232

Internutronal

Journul

of Cardiology,

26 (1990) 232-234

Elsevier

CARD10

10146

The progression of hypertrophic cardiomyopathy: dilatation of the left ventricle with supernormal systolic function Francesco Pelliccia, Cinzia Cianfrocca and Francesco Romeo Deportment

of Cardiologv.

(Received

We performed years

earlier.

The

87%) although that

cardiac

catheterisation

repeat

angiogram

left

ventricular

hypertrophic

cardiomyopathy

Key words:

Left ventricular

the maintenance

of left ventricular

dilatation

should

into a hypokinetic

dilatation;

Hypertrophic

A 4%year-old man was admitted to our Unit in 1981 for episodes of angina and palpitation. Physical examination featured a grade 3/6 mid-systolic murmur. to: Dr. F. 85. 00179 Rome, Italy.

0167.5273/90/$03.50

Pelliccia, Via Tommaso

0 1990 Elsevier

Science

volume

necessarily

cardiomyopathy;

Report

Correspondence

diagnosed

Italv

1989)

as having

hypertrophic

systolic

function

(from 65 to 132 ml/m’).

be considered

a marker

of

cardiomyopathy (ejection

7

fraction:

This case suggests the

progression

of

left ventricle.

Hypertrophic cardiomyopathy is characterised by normal or supernormal systolic function, impairment of diastolic function and reduced left ventricular end-diastolic volume [l]. Progressive cardiac symptoms related to diffuse left ventricle hypokinesia may occasionally develop as part of its natural history. Systolic dysfunction is usually associated with a relative increase in the dimensions of the left ventricular cavity [2], although a few patients may evolve into a phase characterised by marked left ventricular dilatation resembling the morphologic and functional features of a dilated cardiomyopathy [3,4]. Thus, progressive left ventricular dilatation in hypertrophic cardiomyopathy is commonly considered a marker of end-stage disease. We report one patient with hypertrophic cardiomyopathy who showed the maintenance of a normal systolic function up to 7 years despite the angiographic evidence of a progressive increase in the dimensions of the left ventricular cavity.

ghirami

20 September

of a “supernormal”

end-diastolic not

Introduction

Case

of Rome “LA Supienza”,

in a man who had been

showed

there was an increase

progressive

Uniuersit)

10 May 1989: revision accepted

Publishers

In-

Myocardial

function

Noninvasive laboratory evaluation included a resting electrocardiogram showing a prominent biventricular hypertrophy and diffuse repolarisation abnormalities. No supraventricular and/or ventricular ectopic activity was detected by 24-hour ambulatory electrocardiographic monitoring. An echocardiogram demonstrated asymmetric septal hypertrophy (16 mm) and a normal left ventricular end-diastolic diameter (41 mm). At cardiac catheterisation, no pressure gradient across the left ventricular outflow tract was detected. The patient also underwent left ventriculography. Left ventricular end-diastolic and end-systolic volumes, as well as ejection fractions, were calculated from the 30 o right anterior oblique projection of the left ventriculogram using the area/length method. The chosen cycle was taken before the sixth beat following the start of injection of the contrast medium. Extrasystolic and postextrasystolic cycles were excluded. Every value obtained was corrected for body surface area. The patient was in sinus rhythm during the examination. Angiocardiograms (Fig. 1A) showed a rapidly contracting left ventricle (ejection fraction: 71%) and a reduced left ventricular end-diastolic volume (65 ml/m’). No significant mitral regurgitation was detected. The presence of associated coronary arterial disease was excluded by coronary arteriography. The patient was diagnosed as having hypertrophic cardiomyopathy and was treated with propranolol (120 mg/day), which determined an improvement of the symptomatic status. The patient remained clinically unchanged until

B.V. (Biomedical

Division)

233

A

Fig. 1. Left ventricular

end-systolic

(upper)

and end-diastolic (lower) angiographic frames (A), and at the repeat one in 1988 (B).

January 1989, when he was readmitted to our unit because of multiple episodes of chest pain. He did not show any change in physical features. Resting electrocardiography revealed left axis deviation (from + 68” to - 27 Q) and Holter monitoring showed ST-segment downsloping up to 0.3 mV during transient painful episodes. An echocardiogram documented both left atria1 enlargement (from 38 to 47 mm) and left ventricular dilatation (from 50 to 61 mm). There was no evidence of thinning of the left ventricular wall. Haemodynamic assessment showed an increase of left ventricular end-diastolic pressure (from 25 to 41 mm Hg). Additional left ventriculography was performed following the same procedure as the first. The patient was in sinus rhythm at the time of catheterisation. Angiocardiograms (Fig. 1B) revealed the presence of a

at the first cardiac

catheterisation

in 1981

“supernormal” systolic function (ejection fraction: 87%) and an increase of left ventricular cavity size was detected (left ventricular end-diastolic volume: 132 ml/m2). There was no evidence of significant mitral regurgitation. Coronary angiography was performed and showed normal coronary arteries. Discussion

Progression to left ventricular dilatation is commonly thought to be a potential complication in patients with hypertrophic cardiomyopathy [3]. This process can follow acutely a myocardial infarction, or can develop gradually without clinical or electrocardiographic evidence of infarction [4]. Previous reports have highlighted the correlation between left ventricular sys-

234

of a poorly contracting left ventricle, though it probably represents an early stage in the evolution to a hypokinetic phase.

tolic dysfunction and increases in left ventricular dimensions [2-41. Our case provides evidence that left ventricular dilatation can occur in hypertrophic cardiomyopathy regardless of impairment of left ventricular function. Our patient had undergone a stable clinical course and revealed a normal ejection fraction at the repeat angiography, although a progressive enlargement of the left ventricle was noted to be associated with a concomitant increase in left ventricular end-diastolic pressure. These findings suggest that the Frank-Starling mechanism [5] was involved in the maintenance of systolic function within normal limits. The patient was receiving beta-blocker at the time of study, and we cannot rule out, therefore, that this drug could account for some of the dilatation. It is unlikely, nonetheless, that propranolol would cause left ventricular enlargement without affecting contractility. Our case demonstrates that progressive left ventricular dilatation should not always be considered a marker

References Goodwin JF. The frontiers of cardiomyopathy. Br Heart .I 1982;48:1-18. Spirit0 P. Maron BJ, Bonow RO. Epstein SE. Occurrence and significance of progressive left ventricular wall thinning and relative cavity dilatation in hypertrophic cardiomyopathy. Am J Cardiol 1987;59:1233128. Ten Cate FJ, Roelandt J. Progression to left ventricular dilatation in patients with obstructive cardiomyopathy. Am Heart J 1979;97:762-766. Fighali S, Krajcer Z, Edelman S, Leachman RD. Progression of hypertrophic cardiomyopathy into a hypokinetic left ventricle: higher incidence in patients with midventricular obstruction. J Am Co11 Cardiol 1987;9:288-295. Weber KT, Janicki JS. The heart as a muscle-pump system and the concept of heart failure. Am Heart J 1979;98:371376.

Internaitonal

CARD10

Journal of Cardiologv, 26 (1990) 234-236 Elsevier

10147

Pacemaker malfunction due to subcutaneous emphysema Didier Giroud Department

of Medicine,

(Received

We

describe

physiologic venepuncture. anodal

pacing

Accumulation

plate and dysfunction

Key words:

Pacemaker

patient

system.

who

presented

The malfunction

of air within

the generator

20 September

with

1989)

malfunction

two

was due to subcutaneous pocket

of the pacemaker

days

after

emphysema resulted

implantation

produced

in insulation

of a

by subclavian of the unipolar

of the device.

malfunction;

Subcutaneous

emphysema;

introduction

The frequency of pneumothorax complicating subclavian venepuncture varies from 0 to 6% [l-3], but the incidence of subcutaneous emphysema is not known. It Correspondence to: D. Giroud, M.D., Dept. of Radiology, University Hospital, 24, rue Micheli-du-Crest, 1211 Geneve 4,

Subclavian

kj 1990 Elsevier Science Publishers

venous catheterization

may cause pacemaker malfunction due to air entrapment within the generator pocket, resulting in insulation of the unipolar anodal plate. We report such a case. Case

A

Report

70-year-old

atrioventticular

Switzerland. 0167-5273/90/$03.50

Goy

Distrtct Hospttal, LA Chaux-de-Fond.~, Switzerland

19 May 1989; revision accepted

a pacemaker-dependent

dual-chamber

and Jean-Jacques

B.V. (Biomedical

Division)

woman block

and

with chronic

symptomatic pulmonary

complete obstruc-