Extensive Calcification of the Myocardium Report of a Case By A. CARLTON ERNSTENE, M.D., AND JOHN B. HAZARD, M.D. An unusual case of extensive calcification of the myocardium in a 25 year old woman is presented. Presumably it was the result of an earlier severe toxic or septic myocarditis. PA THOLOGIC deposits of calcium may occur in valve leaflets and valve rings, in the pericardial scar of chronic con- strictive pericarditis, and in the myocardium. In each of these regions, the areas of calcifica- tion may be demonstrated by appropriately taken roentgenograms. Their detection often is of considerable diagnostic importance. The most common cause of calcification in the myo- cardium is myocardial infarction, the calcium being deposited during the process of healing of the infarct. In addition, myocardial calcifica- tion may occur as a complication of hyper- parathyroidism' and as a result of focal toxic or inflammatory myocardial necrosis.26 Areas of bone formation may also be present. A re- view of the literature has been presented re- cently by Finestone and Geschickter.6 REPORT OF CASE A white, single woman, aged 25 years, was ad- mitted to the hospital on Nov. 7, 1946 because of progressive dyspnea of two years' duration. At the age of 9 years, she had had scarlet fever of such severity that she was out of school for one year. No cardiac or renal symptoms could be recalled. Four years before admission she had suffered from pneu- monia and had been kept in bed for one month. Two years later there had been an acute upper respira- tory infection, and since that time she had had noticeable shortness of breath on exertion. In Janu- ary, 1946, cough and increasing dyspnea necessitated a 4 weeks' period of rest in bed, but the patient was then able to return to work as a clerk until June. From June onward, dyspnea and cough progressively became more severe, and approximately 10 days be- fore admission a sudden further increase in these symptoms was accompanied by the first appearance of cyanosis and swelling of the face, neck, and ab- domen. From the Cleveland Clinic and the Frank E. Bunts Educational Institute, Cleveland, Ohio. 690 Physical examination revealed a well developed, well nourished young woman with severe orthopnea, cyanosis of the lips and nail beds, and moderate dis- tention of the jugular veins. The temperature was normal, the heart rate 120 per minute, and the blood pressure 80 mm. systolic, and 60 mm. diastolic. There was slight puffiness of the face. Signs of fluid were present over the lower half of the right thorax pos- teriorly, and there were numerous moist rales above this level as well as over the lower lobe of the left lung. The heart was greatly enlarged to the left; its rhythm was regular except for an occasional prema- ture beat, and no murmurs were present. All heart sounds were of average intensity. The liver extended 14 cm. below the costal margin in the right midelavic- ular line and was moderately tender. The edge of the spleen could be felt 4 cm. below the costal margin. There was no peripheral edema. The urine had a specific gravity of 1.020 and con- tained 3 plus albumin and an occasional hyaline or granular cast. The red blood cell count was 6,140,000 per cu. mm., and the hemoglobin content was 16.0 Gm. The leukocyte count was 12,000 per cu. mm. The Wassermann reaction of the blood was negative. An electrocardiogram showed sinus tachycardia with a rate of 116 per minute. The P waves were notched in lead I. The P-R intervals were within normal limits but the duration of the QRS complexes was increased to 0.11 second. There was slurring of QRS in leads I and II, and right axis deviation was present. The T waves were inverted in leads II and III. Portable anteroposterior roentgenograms of the thorax were of poor quality but revealed opacity of the right thorax with slight displacement of the trachea and mediastinum to the left. Extensive areas of increased density, suggestive of calcium deposits, were present within the area of a much enlarged cardiac shadow. Digitoxin, mercurial diuretics, a low sodium diet, the administration of oxygen, and right thoracentesis with the removal of 650 cc. of clear, straw colored fluid resulted in only slight and temporary improve- ment. On the twelfth day in the hospital the patient suddenly developed acute pulmonary edema and died. Postmortem examination revealed the heart to be Circulation, Volume III, May, 1951 by guest on May 20, 2018 http://circ.ahajournals.org/ Downloaded from
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Extensive Calcification of the Myocardium
Report of a CaseBy A. CARLTON ERNSTENE, M.D., AND JOHN B. HAZARD, M.D.
An unusual case of extensive calcification of the myocardium in a 25 year old woman ispresented. Presumably it was the result of an earlier severe toxic or septic myocarditis.
PA THOLOGIC deposits of calcium mayoccur in valve leaflets and valve rings,in the pericardial scar of chronic con-
strictive pericarditis, and in the myocardium.In each of these regions, the areas of calcifica-tion may be demonstrated by appropriatelytaken roentgenograms. Their detection often isof considerable diagnostic importance. Themost common cause of calcification in the myo-cardium is myocardial infarction, the calciumbeing deposited during the process of healingof the infarct. In addition, myocardial calcifica-tion may occur as a complication of hyper-parathyroidism' and as a result of focal toxicor inflammatory myocardial necrosis.26 Areasof bone formation may also be present. A re-view of the literature has been presented re-cently by Finestone and Geschickter.6
REPORT OF CASEA white, single woman, aged 25 years, was ad-
mitted to the hospital on Nov. 7, 1946 because ofprogressive dyspnea of two years' duration. At theage of 9 years, she had had scarlet fever of suchseverity that she was out of school for one year. Nocardiac or renal symptoms could be recalled. Fouryears before admission she had suffered from pneu-monia and had been kept in bed for one month. Twoyears later there had been an acute upper respira-tory infection, and since that time she had hadnoticeable shortness of breath on exertion. In Janu-ary, 1946, cough and increasing dyspnea necessitateda 4 weeks' period of rest in bed, but the patient wasthen able to return to work as a clerk until June.From June onward, dyspnea and cough progressivelybecame more severe, and approximately 10 days be-fore admission a sudden further increase in thesesymptoms was accompanied by the first appearanceof cyanosis and swelling of the face, neck, and ab-domen.
From the Cleveland Clinic and the Frank E.Bunts Educational Institute, Cleveland, Ohio.
690
Physical examination revealed a well developed,well nourished young woman with severe orthopnea,cyanosis of the lips and nail beds, and moderate dis-tention of the jugular veins. The temperature wasnormal, the heart rate 120 per minute, and the bloodpressure 80 mm. systolic, and 60 mm. diastolic. Therewas slight puffiness of the face. Signs of fluid werepresent over the lower half of the right thorax pos-teriorly, and there were numerous moist rales abovethis level as well as over the lower lobe of the leftlung. The heart was greatly enlarged to the left; itsrhythm was regular except for an occasional prema-ture beat, and no murmurs were present. All heartsounds were of average intensity. The liver extended14 cm. below the costal margin in the right midelavic-ular line and was moderately tender. The edge of thespleen could be felt 4 cm. below the costal margin.There was no peripheral edema.The urine had a specific gravity of 1.020 and con-
tained 3 plus albumin and an occasional hyaline orgranular cast. The red blood cell count was 6,140,000per cu. mm., and the hemoglobin content was 16.0Gm. The leukocyte count was 12,000 per cu. mm.The Wassermann reaction of the blood was negative.An electrocardiogram showed sinus tachycardia
with a rate of 116 per minute. The P waves werenotched in lead I. The P-R intervals were withinnormal limits but the duration of the QRS complexeswas increased to 0.11 second. There was slurring ofQRS in leads I and II, and right axis deviation waspresent. The T waves were inverted in leads II andIII.
Portable anteroposterior roentgenograms of thethorax were of poor quality but revealed opacity ofthe right thorax with slight displacement of thetrachea and mediastinum to the left. Extensive areasof increased density, suggestive of calcium deposits,were present within the area of a much enlargedcardiac shadow.
Digitoxin, mercurial diuretics, a low sodium diet,the administration of oxygen, and right thoracentesiswith the removal of 650 cc. of clear, straw coloredfluid resulted in only slight and temporary improve-ment. On the twelfth day in the hospital the patientsuddenly developed acute pulmonary edema anddied.
Postmortem examination revealed the heart to beCirculation, Volume III, May, 1951
greatly enlarged and to weigh 950 Gm. There werenumerous irregular, white projections of calcification
left auricle and ventricle were opened only withdifficulty, and cut sections of their walls revealedextremely extensive, irregular depositions of calcium(fig. 2). Only a small area on the posterior wall ofthe ventricle near the septum was not affected. Theinterventricular septum showed similar massive in-volvement but the right auricle and right ventriclewere affected to a much lesser degree (fig. 3). M\ulti-ple calcific excrescences were present on the endocar-dial surface of the left auricle, left ventricle andpulmonary conus, ranging up to 4 mm. in diameterand 5 mm. in elevation. The extent and degree of thecalcification in the heart as a whole was well demon
FIG. 1. Epicardial surface of the left ventricle andleft auricle. Numerous irregular projections of cal-cium are seen.
FIG. 2. Illustrating the extensive irregular calcifi-cation in the wall of the left ventricle and left auricle.
over the epicardial surface of the left auricle andventricle (fig. 1). The right heart appeared normalexternally except for dilatation of the auricle. The
FIG. 3. The right chambers of the heart have beenopened to demonstrate the much lesser extent of calci -fication in the wall of the right ventricle.
strated by roentgenograms of the fresh, isolatedorgan (fig. 4).The heart valves were normal. There were two
small mural thrombi in the left auricle and a singlethrombus of larger size was present near the apexof the left ventricle. The coronary arteries werenormal. There were 70 cc. of straw colored fluid inthe pericardial sac but the pericardium was normalexcept for two small, irregular roughened areas inthe posterior portion opposite one of the largercalcific protuberances. No adhesions were present.The aorta appeared somewhat small in diameterthroughout its entire length but was otherwisenormal.
Examination of the abdominal viscera and lungsrevealed severe chronic passive congestion. Righthydrothorax was present, and the lower lobe of the
FIG. 4. Roentgeiiogram of the fresh, isolated heartto demonstrate the e\tent almi degree of calcificationof the nivocardium.
right lung showed compression atelectasis and alarge area of recent infarction.
Microscopic examination of the myocardium re-vealed extensive areas of calcification within broadzones of cicatrization (fig. 5, left). Some of thecalcific masses included islands of bone formation(fig. 5, right). Muscle fibers marginal to regions offibrosis were to a considerable extent individuallyseparated by fibrous tissue continuous with thepartly calcified cicatrices. In addition, there werefocal areas of myocardial scarring without accom-panying calcific material. No areas of calcificationwere present in sections of the lungs and kidneys.
DIscussioN-It is of interest that the calcium deposits
in the cardiac area had been recognized byroentgenologic examination before the patient'sadmission to the hospital and the possibilityof chronic constrictive pericarditis with peri-cardial calcification had been suggested. Thepatient's general condition after admission pre-cluded additional detailed roentgenologie andfluoroscopic studies, and the significance of thecalcium deposits was not correctly assessedprior to the patient's death. It was believed,
FIG. 5 Left. Histologic section of the left ventricle my)ocardium showing an extensive area of cal-cification surrounded by a broad zone of dense connective tissue. X70. Right. Wall of left ventricleshowing areas of bone formation in a large calcific mass. X 100.
however, that the great enlargement of theheart was sufficient evidence to exclude chronicconstrictive pericarditis as the cause of theillness. In chronic constrictive pericarditis theheart is surrounded by a firm, vise-like scarwhich interferes with diastolic relaxation of theventricles and prevents hypertrophy and dilata-tion. Roentgenograms show the heart shadowto be of normal size or at the most only slightlyenlarged.The extensive myocardial calcification in the
present case probably resulted from a severetoxic or septic myocarditis but the reason forthe predominant involvement of the left auricleand ventricle and the interventricular septumis not known. The patient had experienced atleast three illnesses that might have been at-tended by acute myocarditis. The first of thesewas severe scarlet fever 16 years before heradmission to the hospital, the second was pneu-monia 4 years before admission, and the thirdwas an acute upper respiratory infection 2 yearsbefore the terminal illness. No decision can bemade as to whether any of these actually wasan etiologic factor. The most severe infectionapparently was the scarlet fever but it wouldseem most unusual for a patient to live for 16years after having experienced such severe dam-age to the myocardium.
SUMMARY
Extensive calcification of the myocardium,involving predominantly the left auricle andventricle and the interventricular septum, wasfound in a 25 year old woman who died ofcongestive heart failure. The etiology of thecondition was not determined but it is probablethat the deposition of calcium occurred duringthe healing phase of an earlier severe toxic orseptic myocarditis.
REFERENCES
1 HANES, F. M.: Hyperparathyroidism due to para-thyroid adenoma, with death from parathormoneintoxication. Am. J. M. Sc. 197: 85, 1939.
2SCHOLZ, T.: Calcification of the heart, its roent-genologic demonstration; review of the literatureand theories of myocardial calcification. Arch.Int. Med. 34: 32, 1924.
'DIAMOND, M.: Calcification of the myocardium ina premature infant. Arch. Path. 14: 137, 1932.
4VANBUCHEM, F. S. P.: Extensive calcification ofthe heart at an early age. Acta med. Scandinav.125: 182, 1946.
5EDELSTEIN, J. M.: Primary, massive calcificationwith ossification of the myocardium. Am. HeartJ. 31: 496, 1946.
6FINESTONE, A. J., AND GESCHICKTER, C. F.: Boneformation in the heart. Am. J. Clin. Path. 19:974, 1949.
is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231Circulation doi: 10.1161/01.CIR.3.5.690
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