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Massive cardiac hypertrophy
MASSIVE
CARDIAC
HYPERTROPHY
A CASE REPORT JOSEPH C. DOAXE,
M.D., AND NORMAN J. SKVERSKP, M.D. PHILADELPHIA,
PA.
T IS not uncommon to find large hearts at the autopsy table, but weights in excess of 1,000 grams are rare, if one may judge by the number of case reports. In the past one hundred years, less than fifty cases have been reported in which the heart weighed more than 1,000 grams. In the majority of instances, such enlarged hearts were found to be the seat of valvular deformities, either single or multiple, of adhesive pericarditis, or of some sort of congenital defect1 In addition, there were usually other abnormalities, such as high blood pressure, chronic pulmonary disease, hyperthyroidism, chronic nephritis, deformities of the t,horax, peripheral arteriovenous fistulae, von Gierke’s disease, myxedema, beriberi, myocardial infarction, and interstitial myocarditis (Fiedler ‘s myoca,rditis) .2-4 In approximately 15 per cent of these cases, however, the cause of the massive hypertrophy was not discovered. These cases have been classed by some pathologists under the heading of idiopathic cardiac hypertrophy. We desire to present a case of cardiac hypertrophy which was discovered clinically and confirmed at autopsy in a patient who had had previous myocardial infarction without actual coronary occlusion. CASE REPORT
A 66-year-old white physician walked into the Jewish Hospital Nov. 22, 1942, stating that, while engaged in his usual professional rounds, he was seized with severe precordial pain which radiated to the left shoulder and down the left arm. He had marked shortness of breath. Three weeks earlier he had a similar attack while attending a wrestling match. At that time he was hospitalized for one day and was discharged symptom free. An electrocardiogram then showed no evidence of recent cardiac damage. The past medical history was irrelevant except that for the preceding year he had had numerous sore throats and had taken small doses of sulfonamides frequently. He was a sthenic person who had been active athletically all of his life. Physical examination disclosed a man who appeared extremely ill. His color was ashen. His skin was cold and clammy. The respirations were 40 per minute. The rectal temperature was 97” F. The blood pressure was 152/92 in the right arm and 142/90 in the left arm. His pulse was weak and thready, and its rate was 150 per minute. The heart sounds were distant. There was a protodiastolic gallop at the base, and occasional extrasystoles were heard. The left border of the heart extended to the anterior axillary line. The lungs were normal. From the Medical Service of the Jewish Received for publication July 5, 1943.
816
Hospital,
Philadelphia,
Pa.
‘Ihe edge of the liver was palpable 4 cm. below the right costal, margin. There was evidence of periphera! arteriosclerosis, but no edema. Emergency treatment consisted of morphine sulfate (34 grain), atropine snlfate (l/lco grain), papaverine hydrochloride (l-grain), and the use of an oxygen tent. The patient had a leucocytosls of 13,000, with a polymorphonuclear count of 90 per cent. The erythrocyte sedimentation rate was 18 mm. in one hour (our normal, 9 mm.). An eleetrocardiogram at this time showed a relatively rapid taehycardia, with left bundle branch block. During the first few days the patient’s eondition remained critical. Gallop rhythm persisted. Dyspnea and cyanosis were marked. The blood pressure fell to 110/70, and moisture appeared at the bases of both lungs. The patient was slowly digitalized, and soon appeared and felt mu.ch improved. Convaieacence was uneventful until the evening of Dee. 11, 1942, when he developed, while speaking to his relatives, sudden, flaccid, right-sided hemiplegia, with aphasca. The intravenous administration of a grain of papaverine resulted in the return of speech in two or three minutes, and he was soon able to move his right arm and leg. ES?the next day> no residual signs remained. On Dec. 20, 1942, the patlent enjoyed a hearty breakfast and sat up in bed to read a newspaper. Twenty minrites later he was found dead. Autopsy was performed two hours after death. The heart weighed 4,450 grams. The left ventricular wall varied from 21 to 26 mm., and the right ventricular wall, from 5 to 6 mm., in thickness. Noderate coronary atherosclerosis was present, but the lnmina of the vessels were very wide; the coronaries measured as much as 1 cm. in eircumferenee. There was no evidence of occlusion. On the lateral aspect of the left ventricle an area of necrosis and mncoid degeneration, w%h hemorrhage, approximately 3 cm. in diameter, was seen. ‘rhe cardiac musculature showed moderate fibrosis, especially near the apex. The papillary muscles were markedly hyper:rophied and fibrotic. Microscopic sections of the heart muscle showed hypertrophy of all fibers. The amount of fibrous interstitial tissue was increased, a’nd active fibroblastic proliferation was present. There was no evidence of perivascular inflammation. Sections of the area of myoeardial necrosis revealed degeneration and necrosis of muscle fibers, invasion 13~ fibroblasts, and early collagen deposition and Iymphocytie infiltration. COMMENT
x zase of massive cardiac hypertrophy is presented, in which there were no valvular lesions or other possible causes for the ei~Iargel~~e~~;~ In considering the diagnostic possibilities, for the sake of completeness, at least, one should not forget myocarditis of unknown origin, as described by Fiedler,*-” which occurs with no other de~no~stra?~ledisease that can be correlated with the cardiac state. The appea.ranee of this zype of interstitial myoearditis in apparently healthy persons who more or less suddenly develop progressive myocardial failure has been described by a number of other investiga.tors. Such patients are prone SOsuddenly develop dyspnea, cyanosis, tachyeardia, weakness, and to die suddenly, as was the case with our patient. In most of the eases reported, however, the patients were between the ages of 20 and 50 years. Microscopic examination of these hearts shows diffuse infl-lra-
818
AMERICAN
HEART
JOURNAL
tion of interstitial tissue by lymphocytes, monocytes, and, to a lesser degree, polymorphonuclear, eosinophile, and plasma cells. Numerous fibroblasts and new blood vessels are also observed. The pathologic and histologic picture in our case was not that of myocarditis. The athletic history of the patient ma.y have had some bearing. The case is of unusual interest, not only because of the size of the heart, but because of the absence of explanatory pathologic changes within the heart itself or elsewhere. REFERENCES
la.
Golden, J. S., and Brams, W. A.: Extreme Cardiac Enlargement, A&f. HEART J. 13: 207, 1937. b. Rosenow, E. C., Jr., and Smith, H. L.: Extreme Cardiac Hypertrophy (Minnesota Med. 22: 739, 1939. 2. Bartels, E. C., and Smith, H. L.: Gross Cardiac Hypertrophy in Myocardial Infarction, Am. J. M. SC. 184: 452, 1932. 3. Fiedler, A.: Ueber akute interstitielle Myokarditis, Centralbl. f. inn. Med. 21: 212, 1900. 4. Englehardt, H. T., and Bruno, F. E.: Fiedler ‘s Myocarditis, New England J. Med. 228: 7, 1943. 5. Saphir, 0.: Isolated Myocarditis, AK HEART J. 24: 167, 1942. 6. Simon, M. A., and Wolpaw, 8.: Acute, Subacute and Chronic Isolated Myocarditis, Arch. Int. Med. 56: 1136, 1935.
CARDIAC
HYPERTROPHY
A CASE REPORT JOSEPH C. DOAXE,
M.D., AND NORMAN J. SKVERSKP, M.D. PHILADELPHIA,
PA.
T IS not uncommon to find large hearts at the autopsy table, but weights in excess of 1,000 grams are rare, if one may judge by the number of case reports. In the past one hundred years, less than fifty cases have been reported in which the heart weighed more than 1,000 grams. In the majority of instances, such enlarged hearts were found to be the seat of valvular deformities, either single or multiple, of adhesive pericarditis, or of some sort of congenital defect1 In addition, there were usually other abnormalities, such as high blood pressure, chronic pulmonary disease, hyperthyroidism, chronic nephritis, deformities of the t,horax, peripheral arteriovenous fistulae, von Gierke’s disease, myxedema, beriberi, myocardial infarction, and interstitial myocarditis (Fiedler ‘s myoca,rditis) .2-4 In approximately 15 per cent of these cases, however, the cause of the massive hypertrophy was not discovered. These cases have been classed by some pathologists under the heading of idiopathic cardiac hypertrophy. We desire to present a case of cardiac hypertrophy which was discovered clinically and confirmed at autopsy in a patient who had had previous myocardial infarction without actual coronary occlusion. CASE REPORT
A 66-year-old white physician walked into the Jewish Hospital Nov. 22, 1942, stating that, while engaged in his usual professional rounds, he was seized with severe precordial pain which radiated to the left shoulder and down the left arm. He had marked shortness of breath. Three weeks earlier he had a similar attack while attending a wrestling match. At that time he was hospitalized for one day and was discharged symptom free. An electrocardiogram then showed no evidence of recent cardiac damage. The past medical history was irrelevant except that for the preceding year he had had numerous sore throats and had taken small doses of sulfonamides frequently. He was a sthenic person who had been active athletically all of his life. Physical examination disclosed a man who appeared extremely ill. His color was ashen. His skin was cold and clammy. The respirations were 40 per minute. The rectal temperature was 97” F. The blood pressure was 152/92 in the right arm and 142/90 in the left arm. His pulse was weak and thready, and its rate was 150 per minute. The heart sounds were distant. There was a protodiastolic gallop at the base, and occasional extrasystoles were heard. The left border of the heart extended to the anterior axillary line. The lungs were normal. From the Medical Service of the Jewish Received for publication July 5, 1943.
816
Hospital,
Philadelphia,
Pa.
‘Ihe edge of the liver was palpable 4 cm. below the right costal, margin. There was evidence of periphera! arteriosclerosis, but no edema. Emergency treatment consisted of morphine sulfate (34 grain), atropine snlfate (l/lco grain), papaverine hydrochloride (l-grain), and the use of an oxygen tent. The patient had a leucocytosls of 13,000, with a polymorphonuclear count of 90 per cent. The erythrocyte sedimentation rate was 18 mm. in one hour (our normal, 9 mm.). An eleetrocardiogram at this time showed a relatively rapid taehycardia, with left bundle branch block. During the first few days the patient’s eondition remained critical. Gallop rhythm persisted. Dyspnea and cyanosis were marked. The blood pressure fell to 110/70, and moisture appeared at the bases of both lungs. The patient was slowly digitalized, and soon appeared and felt mu.ch improved. Convaieacence was uneventful until the evening of Dee. 11, 1942, when he developed, while speaking to his relatives, sudden, flaccid, right-sided hemiplegia, with aphasca. The intravenous administration of a grain of papaverine resulted in the return of speech in two or three minutes, and he was soon able to move his right arm and leg. ES?the next day> no residual signs remained. On Dec. 20, 1942, the patlent enjoyed a hearty breakfast and sat up in bed to read a newspaper. Twenty minrites later he was found dead. Autopsy was performed two hours after death. The heart weighed 4,450 grams. The left ventricular wall varied from 21 to 26 mm., and the right ventricular wall, from 5 to 6 mm., in thickness. Noderate coronary atherosclerosis was present, but the lnmina of the vessels were very wide; the coronaries measured as much as 1 cm. in eircumferenee. There was no evidence of occlusion. On the lateral aspect of the left ventricle an area of necrosis and mncoid degeneration, w%h hemorrhage, approximately 3 cm. in diameter, was seen. ‘rhe cardiac musculature showed moderate fibrosis, especially near the apex. The papillary muscles were markedly hyper:rophied and fibrotic. Microscopic sections of the heart muscle showed hypertrophy of all fibers. The amount of fibrous interstitial tissue was increased, a’nd active fibroblastic proliferation was present. There was no evidence of perivascular inflammation. Sections of the area of myoeardial necrosis revealed degeneration and necrosis of muscle fibers, invasion 13~ fibroblasts, and early collagen deposition and Iymphocytie infiltration. COMMENT
x zase of massive cardiac hypertrophy is presented, in which there were no valvular lesions or other possible causes for the ei~Iargel~~e~~;~ In considering the diagnostic possibilities, for the sake of completeness, at least, one should not forget myocarditis of unknown origin, as described by Fiedler,*-” which occurs with no other de~no~stra?~ledisease that can be correlated with the cardiac state. The appea.ranee of this zype of interstitial myoearditis in apparently healthy persons who more or less suddenly develop progressive myocardial failure has been described by a number of other investiga.tors. Such patients are prone SOsuddenly develop dyspnea, cyanosis, tachyeardia, weakness, and to die suddenly, as was the case with our patient. In most of the eases reported, however, the patients were between the ages of 20 and 50 years. Microscopic examination of these hearts shows diffuse infl-lra-
818
AMERICAN
HEART
JOURNAL
tion of interstitial tissue by lymphocytes, monocytes, and, to a lesser degree, polymorphonuclear, eosinophile, and plasma cells. Numerous fibroblasts and new blood vessels are also observed. The pathologic and histologic picture in our case was not that of myocarditis. The athletic history of the patient ma.y have had some bearing. The case is of unusual interest, not only because of the size of the heart, but because of the absence of explanatory pathologic changes within the heart itself or elsewhere. REFERENCES
la.
Golden, J. S., and Brams, W. A.: Extreme Cardiac Enlargement, A&f. HEART J. 13: 207, 1937. b. Rosenow, E. C., Jr., and Smith, H. L.: Extreme Cardiac Hypertrophy (Minnesota Med. 22: 739, 1939. 2. Bartels, E. C., and Smith, H. L.: Gross Cardiac Hypertrophy in Myocardial Infarction, Am. J. M. SC. 184: 452, 1932. 3. Fiedler, A.: Ueber akute interstitielle Myokarditis, Centralbl. f. inn. Med. 21: 212, 1900. 4. Englehardt, H. T., and Bruno, F. E.: Fiedler ‘s Myocarditis, New England J. Med. 228: 7, 1943. 5. Saphir, 0.: Isolated Myocarditis, AK HEART J. 24: 167, 1942. 6. Simon, M. A., and Wolpaw, 8.: Acute, Subacute and Chronic Isolated Myocarditis, Arch. Int. Med. 56: 1136, 1935.