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Idiopathic myocardial hypertrophy simulating rheumatic heart disease
IDIOPATHIC
EUGENE
MYOCARDIAL RHEUMATIC B. LEVIN,
HYPERTROPHY HEART DISEASE
M.D., AND SAMUEL
SAN FRANCISCO,
SIMULATING
L. COHEN, M.D.*
CALIF.
I
Only twentyDIOPATHIC hypertrophy of the heart is an unusual entity. four cases were collected by Levy and Van Glahn.’ We observed a case of congenital idiopathic hypertrophy of the heart with clinical and radiographic findings compatible with rheumatic mitral valvular disease.The literature does not mention that such a similarity can occur. CASE REPORT E. K., a Z-year-old white male veteran entered this hospital Oct. 31, 1951, with the complaints of fever, productive cough, and pain in left chest. Past history revealed three previous admissions in the last 4 years. On each occasion the complaints consisted of fainting spells associated with palpitation, the seeing of yellow, the hearing of music, and unconsciousness. There were no tongue biting, convulsions, or incontinence. History of previous illnesses revealed childhood complaints questionably compatible with rheumatic fever. Physical examinations reported on each of these admissions showed a well-developed white male with a moderate funnel chest. Auscultation of the lungs revealed transitory wheezes, mainly inspiratory. The heart was enlarged to the left. A Grade 3 systolic murmur was heard best in the third intercostal space to the left of the sternum. The murmur was transmitted to the apex and to a lesser extent to the aortic area. No thrills were present and PZ equaled AZ. The blood pressure varied from 98/68 to 114/66 mm. Hg. Rhythm was alternately regular and irregular. A summation of the laboratory findings revealed a normal blood count, serology, glucose tolerance, and gastrointestinal series. The electroencephalogram revealed nonfocal bursts compatible with epilepsy. Electrocardiographic findings were: left ventricular hypertrophy, abnormal P waves, premature beats, and occasional bouts of auricular flutter with an auriculoventricular block varying from 2 to 1, to 4 to 1. The roentgenogram showed the heart to be 25 per cent above the predicted normal as measured on the height-weight tables. Cardiac fluorosropj with barium swallow revealed definite left auricular and ventricular enlargement (Fig. 1, A and B). On each admission prior to the last admission the patient was treated with quinidine and digitalis. He responded well with abatement of symptoms andconversion to normal sinus rhythm. Coughing or the drinking of cold water sometimes stopped the attacks. Pressure on the carotid sinus and eyeball did not aid. On the final admission all,the findings were similar except for fever, productive cough, and an increased sedimentation rate. He was treated with Gantrisin, quinidine, digitalis, and Dilantin. He rapidly became afebrile and asymptomatic and on the second day was ambulatory. On the evening of Nov. 14,19.51, the patient suddenly developed a slight cough with blood-streaked sputum; he gasped and expired. -____ From the Department of Internal Medicine and Radiology, Veterans Administration, Long Beach. Calif., now Department of Medicine, Letterman Army Hospital, San Francisco, Calif. Recgived for publication March 3. 1954. *Radiologist, Veterans Administration Hospital, Tucson. A&-. 637
638
AMERICAN
HILIRT
A.
JoURN.11.
B.
Fig. 1.-A, Posteroanterior view of the chest. There is evidence of left, ventricular enlargement. questionable enlargement of the left auricle to the right, and a small aort,ic knob shadow. B, Right anterior oblique view of the chest. Note enlargement of the left auricle.
Fig.
S.--Gross
pathologic
specimen of the heart showing thickness of the ventricular
mainly the left wall is 30 mm.
ventricle
and auricle.
The
LEVIN
AND
COHEN:
IDIOPATHIC
MYOCAKDIAL
639
HYPEKTROPHY
Autopsy findings: Lungs showed a marked, frank pulmonary edema. Congestion of the liver, spleen, and kidneys was present. The heart weighed 750 grams and was markedly enlarged. The right ventricle was thickened and measured 5 mm. The left ventricle was also thickened and measured 30 mm. (Fig. 2). All valve measurements and the valve leaflets were normal. The left auricle appeared dilated and the auricular wall appeared somewhat hyperThe left ventricular cavity revealed markedly trophied, but there were no thrombi present. thickened cordae tendineae. Papillary muscles and columna carnae were definitely hypertrophied. The endocardial surface appeared normal and no mural thrombi were present. The ostia of the coronary arteries were patent, and the right coronary artery was of normal size and had no atheromatous changes. The left coronary artery and its branches were somewhat smaller than usual. Cut section into the myocardium of right and left ventricle showed diffuse whitish, fibrotic streakThe aorta revealed very little atheromatous ing. There was no evidence of fresh infarction. change and seemed somewhat hypoplastic.
Fig. 3.-blicroscopic
view
of the
myocardium
of the
left
ventricle
(X60).
Marked
fibrosis
is present.
Microscopic examination: Sections of the right ventricle and left ventricle showed diffuse fibrosis of interstitial areas with many hyperchromatic, blunted, and enlarged nuclei indicating myocardial hypertrophy (Fig. 3). Many of the muscle bundles were completely replaced with fibrous tissue and some show increase in acidophilic staining with loss of striation. The endocardium was not thickened. Careful examination of all sections failed to reveal any evidence of active or old rheumatic heart disease. The lungs showed marked alveolar edema.
640
hMERICAN
HEART
JOURNAL
SUMMARY
This 26-year-old white male patient was intermittently hospitalized for 1 to 2 years prior to death from congestive failure primarily because of cardiac arrhythmias. Autopsy findings revealed only massive cardiac hypertrophy primarily of the left ventricle with fibrosis of the myocardium and evidence of acute pulmonary edema with congestion of the liver, spleen, and kidneys. The etiology of the left ventricular and auricular hypertrophy and fibrosis was not apparent. REFERENCE 1.
Levy,
I<. L.,
and
the Clinical
Von
Glahn,
and
W.
Pathologic
C.:
Cardiac Hypertrophy Features in Ten Adults,
of Unknown AM. HEART
Cause, A Study J. 28:714, 1944.
of
EUGENE
MYOCARDIAL RHEUMATIC B. LEVIN,
HYPERTROPHY HEART DISEASE
M.D., AND SAMUEL
SAN FRANCISCO,
SIMULATING
L. COHEN, M.D.*
CALIF.
I
Only twentyDIOPATHIC hypertrophy of the heart is an unusual entity. four cases were collected by Levy and Van Glahn.’ We observed a case of congenital idiopathic hypertrophy of the heart with clinical and radiographic findings compatible with rheumatic mitral valvular disease.The literature does not mention that such a similarity can occur. CASE REPORT E. K., a Z-year-old white male veteran entered this hospital Oct. 31, 1951, with the complaints of fever, productive cough, and pain in left chest. Past history revealed three previous admissions in the last 4 years. On each occasion the complaints consisted of fainting spells associated with palpitation, the seeing of yellow, the hearing of music, and unconsciousness. There were no tongue biting, convulsions, or incontinence. History of previous illnesses revealed childhood complaints questionably compatible with rheumatic fever. Physical examinations reported on each of these admissions showed a well-developed white male with a moderate funnel chest. Auscultation of the lungs revealed transitory wheezes, mainly inspiratory. The heart was enlarged to the left. A Grade 3 systolic murmur was heard best in the third intercostal space to the left of the sternum. The murmur was transmitted to the apex and to a lesser extent to the aortic area. No thrills were present and PZ equaled AZ. The blood pressure varied from 98/68 to 114/66 mm. Hg. Rhythm was alternately regular and irregular. A summation of the laboratory findings revealed a normal blood count, serology, glucose tolerance, and gastrointestinal series. The electroencephalogram revealed nonfocal bursts compatible with epilepsy. Electrocardiographic findings were: left ventricular hypertrophy, abnormal P waves, premature beats, and occasional bouts of auricular flutter with an auriculoventricular block varying from 2 to 1, to 4 to 1. The roentgenogram showed the heart to be 25 per cent above the predicted normal as measured on the height-weight tables. Cardiac fluorosropj with barium swallow revealed definite left auricular and ventricular enlargement (Fig. 1, A and B). On each admission prior to the last admission the patient was treated with quinidine and digitalis. He responded well with abatement of symptoms andconversion to normal sinus rhythm. Coughing or the drinking of cold water sometimes stopped the attacks. Pressure on the carotid sinus and eyeball did not aid. On the final admission all,the findings were similar except for fever, productive cough, and an increased sedimentation rate. He was treated with Gantrisin, quinidine, digitalis, and Dilantin. He rapidly became afebrile and asymptomatic and on the second day was ambulatory. On the evening of Nov. 14,19.51, the patient suddenly developed a slight cough with blood-streaked sputum; he gasped and expired. -____ From the Department of Internal Medicine and Radiology, Veterans Administration, Long Beach. Calif., now Department of Medicine, Letterman Army Hospital, San Francisco, Calif. Recgived for publication March 3. 1954. *Radiologist, Veterans Administration Hospital, Tucson. A&-. 637
638
AMERICAN
HILIRT
A.
JoURN.11.
B.
Fig. 1.-A, Posteroanterior view of the chest. There is evidence of left, ventricular enlargement. questionable enlargement of the left auricle to the right, and a small aort,ic knob shadow. B, Right anterior oblique view of the chest. Note enlargement of the left auricle.
Fig.
S.--Gross
pathologic
specimen of the heart showing thickness of the ventricular
mainly the left wall is 30 mm.
ventricle
and auricle.
The
LEVIN
AND
COHEN:
IDIOPATHIC
MYOCAKDIAL
639
HYPEKTROPHY
Autopsy findings: Lungs showed a marked, frank pulmonary edema. Congestion of the liver, spleen, and kidneys was present. The heart weighed 750 grams and was markedly enlarged. The right ventricle was thickened and measured 5 mm. The left ventricle was also thickened and measured 30 mm. (Fig. 2). All valve measurements and the valve leaflets were normal. The left auricle appeared dilated and the auricular wall appeared somewhat hyperThe left ventricular cavity revealed markedly trophied, but there were no thrombi present. thickened cordae tendineae. Papillary muscles and columna carnae were definitely hypertrophied. The endocardial surface appeared normal and no mural thrombi were present. The ostia of the coronary arteries were patent, and the right coronary artery was of normal size and had no atheromatous changes. The left coronary artery and its branches were somewhat smaller than usual. Cut section into the myocardium of right and left ventricle showed diffuse whitish, fibrotic streakThe aorta revealed very little atheromatous ing. There was no evidence of fresh infarction. change and seemed somewhat hypoplastic.
Fig. 3.-blicroscopic
view
of the
myocardium
of the
left
ventricle
(X60).
Marked
fibrosis
is present.
Microscopic examination: Sections of the right ventricle and left ventricle showed diffuse fibrosis of interstitial areas with many hyperchromatic, blunted, and enlarged nuclei indicating myocardial hypertrophy (Fig. 3). Many of the muscle bundles were completely replaced with fibrous tissue and some show increase in acidophilic staining with loss of striation. The endocardium was not thickened. Careful examination of all sections failed to reveal any evidence of active or old rheumatic heart disease. The lungs showed marked alveolar edema.
640
hMERICAN
HEART
JOURNAL
SUMMARY
This 26-year-old white male patient was intermittently hospitalized for 1 to 2 years prior to death from congestive failure primarily because of cardiac arrhythmias. Autopsy findings revealed only massive cardiac hypertrophy primarily of the left ventricle with fibrosis of the myocardium and evidence of acute pulmonary edema with congestion of the liver, spleen, and kidneys. The etiology of the left ventricular and auricular hypertrophy and fibrosis was not apparent. REFERENCE 1.
Levy,
I<. L.,
and
the Clinical
Von
Glahn,
and
W.
Pathologic
C.:
Cardiac Hypertrophy Features in Ten Adults,
of Unknown AM. HEART
Cause, A Study J. 28:714, 1944.
of