VOL. 103, No.

MICROCARDIA*
AN UNCOMMON DIAGNOSTIC PROBLEM

By LEONARD E. SWISCHUK, M.D.

OKLAHOMA CITY, OKLAHOMA

]JfICROCARDIA, as a diagnostic prob-

cardia with true cardiac atrophy secondary to
.LY.L lem, is certainly less frequently en- cachexia and undernutrition. The patient con-
countered than cardiac enlargement. How- tinued to do poorly and expired approxi-

ever, when it is seen, the physician must mately 2 years after his initial hospitalization
at the Children’s Memorial Hospital. Consent
consider a variety of causative disorders.
for an autopsy could not be obtained.
Generally speaking, microcardia occurs
with or without associated cardiac atrophy. CASE II. (B.H.). This 4 year old girl was
Microcardia with cardiac atrophy occurs admitted to the Children’s Memorial Hospital,
in association with chronic wasting disease Oklahoma City, on July 20, 1966 with severe
or undernutrition, while microcardia with- dehydration. She had been discharged just 13
out cardiac atrophy is usually seen secon- days previously following evaluation of possi-
dary to a rapid decrease in blood volume ble parental abuse and anemia. The hemo-
e.g., severe dehydration or massive hemor- globin at that time was 8 gm. per soo ml., and
rhage. In both instances, correction of the the hematocrit 25 per cent. The etiology of the
anemia was thought to be on the basis of iron
underlying disease by appropriate therapy
deficiency.
restores the heart size to normal.
At the present admission her temperature
One case illustrating microcardia with was 104#{176} F.,
pulse 180 per minute, respirations
cardiac atrophy and one case illustrating 40 per minute and weight 28 pounds. The pa-
microcardia without cardiac atrophy are tient was unconscious and severely dehydrated.
reported. Signs of shock were present in that the blood
pressure was 70/50 mm. Hg, and the pulse rapid
REPORT OF CASES
and thready. Laboratory studies at the time re-
CASE (P.Z.).
I. This i i year old boy was ad- vealed a sodium of 169 mEq./l., potassium 3.3
mitted to the Children’s Memorial Hospital, mEq./l., chlorides 136 mEq./l., CO, i i
Oklahoma City, on March 28, 1960, 10 months mEq./l., blood urea nitrogen 51 mg./ioo ml.,
after a tonsillectomy. On this admission he pre- hemoglobin 13.9 gm./ioo ml., and hematocrit
sented with a mass behind the right ear, a biop- 41 per cent. Comparison of these values with
sy of which revealed fibrosarcoma. It was those on her previous admission, especially the
thought that the tumor originated in the par- hemoglobin and hematocrit, suggested a
otid gland and had extended into the naso- marked degree of hemoconcentration.
pharynx and regional lymph nodes. Treatment A chest roentgenogram at this time revealed
consisted of a radical neck dissection and a marked decrease in the size of the heart, as
chemotherapy (cytoxan-cyclophosphamide). compared to the roentgenogram obtained dur-
Immediately after the neck dissection and for ing the previous admission (Fig. 2, A and B).
a period of months thereafter, the patient’s It was believed that this represented microcar-
condition was good. However, deterioration dia due to the severe dehydration. Fluid re-
became apparent after this period and the pa- placement, consisting of plasma and intra-
tient lost considerable weight, some 17 pounds venous fluid, was immediately initiated. With
over a month period. He also showed the rehydration, the heart returned to its original
generalized wasting and debilitation of the final size within 4 hours (Fig. 3). Pulmonary
stage of neoplastic disease. Chest roentgeno- edema, probably resulting from the vigorous
grams at this time showed the cardiac silhouette fluid replacement, was present at this time but
to be abnormally small (Fig. i, A and B) and cleared completely on the following day.
it was believed that this represented micro- With rehydration the hemoglobin returned
* From the Departments of Radiology and Pediatrics, University of Oklahoma Medical Center, Children’s Memorial Hospital,
Oklahoma City, Oklahoma.

“5
ii6 Leonard E. Swischuk MAY, 1968

FIG. 1. Case,. (A) The heart is of normal proportions. (B) Fifteen months later, microcardia is present. This
was due to cardiac atrophy as a result of terminal neoplastic disease.

to 8.6 gm./ioo ml., and the hematocrit to 27 proximately 6 weeks after admission and was,
per cent-values quite comparable to those on at that time, normotensive. The heart had re-
the previous admission when the patient’s turned to its original configuration.
state of hydration was normal.
DISCUSSION
The patient’s general condition continued to
improve rapidly and within 72 hours she had A small heart, as seen on chest roent-
completely recovered. She was discharged ap- genography, is usually considered to be of

wF

111G. 2. Case II. (A) The heart is minimally enlarged reflecting the underlying anemia. (B) There is a marked
decrease in cardiac size even though the inspiratory effort is more shallow than that in A. This represents
microcardia in response to severe dehydration.
VOL. 103, No. i Microcardia I 17

little clinical significance and, in the vast first case. It is usually seen as the result of
majority of cases, this is probably a cor- severe starvation or chronic wasting dis-
rect assumption. Small cardiac silhouettes ease such as malignant neoplasms and
are frequently seen in normal asthenic in- chronic infection. This has been substan-
dividuals,4 and an apparent decrease in tiated by Hellerstein and Santiago_Steven-
cardiac size is noted secondary to severe son’ in their comprehensive study of car-
asthmatic attacks and bronchiolitis. In diac atrophy, where they, in fact, showed
these instances, the changes can be ac- that it was most commonly associated
counted for by “squeezing” of the heart and with neoplasm and chronic infection.
“elongation” of the mediastinum secon- Microcardia without cardiac atrophy is
dary to the increased intrathoracic pres- demonstrated by our second case, where a
sure and depression of the diaphragm. marked and rapid decrease in blood vol-
There are, however, diseases which lead time produced a small heart. Patients with
to an actual decrease in cardiac weight this type of microcardia frequently exhibit
(atrophy), with the most commonly cited signs of dehydration and in severe cases,
condition being Addison’s It shock may be present. Dehydration is
is probable that the hypotension and secondary to markedly reduced fluid in-
lowered blood volume encountered in Ad- take or extensive fluid loss, most com-
dison’s disease are responsible for the small monlv due to severe diarrhea or massive
heart. It might be further postulated that hemorrhage. This type of microcardia is
with prolonged decrease in blood pressure not as widely appreciated as is microcardia
and volume, the heart is required to do less with cardiac atrophy, even though it is
work and disuse atrophy ensues. This mentioned in most standard textbooks.”2’4’5
atrophy is evidently reversible as the heart It can be seen at all ages, but as fluid bal-
returns to normal size with appropriate ance is more precarious in the infant and
therapy.”4 young child, it becomes manifest more
Microcardia with cardiac atrophy also readily and more profoundly in this age
occurs as a consequence of undernutrition group. Appropriate fluid therapy readily
and cachexia,”4” and is exemplified by our corrects the condition, but caution should
be employed to avoid overhydration and
pulmonary edema as demonstrated in Case
II.

SUMMARY

The rather rare problem of microcardia

is reviewed.
Two broad groups, namely microcardia
with cardiac atrophy and microcardia
without cardiac atrophy are discussed.
A case illustrating each type is reported
and the etiologic factors are considered.
Leonard F. Swischuk, M.D.
l)epartment of Radiology
University of Oklahoma, Children’s Memorial
Hospital
Soo Northeast Thirteenth Street
Oklahoma City, Oklahoma 73104

REFERENCES

Fic. 3. Case II. Note the return to original heart i. CAFFEV, J. Pediatric X-Ray Diagnosis. 1”ourth
size and the typical “butterfly” pattern of pul- edition. Year Book Medical Publishers, Inc.,
monary edema due to overhydration. Chicago, 1961, p. 387.
ii8 Leonard E. Swischuk MAY, 5968

2. Golden’s Diagnostic Roentgenology. Williams 4. NELSON, E. Editor. Textbook of Pediatrics.

and Wilkins Company, Baltimore, 5964, pp. Eighth edition. W. B. Saunders Company,
244-245. Philadelphia, 5964, pp. 1290-1293.
3. HELLERSTEIN, H. K., and SANTIAGO-STEVENSON, 5. SHANKS, S. C., and KERLEY, P. Editors. A
D. Atrophy of heart; correlative study of Textbook of X-Ray Diagnosis. Third edition.
eighty-five proved cases. Circulation, 5950, I, W. B. Saunders Company, Philadelphia,
93-I 26. 1962, p. 27.