Marfan's syndrome. Aortic complications studied by scanography

Of all the pathological forms and variations of Marfan's syndrome, aortic aneurysm is one of the most important because of the high risk of medial dissection caused by cystic necrosis. The value of computerised axial tomography in the diagnosis of the aortic lesions of Marfan's syndrome was assessed in three clinical cases. Compared to angiography and echocardiography, computerised tomography avoids arterial catheterisation while demonstrating all anatomical changes of the aorta, coronary arteries, pericardium and mediastinum, with significantly less radiation. The point of entry of the dissection was not visualised in two of the cases presented. Therefore, computerised axial tomography should be considered in patients with Marfan's syndrome.     

Angina pectoris, myocardial infarction and sudden cardiac death with normal coronary arteries: a clinicopathologic study of 9 patients with progressive systemic sclerosis

The syndrome of angina pectoris or acute myocardial infarction without obstructive coronary artery disease has been the subject of much interest. We studied nine autopsied patients with progressive systemic sclerosis and evidence of ischemic heart disease but morphologically normal coronary arteries. Three patients had angina pectoris and three others chest pains of unknown etiology, six had ventricular arrhythmias, four had clinically suspected acute myocardial infarction, and eight had sudden cardiac death. At autopsy extensive focal myocardial necrosis was present in seven patients and myocardial scarring in all nine, but all patients had widely patent intramural and extramural coronary arteries. The finding of contraction band myocardial necrosis in seven of the eight patients who experienced sudden death suggests that the myocardial damage was a consequence of reperfusion of focally nonperfused myocardium, and thus due to a myocardial Raynaud's phenomenon. Patients with PSS may provide a model of spasm of intramyocardial vessels causing angina pectoris or myocardial infarction with morphologically normal coronary arteries.     

Radiation heart disease. Analysis of 16 young (aged 15 to 33 years) necropsy patients who received over 3,500 rads to the heart

Certain clinical and necropsy findings are described in 16 young (aged 15 to 33 years) patients who received greater than 3,500 rads to the heart five to 144 months before death. All 16 had some radiation-induced damage to the heart: 15 had thickened pericardia (five of whom had evidence of cardiac tamponade); eight had increased interstitial myocardial fibrosis, particularly in the right ventricle; 12 had fibrous thickening of the mural endocardium and 13 of the valvular endocardium. Except for valvular thickening, the changes were more frequent in the right side of the heart than in the left, presumably because of higher radiation doses to the anterior surface of the heart. In six of the 16 study patients and in one of 10 control subjects, one or more major epicardial coronary arteries were narrowed from 76 to 100 percent in cross-sectional area by atherosclerotic plaque; one patient had a healed myocardial infarct at necropsy and one died suddenly. In 10 patients and in the 10 control subjects, the four major epicardial coronary arteries were examined quantitatively: 6 percent of the 469 five millimeter segments of coronary artery from the patients were narrowed from 76 to 100 percent (controls = 0.2 percent, p = 0.06) and 22 percent were narrowed from 51 to 75 percent (controls = 12 percent). The proximal portion of the arteries in the patients had significantly more narrowing than the distal portions. The arterial plaques in the patients were largely composed of fibrous tissue; the media were frequently replaced by fibrous tissue, and the adventitia were often densely thickened by fibrous tissue. In five patients, there was focal thickening (with or without luminal narrowing) of the intramural coronary arteries. Thus, radiation to the heart may produce a wide spectrum of functional and anatomic changes but particularly damage to the pericardia and the underlying epicardial coronary arteries.     

Atypical giant cell arteritis predominantly involving intramural coronary arteries: a case showing refractory dialysis-related hypotension

Giant cell arteritis involving intramural coronary artery branches is rare, and its clinical features remain poorly understood. We report a 56-year-old hemodialysed patient with a history of mitral valve replacement, who presented with "fever of unknown origin" and intractable hypotension. The antemortem diagnosis was very difficult and the autopsy revealed giant-cell-rich vasculitis in arteries in multiple organs. The heart was most severely involved, in which almost all of the intramural coronary artery branches were infiltrated by many multinucleated giant cells, macrophages, and lymphocytes with luminal narrowing, but the epicardial segments of the coronary arteries were spared. Superimposed on the preexisting valvular heart disease, the vasculitic lesions were thought to play a central role in severe cardiac dysfunction resulting in dialysis-related hypotension, which led to fatal non-occlusive mesenteric ischemia. This case highlights the possibility that giant cell arteritis of intramural coronary arteries could be an uncommon underlying cause of refractory dialysis-related hypotension.     

Fate of intramural coronary arteries after arterial switch operation

BACKGROUND: To evaluate the impact of intramural coronary arteries for the peri-, postoperative and long-term course after arterial switch operation (ASO). METHODS: ASO was performed in 351 patients between 1981 and 2000 with dextrotransposition of the great arteries (d-TGA). Five patients (1.4 %) had an intramural coronary artery. Coronary transfer was performed with a collar under dissection of the commissure without longitudinal splitting of the intramural section. RESULTS: None of these patients died; the intraoperative course was uneventful, and no myocardial ischemic changes were observed. In three patients, follow-up cardiac catheterization after 5, 16 and 53 months revealed an occlusion of the intramural coronary ostium. Exercise electrocardiography and myocardial scintiscan showed myocardial ischemia. Two of these patients underwent a successful internal mammary artery bypass. CONCLUSIONS: The intramural course of coronary arteries in patients with d-TGA is rare and does not cause increased mortality or myocardial infarction rates. However, the risk of coronary occlusion over time seems to be high. Therefore, patients with this condition require selective coronary angiography and frequent exercise investigations. Revascularization with an internal mammary artery bypass may be indicated.     

Smooth muscle contraction bands in the media of coronary arteries: A postmortem marker of antemortem coronary spasm?

To date, no unequivocal morphologic markers have been described that would allow the diagnosis of coronary artery spasm to be made at autopsy. The coronary arteries of 63 adult patients without myocardial infarction were examined at autopsy, and the presence of medial smooth muscle contraction bands in these vessels was correlated with other vascular changes, myocardial pathologic changes and clinical history. These contraction bands have not been reported previously in human coronary arteries, but they were identified in experimental vascular spasm induced with catecholamines. It was found that 47 of the 63 cases were positive for contraction bands. As evidence of an antemortem process, there was a significant correlation between these changes and the presence of nonocclusive microthrombi, found in 25 cases. Contraction bands were also highly correlated with atherosclerotic plaque ruptures and mural plaque hemorrhages, which may be secondary to coronary spasm. In 78.7% of the cases positive for contraction bands, the cause of death was related to a diagnosis possibly associated with high catecholamine levels. On the basis of experimental evidence and the correlations identified in this study, coronary artery medial smooth muscle contraction bands may represent a postmortem marker of antemortem coronary spasm.     

Intimal proliferation of smooth muscle cells as an explanation for recurrent coronary artery stenosis after percutaneous transluminal coronary angioplasty

The pathologic changes in the coronary arteries of three patients who died 5, 17 and 62 days, respectively, after percutaneous transluminal coronary angioplasty were studied. Changes in the vessel wall seen early after angioplasty included focal denudation of the endothelium, splits in the intima extending to and along the inner aspect of the media, focal intimal necrosis and adventitial hemorrhage. Extensive medial dissections were seen in the coronary arteries of the two patients who died 5 and 17 days after coronary angioplasty. Fibrin was deposited on the surface of the intima, within intimal cracks and in areas of intimal and medial necrosis. Focal proliferation of smooth muscle cells was prominent on neointimal surfaces of the coronary artery from the patient who died 17 days after angioplasty. The previously dilated coronary segment from the patient who died 62 days after angioplasty was stenosed by an extensive recent proliferation of smooth muscle cells that were distributed over the entire circumference of the intimal surface as well as within gaps in the old atherosclerotic plaques. This type of intimal proliferation would appear to be responsible for the recurrent coronary artery stenosis that develops in some patients after coronary angioplasty.     

Primary Aortoduodenal Fistula: A Unique Presentation of a Pseudoaneurysm Associated with Cystic Medial Necrosis

A 42-yr-old man who exsanguinated from an acute upper gastrointestinal bleed was found to have a primary aortoduodenal fistula on postmortem examination. The fistula arose in an aortic pseudoaneurysm associated with cystic medial necrosis. Although there was nosuggestion of Marfan's syndrome on physical examination, there was cystic medial necrosis of not only the involved aorta, but also other systemic arteries. Primary aortoduodenal fistula is a rare cause of acute upper gastrointestinal bleeding and is usually associated with atherosclerotic disease of the aorta. This is the first report of a pseudoaneurysm associated with cystic medial necrosis presenting as an aortoduodenal fistula.     

The clinical recognition of dissecting aortic aneurysm.

The clinical, roentgenologic and laboratory findings in 124 patients with dissecting aneurysm of the aorta are reported. In 53 patients the dissection occurred in the ascending aorta ("proximal" dissection), and in 71 patients the site of origin was the descending thoracic aorta ("distal" dissection). Certain distinct clinical differences between the groups were apparent. Although hypertension was an important predisposing factor, it was significantly more common in distal dissection, as was atherosclerosis. Back pain and hypertension on hospital presentation characterized patients with distal dissection. Conversely patients with proximal dissection were younger and had a significantly higher incidence of Marfan's syndrome, cystic medial necrosis, anterior chest pain, pulse deficits, neurologic compromise, aortic insufficiency and congestive heart failure. In both groups, syncope appeared to correlate well with the occurrence of cardiac tamponade. Chest roentgenograms almost always showed an abnormal aortic contour. Aortic angiography, when performed, was usually confirmatory of the diagnosis.     

Anomalous Intramural Course of Coronary Arteries in Congenital Heart Disease—Three Case Reports and Review of the Literature

Anomalies of the coronary arteries are commonly associated with congenital heart disease (CHD). Anomalies include variations in number, shape, and location of the origin, as well as the course of the coronary artery. An intramural course of one of the coronary arteries is a rather rare condition. Most cases of anomalies are of no clinical relevance and are found incidentally during evaluation for other cardiac conditions. However, they become of importance when cardiac surgery/intervention, especially valve replacement, needs to be performed. We conducted a literature review for coronary anomalies with an intramural course in patients with CHD and include three cases from our own clinic. Note that transposition of the great arteries seems to be the condition most frequently associated with an intramural course of one of the coronary arteries. In case of a suspected anomaly, transesophageal echocardiography presents a valuable tool to visualize the origin and course of the coronary artery, as well as the relationship to important anatomical structures like the aortic or pulmonary valve. Alternatively, cardiac magnetic resonance imaging can be used. Although rare, both cardiologists and surgeons need to be aware of intramural courses of coronary arteries to prevent accidental trauma to them and thereby, reducing the risk for the patient.     

Coronary Artery Manifestations of Fibromuscular Dysplasia

Fibromuscular dysplasia (FMD) involving the coronary arteries is an uncommon but important condition that can present as acute coronary syndrome, left ventricular dysfunction, or potentially sudden cardiac death. Although the classic angiographic “string of beads” that may be observed in renal artery FMD does not occur in coronary arteries, potential manifestations include spontaneous coronary artery dissection, distal tapering or long, smooth narrowing that may represent dissection, intramural hematoma, spasm, or tortuosity. Importantly, FMD must be identified in at least one other noncoronary arterial territory to attribute any coronary findings to FMD. Although there is limited evidence to guide treatment, many lesions heal spontaneously; thus, a conservative approach is generally preferred. The etiology is poorly understood, but there are ongoing efforts to better characterize FMD and define its genetic and molecular basis. This report reviews the clinical course of FMD involving the coronary arteries and provides guidance for diagnosis and treatment strategies.     

Spontaneous coronary artery dissection: a neglected cause of acute myocardial ischaemia and sudden death.

Spontaneous coronary artery dissection is a rare cause of acute myocardial ischaemia. Eight consecutive fatal cases which occurred in women aged 34-54 years (mean 43) are described. The dissection involved the left anterior descending coronary artery in four, the left main trunk in two, the right coronary artery in one, and both left anterior descending and circumflex arteries in one. The clinical presentation was sudden death in six cases, and acute myocardial infarction in two. Diagnosis was made at necropsy in every case but one, in which coronary dissection was diagnosed during life by selective coronary angiography. The only ascertained risk factor was hypertension in one patient; none of the women was in the puerperium, and Marfan syndrome was excluded in all. Histology showed a haematoma between the coronary tunica media and adventitia, that flattened and occluded the lumen; a coronary intimal tear was detected in only two cases. Unusual histological findings were cystic medial necrosis in one case, eosinophilic inflammatory infiltrates in four, and angiomatosis of the tunica adventitia in one. Patients dying of spontaneous coronary dissection are usually middle aged women, with no coronary atherosclerosis and apparently no risk factors. Spontaneous coronary artery dissection is unpredictable, and sudden death is the usual mode of clinical presentation. Prompt diagnosis and life saving treatment is far from being achieved.     

Connexion ectopique de l’artère coronaire gauche dans le sinus controlatéral avec ou sans passage intramural : comment et pourquoi les différencier ?

Among the wide spectrum of congenital abnormalities of coronary arteries, a left coronary artery connected with the contralateral sinus is considered as an anatomical high-risk while associated with a proximal preaortic course. The ectopic connection may be associated with or without intramural pathway regarding the aortic wall. An intramural pathway is a characteristic to be considered because it is able to be involved in myocardial ischemia-related adverse events of which the sudden death. Therefore, making an identification of an intramural pathway is essential for the ectopic connections of the left coronary artery associated with a preaortic course.     

Hemorrhagic myocardial infarction associated with aortocoronary bypass revascularization.

Experimentally, hemorrhage and extension of myocardial infarction occur commonly when there is reperfusion after coronary artery occlusion. To investigate this hazard in a clinical setting, we compared the histopathologic picture of myocardial infarction in 44 patients who had undergone aortocoronary bypass: 14 (Group I) had myocardial infarction that predated aortocoronary bypass by 1 to 7 days; 13 (Group II) had infarction 1 to 14 days after the surgery; and 17 (Group III) had infarction 15 to 90 days postoperatively. All 44 patients had two or more coronary arteries with luminal narrowing of more than 75 per cent and patent vein grafts to arteries supplying areas of infarction. Hemorrhagic infarcts were present in 57 per cent of patients (eight of 14) in group I and 38 per cent of patients (five of 13) in Group II, contrasting with 6 per cent of patients (one of 17) in Group III (P < 0.005 and P < 0.05, respectively). In hemorrhagic infarcts, the extravasated blood formed irregular intramural dissecting tracts beyond the area of infarction, and foci of myocardial necrosis were present in the border zones. Infarcts affected more than 50 per cent of the left ventricular muscle in 64 per cent of cases of hemorrhagic infarction and in 13 per cent of cases of nonhemorrhagic infarction (P < 0.05). The prevalence of hemorrhagic infarction after revascularization may account for the high mortality of evolving and perioperative myocardial infarction associated with aortocoronary bypass, and this finding militates against wholesale immediate revascularization in patients who have uncomplicated myocardial infarction.     

Obstructive intramural coronary amyloidosis and myocardial ischemia are common in primary amyloidosis

Purpose

The purpose of this study was to determine the prevalence of clinical syndromes and pathologic changes of myocardial ischemia due to obstructive intramural coronary amyloidosis among patients with primary amyloidosis and cardiac involvement.

Subjects and methods

Medical records and pathologic specimens were reviewed from 96 patients with primary amyloidosis and cardiac involvement at autopsy or after cardiac transplantation during a 20-year period. Medical records were reviewed for patient demographic and clinical characteristics, including evidence for syndromes of myocardial ischemia. Pathologic specimens were examined for obstructive intramural coronary amyloidosis and microscopic changes of myocardial ischemia.

Results

Obstructive intramural coronary amyloidosis was present in 63 of 96 patients (66%). Microscopic changes of myocardial ischemia were more common in patients with obstructive intramural coronary amyloidosis (86%) than in those without (52%) (P <.001). In the 76 patients without coexistent severe epicardial coronary atherosclerosis, changes of myocardial ischemia were more common in those with obstructive intramural coronary amyloidosis (83%) than in those without (45%) (P <.001). In patients who had tissue available for review, none had obstruction of epicardial coronary arteries from amyloid. Syndromes of myocardial ischemia affected 16 patients (25%) with obstructive intramural coronary amyloidosis but only 2 patients (6%) without (P = .027). For 11% of the patients with obstructive intramural coronary amyloidosis, a syndrome of myocardial ischemia consisting of acute myocardial infarction or angina pectoris was the first manifestation of primary amyloidosis.

Conclusion

Most patients with primary systemic amyloidosis and cardiac involvement have obstructive intramural coronary amyloidosis and associated microscopic changes of myocardial ischemia. Syndromes of myocardial ischemia may occur in these patients.     

A case of an intramural coronary artery associated with transposition of the great arteries

One case of an intramural coronary artery in association with transposition of the great arteries is presented. A 16-month-old infant was admitted for the second-stage operation of complete transposition after a subclavian patch plasty for the aortic isthmus stenosis, division of the persisting arterial duct, and pulmonary artery banding had been performed in the first-stage operation soon after birth. The primarily planned arterial switch operation was, however, not performed because of an unusual coronary artery morphology and pattern--a separated origin of both coronary arteries from the posterior, "facing" sinus of Valsalva and an intramural course of the left anterior descending artery. This very rare case of coronary artery variation is presented from the surgical point of view.     

Coronary anatomy and arteriography in patients with unstable angina pectoris.

A prospective series of 188 patients with the syndrome of unstable angina pectoris undergoing coronary arteriography was reviewed to determine the spectrum of anatomic coronary artery disease, suitability for coronary revascularization and in-hospital morbidity and mortality. Thirty-two patients demonstrated normal to moderately diseased coronary arteries. None of these patients sustained myocardial infarction or died. Twenty patients (10.6 percent) had normal coronary arteriograms. Of the 156 patients having severe coronary artery disease (greater than 70 percent stenosis), 20 patients (13 percent) had left main coronary artery disease. One hundred forty-two patients (91 percent) were potential candidates for coronary surgery; 14 were not candidates because of distal vessel disease or poor left ventricular function. During cardiac angiography or in the subsequent hospital period 12 patients sustained a myocardial infarction and 7 of these died. Of these seven, six had left main coronary artery disease and one had three vessel disease. In three patients who died (1.9 percent of those with severe coronary artery disease) the death may have been related to cardiac catheterization because evidence of myocardial necrosis began within 24 hours of study. Thus, patients with the syndrome of unstable angina pectoris usually presented with severe coronary artery disease and were candidates for coronary revascularization. The anatomic severity of coronary artery disease appeared to be the most important factor contributing to myocardlal infarction or death after cardiac catheterization. Mortality after catheterization was primarily associated with left main coronary artery disease.     

Hemodynamic principles in the control of coronary blood flow

The effects of atherosclerotic epicardial stenoses on coronary vascular resistance can be understood in terms of basic principles of fluid mechanics. Resistance is directly related to the pressure drop across the stenosis and inversely related to flow. Even with a fixed anatomic stenosis, however, resistance is not fixed; it increases as flow across the stenosis increases. This exacerbates the pressure drop across the stenosis that develops as a result of flow; at high flows, large pressure drops can occur. This characteristic of flow through stenotic lesions can contribute to a "steal" phenomenon between either epicardial or intramural coronary arteries. Studies have also shown the clinical importance and influence of dynamic alterations in coronary resistance, occurring either at the large or small vessel level. In addition, compressive forces exerted by the myocardium or by elevated intraventricular pressures can increase coronary vascular resistance, and thus interfere with myocardial perfusion. All of these factors must be considered in order to obtain a comprehensive understanding of the mechanisms leading to myocardial ischemia and, therefore, to the clinical syndrome of angina pectoris.     

Intramural ("small vessel") coronary artery disease in hypertrophic cardiomyopathy

Many patients with hypertrophic cardiomyopathy have signs and symptoms of myocardial ischemia and dysfunction. Although hypertrophy and increased left ventricular pressure can account for such abnormalities, altered small intramural coronary arteries have also been described in such patients. To determine the prevalence and extent as well as the clinical relevance of abnormal intramural coronary arteries, a histologic analysis of left ventricular myocardium obtained at necropsy was performed in 48 patients with hypertrophic cardiomyopathy (but without atherosclerosis of the extramural coronary arteries) and in 68 control patients with either a normal heart or acquired heart disease. In hypertrophic cardiomyopathy, abnormal intramural coronary arteries were characterized by thickening of the vessel wall and a decrease in luminal size. The wall thickening was due to proliferation of medial or intimal components, or both, particularly smooth muscle cells and collagen. Of the 48 patients with hypertrophic cardiomyopathy, 40 (83%) had abnormalities of intramural coronary arteries located in the ventricular septum (33 patients), anterior left ventricular free wall (20 patients) or posterior free wall (9 patients); an average of 3.0 +/- 0.7 abnormal arteries were identified per tissue section. Altered intramural coronary arteries were also significantly more common in tissue sections having considerable myocardial fibrosis (31 [74%] of 42) than in those with no or mild fibrosis (31 [30%] of 102; p less than 0.001). Abnormal intramural coronary arteries were also identified in three of eight infants who died of hypertrophic cardiomyopathy before 1 year of age. In contrast, only rare altered intramural coronary arteries were identified in 6 (9%) of the 68 control patients (0.1 +/- 0.05 abnormal arteries per section; p less than 0.001) and those arteries showed only mild thickening of the wall and minimal luminal narrowing. Moreover, of those patients with abnormal intramural coronary arteries, such vessels were about 20 times more frequent in patients with hypertrophic cardiomyopathy (0.9 +/- 0.2/cm2 myocardium) than in control patients (0.04 +/- 0.02/cm2 myocardium). Hence, abnormal intramural coronary arteries with markedly thickened walls and narrowed lumens are present in increased numbers in most patients with hypertrophic cardiomyopathy studied at necropsy and may represent a congenital component of the underlying cardiomyopathic process.(ABSTRACT TRUNCATED AT 400 WORDS)     

Cystic medial necrosis of the aorta in patients without Marfan's syndrome: surgical outcome and long-term follow-up

A retrospective analysis was performed to determine the surgical outcome and long-term follow-up of patients with documented cystic medial necrosis of the aorta. Ninety-three patients were diagnosed as having cystic medial necrosis at the Cleveland Clinic between July 1963 and December 1987 (72% men aged 26 to 77 years, mean 55). Patients who met the standard diagnostic criteria for Marfan's syndrome were deliberately excluded. Sixty-eight percent of the patients had a diastolic murmur and chest roentgenogram revealed a dilated aortic arch in 58% and cardiomegaly in 63%. Cardiac catheterization in 76 patients demonstrated aortic root dilation in 78%, aortic regurgitation in 72%, aortic dissection in 32% and coronary artery disease in 32%. Ninety patients underwent surgery including composite graft repair with reimplantation of the coronary arteries in 34%. Follow-up, obtained on 90 (97%) of the 93 patients, ranged in duration from 0 to 137 months (mean 29). Thirty-four of the 90 patients died (age range 30 to 75 years, mean 60). Ninety-four percent of the known causes of death were related to the cardiovascular system; 65% were the result of aortic dissection or rupture or sudden death. Ninety-six percent of survivors were in New York Heart Association functional class I or II. Overall estimated survival at 1, 3 and 5 years was 72.2%, 63.5% and 57.4%, respectively. Actuarial survival in patients who underwent composite graft reconstruction was 84% at 5 years. The presence of a diastolic murmur at initial presentation was associated with a poor prognosis (p = 0.03).(ABSTRACT TRUNCATED AT 250 WORDS)