Cardiac Rhabdomyoma in a Newborn. Two-Dimensional Echocardiographic Diagnosis

Case

Reports

Cardiac

Rhabdomyoma

Two-Dimensional Suheyla

a Newborn

Echocardiographic

OZKUTLU, M.D.,*

Ilhan

in

Muhsin

PASAOGLU, M.D.,**

Semra

Diagnosis

SARACLAR, M.D.,* ATALAY, M.D.,***

and Ayse AYHAN, M.D.**** SUMMARY Primary

cardiac

to surgery, formed

to

confirm

the

a 2-day-old

congenital

heart

multiple

for the

method

severely

without

prior

Additional Cardiac

baby

disease that

diagnosed and

Indexing

the by

in the

of primary newborns

catheterization,

newborn

period.

angiocardiography

have

echocardiographic clinical

impression

two-dimensional

confirmed

two-dimensional

diagnosis

rhabdomyoma

rare

and

with

symptomatic cardiac

quite

two-dimensional

rhabdomyoma

is emphasized technique

are

catheterization

this report as

tumors

cardiac

by

surgery

may thus

tumors. be

taken

minimizing

per-

findings. of severe

In

cyanotic

echocardiography is presented.

echocardiography cardiac

Prior been

is

a very

By means directly

It useful of this

to surgery

complications.

Words: Newborn

Two-dimensional

echocardio-

graphy

RIMARY cardiac tumors are quite rare in the newborn period.1) Previously, they had been diagnosed only at autopsies due to their nonspecific clinical signs and symptoms.1) More recently the number of diagnosed cases has increased with the extensive use of echocardiography.2)-11) Prior to surgery, cardiac catheterization and angiocardiography have been performed to confirm the two-dimensional echocardiographic findings.12) It is known that patients with these tumors may experience rhythm disturbances and/or embolization as a complication of cardiac catheterization and angiocardiography. During catheterization, deaths due to rhythm disturbances From the Departmentof Cardiology,HacettepeUniversity,Instituteof Child Health,Ankara, Turkey. *Professorof Pediatricsand PediatricCardiologist , HacettepeUniversity,Instituteof Child Health. **AssociateProfessor of Cardiovascular Surgery. ***Pediatric Cardiologist . ****AssociateProfessorofPathology . Addressfor reprints: SuheylaOzkutlu,M.D., Departmentof PediatricCardiology,Hacettepe University,Ankara,Turkey. Receivedfor publicationJuly 20, 1990. AcceptedAugust16, 1990. 391

392 have

been

cardiac

reported.2),13)

Before

catheterization

noninvasive

anotic

for

methods

In

this

we

congenital

A

a case

disease,

in

two-dimensional

2-day-old

with

the

whom

clinical

multiple

the

other

1991

risks

diagnosis

infant

impression

cardiac

echocardiography

female

also

newborns,

May

of

by

only

important.

CASE

and

considering

symptomatic

present

heart

by

surgery,

severely

becomes

paper,

diagnosed

and

of

severe

cy-

rhabdomyomas confirmed

were

by

surgery.

REPORT

was

admitted

to

the

to

be

hospital

with

cyanosis

and

weighed

tachypnea. On

physical

3300g.

The

and

2/6

about

4cm Hb

severe

was

blood was

was

heard the

17.4g/dl

cyanotic

the costal

and

and the

tumors

heart

rate

was

160bpm,

ejection

The

liver

8400/mm3.

addition

to

right

ventricular

the

baby

cardiac

died failure.

be

on

murmur

was

palpable

Chest

x ray

axis

deviation

the

showed and

was

right

atrial

day.

At

the

size

whole

position

outflow

ven-

another tract

operation,

mass

(Figs.

a

measuring

1, 2).

and

was

and the

gray-white

25•~50•~15mm excised

interventricular

through

arising

right

enlargement.

10•~15mm

excised

parasternal, in

the

axis

valve on

measuring

tract,

into

and

tricuspid seen

the

50•~23mm

long

second

was

in

ventricular

septum

mass

tumor,

mass

and

the

through

interatrial

left

ventricular

small

second

a

extending

parasternal the

interventricular

outflow the

examination

and

into

performed

very

The

to

count in

modified

right

visualized

another

The and

the

was

from

readily

of

term

systolic

border.

revealed

extending

was

arising

incision

blood

positions

in

size

Surgery

left

sternal

full

margin.

septum

there

excised.

The A

echocardiographic

and

in

addition,

Also,

left

hypertrophy

apical

cavity

was

dyspneic. 80mmHg.

white

interventricular

9•~13mm

mass

noted

enlargement.

subcostal

tricular

and

over

Two-dimensional

from

was

was

right

ventricular

atrial

she

pressure

below

right

right

examination, baby

systolic

grade

In

Jpn. Heart J.

OZKUTLU, SARACLAR, PASAOGLU, ATALAY, AND AYHAN

in

toto.

septum extending

mitral

valve

and

into

the

following

septum. 10

hours

after

surgery

Histopathologic

rhabdomyomas

due

study

(Fig.

of

to the

uncontrolled

arrhythmia

specimens

showed

the

3).

DISCUSSION Primary ECG

and

cardiac telecardiographic

tumors,

with findings,

their

nonspecific may

mimic

clinical severe

manifestations, valvular

or

myo-

Vol.32 No.3

CARDIAC RHABDOMYOMA IN A NEWBORN

Fig. originating right

1.

Parasternal from

the

ventricle;

atrium;

IVS

LV=left

long-axis

view.

extending

into

Arrows

indicate

ventricle;

IVS=interventricular

the right

the

ventricular

393

tumor

cavity. septum;

mass RV=

LA=left

AO=Aorta.

Fig. 2. Modified parasternal long-axis view. In addition to the right ventricular tumor, illustrated in Fig. 1 a smaller tumoral lesion is seen in the left ventricular outflow tract (arrows). RV=right ventricle; PM=papillary muscle; vein.

cardial even

IVS=interventricular

diseases, septicemia

formed

and

especially

and

metabolic

echocardiography

congenital Usually

heart

septum;

in the on

atrium;

newborn

disorders.

based

LA=left

period,

In

the

a clinical

PV=pulmonary

respiratory

patient

impression

distress

presented

we per-

of severe

cyanotic

disease.

rhabdomyomas

are

multiple

and

have

or

a distinct

preference

394

OZKUTLU, SARACLAR, PASAOGLU, ATALAY, AND AYHAN

Fig. myoma

for

the

an

Microscopic

often

extending calls

leading

the

been

one-half

looked

sional

The

and diagnoses

rhabdomyoma

case

graphy was and location

confirmed of tumors

the

and

surgical

In who

the

patients,

with

diagnosed

obvious

the

have and

following

of this

only

Surgical inflow

and

by

due

2 patients tumors

with have

been

treatment outflow

confirmed by by

by

two-dimenautopsy.6),11) echocardio-

is indicated tract

was

with

performed

ventricular

1 right

died.

and on

inflow

cardiac

patients

fibroma10),15)

in all

obstruction

primary

Three

survived;

has

these cases the number were in correlation with

to the right

reported.

disease

present.

two-dimensional In

cavity, is present

by two-dimen-

confirmed

surgery

11 newborns been

was this

demonstrated

also

Emergency

literature,

surgery

evidence

have

into

ventricle

ventricular

rhabdomyoma

were

left

sclerosis

angiocardiography

cham-

location

in the

right

myo-

cardiac

tuberous

nor at autopsy

2 cases

to the

of the

to the Since

of intrauterine

findings.

with

medical

rhabdomyoma3),13),14)

1991

rhabdo-

intramural

of a mass

rhabdomyoma

of severe heart failure outflow obstructions.

underwent

their

septum

arrhythmias.12)

patient because left ventricular

clear

be limited

deformity

from

surgically as in ours.8) found by echocardiography

autopsy

babies

threatening

the

may

of rhabdomyoma,

with

in

symptomatic

and

detection

clinically

cases

echocardiography

Another

showing

lesions

extend The

cases

echocardiography

sional

life

may

Neither

some

surgery.4),5),7)

tumor

of rhabdomyoma.

of the

for.12)

Recently,

The

interventricular

for consideration

in over

the

to compression they

position.12)

from

of

walls.12)

Alternatively, intracavitary

section

Heart J.

May

H-E•~115.

interventricular

cardium, bers.

3. cells.

Jpn.

with

and

tumors multiple

The

ventricular

our

other myxo-

Vol.32 No.3

CARDIAC RHABDOMYOMA IN A NEWBORN

ma,16)

1 right

atrial

myomas.7),8) In the

other

patient

origin

of the

anterior

other

two

tion

and

masses,

period

tumor

with

multiple

leaflet

surgery results

not

or tumors,

the

mitral

valve

could

be left

would

level

fatal

the

and

depend

tumor

as they

were

to remove

for primary

of myocardial

and

a mass

cardiac mainly

function

and

3 rhabdo-

was

excised.7)

attached

was successfully

be required

of surgery

always

teratoma17)

rhabdomyoma

rhabdomyomas,

of the

it was thought,

the are

1 intrapericardial

patients

with

complicated

As seen, born

fasciitis,9)

In 2 of the

395

to the

excised.

The

in a stable

posi-

them. tumors

on the the

in the

location

feasibility

newof the

of exci-

sion. very

Consequently,

two-dimensional

useful

for

allows

method

accurate

its relationship tion. due

to anatomic

of the structures

symptomatic

by only

to cardiac

of primary extent and

newborns

two-dimensional

catheterization

echocardiographic

diagnosis

determination

Severely

diagnosis

the

and go

is

of the

heart

and

location

of the

lesion

and

measurement may

echocardiography

and

examination

tumors

of myocardial

directly

to

to avoid

surgery

a

funcafter

complications

angiocardiography.

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OZKUTLU, SARACLAR, PASAOGLU, ATALAY, AND AYHAN

Jpn. Heart J. May

1991

myoma of the heart in infancy and childhood. Am J Cardiol 30: 95, 1972 14. Kuehl KS, Perry LW, Chandra AL, et al: Left ventricular rhabdomyoma: a rare cause of subaortic stenosis in the newborn infant. Pediatrics 46: 464, 1970 15. Oliva PB, Breckinridge JC, Johnson ML, Brantigan CO, O'Meara OP: Left ventricular outflow obstruction produced by a pedunculated fibroma in a newborn. Chest 74: 590, 1978 16. Balsara RK, Anastasios PJ: Myxoma of right ventricle. Chest 83: 145, 1983 17. Van der Hauwaert LG: Cardiac tumours in infancy and childhood. Br Heart J 33: 125, 1971