Isolated coronary periarteritis: Report of a case of unexpected death in a young pregnant woman

Isolated Coronary Periarteritis : Report of a Case of Unexpected Death in a Young Pregnant Woman

JACQUES H . AHRONHEIM, MS, MD

Jackson, Michigan

A case of sudden unexpected death in a 23 year old woman in her 8th month of pregnancy is reported . Death occurred from isolated coronary periarteritis with thrombosis. It is believed that the thrombosis was secondary to the adventitlai Infiltrate, which was of a type suggesting a hypersensitivity factor. It is proposed that pathogenetically this case is identical to reported cases of dissecting hemorrhage or aneurysm of the coronary artery and represents an entity different from other forms of Inflammatory coronary disease .

This is the case of a young woman in the 8th month of pregnancy who died unexpectedly from isolated thrombosing coronary periarteritis .

Case Report

From the Office of the Medical Examiner, Jackson County, Jackson, Michigan. Manuscript received October 14, 1976 ; revised manuscript received November 24, 1976, accepted December 1, 1976 .

Address for reprints : Jacques H . Ahronheim, MD, 569 Wildwood Avenue, Jackson, Michigan 49201 .

The patient was a 23 year old white primipara in the 8th month of an essentially uneventful pregnancy . The date of expected delivery was November 28, 1974 . She had received the usual prepartum care . Her previous medical history was normal. There was no family history of cardiovascular disease but one sister had bronchial asthma . Her weight was 113 .4 kg when she was first seen by her physician in May 1974 but had decreased to 90 kg by September . Her blood pressure increased from 120/80 to 140/70 mm Hg during the same period . A blood count in June was normal with 1 percent eosinophils . The patient arrived at the emergency room of W . A. Foote Memorial Hospital, Jackson, Michigan, at 8 :35 PM on October 5,1974, complaining of nausea and retrosternal pain . Her temperature was 98 .6 F, blood pressure 160/80 mm Hg and pulse rate 100/min . Blood count revealed a mild anemia with 1 percent eosinophils . An electrocardiogram showed no diagnostic changes . She was given an antacid and Valium ® , 10 mg, and sent home at 10 :20 PM . At 11 :50 PM, when she returned with the same complaints, she was given Demerol ®, 75 mg, and again sent home with the diagnosis of anxiety. At 5 :45 the next morning she was dead on arrival at the hospital . The man with whom she lived stated that she woke up screaming, then fell back in bed and remained unresponsive . When the ambulance arrived, her pupils were dilated and fixed and resuscitation attempts were unsuccessful . Autopsy findings: There was evidence of resuscitation trauma on the anterior chest wall . The heart weighed 385 g . In the left anterior descending coronary artery, close to its origin, there was an apparent postmortem clot . The myocardium and aorta appeared normal . In the uterus there was a normally developed male fetus measuring 47 cm in length . The rest of the organs appeared normal . No cause of death was established on gross examination . On microscopic examination, the clot in the left anterior descending coronary artery was found to be a mixed thrombus occluding the lumen (Fig . 1) . The intima and subintimal stroma were moderately thickened and the stroma showed localized edema . A few lipophages were seen under the endothelial l ining . in one place, the thrombus was loosely attached to the intima and a small infiltrate of neutrophils was found superficially invading the intima . The muscular coat was normal . The adventitia showed moderate fibrous thickening and was densely infiltrated with cells that formed a cuff around the artery (Fig . 1) and consisted of neutrophils, eosinophils, lymphocytes and occasional histiocytes (Fig . 2) . Verhoeff's elastic stain revealed some flattening of the internal elastic membrane . A Van Gieson stain confirmed the increase in fibrous tissue in the subintimal

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FIGURE 1 . Occlusion of coronary artery by thrombus . Subintimal edema and a perivascular infiltrate are present . (Hematoxylin-eosin X40, reduced by 10 percent .)

stroma and adventitia. A mucicarmine stain was noncontributory . No changes were evident in the myocardium, intramural coronary branches or thoracic aorta . The diagnosis was acute thrombosis and periarteritis of the left anterior descending coronary artery causing sudden death in a young pregnant woman .

Review of Literature Nonatherosderotic coronary occlusion is rare in the clinical and pathologic material of the average community hospital . When it does occur, it is likely to come under the jurisdiction of the medical examiner, as it did in this case, and is therefore of particular interest to the forensic pathologist . In view of the suddenness of death, the majority of these cases have not been investigated clinically, depriving the clinician of valuable information and permitting only limited scientific study . There are numerous types of nonatherosclerotic coronary disease . Cheitlin et al .' present a long list of mostly unusual

pathologic entities including specific and nonspecific coronary arteritis, trauma, metabolic disease, embolism, congenital anomalies and many others . Some cases are morphologically indistinguishable from atherosclerosis . Vasicka and Line reported the case of a 21 year old woman who died postpartum from intimal thickening of a coronary artery causing almost complete occlusion . Young et al .' reported a similar nonfatal case of an 18 year old girl who had severe angina pectoris ; coronary stenosis was demonstrated at cardiac catheterization and angiography, and she was successfully treated with bypass surgery . Coronary disease due to tuberculosis is occasionally found in parts of the world where the latter disease is still common . 4 Lupus erythematosus involving the coronary arteries was reported by Bonfiglio et al . 5 Takayasu's disease, which focally involves the aorta, may extend into the coronary arteries where it presents as an inflammatory and degenerative process that gradually affects all layers of the coronary wall. 6- s A fairly new disease entity, first described by Kawa-

FIGURE 2 . Perivascular infiltrate consisting of neutrophils, eosinophils, lymphocytes and histiocytes . (Hematoxylin-eosin, oil immersion X 1000, reduced by 10 percent .)

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FIGURE 3. Dissecting hemorrhage in wall of coronary artery in a previously reported case's showing striking similarity of the perivascular Infiltrate with that shown in Figure 1 . (X35, reduced by 20 percent .) (Reproduced with permission of the editor of the Archives ofPathology.1 e )

saki 9 in 1967, is acute febrile mucocutaneous lymph node syndrome . It is found in infancy and early childhood and closely resembles infantile periarteritis nodosa . Fatal cases uniformly show occlusion of the coronary arteries and, in some instances, coronary aneurysms .'°•' I Immunologic factors may be the underlying cause of coronary disease in some of the collagen diseases . In rheumatoid arteritis the disease may become manifest in the intramural branches of the coronary arteries, which are narrowed and show signs of healed angiitis, although the extramural branches may be normal ." Zeek,ls in her classification of necrotizing angiitis, lists allergic granulomatous, rheumatic and hypersensitivity angiitis commonly affecting the heart . Saphir et al . 14 found changes suggesting old periarteritis in the intramural branches of the coronary arteries in 32 of 82 cases and a history of hypersensitivity in 12 of 100 cases of coronary atherosclerosis . Immunologic factors having a bearing on inflammatory coronary disease are suggested by several experimental studies .'s-17

Discussion The principal feature of this case was the unexpected death from coronary thrombosis of nonatherosclerotic origin of a young woman in the last trimester of pregnancy . The main pathologic changes were in the adventitia, which showed an inflammatory infiltrate of a type suggesting a hypersensitivity factor . The finding was isolated and found in no other part of the body or any other level of the coronary system . The localized involvement and the lack of changes in the muscular coat distinguish this case from other cases of nonatherosclerotic: coronary disease . In 1959 Wagman and I reported the sudden death of a 41 year old woman from what we called "dissecting hemorrhage in the media of

the coronary artery ." The artery manifested the identical inflammatory infiltration in the adventitia found in the present case . In retrospect and in the light of the present findings, I now consider that both cases represent the same entity . The striking similarity of the adventitial changes in the two cases is shown in Figures 1 and 3 . If this interpretation is correct, one may assume that the adventitial infiltrate is the primary pathologic finding and the intramural hemorrhage and the thrombosis in the respective cases are secondary . It then appears justified to identify as the same entity previously reported cases diagnosed as dissecting hemorrhages or hematomas in coronary arteries19 22 or, perhaps not quite correctly in the strict sense of the term, as aneurysms. 23,24 All these cases are isolated and uniformly show the identical adventitial infiltrate of neutrophils, eosinophils, lymphocytes and histiocytes . A relation probably exists between the pregnancy and the fatal disease in this case . Katz25 found only one case of "Herzmuskelmalazie," presumably myocardial infarct, in 95 cases of sudden death during pregnancy and puerperium . More recently, several such cases have been reported in pregnant and postpartum women . Asuncion and Hyun 22 added 3 cases to 7 reported since 1952 ; of these 10 cases, 2 occurred during the 9th month of pregnancy, 7 during the immediate postpartum period and only 1 as late as 80 days post partum .

Acknowledgment The courtesy of the administration of The W . A. Foote Memorial Hospital, Jackson, Michigan, in granting me use of the hospital emergency record is gratefully acknowledged .

References 1. 2. 3.

Cheitlin MD, McAllister HA, deCastro CM : Myocardial infarction without atherosclerosis . JAMA 231 :951-959, 1975 Vaslcka Al, Lin TJ : Fatal coronary artery disease during early post partum period . Am J Obstet Gynecol 77 :899-904, 1959 Young JA, Sengupta A, Khaja F : Coronary arterial stenosis, angina

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pectoris and atypical coarctation of the aorta due to nonspecific arteritis. Am J Cardial 32 :356-360, 1973 Kinare SG, Bhatia BI : Tuberculous coronary arteritis with aneurysm of the ventricular septum . Chest 60 :613-616, 1971 Bonfiglie TA, Bettl RE, Hagstrom JWC: Coronary arteritis, oc-

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clusion and myocardial infarction due to lupus erythematosus . Am Heart J 83 :153-158, 1972 6 . Lands A, LaPorla A: Takayasu arteries . Arch Pathol Lab Med 100 :437-440, 1976 7 . Rosen N, Gallon E Takayasu's arterlts of coronary arteries . Arch Pathol 94:225-229, 1972 8 . Salto Y, Hlrota K, No I, et al : Clinical and pathological studies of five autopsied cases of aortitis syndrome. Jpn Heart J 13 :107-117, 1974 9 . Kawasaki T: Acute febrile mucocutaneous lymphoid involvement with specific desquamation of the fingers and toes in children . Jpn J Allerg 16:178-181, 1967 10 . Tanaka N, Sekimoto K: Kawasaki disease . Arch Pathol Lab Med 100 :81-86, 1976 11 . Yanagisawa M, Kobayashi N, Matsuya S : Myocardial infarction due to coronary thromboarteritis following acute febrile mucocutaneous lymph node syndrome in an infant . Pediatrics 54:277-281, 1974 12 . Case Records of the Massachusetts General Hospital . N Engl J Med 285 :1250-1257, 1971 13 . Zeek MP: Periarteritis nodosa : a critical review . Am J Clin Pathol 22 :777-790, 1952 14 . Saphir 0, Ohdnger L, Wong R : Changes in the intramural coronary branches in coronary arteriosclerosis . Arch Pathol 62 :159-170, 1956 15 . Harkavy J : Cardiac manifestations due to hypersensitivity . Ann

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Allergy 28:242-251, 1970 16 . Porter DD, Larsen AE, Porter HG : The pathogenesis of aleutian disease of mink . Am J Pathol 71 :231-238, 1973 17 . Leuchtenberger C, Leuchtenberger R : Differential response of Snell's and C57 black mice to chronic inhalation of cigarette smoke . Oncology 29:122-138, 1974 18 . Ahronhelm JH, Wagman CF : Dissecting hemorrhage in media of coronary artery. Arch Pathol 67 :19-23, 1959 19 . Lovitt WV, Corzine WJ: Dissecting intramural hemorrhage of anterior descending branch of left coronary artery . Arch Pathol 54: 458-462,1952 20 . Nalbandlan RM, Chosen JL : Intramural (intramedial) dissecting hematomas in normal or otherwise unremarkable coronary arteries . Am J Clin Patrol 43 :348-356, 1965 21 . Palomino SJ : Dissecting intramural hematoma of left coronary artery In the puerperium . Am J Clin Pathol 51 :119-125, 1969 22 . Asuncion CM, Hyun J : Dissecting intramural hematoma of the coronary artery in pregnancy and the puerperium . Obstet Gynecol 40:202-210, 1972 23 . Roy JJ, Klein HZ: Dissecting aneurysm of the coronary artery . JAMA 218 :1047-1048, 1971 24 . Smith JC : Dissecting aneurysms of coronary arteries . Arch Pathol 99 :117-121, 1975 25 . Katz H : Ueber den ploetzlichen natuerlichen Tod In Schwangerschaft, Geburt and Wochenbett . Arch Gynaek 115 :283-288, 1922

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