Eosinophilic coronary periarteritis (vasospastic angina and sudden death), a new type of coronary arteritis: report of seven autopsy cases and a review of the literature.

A previously reported autopsy case of eosinophilic coronary periarteritis (ECPA, or isolated eosinophilic coronary periarteritis, IECPA), and an additional six autopsy cases of ECPA are reported. In addition, another four autopsy cases of ECPA reported in the literature are discussed. Fifteen cases of ECPA with spontaneous coronary dissection (hematoma), which appeared in the literature from 1987 to 2011, are also reviewed. The characteristic clinico-pathological findings of ECPA are: (a) variant angina (Prinzmetal's vasospastic angina) appeared mainly from evening to early in the morning; (b) allergy or allergic history could be identified in only three of a total of 11 cases; (c) sudden unexpected death (sudden cardiac death) usually occurred early in the morning; (d) eosinophilic inflammation limited to the adventitia and periadventitial soft issue appeared in the epicardial large coronary arteries, chiefly in the left coronary anterior descending artery; (e) fibrinoid necrosis or granuloma could not be found in or around the inflammatory area; (f) no type of vasculitis could be found in any other tissues or organs (i.e., localized and non-systemic periarteritis); (g) ECPA was frequently accompanied by spontaneous coronary arterial dissection (SCAD) in the affected wall; and (h) ECPA without SCAD appeared mainly in men (male/female ratio was 8:3), while EPCA with SCAD appeared in almost all female cases (male/female ratio was 1:14). Although the etiology and pathogenesis are still unknown, we believe that ECPA (with or without SCAD) might be a distinct new type of coronary arteritis.

Prinzmetal's angina in a patient with angiosarcoma of the right cardiac chambers.

Variant (Prinzmetal's) angina pectoris is a clinical syndrome characterized by the presence of angina at rest, coinciding with a transient ST-segment elevation. This syndrome is often caused by vasospasm, either on a normal coronary artery or at the site of a coronary atherosclerotic stenosis. We describe a classic case of variant angina associated with an angiosarcoma of the right heart chambers.

Gender difference of clinical characteristics in Chinese patients with spontaneous variant angina.

BACKGROUND: Spontaneous attack of variant angina (VA) is a unique component of coronary artery disease (CAD), and associated with severe cardiac events. However, no data are available regarding sex differences in Chinese patients with spontaneous attacks of VA. Accordingly, the present retrospective study was initiated to evaluate the Clinical characteristics of Chinese female patients with spontaneous attacks of VA. METHODS: From January 2003 to January 2008, a total of 209 patients were diagnosed to have had a spontaneous attack of VA at Fu Wai Hospital. Of them, 27 were female, and their clinical findings were collected and compared with male patients for aspects of risk factors, clinical features and angiographical findings. RESULTS: Spontaneous attacks of VA was relatively uncommon in female (12.9%) compared with male patients. The female patients were less likely to have a history of smoking (14.8% vs. 79.7%, P < 0.001), more likely to have a family history of CAD (33.3% vs. 11.0%, P < 0.01), and to have had a greater incidence of ventricular fibrillation during attack (11.1% vs. 2.2%, P < 0.05). There were no significant differences in other characteristics between the two groups. CONCLUSION: Chinese female patients who experienced a spontaneous attack of VA had the characteristics of less smoking history, more family history of CAD and higher occurrence of ventricular fibrillation than male patients.

Histological evaluation of coronary plaque in patients with variant angina: relationship between vasospasm and neointimal hyperplasia in primary coronary lesions.

OBJECTIVES: This study was designed to determine whether coronary vasospasm in patients with variant angina pectoris (VAP) may produce focal organic lesions at the site of vasospasm that would contribute to disease progression. BACKGROUND: Recent clinical angiographic and experimental studies have demonstrated the potential role of vasospasm in the worsening of organic coronary stenosis. METHODS: We studied histologically the coronary plaques obtained at atherectomy in 202 patients with moderate to severe coronary stenosis. This population included 22 patients with VAP, 100 patients with chronic stable angina and 80 patients with restenosis following angioplasty or atherectomy. Diagnosis of VAP was based on both the clinical feature of angina at rest associated with ST elevation and a positive response to acetylcholine provocation test. RESULTS: The most common histological appearance in 92% of patients with stable angina was hypocellular fibroatheromatous plaques, whereas neointimal hyperplasia was the characteristic feature of the plaque observed in 90% of patients with restenosis. The coronary specimens at the site of spasm in 15 of the 22 patients (68%) with VAP demonstrated intimal injuries such as neointimal hyperplasia (15), thrombus formation (2), and intimal hemorrhage (3). Neointimal hyperplasia was significantly more common in the patients with VAP as compared with those with stable angina (68% vs. 8%; p < 0.0001). A rapid progression of organic stenosis within three years was angiographically found in 5 of the 22 patients with variant angina. In all five cases, neointimal hyperplasia was the main contributor to the worsening of the organic lesion at the site of spasm. These histological findings in patients with VAP extremely resembled those in restenosis. Except for vasospasm, no factors significantly predicted the presence of neointimal formations in primary coronary lesions. CONCLUSIONS: Coronary vasospasm may provoke vascular injury that leads to the formation of neointima in VAP patients similar to that seen with restenosis. Coronary spasm may thus play a key role in the rapid coronary stenosis progression in certain patients with VAP.

Angioscopy in variant angina: coronary artery spasm and intimal injury.

Studies in pigs and dogs show that intimal injury is related to coronary artery spasm; it is not known whether intimal injury is related to coronary artery spasm in human beings. We examined intima at the site of coronary artery spasm by percutaneous transluminal coronary angioscopy in 10 of 13 consecutive patients with variant angina. Coronary artery spasms occurred spontaneously or were induced by intracoronary acetylcholine (10-100 micrograms). Angioscopy showed intimal injuries (haemorrhage, flap, thrombus, or ulcer) in 4 of the 10. We suggest that intimal injury is related to coronary artery spasm in human beings.

Clinical characteristics of patients with spontaneous remission of variant angina.

To determine the factors influencing the spontaneous remission of variant angina, clinical characteristics were examined in 75 Japanese patients with variant angina. Spontaneous remission was defined as an absence of angina at rest for at least 3 months after withdrawal of treatment with calcium antagonists. This remission occurred in 12 patients (16%) (remission group), while angina persisted despite treatment with calcium antagonists and nitrates in 33 patients (44%) (persistent angina group). The remaining 30 patients (40%) were angina-free under treatment with calcium antagonists and/or nitrates (angina-free on treatment group). The prevalence of significant coronary artery stenosis (> 75%) was significantly higher in the remission group than in the persistent angina group (44% vs 7%, p < 0.05). The prevalence of cessation of smoking was significantly higher in the remission group than in the persistent angina group (92% vs 39%, p < 0.01). Age, gender, other coronary risk factors, disease activity of variant angina and site of myocardial ischemia during anginal attacks were not statistically different among the 3 groups. There data indicate that remission of variant angina occurs more frequently in patients with than in those without significant coronary artery stenosis and that cessation of smoking is an important factor for remission of variant angina.

Sympathectomy in the treatment of angina and arrhythmias.

Sympathectomy has been used as treatment for several different cardiac conditions. These include classic angina pectoris, Prinzmetal's angina, paroxysmal atrial tachycardia, ventricular tachycardia, and long QT syndrome. To understand the rationale of such treatment, the innervation of the human heart is reviewed with discussion of the cardiac plexus and coronary innervation. Results in published studies are summarized and discussed.

[Intimal coronary fibrous dysplasia and Prinzmetal's angina]. / Dysplasie fibreuse intimale coronaire et angor de Prinzmetal.

It is currently considered as exceptional for there to be arterial pathology other than atheroma in Prinzmetal angina. The authors have found five cases of coronary fibrous dysplasia in the literature. They add a case, in a woman, of intimal fibrous dysplasia diffusely involving the three main coronary vessels. Coronary arteriography showed that spasm of the right coronary artery was the cause of attacks with the clinical and electrocardiographic features of Prinzmetal angina. The whole coronary tree was otherwise normal angiographically and also macroscopically at autopsy. The dysplasia was only discovered by histological study of serial sections of the three coronary vessels. Histological examination showed no evidence of an atheromatous lesion. The authors place these coronary artery lesions within the general classification of arterial fibrous dysplasias. It remains hypothetical whether there is a relationship between the occurrence of spasm and this very special histological feature of the coronary artery wall.

[Prinzmetal's angina. Histological study of peroperative coronary specimens. Apropos of 2 cases]. / Angor de Prinzmetal. Etude histologique coronaire de prlèvements per-opératories. A propos de deux cas

The results of histological examination of two coronary artery biopsy specimens removed at operation are presented. There were unusual findings, especially of a highly cellular and oedematous fibroblastic endarteritis, of the lack of calcification, and of the presence of marked medial sclerosis. These unusual histological findings are the result of solitary variable stenoses of the coronary arterial tree, and very different from the histological findings in the atheromatous lesions which are usually found when Prinzmetal's angina is super-imposed on lesions which are multiple and diffuse.

Conduction system in a patient with Prinzmetal's angina and transient atrioventricular block.

His bundle recordings obtained during and between attacks of Prinzmetal's variant angina and transient atrioventricular (A-V) block were followed by a comprehensive serial section study of the conduction system in a 33 year old woman. Recordings between attacks showed normal A-H and H-V intervals. During an attack there was block proximal to the His bundle recording site. Pathologic studies revealed severe narrowing of the right coronary artery. Arteriolosclerosis of the heart was diffuse. Insignificant changes were found in the approaches to the A-V node and the A-V node itself. Major changes found in the left bundle branch had no counterpart in the electrocardiogram; the discordance in these findings is discussed.

Prinzmetal's variant angina.

Two patients are described with a typical Prinzmetal's variant angina. Both patients were young, active males with angina of recent onset. They experienced angina only at rest and in one patient the angina had a cyclic character appearing only between 4 and 10 a.m. In both patients there was no correlation whatsoever between the angina pectoris and effort, emotion or change in temperature and the angina reacted promptly to nitroglycerine sublingually. The number of attacks and the intensity of the pain were increasing rapidly over a short period of time in both cases. The stenoses in both patients did not exceed 50% of the lumen of the coronary arteries, but coronary surgery has been performed on clinical grounds because both patients were completely disabled and one of them suffered from life-threatening dysrhythmias. For respectively 18 and 6 months after saphenous vein bypass surgery both patients have been symptom-free and able to resume their work. The problems concerning the diagnosis Prinzmetal's variant of angina pectoris are discussed.