Pulmonary arterial aneurysms in Marfan's syndrome

International

Journal

of Cardiology,

21 (1988)

79

79-82

Elsevier

IJC 07494

Pulmonary arterial aneurysms in Marfan’s syndrome Laurence J. Disler, Pravin Manga and John B. Barlow Department

of Cardiology,

Johannesburg

Hospital

and Uniuersity

Johannesburg,

of the Witwatersrand

Medical

School,

South Africa

(Received 29 March 1988; accepted 16 April 1988)

We report of the

the case

pulmonary

of a 23-year-old

arteries.

The

man with Marfan’s

importance

and

possible

Pulmonary

arterial

syndrome

and saccular

complications

aneurysms

of this

finding

are

feature

of Marfan’s

discussed. Key words:

Marfan’s

syndrome;

aneurysm

Introduction Dilatation syndrome

of the pulmonary

trunk

is a recognized

although

may

occur

[l]

pulmonary

arteries

such saccular

rupture

have, to our knowledge,

aneurysms

with a 7-year

and often

[2]. Saccular not previously

benign

aneurysms

of the

been reported.

left

and

We report

right

a case of

follow-up.

Case Report A 23-year-old follow-up. intolerance arterial

Caucasian

and a diagnosis

aneurysms

Physical

examination

and mild pectus

seen

and bounding

revealed

excavatum

at the

in character.

normal.

A grade 2/4 aortic 2/4

ejection

Correspondence 2193,

0167-5273/88/$03.50

a Marfanoid

Austin

systolic

The

Flint

appearance palate.

blood

pressure

was

was no cardiomegaly. murmur

mid-diastolic which

incompetence

history

of Marfan’s

Straightening

150/80 The

University

Science

Publishers

and pulmonary arachnodac-

of the thoracic

regular,

venous

sounds

at the left parastemal at the apex.

was also heard.

Division)

were

border

A grade

There

of the Witwatersrand,

B.V. (Biomedical

spine

of equal volume

mm Hg. The jugular

South Africa.

0 1988 Elsevier

routine

syndrome.

1st and 2nd heart

was audible

to the neck

to: Dr. L.J. Disler, Dept. of Cardiology,

for

of mild effort

with long extremities,

was present

murmur

radiated

Clinic

complaining

The pulses were 80/minute,

early diastolic

murmur

Cardiac

with aortic

was no family

and a high arched

There

Outpatient

to this hospital

syndrome

were noted.

was not elevated.

and a grade

of Marfan’s

small joints

pressure

Parktown

was

had been made. There

tyly, hyperextensible

aortic

man

At the age of 16, he had been admitted

2/6

were no

York

Road,

80 signs of cardiac failure. Duroziez’ sign was negative. The chest X-ray showed antero-posterior compression of the heart due to the pectus excavatum. There was no cardiomegaly. The ascending aorta was dilated and there were saccular dilatations of the left and right pulmonary arteries (Fig. 1). The electrocardiogram showed sinus rhythm, and poor R wave progression with left ventricular hypertrophy on voltage criteria. An echocardiogram confirmed a dilated aortic root, measuring 4.9 cm in diameter but showed no evidence of aortic dissection. Mitral valve prolapse was noted. Left ventricular function was normal. Ophthalmological assessment including slit lamp examination confirmed the presence of myopia but

Fig. 1. Frontal

chest X-ray

showing

a dilated ascending aorta and saccular right pulmonary arteries.

dilatations

of the left and

Fig. 2. Pulmonary angiogram showing saccular aneurysms of the left and right pulmonary arteries. A small diverticulum

there were no signs of lens dislocation. and diastolic showed

large

diverticulum

pressures saccular

aneurysms

trunk is noted.

At right heart catheterisation

were normal

of the pulmonary

of the pulmonary

with a mean of

the

trunk (Fig.

left

and

the pulmonary

of 22 mm Hg. Pulmonary right

pulmonary

systolic

arteriography

arteries

and

a small

2).

Discussion Significant

pulmonary

arterial

dilatation

of the proximal

dilatation

of the pulmonary

unlike patient trunk involve

that of so-called is unusual is unaffected the junction

involvement

pulmonary

trunk has also been reported

“congenital

in that, in contrast apart

is uncommon

trunk with or without

from

idiopathic

reported

of the pulmonary cases,

of a small diverticulum

of the left and right pulmonary

syndrome.

arteries

Diffuse

may occur [l]. Saccular

[3], as well as a clinical

dilatation”

to previously

the presence

in Marfan’s dissection

the proximal (Fig.

picture

trunk

not

[4]. Our

pulmonary

2). The aneurysms

with their lobar

divisions.

Cystic

82

medial necrosis occurring at the branching points of the pulmonary arteries, areas of potential weakness in the arterial wall, may possibly explain their unusual site. Pulmonary arterial aneurysms may be complicated by tracheobronchial compression in infancy due to immaturity of the walls of the airways [5]. Of great concern in this patient is the possibility of rupture. The aneurysms have presumably occurred at sites of extreme weakness in the pulmonary arterial wall and could potentially rupture despite the normal pulmonary arterial pressure. This complication has only once previously been reported in a 23-year-old woman with Marfan’s syndrome and fusiform dilatation of the pulmonary trunk [2]. In the case reported here, very little increase in the size of the aneurysms has been noted in a 7-year follow-up period with serial X-rays. Nonetheless, close follow-up of this patient will still be needed.

References 1 McKusick VA. In: Heritable disorders of connective tissue. 3rd ed. St Louis, MO: The CV Mosby Company, 1966;38-149. 2 Anderson M. Pratt-Thomas HR. Marfan’s syndrome. Am Heart J 1953;46:911-917. 3 Childers RW, McCrea PC. Absence of the pulmonary valve. A case occurring in the Marfan syndrome. Circulation 1964;29:598-603. 4 Perloff JK. The clinical recognition of congenital heart disease. 2nd ed. Philadelphia: Saunders, 1978;222-227. 5 Lakier JB. Stanger P, Heymann MA, Hoffman JIE, Rudolph AM. Tetralogy of Fallot with absent pulmonary valve. Natural history and hemodynamic considerations. Circulation 1974;50:167-175.

International Elsevier

Journal

of Cardiology, 21 (1988) 82-84

IJC 07495

Renal effects of calcium channel blockers in vivo S.R. Mittal ‘, A.K. Mathur Departments

* and H. Parchwani

3

of ’ Cardiology, ’ Pharmacology and 3 Biochemistry, J. L. N. Medical College and Hospital, Ajmer (Rojasthan),

India

(Received 7 April 1988; accepted 16 April 1988)

Daily output seven

days

of urine

of oral

was measured

therapy

with

in three groups

nifedipine,

verapamil

of five rabbits and

diltiazem.

Correspondence to: Dr. S.R. Mittal, Dept. of Cardiology, J.L.N. X1/101, Brahampuri, Ajmer (Rajasthan), India 305 001. 0167-5273/88/$03.50

each, before

and during

All the drugs

caused

Medical College and Hospital,

0 1988 Elsevier Science Publishers B.V. (Biomedical Division)