemiblock; LA!!‘, lateral wall; LBEE, left bundle branch block; LIFT, left parasternal systolic lift; MR, mitral regurgitation; MVR, mitral valve repair; N, normal; NSVT, nonsustained ventricular tachycardia; OE, peripheral edema; POLYM, polymorphous; PM, pacemaker; P MURM, systolic ejection murmur in pulmonary area; PROP, propafenone; QRS-, low QRS voltage in peripheral leads; RAE, right atrial enlargement; RBBB, right bundle branch block; ST+, ST segment elevation; SUPRAV T, supraventricular tachycardia; SVT, sustained ventricular tachycardia; T-, negative T waves; TR, tricuspid regurgitation: VEB, ventricular ectopic beats; VERAP, verapamil; +, increased; 0, absent; l”AVB, first-degree atrioventricular block; Z”SP, widely split second-degree heart sound; 3”. third heart sound; 4’, fourth heart sound.

ventricles were analyzed qualitatively and quantitatively. In the qualitative analysis the study focused on bulges of the ventricular walls and on wall motion abnormalities.” Regional wall motion was evaluated semiquantitatively by focusing on endocardial motion and myocardial thickening. In the quantitative analysis, left ventricular end-diastolic and end-systolic volumes and ejection fraction were calculated from the apical four-chamber view by means of an area-length single-plane method.lg Right ventricular dimensions and systolic function were evaluated from the same view, measuring the right ventricular end-diastolic

and end-systolic areas and fractional shortening of these areas.lg All measurements were obtained from the mean of three beats and were normalized for body surface area. Normal values were obtained from 41 normal subjects were studied in our laboratory. lg The limits of normality considered the mean -t two standard deviations of the normal values. After the first evaluation (including two-dimensional echocardiography), patients were divided into two groups: Group 1, right ventricular dysplasia with a normal left ventricle; and group 2, right ventricular dysplasia associ-

Volume Number

123 3

Table

LVabnorrnalities

in RV dysplasia

713

I. Cont’d

Patient Age (yr)

Sex

Clinical presentation

20138 F 21/29 M

SUPRAV MURM

22141 M

NSVT

Physical findings T

N 2SP, P MURM N

ECG T -

A71-V3

N Flat

Arrhythmia morphology

Heart size O-ray)

Follow-up duration

0 0

N N

1 yr, 8 mo

LVBBB

N

2 yr, 2 mo

LVBBB

+

7 Yr

Clinical course SUPRAV

T

ASYM, VEB SVT, HF,

AF

TA’4Vs

23/37

M

24151 M 25137 M Group 2 26146 M 27153 F

ABN ECG+ ABN x-ray ABN x-ray SUPRAV T

2”SP

T

- Nl-V.+,

CW

N N

N N

LVBBB 0

+ N

9 Yr

NSVT HF

N

T - /VI-V3 T flat LAT, T - V1-V3, LAH, QRST - /D3-aVF, T flat V5V7 N T flat V5V7, DzaVL 1” AVB, LBBB tW T flat V3V,5, T - v1v2; QRST flat VsV7, T - V1V4; QRSASP REP T - V3V5 T flat V4V6, QDeVF, QRSLAH

POLYM POLYM

N N

1 yr, 6 mo 4 yr

ASYM, VEB NSVT, HF, death

POLYM

N

3 yr,

POLYM POLYM

N +

4 yr 3 yr, 11 mo

POLYM

+

15 yr

ASYM, VEB (P) ASYM, VEB ASYM, VEB (AMIO) PM, MVR BEV (AMIO)

POLYM

+

POLYM

N

2 yr

NSVT 03)

LVBBB LVBBB POLYM

N N N

18 yr, 6 mo

LVBBB LVBBB

HF, SVT (AM10 + VERAP) SVT (AMIO)

G

POLYM

N

3“4’ LIFT OE, IVP, TR

28/33

M

SVT

LIFT

29/19 30/59

M F

VEB SVT

4”, OE

31/31

F

HF

MR

32/23

F

SVT

N

33/18

mo

NSVT

N

34148 M 35/21 M 36/19 M

SVT NSVT SVT

N N N

37/37 38/25

M M

SVT NSVT

N

39/48

M

ABN

N

ECG

3O4” LiFT, ;VP

LIFT, SP 4O

2”

T - A’l-V3 T - N,-V3, cW

ated with minor left ventricular abnormalities (presenceof localized left ventricular wall motion abnormalities and/or mild left ventricular dysfunction with ejection fraction 45% to 51%). Patients in both groups were followed clinically and by meansof serial two-dimensionalechocardiowpb. RESULTS

Right ventricular dysplasia was diagnosed in 39 patients. Their clinical, echocardiographic, and hemodynamic data are shown in Tables I to III. There were 23 men and 16 women. Their ages ranged from 5 months to 59 years (mean age 33.7 + 14.6 years). In three families more than one member was affected (patients 8,9,13,23,24,31,36, and 39). Two other pa-

11 mo

2 yr, 11 mo

tients had a relative (the father) with myocardial disease (dilated cardiomyopathy in patient 3 and myocarditis in patient 15). The majority of patients (26) were initiahy evaluated for complex ventricular arrhythmias in the absence of overt heart disease: ventricular tachycardia in 22 (sustained in 12, nonsustained in 10) and frequent ventricular ectopic beats in four. In an additional eight patients ventricular arrhythmias were also observed during Holter monitoring. In 22 patients the ventricular ectopic beats were monomorphic with a left bundle branch block pattern; they were polymorphic in the other 12. Two patients had paroxysmal supraventricular tachycardia. Five had congestive heart failure, predominantly on the right side. Six were asymptomatic but

7 14

Finamonti

et al.

Table

II. Echocardiographic

March 1992 Heart Journal

American

data

LVEDDI (cmlm2)

SF (%)

RVEDDI (cmlm2)

LVEDVI (ccIm2)

EF (%I

RVEDAI (cm2/m2)

CF (%I

2 3 4 5 6 I 8

3.1 2.8 2.7 3.2 2.8 2.6 2.7 3.4*

40 34 38 34 37 31 37 45

1.6 1 1.4 1.6 1 2* 1.7 1.5

33 40 N 51 36 26 36 81*

71 78 N 63 63 66 66 54

11 12 10 15* 12 20* 18* 22*

40 31 32 53 57 24* 37 39

9 10

3.2 2.5

42 34

0.9 1.6

N N

N N

20* INCR

38 D

0 0

AP B AP+LAT+ANTB

11 12

1.8 2.8

61 33

3.5* 3.3*

N 27

N 72

INCR 26*

D 26*

0 0

SEV DIFF SEV DIFF

HYPO HYPO

+ DIL + DIL

13

3.8*

27*

2.6*

48

69

25*

15*

0

SEV DIFF

HYPO

+ DIL

14 15

3 2.8

44 40

1.6 1.4

43 24

55 67

18* 12

26+ 41

0 0

AP HYPO AP HYPO

16 17 18 19 20 21 22 23 24 25

2 2.6 2.8 3.2 2.8 2.3 2.7 2.8 2.9 2.9

31 49 32 43 39 41 40 27* 35 33

2.9* 1.4 0.9 1.2 1.7 2.1* 1.5 2.6* 1 1.5

29 57 45 51 67* 37 33 34 N 93*

67 70 80 77 68 67 68 57 N 54

23* 15 N 19* 9 21* 12 20* N 20*

10* 46 N 24’ 37 47 54 24* N 23*

0 0

NVP 0 0 0 0 0 0 0

DIFF HYPO+ DIL + MULT APtANTB AP+LATB DIFF HYPO + DIL AP B AP+INFB ANT HYPO AP+LAT+INF+OUTB INF B ANT + LAT+ INF + AP B

26 27

2.8 3.1

27* 26*

1.8* 1.4

46 43

65 48*

17* 16*

28 18*

AP HYPO INF + AP HYPO

AP+INF+OUTB AP+INF+LAT+OUTB

28 29 30 31

2.5 3.4* 2.8 3.6*

21* 22* 25* 32

1.4 1.2 l.S* 1.2

59 73* 31 70*

56 57 57 45*

13 21* 14 19*

31 32 29 31

INF HYPO IVS HYPO POST HYPO AP + IVS HYPO

AP+INFB AP B APB AP+LAT+OUTB

32

2.8

28

1.2

48

52

14

29

33

2.8

28

0.7

32

47’

13

31

AP + POST + INF HYPO IVS B DIFF HYPO

LAT + INF +ANTB INF+APB

34 35 36 37

3.7* 3.1 3.4* 3.5*

29 27* 15s 23*

0.6 1.4 1.6 2*

63* 58* 62* 58*

51* 47* 49’ 45*

11 15* 17* 15*

58 20* 16* 16*

ANT B ANT + LAT + INF INF+APB ANT + INF + LAT

38 39

3.3 3.2

27* 22*

1.1 1.4

73* 82%

55 55

18* 14

33 33

IVS akinesis DIFF HYPO DIFF HYPO DIFF HYPO AP+ANTB INF HYPO POST HYPO

Patient Group

LV ABN

RV ABN

1

1

AP+INFB AP B AP+INFB AP B APB AP+INF+LATB AP B APB

D

Group 2

AP B

+ AP

ANT+LAT+APB ANT + INF + OUT

B B

B

ANT, Anterior wall; AP, apical; B, bulge; CF, right ventricular contraction fraction of area (normal values ~28%); D, decreased (semiquantitative data); DIFF, diffuse; DIL, dilatation; DYSK, dyskinesis; EF, left ventricular ejection fraction (normal values 2 52 %); HYPO, hypokinesis; INCR, increased (semiquantitative data); INF, inferior wall; IVS, interventricular septum; LAT, lateral wall; LV ABN, left ventricular abnormalities; LVEDDI, left ventricular end-diastolic diameter index (normal values I 3.3 cm/m2); LVEDVI, left ventricular end-diastolic volume index (normal values z 51 ml/m2); MULT, multinle: MVP. mitial valve urolawe: N, normal (semiquantitative data); NO CH, no changes; OUT, outffow; POST, posterior wall; RV ABN, right ventricular abnormalities; RVEDfl,‘right ventricular end-diastolic area index (normal values 5 14 cm2/m2); RVEDDI, right ventricular end-diastolic diameter index (normal values I 1.7 cm/m*); SEV, severe; SF, shortening fraction (normal values 2 28% ). *Abnormal data (see text for explanation). _I

_

Volume Number

123 3

LV abnormalities

Follow-up

echocardiographic

LVEDDI (cm/m2)

Two-dimensional SF (%)

RVEDDI (cmlm2)

LVEDVI (cclmd

EF

(%)

RVEDAI (cm2/mz)

CF (%I

LV

ABN

33 76 46 86 81 96 77 84

3.4* 2.6 2.6 3.2 2.7 2.3 2.7 2.7

41 47 33 35 31 48 43 33

0.6 1.2 1.4 1.3 0.9 2.1 1.7 0.8

25 51 49 55 56 39 31 56

62 82 57 66 57 75 74 62

10 17* 11 15* 12 19* 17* 17*

43 23* 50 22* 39 24* 17* 24

0 0 0 0 0 AP DYSK 0 0

51 44

2.6 2

33 38

1.2 2.7*

50 ?

50* ?

14 20*

35 8*

0 0

29

3.4*

15*

3.4*

45

38*

26*

29

41

3.4*

28

2.9*

40

40*

28*

lo*

70 51

2.8 2.7

40 15’

1.5 2.7*

34 25

59 30*

19* 27*

24 14*

33

2.7

46

0.9

53

69

15*

16 20

2.7 2.9

39 38

1.1 2.0*

50 54

68 71

26 78 36

2.6 2.6 2.8

36 30 21*

1.2 2.5” 1.1

40 44 87*

19 20

2.8 3.1

32 12*

1.4 2.3*

47 37 45 78

2.7 3.5 2.7 3.7*

28* 22* 33 25*

1.3 0.9 1.9* 1.2

24

80 11

3.2

3.4 3.4

715

examination

M-mode Follow-up MO.

in RV dysplasia

24*

14* 16

1.3

1.4 1.3

RVABN

AP+INF+ANTB AP+ANTB NO CH AP+INF+LATB NO CH NO CH AP+INF+LATB AP+LAT+ANT+ INF B NO CH AP+LAT+ANT+ INF + OUT

B

POST HYPO IVS DYSK IVS + AP HYPO 0 IVS B DIFF HYPO

NO CH DIFF HYPO + DIL; AP+INF+LATB

34

0

NO CH

17* 13

25* 27*

0 0

NO CH AP+INFB

62 65 32*

14 21* 17*

29* 11* 12*

0 0 IVS HYPO

ANT + INF NO CH ANT + INF

57* 48

53 40*

17* 19*

28 19*

NO NO

58 74 56 79*

57 49* 61 48*

12 16* 15 14*

36 27* 36 17*

NO CH INF HYPO + AP AK + THR NO CH NO CH NO CH NO CH

100*

95* 71*

49*

36* 37*

had abnormalities that were seen on physical examination, ECG, or chest x-ray. Echocardiography (Table II). At the time of the first echocardiogram, 25 patients were classified intogroup

16

16* 18*

23*

6” 25*

DIFF

HYPO

NO CH NO CH

NO

CH

NO CH

B B

CH CH

NO CH NO CH NO CH AP+LAT+ INF + OUT

ANT+AP+ INF OUT

B

B

NO CH NO CH

1. By definition the left ventricle was normal in all of them. In 15 patients the right ventricle showed only localized changes. In the other 10 fractional shortening of areas of the right ventricle was decreased and

7 16

Pinamonti

et al.

Table

III. Hemodynamic CI

data

(Llmlm2)

(mm Hg)

RVEDP (mm Hg)

3 4.3 2.6

10 6 12

5 2 10*

121* 56 55

63 65 81

0 0 0

AP+INFB AP HYPO ANT + LAT

7

3.3

12

lo*

69

62

0

AP+INFB

8 10 11

4.9 2* -

6 8

5 6 9*

96 94 -

56 80 -

0 0 0

ANT -

Patient Group 1 3 6

March 1992 Heart Journal

American

LVEDP

LVEDVI

(mllm2)

EF

(%o)

LV ABN

RV ABN

R VEMB

L VEMB

I

-

12

1.8*

13

2.4*

13*

I

110

67

14

2.3*

7

6

74

67

15

4

10

3

82

58

16

2.2*

4

-

77

63

17 18 20 22

3.5 3.4 5.2 4.0

4 4 7 5

3 1 3 -

36 85 77 17

81 63 15 59

11

4

10 0

55

101*

47*

23 Cardiac catheterization 1.6 10 Followup 14 yr

-

performed 18*

during

MILD MOD

MR MR 0

INF

D 0 0

MVP 0 0 0

+ INF

B

FAT + FIBR t FAT + FIBR FIBR + INFILTR i FIBR + HPT

DIFF HYPO + DIL DIFF HYPO + DIL DIFF HYPO

HPT + FIBR + FAT + INFILTR FIBR + INFILTR

DIFF HYPO + DIL DIFF HYPO + DIL AP B AP B AP HYPO DIFF HYPO + DIL AP+INF+ OUTF B

+

FIBR + FAT Normal k HPT FIBR i: FAT + FIBR + HPT

follow-up 21*

AP HYPO, ANT B, MR3+ AP HYPO, MOD MR

DIFF HYPO+ DIL, OUT TR3 DIFF HYPO

FIBR, OE, f HPT

B,

10

lo*

256*

49*

4.3

20*

lo*

124*

43*

DIFF HYPO, IVS B

DIFF

HYPO

FIBR + FAT+ INFILTR+ HPT FIBR + FAT

1.7*

17s

3

53

47*

OUT

B

FAT

28

3.6

14*

3

97

57

30

2.1

20*

5

138*

48*

31

5.0

20*

10*

104

51*

32

2.9

15*

12*

96

53

33

4.4

14*

4

90

51*

34 35

6.0 5.7

7 9

-

adequate 71

63

36

4.0

14*

-

154*

35*

37

4.5

10

3

83

24*

38

4.4

15*

4

103

57

AP + INF HYPO AP + INF HYPO POST HYPO; 21NFD AP HYPO+ MOD MR INF aneurysm AP + ANT HYPO Not adequate AP HYPO + INF D ANT + INF D DIFF HYPO+ APB 0

24 FollowUP 9 yr Group 2 21

k FIBR + HPT

B

3.1

13 FollowUP 8 Y=

FIBR

6

Not

+ FIBR

AP+INFB

k FIBR

DIL

FAT++

+ HYPO +APB -

FAT

AP+INFB

FAT

+ FIBR

AP+INFB

FIBR,

HPT

AP HYPO AP B

FAT, kFIBR t- FIBR

DIFF

FIBR, HPT, + FAT *FAT, + FIBR

INFB+ DIFF AP+INFB

HYPO

HYPO

+ FIBR

+FIBR

CI, Cardiac index; D, diverticulum; HPT, hypertrophy; INFILTR, infiltration; LVEDP, left ventricular end-diastolic pressure; MOD, moderate; right ventricular end-diastolic pressure; 0, absent; -, not measured/not performed; RVEMB, right ventricular endocardial muscle biopsy; LVEMB, tricular endocardial muscle biopsy; FAT, fatty infiltration; FIBR, fibrosis; other abbreviations as in Table II. *Abnormal data.

RVEDP,

left ven-

Volume Number

123 3

the cavity was dilated, often in association with multiple bulges of the walls. The remaining 14 patients (group 2) were characterized by involvement of both ventricles; in 10 patients the right ventricle showed localized wall bulges, and in the other four multiple wall bulges were associated with global right ventricular dilatation and depressed systolic function. The left ventricle demonstrated localized wall motion abnormalities only in eight patients (Fig. l), localized changes in association with a slightly depressed ejection fraction in four (Fig. 2), and a moderate diffuse hypokinesis in the remaining two. In no patient was the left ventricular cavity significantly enlarged (maximal end-diastolic volume index 82 cc/mg). Cardiac catheterization (Table III). Twenty-eight patients (17 in group 1 and 11 in group 2) underwent cardiac catheterization, including left ventricular angiography in all and coronary angiography in all except the very young (six patients). The coronary arteries were normal in all of them. Right ventricular angiography was performed in 25 patients, and findings were considered abnormal in all of them. They all had one or more akinetic or dyskinetic areas or discrete bulges and/or diffuse hypokinesia (Table III). Two patients had tricuspid regurgitation, which was mild in one and moderate in the other. Right ventricular end-diastolic pressure was elevated (>8 mm Hg) in six patients. None of the patients in group 1 showed any abnormalities on left ventricular angiography. Left ventricular end-diastolic pressure was slightly elevated (13 mm Hg) in only one patient (No. 13). Two patients had mild mitral regurgitation, and one had mitral valve prolapse. Nine of the 11 patients in group 2 who underwent catheterization showed some abnormalities on left ventricular angiography; localized wall motion abnormalities were present in eight and mild-to-moderate depression of the ejection fraction in six. In one patient (No. 38) the left ventricle was judged normal, and in another patient the angiogram was inadequate because of the presence of ventricular tachycardia. Left ventricular end-diastolic pressure was elevated (>12 mm Hg) in eight patients, and one patient had moderate mitral regurgitation. In addition, three patients (Nos. 15, 30, and 35) had one or two small diverticula of the diaphragmatic wall of the left ventricle (Fig. 3). Endomyocardial biopsy. Percutaneous endomyocardial biopsy was performed in 25 patients-from the right ventricle in 19, from the left in three, and from both ventricles in three (Table III). Right ventricular samples showed the presence of moderate-to-severe fatty infiltration in 11 patients, which was associated with fibrosis in six of them. Isolated interstitial fibrosis was present in the other five. In addition,

LV abnormalities in RV dysplasia

717

Fig. 1. A, Patient No. 32. Two-dimensional echocardiogram, apical four-chamber view, systolic frame. Right ventricle (RV) is dilated and diffusely hypokinetic. Akinetic bulge (B) is present at lateral wall. In addition, small akinetic bulge is seen at level of apical third of interventricular septum. LV, Left ventricle. B, Parasternal long-axis view during systole from same patient as in 1A. Basal third of left ventricular posterior wall (PW) is markedly hypokinetic (HYPO). IVS, Interventricular septum; A, aorta; LA, left atrium; other abbreviations as in A.

mild-to-moderate inflammatory infiltration was observed in four patients. In the remaining patients nonspecific changes were present, and in one the biopsy results were considered normal. Left ventricular samples showed the presence of interstitial fibrosis in all patients (mild in two), associated with hypertrophy and attenuation of myocells. Follow-up. Thirty-one patients had clinical follow-up for periods ranging from 1 month to 23 years (mean 84.1 + 66.1 years) (Table I). Serial two-di-

March

7 18

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et al.

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Heart

1992 Journal

Fig. 2. A, Patient No. 36.M-mode echocardiogramat ventricular level. Left ventricle is mildly dilated (left ventricular end-diastolic diameter index = 3.4 cm/m2) and septum and posterior wall are hypokinetic; shortening fraction is 15% . B, Two-dimensional echocardiogram,magnified apical four-chamber view, of samepatient as in A. Akinetic rounded bulge (B) is seenat left ventricular apex. C, Modified subcostal four-chamber view during systole from samepatient asin A and B. Two akinetic bulges(B) are present at level of basalthird of right ventricular diaphragmatic wall. RV, Right ventricle; other abbreviations as in A.

mensional echocardiographic examinations were done in 30 patients (follow-up ranging from 11 to 96 months, mean 49.5 +- ‘25.4months) (Table II). Three patients underwent cardiac catheterization during the follow-up period (Table III). Group 1. Twenty-two patients in group 1 were fol-

lowed-up. New abnormal right ventricular areas were seen in 10 patients; in 7 of the 10 this was associated with enlargement of the cavity and a decrease in global systolic function. Six patients had left ventricular involvement (associated with worsening of right ventricular function in three). In four paradoxical

Volume Number

123 3

LV abnormulities

in RV dysplasia

719

Fig. 3. Patient No. 35. Left ventricular angiogram, right anterior oblique projection, systolic frame. Small dyskinetic diverticulum is present at level of inferior wall (arrow).

septal motion with decreased systolic thickening and diffuse hypokinesis of the left ventricular free wall without chamber dilatation developed slowly during follow-up (Fig. 4). In patient 6 a small area of dyskinesis appeared at the left ventricular apex with normal contraction of the remaining parts of the left ventricle. The remaining patient (No. lo), who initially had ventricular tachycardia, had progressive (12 years) development of clinical signs of right ventricular failure refractory to medical treatment; she is now awaiting heart transplantation. In this patient the left ventricle was not adequately visualized by two-dimensional echocardiography during followup. Cardiac catheterization was repeated and results of left ventricular angiography, considered normal 14 years earlier, showed apical hypokinesis, a small dyskinetic bulge at the level of the anterolateral segment, and mild depression of the ejection fraction (47%). Moderate mitral regurgitation was also present. The right ventricle was massively dilated and severely hypokinetic. In addition, there was an aneurysm of the outflow tract. Severe tricuspid regurgitation was present. The diastolic pressures in the right atrium, right ventricle, and pulmonary artery were similar. Four patients died during follow-up. One of them, a &month-old infant, died in low cardiac output 1 month after clinical presentation. Two other patients died after a long period of chronic right-sided heart

failure. One remaining patient (No. 15) died suddenly at the age of 18 years. Postmortem examination was performed in two patients (Nos. 13 and 15). In patient 13 massive fatty infiltration of the right ventricular free wall was evident, associated with fibrosis and a marked decrease in contractile elements with fibrosis was also (Fig. 5, A). Fatty infiltration present at the left ventricular apex (Fig. 5, B), which appeared hypokinetic at the last echocardiographic and angiographic study. Interstitial and subendocardial fibrosis, hypertrophy, and attenuation of myocytes were observed in the remaining left ventricular walls. In patient 15 the outer half of the right ventricular wall was totally replaced by adipose tissue. In the inner half only extremely atrophic myocells were present, and they appeared to be immersed in fibrous tissue. The endocardium was thickened. In the left ventricle the predominant feature was atrophy of myocytes of the inner third, whereas the myocells were only slightly attenuated in the remainder of the wall,< The subendocardial region was fibrotic. Group 2..,Follow-up data were available from nine patients in .group 2. In two of them new abnormal right ventricular areas appeared, and in three worsening of right ventricular function was observed without clinical signs of cardiac failure. Another patient was readmitted to the hospital for severe heart failure and low cardiac output. Worsening of left ventricular function (ejection fraction 48 to 40% )

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Fig. 4. A, Patient No. 15. Three subsequent M-mode tracings at ventricular level. In 1984 M-mode tracing was normal. Hypokinesis at right ventricular apex was observed on two-dimensional examination. One year later, right ventricle was dilated and interventricular septum showed abnormal motion and less thickening; shortening fraction was mildly depressed. At the third examination (1988), left ventricular function further worsened and there was new severe hypokinesis of posterior wall. No significant left ventricular dilatation was observed. Percentages inside left ventricle indicate shortening fraction of left ventricle. B, Two-dimensional echocardiogram obtained in 1988 from same patient as in A. Modified apical four-chamber view. Right ventricle (RV) is markedly dilated, diffusely hypokinetic, and shows multiple bulges in walls. C, Two-dimensional echocardiogram obtained in 1988 from same patient as in A and B. Parasternal longaxis view, systolic frame. Akinetic bulge (B) is present at septal level. Dilatation of right ventricle (RV) is also evident.

chamber enlargement and formation of a thrombus at the left ventricular apex were observed. The patient died in congestive heart failure a few months later. No autopsy findings were available. Worsening of left ventricular function was also observed in another three patients. In one (patient 36), whose mother and brother had typical right ventricinvolvement at ular dysplasia with left ventricular

without

postmortem

examination,

clinical

signs of heart

fail-

ure appeared, which required digitalis and diuretics. DISCUSSION

In the past decade several investigators have used different terms to describe some patients with “idiopathic” right ventricular disease, such as right ventricular

dysplasia,’

right

ventricular

(dilated)

cardi-

Volume Number

123 3

LV abnormalities in RV dysplasia

72 1

5. Patient No. 13. Postmortem study. A, Histologic specimenfrom right ventricular free wall. Severe fatty infiltration and subendocardialfibrosis are present. Few contractile elements are seen, and they are usually within trabeculae. (Trichromic stain. Original magnification ~4.5.) B, Severe fibrosis and fatty infiltration are present at left ventricular apex. (Trichromic stain. Original magnification x40.)

Fig.

omyopathy,20 parchment heart,3 and lipomatous infiltration of the right ventricle.21 Although there is not yet complete agreement in the literature, it is presently thought that these entities do not all represent separate disorders but rather different expressions of the same disease.22 Considering that the cause of this disease is still unknown, some investigators have proposed that it be considered a cardiomyopathy.2, 23 In this report we have adopted the term “right ventricular dysplasia” because it is the most widely used, although it is probably not correct from an etymologic standpoint (dysplasia, from the greek dysplassein, means abnormality of development). In this disease the pathologic changes in the right ventricle

are usually characterized by marked atrophy of myocardial fibers, which are frequently replaced by adipose or fibroadipose tissue.lv 2, 23 In some instances the pathologic changes are widespread and the right ventricle is globally dilated and hypokinetic, whereas in others only localized abnormalities are present, usually in the form of asynergic areas, bulges, or aneurysms, which can be recognized on echocardiography or angiography.i, 3, 11-13,2o Ventricular tachycardia, characteristically with a left bundle branch block pattern, is the predominant clinical manifestation in most reported cases; thus the disease is also known as “arrhythmogenic” right ventricular dysplasia.’ However, other casesreported in the literature had “signs and symptoms other than

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Table

IV. Left ventricular

American

involvement

March 1992 Heart Journal

in right ventricular cardiomyopathy: Data from the literature

No. of Reference

Sega112* (1950) Arcilla and Gasulz6 (1961) Viola et a1.33(1970)

1

Waller et a1.3 (1980)

2

Halphen et al.4 (1981)

Fitchett et aLzo (1984)

end-diastolic

TLVEDP

(14 mm Hg)

Histologic: Myocardial to RV)

tLVEDP

(15 mm Hg); ANGIO:DIL

1

degeneration

(similar

+ HYPO Gross + HISTOL.: Massive fatty infiltration Gross: Paper-thin IV Septum; Histologic: Focal Fibrosis, Fatty infiltration, myocardial degeneration

ECHO: POST wall HYPO ANGIO: DIL; apex aneurysm ANGIO: POST wall AK (case 2)

Gross: Apex aneurysm (case 1); POST wall thinned (case 2) Histologic: Fibrosis + fatty infiltration + few myocardial fibers (case 1)

ANGIO: DIL (EDV 263 cc); INF + ANT + LAT HYPO-Bulge tCWP’(18 mm Hg) ECHO: INF HYPO ANGIO: Small apex aneurysm RNA: LV DIL, 1EF, septal HYPO (2 cases) =/J EF during effort (4 cases) ANGIO: tLVEDP (14-25 mm Hg) (6 cases); MILD DIL and/or mild DIFF HYPO

Hoback et a1.27 (1981) Marcus et al.l (1982) Baran et al.ls (1982) Manyari et a1.15(1983)

tricular

Gross: All 4 chambers “parchment like” Gross: DIL, HPT, endocardial thickening

AV block

Cherrier et aL30 (1979)

Higuchi et a1.s (1984)

Pathologic findings

tCWP; ANGIO: DIL

French et a1.2s (1975) Letac et a1.2s (1976) Bharati et aLz5 (1978)

AEN, Abnormalities; DIL, left ventricular

Clinical findings

cases

Histologic: Focal fibrosis ANT LAT Wall

Gross: “Gross ABN” (2 cases); moderate DIL (1 case) Histologic: FIB (2 cases); HPT + inflammatory (1 case)

infiltration

RNA: During follow-up: LVEF 51%->41%->24%

AK, akinesis; Angie, left ventricular angiography; ANT, anterior; dilatation; FIB, fibrosis; HPT, hypertrophy; HYPO, hypokinesis; pressure; NMR, nuclear magnetic resonance; POST, posterior;

ventricular tachycardia, including cardiomegaly or right ventricular failure of unknown etiology.“l This spectrum of clinical evidence was also observed in our series (Table I). Initially right ventricular dysplasia was considered a diseasestrictly and exclusively localized to the right ventricle, although isolated reports suggested that the left ventricle may be involved occasionally (Table IV). The left ventricular abnormalities associated with right ventricular dysplasia are usually characterized by the presence of asynergic areas, most frequently located at the apex or the inferoposterior wall 1l3,4,6 I13I31I8725,3oor by diffuse hypokinesia and dilaiation.4, I3 They may be detected more frequently by radionuclide studies during exercise.5 In some instances results of histologic studies of the left ventricle, available in a few cases (Table IV), showed abnormalities similar to those found in the affected right ventricle, L 3*31 whereas in others predominant fibrosis was present. 2r6 20,25,32 Results of our study demonstrate that in patients with typical right ven-

AV, atrioventricular; CWP, capillary INF, inferior; IV, interventricular; RNA, radionuclide angiography.

wedge

LAT,

pressure; DIFF, diffuse; lateral; LVEDP, left ven-

tricular dysplasia, left ventricular involvement is not unusual. Six patients who at their first evaluation had typical right ventricular abnormalities and a normal left ventricle had progressive left ventricular involvement characterized by diffuse hypokinesis without dilatation (five patients) (Fig. 4) or by localized asynergy (one patient). It is difficult to classify the group of patients (group 2) with typical morphologic right ventricular abnormalities, who had slight left ventricular involvement at the initial examination. In some the left ventricular changes were localized and minor, but in others the left ventricle showed evidence of wall motion abnormalities, a slight decrease in the ejection fraction, or both. In some of these latter patients, as observed by other investigators,4, l3 the differentiation between right ventricular dysplasia with left ventricular involvement and dilated cardiomyopathy may be difficult. Some additional observations may be made. In some instances a peculiar abnormality was observed

Volume Number

Table

123 3

LVabnormulities

723

in RV dysplasia

IV. Cont’d No. of Reference

cases

Purcaro et al6 (1984)

1

Ibsen et a1.2s (1985)

2

Webb et al7 (1986)

4

Yutani et a1.32(1987)

1

Hasumi et al.“‘j (1987) Blomstrom-Lindqvist et a1.g (1987) Casolo et a13* (1987) Blomstrom-Lundqvist et a1.3” (1988) Daubert et a1.13 (1988)

6

Nava et a1.s7 (1988)

17

Thiene et a1.2 (1988)

4

Blomstrom-Lundqvist et al.” (1988)

5

Clinical

findings

Pathologic

findings

ECHO and ANGIO: Apex aneurysm; ~LVEDP (15 mm Hg) ANGIO: Normal volume but /EF (1 case)

Histologic: FIB + HPT + myocardial degeneration + endocardial thickening Histologic: Similar ABN but less than RV (1 case)

ANGIO: -JEF (2 cases): 36% in one; INF DYSK (1 case) RNA: =/j EF during effort (1 case) ECHO: DIFF HPT; during follow-up: EF 50% (ANGIO) -22% (RNA) ANGIO: DIL (1 case) JEF: 45%) 38% (2 cases) ECHO: During follow-up “LV ABN” (1 case)

Gross: Moderate DIL Histologic: FIB + myocardial disarray (similar to RV?)

NMR: Increased signal from LV (fatty infiltration ?) ANGIO: Mild-moderate DIL (3 cases); JEF (43 70) (1 case); Wall notion ABN (4 cases) ANGIO: DIL (3 cases): In 1 case EDV = 222 cc; DIFF HYPO (2 cases); Wall motion ABN (4 cases) ANGIO and/or ECHO: Wall notion ABN and/or mild DIL

Gross: “LV involvement at operation or necropsy” (3 cases)

Histologic: Fibrosis (6 cases) Histologic: Focal apical fatty infiltration cases), scattered inflammatory infiltration + patchy FIB (2 cases)

(2

ECHO: Wall motion ABN at apex or POST wall

on left ventricular angiography, characterized by the presence of one or more discrete small diverticula of the left ventricular wall (Fig. 3). It is not certain whether this finding is incidental or it corresponds to a localized muscle defect, possibly similar to that in the abnormal zones of the right ventricle. In most patients with right ventricular dysplasia and left ventricular involvement the ventricular ectopic beats were polymorphic, whereas most patients with a normal left ventricle had a typical left bundle block pattern of ectopic beats (Table I). Similar observations were reported by others.7-g,38 Progression of the diseasehas not been studied extensively. Higuchi et a1.8reported one case of right ventricular dysplasia initially characterized by ventricular tachycardia with a left bundle branch block pattern and isolated right ventricular abnormalities. During a follow-up period of 17 years, right ventricular function deteriorated followed by the appearance of progressive left ventricular dysfunction. Ventricular tachycardia recurred with both left and right bundle branch block morphology. Blomstrom-Lundqvist et a1.,gin a follow-up study of 15 patients with

right ventricular dysplasia, observed signs of structural progression of the disease in a significant number of cases. Three patients had insidious development of severe right congestive heart failure and four had new abnormal physical findings. In six patients cardiac enlargement was seen on chest x-ray, whereas the majority had an increase in ECG abnormalities. Nava et al.1° found that two members of a family with right ventricular dysplasia had no abnormalities when they were first examined at 11 years of age but had overt disease 4 years later. Progression of the disease was observed in more than one third of our patients: in 10 (37%) right ventricular function deteriorated, and in 10 (37%) left ventricular involvement appeared or progressed. Progression of the diseasein both ventricles was observed twice (Fig. 4). Of the five patients who died, four showed the appearance or worsening of signs of left ventricular involvement on follow-up (echocardiographic and/or postmortem examination). In conclusion, right ventricular dysplasia includes a spectrum of abnormalities that are localized in some instances and widespread in others. Left ven-

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tricular involvement may be present in a substantial percentage of patients in the form of localized wall motion abnormalities, mild depression of left ventricular function, or both. In some cases the disease appears to be progressive, eventually causing widespread involvement of both ventricles. Advanced phases of the disease may be difficult to distinguish from dilated cardiomyopathy. REFERENCES

1. Marcus FI, Fontaine GH, Guiraudon G, Frank R, Laurenceau JL, Malergue C, Grosgogeat Y. Right ventricular dysplasia: a report of 24 adult cases. Circulation 1982;65:384-98. 2. Thiene G, Nava A, Corrado D, Rossi L, Pennelli N. Right ventricular cardiomyopathy and sudden death in young people. N Engl J Med 1988;318:129-33. 3. Waller BF, Smith ER, Blackbourne BD. Arce FP. Sarkar NN. Roberts WC. Congen&l hypoplasia of portions bf both right and left ventricular myocardial walls. Clinical and necropsy observations in two patients with parchment heart syndrome. Am J Cardiol 1980;46:885-91. 4. Halphen C, Beaufils P, Azancot I, Baudouy P, Manne B, Slama R. Tachycardies ventriculaires r&idivantes par dysplasie ventriculaire droite. Association B des anomalies du ventricule gauche. Arch Mal Coeur 1981;‘74:1113-8. 5. Manyaride, Klein GJ, Gulamhusein S, Boughner D, Guiraudon GM, Wyse G, Mitchell LB, Kostuk WJ. Arrhythmogenic right ventricular dysplasia: a generalized cardiomyopathy? Circulation 1983;68:251-7. 6. Purcaro A, Capestro F, Ciampani N, Breccia Fratadocchi G, Belardinelli R, Murer B, Schicchi F, Massini C. La displasia ventricolare destra aritmogena. A proposito di 3 osservazioni. G Ital Cardiol 1984;14/II:1087-96. 7. Webb JG, Kerr CR, Huckell VF, Mizgala HF, Ricci DR. Left ventricular abnormalities in arrhythmogenic right ventricular dysplasia. Am J Cardiol 1986;58:568-70. 8. Higuchi S, Caglar NM, Shimada R, Yamada A, Takeshita A, Nakamura M. Is-year follow-up of arrhythmogenic right ventricular dysplasia. AM HEART J 1984;108:1363-5. 9. Blomstrom-Lundqvist C, Sabel C-G, Olsson SB. A long term follow-up of 15 patients with arrhythmogenic right ventricular dysplasia. B; Heart J 1987;58:477-88: 10. Nava A. Thiene G. Canciani B. Scoenamielio R. Daliento L. Buja G,‘Martini B; Stritoni P, ‘Fasoc G. f&nil&l occurrence of right ventricular dysplasia: a study involving nine families. J Am Co11 Cardiol 1988,12:1222-8. 11. Blomstrom-Lundqvist C, Beckman-Suurkula M, Wallentin I, Jonsson R, Olsson SB. Ventricular dimension and wall motion assessed by echocardiography in patients with anhythmogenic right ventricular dysplasia. Eur Heart J 1988;9:1291-1302. 12. Robertson JH, Bardy GH, German LD, Gallagher JJ, Kisslo J. Comparison of two-dimensional echocardiographic and angioaraphic right ventricular dvs- - _ findings in arrhythmogenic plasia. Am J Car&o1 1985;&1506-8. 13. Daubert C. Descaves C. Fouleoc J-L. Bourdonnec C. Laurent M, Gouffablt J. Critical arm&is of cineangiographic criteria for diagnosis of arrhythmogenic right ventricular dysplasia. AM HEART J 1988;115:448-59. 14. Conte MR, Presbitero P, Gaita F, Tanga M, Massobrio N, Orzan F, Brusca A. L’aspetto angiogra&o della displasia aritmogena de1 ventricolo destro. G Ital Cardiol 1989:19:580-4. 15 Daliento L, Rizzoli G, Thiene G, Nava A, Rinuncini M, Chioin R, Dalla Volta S. Diagnostic accuracy of right ventriculography in arrhythmogenic right ventricular cardiomyopathy. Am J Cardiol 1990;66:741-5. 16. Sahn DJ, De Maria A, Kisslo J, Weyman A. The Committee on M-Mode Standardization of the American Society of Echocardiography recommendation regarding quantitation in

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22. 23. 24. 25.

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March 1992 Heart Journsl

M-mode echocardiographic measurements. Circulation 1978; 58:1072-83. Tajik AJ, Seward JB, Hagler DJ, Mair DD, Lie JT. Twodimensional real time ultrasonic imaging of the heart and great vessels. Mayo Clin Proc 1978;53:271-303. Baran A, Nanda NC, Falkoff M, Barold SS, Gallagher JJ. Two-dimensional echocardiographic detection of arrhythmogenic right ventricular dysplasia. AM HEART J 1982; 103:1066-7. Pinamonti B, Alberti E, Cigalotto A, Dreas L, Salvi A, Silvestri F, Camerini F. Echocardiographic findings in myocarditis. Am J Cardiol 1988;62:285-91. Fitchett DH, Sugrue DD, McArthur CG, Oakley CM. Right ventricular dilated cardiomyopathy. Br Heart J 1984;51:25-9. Voigt J, Agdal N. Lipomatous infiltration of the heart. An uncommon cause of sudden, unexpected death in a young man. Arch Path01 Lab Med 1982;106:497-8. Nava A, Thiene G. Displasia o cardiomiopatia aritmogena? G Ital Cardiol 1990,20:562-3. Maron BJ. Right ventricular cardiomyopathy: another cause of sudden death in the young. N Engl J Med i988,318:178-80. Segall HN. Parchment heart (Osler). AM HEART J 1950; 40:948-50. Bharati S, Ciraulo DA, Bilitch M, Rosen KM, Lev M. Inexcitable rieht ventricle and bilateral bundle branch block in Uhl’s disease. Circulation 1978;57:636-44. Arcilla RA, Gasul BM. Congenital aplasia or marked hypoplasia of the myocardium of the right ventricle (Uhl’s anomaly). J Pediatr 1961;58:381-8. Hoback J, Adicoff A, From AHL, Smith MK, Shafer R, Chesler E. A report of Uhl’s disease in identical adult twins. Evaluation of right ventricular dysfunction with echocardiography and nuclear angiography. Chest 1981;79:306-10. Ibsen HHW, Baandrup U, Simonsen EE. Familial right ventricular dilated cardiomyopathy. Br Heart J 1985;547156-9. French JW. Baum D. PODD RL. Echocardiopranhic findines in Uhl’s anomaly. Am j Cidiol 1975;36:349-g3. * Cherrier F, Floquet J, Cuilliere M, Neimann JL. Les dysplasies ventriculaires droites. A propos de 7 observations. Arch Ma1 Coeur 1979;72:766-73. Letac B, Tayot J, Barthes P. Infiltration graisseuse du coeur et maladie de Uhl. A propos d’une observation de lipomatose cardiaque. Arch Ma1 Coeur 1977;70:107-13. Yutani C, Imakita M, Ishibashi-Ueda H, Nagata S, Sakakibara H, Nimura Y. Uhl’s anomaly as a result of progression to ventricular dilation from hypertrophic cardiomyopathy. Acta Path01 Jpn 1987;37:1477-88. Viola AP, Adaro FVM, Roncoroni AJ. Idiopathic myocardiopathy resulting in failure of contractility of the right ventricle. Am J Med 1970;48:235-8. Casolo GC, Poggesi L, Boddi M, Fazi A, Bartolozzi C, Lizzadro G, Dabizzi RP. ECG-gated magnetic resonance imaging in right ventricular dysplasia. AM H-EART J 1987;113:1245-8.Blomstrom-Lundqvist C, Selin K, Jonsson R, Johansson SR, Schlossman D, Olsson SB. Cardioangiographic findings in patients with arrhythmogenic right ventricular dysplasia. Br Heart J 1988,59:556-63. Hasumi M, Sekiguchi M, Hiroe M, Kasanuki H, Hirosawa K. Endomyocardial biopsy approach to patients with ventricular tachycardia with special reference to arrhythmogenic right ventricular dysplasia. Jpn Circ J 1987;51:242-9. NavaA,Ma&B,ThieneG,BujaG,CancianiB,Scognamiglio R, Miraglia G, Corrado D, Boffa GM, Daliento L, Scattolin G, Fasoli G, Stritoni P, Dalla Volta S. La displasia aritmogena de1 ventricolo destro. Studio su una popolazione selezionata. G Ital Cardiol 1988:18:2-g. Nava A, Scogn&iglio R, Thiene G, Canciani B, Daliento L, Buia G. Stritoni P. Fasoli G. Dalla Volta S. A DolvmorDhic form oi familial arihythmogenic right ventricular dysplasia. Am J Cardiol 1987;59:1405-9.

Left ventricular involvement in right ventricular dysplasia.

Right ventricular dysplasia, a heart muscle disease of unknown cause, anatomically characterized by variable replacement of myocardial muscle with adi...
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