Cholesterol pericarditis

Case Reports Cholesterol Pericarditis* ERNEST W . CROW, M .D . Wichita, Kansas

I

N 1919, Alexander' reported a case of peri-

hospital admission although his symptoms had become progressively more severe .

cardial effusion with a "gold paint" ap-

pearance due to the presence of cholesterol crystals .

Physical examination on admission revealed that the blood pressure was 100/78 mm . Hg in both arms and the pulse rate 72 per minute . No abnormality of the eyes, ears, nose or throat was noted . The thyroid gland was normal in size, but neck veins were distended . Cardiac dullness extended from the right

Since this classic description, only a

few additional cases have been reported . The present case is added to the literature because of its rarity and since it presents some other important features : (1) an unusually long dura-

mid-clavicular line to the left anterior axillary line . Cardiac sounds could barely be heard and precordial pulsation was not present . Peripheral pulses were normal and a paradoxic pulse was not present . The liver was palpable about 6 cm . below the right costal margin, did not pulsate, was moderately tender, firm and had a sharp edge . Signs of as cites were not present . Rectal examination was normal . Edema

tion of pericardial effusion, probably lasting eight years and possibly sixteen years ; (2) striking freedom from symptoms for many years, in spite of a large pericardial effusion ; and (3) apparent arrest after thoracotomy and pericardial biopsy . CASE REPORT

was present up to the knee level and severe varicosities, involving the superficial saphenous system, were present bilaterally . Laboratory Data . Urinalysis, red cell count, hemoglobin, hematocrit, white cell count, differential, fasting blood sugar and urea nitrogen were normal . The Kline test was negative . Serum glutarnic oxaloacetic

On July 18, 1956, a forty-one year old aircraft manufacturing executive was admitted to the hospital with complaints of wheezing, shortness of breath and fatigue . In 1940 he consulted a physician because of fatigue and was found to have a pericardial effusion . Three pericardial aspirations were performed . The patient recalled that the fluid was straw-colored . Bedrest was prescribed for ten months with a presumptive diagnosis of tuberculosis . Although he was told that the effusion was still present, he chose to return to work and has worked full time since then . Ile was admitted to the hospital in 1948 for treatment of a perirectal abcess, at which time an x-ray film of the chest was taken (Fig . 1, May 26, 1948) . He was again hospitalized in 1949 for a perirectal abscess and had an incision of the abscess and saucerization of the fistula . From 1948 to 1955 he was seen regularly by his physician, who fluoroscoped him frequently and each time an enlarged cardiac shadow was demonstrated .

transaminase was 26 units and the sedimentation rate (Wintrobe) 25 mm . per hour . L . E . cell study was negative on five occasions . C reactive protein was negative . Serum calcium was 9 .4 mg . per cent, cholesterol 162 mg. per cent and cholesterol esters 88 mg . per cent . Thymol turbidity, serum bilirubin, alkaline phosphatase and cephalin flocculation were normal . Protein-bound iodine was 4 .5 pg. per cent . A .S .O . titer measured 166 Todd units . Electrophorctic study showed a total protein of 7 .95 gin . The various particles in per cent of total were : alpha globulin, 23 per cent ; beta globulin, 21 per cent ; alpha 2 globulin, 14 per cent ; alpha 1 globulin, 10 per cent ; albumen, 32 per cent ; P"t uptake was 36 per cent in twenty-four hours and 20 per cent in six hours . I's' excretion was 58 per cent in twenty-four hours . The histoplasmin and coccidioidin skin tests were negative . The tuberculin skin test (P.P.D .) (0.001 mg .) was 1 plus . The electrocardiogram dated July 19, 1956, is

He felt well until about April 1956, when he began to note swelling of his legs, shortness of breath on exertion, fatigue, orthopnea and night sweats . He recalled a respiratory infection about December 1955, and again in April 1956 . Varicose veins had been noted for one year . He worked until the day of his

• From the Department of Medicine, Wesley Hospital, Wichita, Kansas . 120

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Fro . 1 . Teleroentgenograms . Note the large cardiac silhouette present in 1948, the further increase in size apparent on the 1956 posteroanterior and lateral film, and the striking reduction seen in 1957 following surgery .

shown in Figure 2, Posteroanterior and lateral x-ray film of the chest, dated July 29, 1956, is shown in Figure 1 . Venous pressure was 17 .2 cm . of water in Circulation time, arm-to-lung, was the right arm .

twenty-four hours, following which he began to have a temperature of 100 to 103 °F ., accompanied by chilly sensations . He was given procaine penicillin, 600,000 units twice a day, for five days .

nine seconds by the ether method and the arm-totongue time was twenty seconds, using Decholine . On July 21, 1956, pericardial paracentesis was perThe fluid formed and 2 .600 cc . of fluid removed .

He left the hospital on August 1, 1956, without being totally relieved, although he was somewhat improved . The fever gradually disappeared and there was nearly complete disappearance of edema of the lower extremities . Further Course and Surgical Findings : On October 1, 1956, the patient had an episode of vomiting and diarrhea followed by daily temperature elevations to 100 to 102 ° F ., which continued until he was rehospitalized on October 19, 1956 . He experienced progressive dyspnca and orthopnea during the week prior to admission and ankle edema appeared three to four days prior to admission . Physical findings were essentially those of his previous admission . On October 19 and October 21, 1956, pericardial aspirations were done with removal of 1,000 cc . of

was the color of strong coffee, somewhat cloudy, and suspended in the fluid were innumerable. tiny goldcolored flecks, giving it the appearance of gold paint . Examination of the pericardial fluid showed the specific gravity to be 1 .022, and the cell count 11,623 with 43 per cent lymphocytes and 57 per cent segmented cells . Total protein was 6 .9 gm . per cent . A smear for acid-fast bacillus was negative, as was a culture and guinea pig inoculation . Microscopic examination of of the centrifuged material showed numerous cholesterol crystals (Fig . 3) . Following aspiration, the patient felt well for about JANUARY 1961



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1-17-58 Electrocardiograms before and after surgery,

fluid each lime . On October 26, 1958, thoracotomy and pericardial biopsy were performed . Laboratory tests on the pericardial fluid removed at the time of surgery were as follows : Cholesterol crystals, numerous ; sugar, 72 mg . per cent ; total protein, 6 .0 mg. per cent ; chloride . 612 mg . per cent ; specific gravity, 1 .018 ; white blood cell count, 280 per cu . mm . ; differential : polymorphonuclear leucocytes, 36 per cent ; lymphocytes, 64 per cent ; red blood cell count, 240,000 per cu . mm . ; malignant cells, negative ; total fat, 1,100 mg . per 100 cc. ; culture, negative . On October 27, 1956, serum cholesterol was 160 mg . per cent and total fat, 1,200 mg . per 100 cc.

Specimens for biopsy were taken from the epicardial and parietal pericardium and were stained for fungi by the Schiff technic . None was found . Microscopic examination of the tissue revealed proliferative fibrosis which, in some areas, revealed hyalinization . Foci of lymphocytes, plasma cells and foreign body giant cells were scattered through the tissue . There were tiny areas of hemorrhagic extravasation . Several thick-walled blood vessels of the capillary type were preset t . So tumor cells were seen . Cleft-like spaces characteristic of cholesterol deposition were present . No tubercles were seen and no acid-fast bacilli identified (Fig . 4) . Postoperatively the patient had daily fever but was dismissed from the hospital on -November 10, 1956 . During hospitalization, following surgery, he was given 400,000 units of procaine penicillin and 0 .5 gm . of streptomycin twice daily for eleven days . lie had a temperature of from 100 to 102 ° r . daily for six weeks following discharge. However, edema of the lower extremities, ascites and liver size diminished while he was still in the hospital and by March 1957 he was feeling well, worked full time and had only a trace of edema of the legs . By July 1957 the liver was no longer palpable and only an occasional trace of edema of the lower extremities was noted . No further symptoms have developed to date, and subsequent

FIG . 3 . Photomicrograph of centrifuged fluid from pericardial cavity showing cholesterol crystals .

x-ray films of the chest show no change in cardiac size (film dated July 16, 1957, Fig . 1) . The electroTHE AMERICAN JOURNAL OF CARDIOLOGY

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B FIG . 4 . A, photomicrograph of visceral pericardium showing cholesterol clefts and fibrous thickening ; hematoxylin and eosin X100 . B, high power photomicrograph showing cholesterol clefts and hyalinized fibrous tissue ; hematoxylin and eosin X44 .

cardiogram continued to show abnormal T waves (Fig . 2, January 17, 1958) . COMMENTS

The etiology of cholesterol pericarditis is unknown . In three cases reported,' , ', ' each author suggests myxedema as the etiology, but in none of the remaining cases has this been present . In our case there was no clinical evidence of hypothyroidism and the proteinbound iodine and 1 151 studies were normal . All authors have suspected tuberculosis, but in only one case s has the tubercle bacillus been found . In the case herein reported, a careful search for tubercle bacillus in the fluid, by smear, culture and guinea pig inoculation, was negative . In addition, biopsy tissue of pericardium, both visceral and parietal, showed nothing typical of tuberculosis . Review of the sections taken from the rectal fistula did not show characteristics of tuberculosis, Hemopericardium has been suggested as a possible cause and, indeed, some experimental support for this has been presented by Ehrenhaft, 10 who also demonstrated that the lipid fraction of blood more consistently produced a pericardial reaction when injected into the pericardium than did whole blood . This reaction, however, was not typical of cholesterol pericarditis. Idiopathic pericarditis of benign type is now JANUARY

1961

frequently recognized, and occasionally has chronic manifestations ." Large asymptomatic pericardial effusions have been reported in two brothers," and the fluid in one contained cholesterol crystals . The author suggested that the cholesterol crystals may have resulted from the very slow absorptive properties of the pericardium allowing slow degradation of lipoprotein complexes . Five of the patients reviewed died 2,1.7-9 but six were alive at the time they were reported . Of these, one' improved with administration of thyroid extract ; the patient presented herein and one others improved after thoracotomy and pericardial biopsy ; another patient improved after repeated aspirations,' twos," improved after pericardiectomy, and one 12 improved after aspiration and antituberculosis therapy . The removal of the entire parietal pericardium, if feasible, might remove the dangers of constrictive pericarditis . It is interesting to note, however, that none of the patients autopsied or surgically explored has shown constrictive pericarditis, so proof that this may result is still lacking . A remarkable feature of this case is the duration of effusion . There is little doubt that the patient had continuous effusion from 1948 until surgery in 1956 . It is possible that it was continuously present from 1940 to 1956 al-

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Some

2 . DANIEL, G . and PUDER, S . Pcricarditis and pleuritis

other reported casess• i 2,1s were also of long duration, but none beyond eight years . This

cholesterinea . Virchuw's Arch. path . Anat ., 284 : 853, 1932 . Am . 3 . MERRILL, A . J . Cholesterol pericarditis . Heart J ., 16 : 505, 1938 . 4 . HOWARD, .J . W. Myxedema with cholesterosis and massive pericardial effusion . Delaware M . J, 16 : 150, 1946 . 5 . ADA, A . E . W ., JONES, 0. S . and SIIEERAN, A . D . Cholesterol pcricarditis . .7. T horacic Surg ., 20 : 28, 1950 . 6 . CREECH, 0 ., HICKS, W . M ., SNYDER, H . B . and ERICKSON, E, E . Cholesterol pericarditis, successful treatment by pericardiectomy . Circulation, 12 : 193, 1955 . 7 . MoE, A . S. and CAMPOS, F. J. Cholesterol pericarditis . Ann . lot . Med ., 47 : 817, 1957 . 8 . SANDRI . S . and CAVmcas, E . Cholesterol pericarditis . Athena, 23 : 88, 1957 . 9 . CosTEAS, F ., YATZIDIS, H ., CosoAKus, A . and MICRAELIDES, G. Cholesterol pericarditis, complete anatomical and clinical study . Presre used., 65 : 336, 1957 . 10 . EHRENHAFr, J . L . and TABER, R . E . Hemopericardium and constrictive pcricarditis . J. Thoracic Surg., 24 : 355, 1952 . 11 . FRIEDBERG, C . K . Diseases of the Heart, p . 603 . Philadelphia and London, 1956 . W. B . Saunders Co . 12 . ArrxEN, G . J . Cholesterol pericarditis . Scottish M . J ., 4 : 254, 1957 . 13, GENECIN, A . Chronic pericardial effusion in brothers with a note on cholesterol pericarditis . Am . J . ., 26 : 496, 1959 . Med

though

this

cannot

be

documented .

emphasizes the remarkable adaptability the circulation to a slowly developing pericardial effusion . case

of

The patient showed improvement following biopsy. Why this occurred

thoracotomy and is not clear .

The possibility that antibiotics,

given postoperatively, could have influenced the course

of

the disease must be considered .

This seems unlikely since he continued to have a daily fever for six weeks after all antibiotics were stopped . None

of

the patients reported, as well as the

present one, showed a significantly elevated serum cholesterol . SUMMARY An unusual case

of

cholesterol pericarditis is

presented and the literature reviewed . The etiology is still unknown and the treatment is not established . REFERENCES 1 . ALEXANDER, .I . S . A pericardial effusion of "gold paint" appearance due to the presence of cholesterin . Brit . M. J ., 2 : 463, 1919 .

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