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Spontaneous Coronary Arterial Dissection and Isolated Eosinophilic Coronary Arteritis: Sudden Cardiac Death in a Patient With a Limited Variant of Churg-Strauss Syndrome

1992; Elsevier BV; Volume: 67; Issue: 8 Linguagem: Inglês

10.1016/s0025-6196(12)60801-5

1942-5546

John C. Hunsaker, William N. O’Connor, J. T. Lie,

Vascular Anomalies and Treatments

Isolated eosinophilic coronary arteritis expressed as a limited variant of the Churg-Strauss syndrome (allergic granulomatosis and angiitis) is a rare condition. Equally as rare is the entity of isolated spontaneous coronary arterial dissection associated with eosinophilic arteritis. A 57-year-old woman with a history of asthma and recurrent hypersensitivity (anaphylactoid) reactions to various exogenous allergens was found dead in her home; no premonitory complaints had been noted during the preceding days. Autopsy revealed focal occlusion of the left anterior descending and first diagonal coronary arteries by discrete dissecting hematomas of the media as the cause of sudden and unexpected death. Histologically, the affected arterial wall showed eosinophilic inflammation characteristic of this limited expression of the Churg-Strauss syndrome. To our knowledge, sudden cardiac death caused by arterial dissection in isolated eosinophilic coronary arteritis has not previously been reported. Isolated eosinophilic coronary arteritis expressed as a limited variant of the Churg-Strauss syndrome (allergic granulomatosis and angiitis) is a rare condition. Equally as rare is the entity of isolated spontaneous coronary arterial dissection associated with eosinophilic arteritis. A 57-year-old woman with a history of asthma and recurrent hypersensitivity (anaphylactoid) reactions to various exogenous allergens was found dead in her home; no premonitory complaints had been noted during the preceding days. Autopsy revealed focal occlusion of the left anterior descending and first diagonal coronary arteries by discrete dissecting hematomas of the media as the cause of sudden and unexpected death. Histologically, the affected arterial wall showed eosinophilic inflammation characteristic of this limited expression of the Churg-Strauss syndrome. To our knowledge, sudden cardiac death caused by arterial dissection in isolated eosinophilic coronary arteritis has not previously been reported. Isolated eosinophilic coronary arteritis as a limited variant of the Churg-Strauss syndrome that causes sudden death was first described by Lie and Bayardo1Lie JT Bayardo RJ Isolated eosinophilic coronary arteritis and eosinophilic myocarditis: a limited form of Churg-Strauss syndrome.Arch Pathol Lab Med. 1989; 113: 199-201PubMed Google Scholar in 1989. Later that year, a similar case was independently reported by Kajihara and associates.2Kajihara H Kato Y Takanashi A Nakagawa H Tahara E Otsuki T Tsuchioka Y Amioka H Okamoto M Matsuura H Kajiyama G Periarteritis of coronary arteries with severe eosinophilic infiltration: a new pathologic entity (eosinophilic periarteritis)?.Pathol Res Pract. 1989; 184: 46-52Crossref Scopus (21) Google Scholar We have encountered a third case that, to our knowledge, is also the first case of this rare entity that shows acute arterial dissection and occlusion of the affected coronary artery by the dissecting hematoma. A 57-year-old postmenopausal woman actively employed as a nursing instructor was found dead at home during predawn hours. She had expressed no specific complaint during the preceding days but was reported to have experienced intermittent chest discomfort associated with a productive cough during several months previously, for which she had sought no medical attention. This woman had a lifelong history of allergies to numerous agents, including the following: penicillin, sulfa drugs, tetracycline, morphine sulfate, local anesthetics, pollens, weeds, fish, avocado, and fresh pineapple. Approximately 5 years before her death, she was hospitalized because of an anaphylactoid reaction characterized by cutaneous hives, hypotension and near-syncope, and mild dyspnea. This reaction was thought to be attributable to oral administration of cefaclor3Saxon A Beall GN Rohr AS Adelman DC Immediate hypersensitivity reactions to beta-lactam antibiotics.Ann Intern Med. 1987; 107: 204-215Crossref PubMed Scopus (347) Google Scholar, 4VanArsdel Jr, PP Miller S Antimicrobial treatment of patients with a penicillin allergy history (abstract).J Allergy Clin Immunol. 1990; 85: 188Google Scholar (a semisynthetic cephalosporin antibiotic), which she had been taking for several days for treatment of "flu" symptoms. Her condition responded rapidly to standard therapy for anaphylaxis. On admission, findings on physical examination disclosed focal wheezing. Results of pertinent laboratory studies included an unremarkable leukocyte count with no peripheral eosinophilia, and pulmonary function studies during hospitalization confirmed a mild to moderate constrictive disorder. She was moderately obese (weight, 105 kg) and had a lengthy history of cigarette smoking (1 pack per day since age 19 years). On autopsy, foul play was ruled out and postmortem toxicology was positive only for subtherapeutic levels of phenobarbital (an ingredient of Tedral SA, which she took regularly for treatment of the bronchospastic disorder). Death was attributed to eosinophilic coronary arteritis and luminal occlusion by dissecting hematoma of the media. The macroscopic findings relevant to the cause of death were limited to the heart, which was slightly enlarged (weight, 430 g). Coronary arterial origin and distribution were normal. Epicardial and pericardial surfaces were unremarkable except for focal perivascular hemorrhage overlying the distal region of the proximal left anterior descending coronary artery. The proximal segment of the left anterior descending coronary artery slightly distal to the bifurcation of the left circumflex coronary artery exhibited focal, near-critical (70 to 75%) stenosis because of an eccentric atherosclerotic plaque. The left main, left circumflex, and right coronary arteries were widely patent and free of aneurysmal change. Immediately distal and adjacent to the focus of stenosis within the left anterior descending coronary artery was a grossly apparent 2.5-cm-long occlusion of the arterial lumen caused by medial dissection. The intramural hematoma was expansive and completely compressed the vascular lumen focally (Fig. 1). A separate focus with identical occlusive features was apparent within the midsegment of the first diagonal branch arising from the left anterior descending coronary artery. This separate focus of medial dissection was adjacent to a region of 60% eccentric stenosis caused by atherosclerotic plaque. No intraluminal thrombosis was present. The wall of the left cardiac ventricle was 1.4 cm in thickness concentrically, and multiple sections showed patchy subendocardial fibrosis of the left lateral free wall and one focus of virtually transmural scar within the midsegment of the same region. No acute myocardial infarct was evident. Moderate, focally ulcerative aortic atherosclerosis and mild nephrosclerosis were noted. Physical trauma was not evident. The histologic appearance of both involved coronary arterial segments was similar. The intramural dissection appeared as a partially encircling, crescentic separation near the junction of the outer media and adventitia (Fig. 2). A diffuse inflammatory cell infiltrate, composed of numerous bilobed eosinophils, considerably smaller numbers of plasma cells and lymphocytes, and only rare multinucleated giant cells, invested the periadventitial and outer medial wall (Fig. 3). The affected arterial wall displayed fibrinoid necrosis. No microorganisms were identified. The cardiac myocytes exhibited features of moderate hypertrophy, and the intramural coronary vessels were normal. No histologic evidence of cystic medionecrosis of the aorta or of any of the major epicardial coronary arteries was noted.Fig. 3High-power view of mixed-cell inflammatory infiltrate with prominent eosinophils, histiocytes, and multinucleated giant cells. (Hematoxylin-eosin; ×400.)View Large Image Figure ViewerDownload (PPT) The combined weight of the lungs was 1,070 g because of mild hyperemia and edema. The bronchi and bronchioles were grossly patent and, on microscopic examination, had no specific histologic abnormalities except for mild hyaline thickening of the basal lamina and hypertrophy of the submucosal smooth muscle. No visceral eosinophilia, extracardiac necrotizing arteritis, or granulomatosis was evident. Initially, Churg-Strauss syndrome (allergic granulomatosis and angiitis) was considered a systemic disease almost always characterized by the clinical triad of a history of asthma and allergy, peripheral eosinophilia, and systemic eosinophilic vasculitis of both small arteries and veins.5Churg J Strauss L Allergic granulomatosis, allergic angiitis, and periarteritis nodosa.Am J Pathol. 1951; 27: 277-301PubMed Google Scholar Pulmonary involvement was reported in up to 50% of the cases.6Lie JT The classification of vasculitis and a reappraisal of allergic granulomatosis and angiitis (Churg-Strauss syndrome).Mt Sinai J Med. 1986; 53: 429-439PubMed Google Scholar Extravascular granulomatous inflammation was a common feature in the original description but was unnecessary for diagnosis.7Lanham JG Elkon KB Pusey CD Hughes GR Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome.Medicine. 1984; 63: 65-81Crossref PubMed Scopus (1046) Google Scholar The most common extrapulmonary involvement has been noted in the heart (coronary arteries or myocardium),6Lie JT The classification of vasculitis and a reappraisal of allergic granulomatosis and angiitis (Churg-Strauss syndrome).Mt Sinai J Med. 1986; 53: 429-439PubMed Google Scholar skin, peripheral nerves, spleen, and gastrointestinal tract; less frequently, the skeletal muscle and the urogenital system have been involved.8Lie JT Coronary vasculitis: a review in the current scheme of classification of vasculitis.Arch Pathol Lab Med. 1987; 111: 224-233PubMed Google Scholar Recent investigators opine that the Churg-Strauss syndrome is within a spectrum of systemic vasculitides including polyarteritis nodosa and Wegener's granulomatosis, but current classification schemes distinguish each of these overlapping entities by histopathologic and clinical criteria.6Lie JT The classification of vasculitis and a reappraisal of allergic granulomatosis and angiitis (Churg-Strauss syndrome).Mt Sinai J Med. 1986; 53: 429-439PubMed Google Scholar Since the original concept of systemic involvement, support has been increasing for a limited or isolated expression of the Churg-Strauss syndrome.9Nissim F Von der Valde J Czernobilsky B A limited form of Churg-Strauss syndrome: ocular and cutaneous manifestations.Arch Pathol Lab Med. 1982; 106: 305-307PubMed Google Scholar Isolated eosinophilic angiitis (a limited variant of Churg-Strauss syndrome) of the coronary artery was first described in 1989 by Lie and Bayardo;1Lie JT Bayardo RJ Isolated eosinophilic coronary arteritis and eosinophilic myocarditis: a limited form of Churg-Strauss syndrome.Arch Pathol Lab Med. 1989; 113: 199-201PubMed Google Scholar they reported the sudden and unexpected death of a 39-year-old man who had chronic asthma, splenic eosinophilia, and isolated eosinophilic coronary panarteritis and myocarditis. Later that year, Kajihara and associates2Kajihara H Kato Y Takanashi A Nakagawa H Tahara E Otsuki T Tsuchioka Y Amioka H Okamoto M Matsuura H Kajiyama G Periarteritis of coronary arteries with severe eosinophilic infiltration: a new pathologic entity (eosinophilic periarteritis)?.Pathol Res Pract. 1989; 184: 46-52Crossref Scopus (21) Google Scholar described a second case of isolated eosinophilic angiitis of the coronary artery in a 40-year-old man who died after hospitalization for unstable angina pectoris. Luminal stenosis (up to 85%) of the right coronary artery was characterized microscopically by profuse eosinophilic periarteritis and irregular fibrotic thickening of the intima. This latter group of investigators speculated that such changes were due to immune or hypersensitivity mechanisms. Coronary artery dissection attributed to an established proximate cause or association occurs often and may be an extension of aortic dissection.10Shin P Minamino T Onishi S Kitamura H Dissecting aneurysms of the coronary arteries.Acta Pathol Jpn. 1982; 32: 713-724PubMed Google Scholar, 11Lantos G Sos TA Sniderman KW Saddekni S Hilton S Dissecting hematoma of the thoracic aorta extending into a coronary artery: angiographic demonstration.Radiology. 1980; 135: 329-330PubMed Google Scholar It is also associated with other conditions, such as arteritis (for example, Takayasu's arteritis12Rose AG Sinclair-Smith CC Takayasu's arteritis: a study of 16 autopsy cases.Arch Pathol Lab Med. 1980; 104: 231-237PubMed Google Scholar or Kawasaki disease13Landing BH Larson EJ Are infantile periarteritis nodosa with coronary artery involvement and fatal mucocutaneous lymph node syndrome the same? Comparison of 20 patients from North America with patients from Hawaii and Japan.Pediatrics. 1977; 59: 651-662PubMed Google Scholar), degenerative diseases, and metabolic abnormalities. Various types of blunt thoracic trauma may also produce coronary arterial dissection;14Hedinger C Contusio cordis mit Spätruptur der linken Herzkammer.Cardiologia. 1947; 12: 46-53PubMed Google Scholar, 15Vlay SC Blumenthal DS Shoback D Fehir K Bulkley BH Delayed acute myocardial infarction after blunt chest trauma in a young woman.Am Heart J. 1980; 100: 907-916Abstract Full Text PDF PubMed Scopus (43) Google Scholar one case report attributed the dissection to forceful external chest compression during cardiac resuscitation.16Baker PB Keyhani-Rofagha S Graham RL Sharma HM Dissecting hematoma (aneurysm) of coronary arteries.Am J Med. 1986; 80: 317-319Abstract Full Text PDF PubMed Scopus (25) Google Scholar Other reported iatrogenic traumatic causes include coronary artery bypass grafting, replacement of the aortic valve,17Bramlet DA Behar VS Ideker RE Aneurysm of a saphenous vein bypass graft associated with aneurysms of native coronary arteries.Cathet Cardiovasc Diagn. 1982; 8: 489-494Crossref PubMed Scopus (27) Google Scholar, 18Bulkley BH Roberts WC Dissecting aneurysm (hematoma) limited to coronary artery: a clinicopathologic study of six patients.Am J Med. 1973; 55: 747-756Abstract Full Text PDF PubMed Scopus (67) Google Scholar, 19Bulkley BH Roberts WC Isolated coronary arterial dissection: a complication of cardiac operations.J Thorac Cardiovasc Surg. 1974; 67: 148-151PubMed Google Scholar coronary angiography,20Zelinger AB Shulruff S Pouget JM Significant left main stenosis following asymptomatic dissection during coronary arteriography.Chest. 1983; 83: 568-569Crossref PubMed Scopus (19) Google Scholar, 21Weiner RD Boston BA Mintz GS Bemis CE Kimbiris D Iskandrian AS Catheter-induced coronary artery dissection (abstract).Circulation. 1981; 64: IV-108Google Scholar and percutaneous transluminal coronary angioplasty.22Cowley MJ Dorros G Kelsey SF Van Raden M Detre KM Acute coronary events associated with percutaneous transluminal coronary angioplasty.Am J Cardiol. 1984; 53: 12C-16CAbstract Full Text PDF PubMed Scopus (339) Google Scholar, 23Düber C Jungbluth A Rumpelt H-J Erbel R Meyer J Thoenes W Morphology of the coronary arteries after combined thrombolysis and percutaneous transluminal coronary angioplasty for acute myocardial infarction.Am J Cardiol. 1986; 58: 698-703Abstract Full Text PDF PubMed Scopus (47) Google Scholar In contrast, spontaneous or primary dissection of a normal or diseased coronary artery is exceedingly rare.24Robinowitz M Virmani R McAllister Jr, HA Spontaneous coronary artery dissection and eosinophilic inflammation: a cause and effect relationship?.Am J Med. 1982; 72: 923-928Abstract Full Text PDF PubMed Scopus (218) Google Scholar, 25Nalbandian RM Chason JL Intramural (intramedial) dissecting hematomas in normal or otherwise unremarkable coronary arteries: a "rare" cause of death.Am J Clin Pathol. 1965; 43: 348-356PubMed Google Scholar The pathogenesis of such a catastrophic event has yet to be completely elucidated but is probably multifactorial. Of the slightly more than 60 reported cases since the first description by Pretty26Pretty HC Dissecting aneurysm of coronary artery in a woman aged 42: rupture.Br Med J. 1931; 1: 667Crossref Scopus (2) Google Scholar in 1931, most have occurred in middle-aged women. A single article described its occurrence in a 6-year-old boy.27Trillo AA Scharyj M Prichard RW Coronary artery aneurysm and myocardial infarction resulting in sudden death of a 6-year-old child: a case report.Am J Forensic Med Pathol. 1980; 1: 349-354Crossref PubMed Scopus (12) Google Scholar The mean age of affected men and women was 49 years (range, 21 to 79 years) and 38½ years (range, 17 to 60 years), respectively. Slightly less than a third of the women died in the peripartum28Asuncion CM Hyun J Dissecting intramural hematoma of the coronary artery in pregnancy and the puerperium.Obstet Gynecol. 1972; 40: 202-210PubMed Google Scholar or postpartum period24Robinowitz M Virmani R McAllister Jr, HA Spontaneous coronary artery dissection and eosinophilic inflammation: a cause and effect relationship?.Am J Med. 1982; 72: 923-928Abstract Full Text PDF PubMed Scopus (218) Google Scholar (14 to 80 days after parturition); this finding prompts the speculation of possible medial weakening associated with hormonal changes and parturient strain of pregnancy. Neither systemic hypertension nor coronary atherosclerosis29Forker AD Rosenlof RC Weaver WF Carveth SW Reese HE Primary dissecting aneurysm of the right coronary artery with survival.Chest. 1973; 64: 656-658Crossref PubMed Scopus (36) Google Scholar with associated risk factors for coronary artery disease was considered substantially causative in these cases. One woman had generalized sarcoidosis,30Ehya H Weitzner S Postpartum dissecting aneurysm of coronary arteries in a patient with sarcoidosis.South Med J. 1980; 73: 87-88Crossref PubMed Scopus (33) Google Scholar and marfanoid features were noted twice.31Kaufman G Engelbrecht WJ Hemorrhagic intramedial dissection of coronary artery with cystic medial necrosis.Am J Cardiol. 1969; 24: 409-413Abstract Full Text PDF PubMed Scopus (20) Google Scholar In approximately a third of the cases reported or reviewed by Robinowitz and colleagues,24Robinowitz M Virmani R McAllister Jr, HA Spontaneous coronary artery dissection and eosinophilic inflammation: a cause and effect relationship?.Am J Med. 1982; 72: 923-928Abstract Full Text PDF PubMed Scopus (218) Google Scholar histopathologic examination showed aortic cystic medionecrosis, and separate single case reports32Ashley PF Dissecting aneurysm of the coronary artery.Del Med J. 1965; 37: 86-88PubMed Google Scholar, 33Wisecarver J Jones J Goaley T McManus B "Spontaneous" coronary artery dissection: the challenge of detection, the enigma of cause.Am J Forensic Med Pathol. 1989; 10: 60-62Crossref PubMed Scopus (17) Google Scholar described changes suggestive of that condition. Predisposing factors that underly primary coronary artery dissection are not well defined but are postulated to result from degeneration of the media in the wake of myriad ischemic, biochemical, or hemodynamic conditions or exertional stress.34Uehlinger E Das spontane intramurale Hämatom und Aneurysma dissecans der normalen Coronararterie.Schweiz Med Wochenschr. 1947; 77: 608-611PubMed Google Scholar Virmani and co-workers35Virmani R Forman MB Robinowitz M McAllister Jr, HA Coronary artery dissections.Cardiol Clin. November 1984; 2: 633-646PubMed Google Scholar reported spontaneous dissection of a single coronary artery in 11 women with normal-sized hearts. Microscopic findings consisted of outer medial dissection of the affected artery and mixed inflammation composed of bilobed eosinophils, some polymorphonuclear leukocytes, lymphocytes, and plasma cells limited to the adventitia only. Various authors who have addressed the cause of spontaneous coronary artery dissection have postulated several pathogenetic mechanisms. Noting a predominant eosinophilic component of the adventitial or periadventitial inflammatory cellular infiltrate, some investigators24Robinowitz M Virmani R McAllister Jr, HA Spontaneous coronary artery dissection and eosinophilic inflammation: a cause and effect relationship?.Am J Med. 1982; 72: 923-928Abstract Full Text PDF PubMed Scopus (218) Google Scholar, 36Barrett DL Isolated dissecting aneurysm of the coronary artery: report of a case apparently due to hypersensitivity angiitis.Ohio Med J. 1969; 65: 830-832PubMed Google Scholar have speculated that medial dissection is caused primarily after release of lytic enzymes by the eosinophils. In contrast, other investigators37Dowling GP Buja LM Spontaneous coronary artery dissection occurs with and without periadventitial inflammation.Arch Pathol Lab Med. 1987; 111: 470-472PubMed Google Scholar have suggested that adventitial eosinophilic inflammation represents a reactive or secondary phenomenon temporally subsequent to the medial dissection itself, possibly induced by histamine released from periadventitial mast cells.38Whitehead R Dunnill MS Primary dissecting aneurysms of coronary arteries.J Pathol. 1969; 99: 33-37Crossref PubMed Scopus (13) Google Scholar Recently, Virmani and Forman39Virmani R Forman MB Coronary artery dissections.in: Virmani R Forman MB Nonatherosclerotic Ischemic Heart Disease. Raven Press, New York1989: 325-354Google Scholar exhaustively reviewed and tabulated the reported cases up to 1987. Speculating on the issue of "inflammatory" cause versus effect in coronary artery dissection, these authors suggested that release of multiple chemical mediators by adventitial or periadventitial eosinophils effects elastolysis, collagenolysis, and myocytolysis of vascular smooth muscle in the arterial media. Furthermore, the absence of eosinophilic inflammation in dissections of the epicardial coronary arteries attributable to trauma or as an extension of aortic dissection is cited as supportive evidence for the theory that eosinophilia associated with spontaneous coronary artery dissection is primary, not reactive. The findings reported herein are important for several reasons. First, relative to the cause of the angiitic process, current theory maintains that the Churg-Strauss syndrome legitimately belongs in the spectrum of systemic vasculitides with increasing refinement of distinguishing clinical and pathologic characteristics among the groupings.6Lie JT The classification of vasculitis and a reappraisal of allergic granulomatosis and angiitis (Churg-Strauss syndrome).Mt Sinai J Med. 1986; 53: 429-439PubMed Google Scholar The origin of the Churg-Strauss syndrome is allergic, a conclusion buttressed by the virtually invariable history of an underlying allergic, atopic, or asthmatic diathesis.40Koss MN Antonovych T Hochholzer L Allergic granulomatosis (Churg-Strauss syndrome): pulmonary and renal morphologic findings.Am J Surg Pathol. 1981; 5: 21-28Crossref PubMed Scopus (54) Google Scholar Although a discussion of the pathogenesis of the syndrome itself is beyond the scope of this brief report, the weight of accumulating evidence strongly implicates an unequivocal association with either allergic or immune-related factors, as encapsulated in the differential diagnostic triad. Thus, the therapeutic and prognostic implications of such distinctions are important. Treatment with corticosteroids is effective in the Churg-Strauss syndrome and is associated with considerably more favorable results than therapeutic approaches for other related vasculitides such as polyarteritis nodosa or Wegener's granulomatosis.7Lanham JG Elkon KB Pusey CD Hughes GR Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome.Medicine. 1984; 63: 65-81Crossref PubMed Scopus (1046) Google Scholar, 41Chumbley LC Harrison Jr, EG DeRemee RA Allergic granulomatosis and angiitis (Churg-Strauss syndrome): report and analysis of 30 cases.Mayo Clin Proc. 1977; 52: 477-484PubMed Google Scholar