Hugh A. McAllister

Sudden Death as a Complication of Anomalous Left Coronary Origin From the Anterior Sinus of Valsalva A Not-So-Minor Congenital Anomaly

Both coronary arteries arising as a single or double vessel from the same sinus of Valsalva has been considered a minor congenital anomaly not affecting longevity. There are rare case reports in the literature of sudden death in young males with this anomaly of coronary origin. We have reviewed 51 such cases from the Armed Forces Institute of Pathology congenital heart disease accessions. There were 33 patients in whom both coronaries arose from the anterior sinus of Valsalva either as a single or double vessel and 18 in whom they arose from the left coronary sinus of Valsalva. Out of the 33 patients in whom the coronaries arose from the anterior sinus of Valsalva, 9 (27.3%) died sudden, unexplained deaths. There were no sudden unexplained deaths among the patients in whom both coronaries arose from the left sinus of Valsalva. It is evident that where the left coronary artery does not pass acutely posterior and leftward between the pulmonary artery and the aorta there is no risk of sudden death. All patients who died suddenly were male. The suggested mechanism for sudden death in these cases is that the acute leftward passage of the coronary artery along the aortic wall causes the entrance into the left coronary svstem to be slit-like. Under circumstances of increased cardiac activity with increased expansion of the pulmonary artery and aorta with exercise, there is stretching of the left coronary artery and a flap-like closure of the orifice of the left coronary with sudden, fatal myocardial ischemia.We also present the first case where this anomaly was recognized in a 14-year-old boy and a surgical attempt was made to correct the problem by creating a non-collapsible funnel-like opening into the left coronary artery.

Spontaneous coronary artery dissection and eosinophilic inflammation: A cause and effect relationship?

Spontaneous coronary artery dissection is described in eight patients 26 to 47 years of age. Six died suddenly, and two died after the onset of chest pain. All had normal heart weights and all had dissection of left anterior descending coronary artery, which occurred mainly in the outer one third of the media. The adventitial of the dissected artery contained inflammatory infiltrates which were predominantly eosinophilic granulocytes. Forty-six cases previously published in 32 reports are reviewed. It is suggested that adventitial eosinophilic infiltrate may be responsible for spontaneous coronary artery dissection.

Congenital bicuspid aortic valve after age 20.

The bicuspid aortic valve is recognized as a frequent cause of aortic stenosis in adults. Aortic stenosis has been reported to occur in as many as 72 percent of adults with a congenital bicuspid aortic valve, with peak incidence occurring in the 5th and 6th decades of life. Review of the clinical records of 152 patients aged 20 years and older found to have a bicuspid aortic valve at autopsy revealed aortic stenosis in only 28 percent. The incidence of aortic stenosis increased progressively with age; 46 percent of patients over age 50 years and 73 percent over age 70 years had some degree of stenosis. The stenotic valves were obstructed by nodular, calcareous masses but commissural fusion was present in only eight cases. The largest group of patients in the series (40 percent) died of infective endocarditis; 77 percent of these were under age 50 years. Primary aortic regurgitation without infective endocarditis was uncommon. Thirty-two percent of the patients in this series had an apparently normally functioning aortic valve, and this rate remained relatively constant with increasing age; 37 percent of patients over age 50 years and 27 percent over age 70 years had an apparently normal valve. The bicuspid aortic valve in patients over age 20 does not invariably become stenotic or insufficient.

Acute takeoffs of the coronary arteries along the aortic wall and congenital coronary ostial valve-like ridges: association with sudden death.

Congenital coronary artery anomalies have been associated with sudden death. Twenty-two patients who were victims of sudden death (mean age 46) and who had no significant anatomic cause of death were examined at autopsy and compared with 19 patients who died of known causes (control group). The hearts of these 41 patients were examined for abnormalities of acute angle takeoff of the coronary artery and presence of ostial valve-like ridges. Of the 22 patients who died suddenly, 13 (59%) had acute angle takeoff of the coronary artery and 9 (41%) had ostial valve-like ridges. Of the 19 control subjects, 4 (21%) had acute angle takeoff and only 2 (11%) had an ostial valve-like ridge. The difference was statistically significant (p = 0.015 and 0.031, respectively). It is suggested that aortic root dilation may compress coronary arteries with acute angle takeoff and that ostial valve-like ridges may act as occlusion valves. Thus, either may cause acute obstruction of the proximal coronary artery and lead to sudden death. A very lethal combination for sudden death would be the presence of severe coronary artery disease, an acute angle takeoff and an ostial valve-like ridge.

Pericardial cysts. A radiologic-pathologic correlation and review

Pericardial cysts are generally described as round radiodensities typically found at the right cardiophrenic angle in asymptomatic individuals. A review of all cases of pericardial cysts from the files of this Institute reveals that approximately one third of the cysts are found in other locations and that approximately one third of patients have symptoms of chest pain, dyspnea, or persistent cough. The radiographs of 41 patients show that in all but 6 of the cases the cyst is visualized as a round radiodensity touching both the hemidiaphragm and the anterior chest wall. Surprisingly, 15 of the 41 occurred on the left border of the heart. The six cysts significantly above the diaphragm were difficult to diagnosis radiologically and were usually mistaken for thymomas or pulmonary masses; two such cysts caused bronchial obstruction. In general, the possibility that a mass in either anterior cardiophrenic angle is a pericardial cyst should be strongly considered, even if the mass is on the left side and even if the patient is symptomatic.

Drug related vasculitis: Clinicopathologic correlations in 30 patients*

Drug related vasculitis has variously been described as necrotizing hypersensitivity or allergic angiitis or microscopic panarteritis nodosa. We reviewed tissue sections from 30 patients with validated drug hypersensitivity and vasculitis in order to precisely define this entity. No evidence of necrotizing vascular lesions or of fibrinoid associated with necrosis was found. The vascular lesions in all 30 patients involved small arteries, arterioles, capillaries, and venules. The inflammatory infiltrate consisted primarily of mononuclear cells and prominent numbers of eosinophils and was present in all three layers of the involved vessel walls. Clinically the patients developed either localized or systemic vasculitis, which could not be predicted on the basis of the associated drug. The findings of a skin rash, fever, or eosinophilia and the development of symptoms consistent with a hypersensitivity reaction while medication was being taken were all suggestive of the diagnosis of drug related vasculitis.

Intravenous pyogenic granuloma. A study of 18 cases.

A series of 18 intravenous pyogenic granulomas (IVPG) was studied. The lesions were limited to the neck and upper extremity and appeared as nonspecific tumors. Most had a clinical duration of 2 months or less. Treatment was by excision and there were no recurrences. IVPG consists of an intraluminal polyp attached to the wall of the vein by a fibrovascular stalk. The characteristic appearance is of lobules of capillaries separated by a fibromyxoid stroma. The organization and histologic detail are similar to those of pyogenic granulomas that are uncomplicated by the changes of inflammation. Our observations suggest that IVPG develops in or adjacent to the wall of the involved vein. It probably receives its blood supply from a small artery that enters the lesion in the region of the stalk.

Drug related myocarditis: I. Hypersensitivity myocarditis

The purpose of this study was to characterize in detail the morphologic changes in one form of drug related myocarditis: hypersensitivity myocarditis. We reviewed the clinical histories and microscopic findings in 24 patients with validated drug hypersensitivity and myocarditis. The myocarditis in these patients was characterized by a patchy interstitial inflammatory infiltrate rich in eosinophils, focal myocytolysis, prominent perivascular infiltrates, an absence of myocardial fibrosis, and the uniform features of the myocarditis in each patient. Twenty of the 24 patients died suddenly and unexpectedly. In retrospect, the presence of signs and symptoms of hypersensitivity (skin rash, fever, eosinophilia, and malaise) and nonspecific cardiac findings (electrocardiographic changes, tachycardia, or serum enzyme elevations) were suggestive of hypersensitivity myocarditis in these patients. Since early diagnosis is vital in hypersensitivity myocarditis, the presence of nonspecific cardiac findings in a patient receiving any drug associated with hypersensitivity should alert the clinician to the possibility of drug related myocarditis.

Acquired coronary arterial aneurysms: an autopsy study of 52 patients

In the past decade most studies of coronary arterial aneurysms have been clinical; few have focused on morphology and etiopathogenesis. The subjects of the present autopsy study were 52 patients, 5 months to 80 years of age, with coronary arterial aneurysms. Patients were divided into two groups: 38 with atherosclerotic coronary aneurysms and 14 with aneurysms secondary to inflammation. Of the 38 patients with atherosclerotic aneurysms, 20 (53 per cent) had histories of ischemic heart disease; the aneurysms were in the right coronary artery in 18 (47 per cent), the left coronary artery in 13 (35 per cent), and in the right and left coronary arteries in seven (18 per cent). Of the four major coronary arteries, the average number of severely narrowed arteries (reduction of more than 75 per cent) in cross-sectional luminal area) was 1.8/patient; aortic aneurysms were present in eight of these patients (24 per cent). Of the 14 patients with coronary aneurysms secondary to inflammation, four had histories of ischemic heart disease; 10 had histories of an influenza-like syndrome. Isolated left coronary arterial aneurysms were seen in six of these patients (43 per cent), while eight (51 per cent) had multiple right and left coronary arterial aneurysms. The average number of severely narrowed coronary arteries in this group was 1.5/patient, and only one patient had an aortic aneurysm. Therefore, patients with atherosclerotic aneurysms are more often symptomatic; they have increased heart weights and equal numbers of coronary arterial aneurysms in the right and left vessels, and the majority (89 per cent) have single aneurysms with thrombi in the lumen. Patients with coronary arterial aneurysms secondary to inflammation are younger; the majority of these patients have a prodromal influenza-like syndrome, a low incidence of ischemic heart disease, and multiple coronary arterial aneurysms.

Cardiac fungal infections: Review of autopsy findings in 60 patients

An autopsy study of 60 patients with fungal infections of the heart was undertaken. The patients ranged in age from 2 months to 79 years. Fifteen of the patients had undergone cardiac surgery; neoplasms were found in 13, renal failure in eight, bacterial infections in five, liver disease in five, gastrointestinal disorders in five, and immune disease in four; two had been intravenous drug abusers; other miscellaneous disorders were observed in three. The fungal infection was limited to the myocardium in 27 patients and to the endocardium in 17 patients. Myocardium and endocardium were involved in nine patients and pericardium and myocardium in five; two patients had pericarditis alone. The most frequent organism was Candida (62 per cent). Aspergillus (12 per cent) and Phycomycetes (12 per cent) were also found frequently. In 51 patients (85 per cent) other deep organs, usually lung, kidney, brain, or spleen were involved. Cultures for fungus had been positive in 26 patients prior to death, and postmortem cultures were positive in 29 patients. Patients who had undergone cardiac surgery had a higher incidence of endocarditis (93 per cent), with Candida (53 per cent) being the most frequent cause. Patients who had received antineoplastic drugs, antibiotics, or corticosteroids had a higher incidence of myocarditis (79 per cent), again most often due to Candida (60 per cent).