Grover M. Hutchins

Cardiac sarcoid: a clinicopathologic study of 84 unselected patients with systemic sarcoidosis.

SUMMARY Although sarcoid may involve the myocardium, there is little information on its incidence or significance. We studied 84 consecutive autopsied patients with sarcoidosis. The patients ranged in age from 18-80 years (average 46 years) and 61% were women; 23 (27%) of them had myocardial granulomas. In eight (35%) these were clinically silent, and in 15 (65%) there was a history of heart failure and/or arrhythmias and conduction defects. Of the 23 patients, only four (17%) had grossly evident, widespread myocardial lesions: three of these four (75%) had documented arrhythmias. All four had sudden, unexpected death at an average age of 36 years; in only two had sarcoid been suspected during life. The other 19 patients (83%) had microscopically evident granulomatous involvement. Of these, eight (42%) had a rhythm or conduction disturbance and three (16%) sudden death, although none of those who suffered sudden death had a recognized rhythm or conduction disturbance. Nine (15%) of those without cardiac sarcoidosis had a rhythm or conduction disturbance and eight (13%) suffered a sudden death.The results show that although myocardial involvement occurs in at least 25% of patients with sarcoid, it most often involves a small portion of myocardium and is clinically silent. Since some of the 61 patients in whom myocardial lesions were not identified may still have had small microscopic granulomas, the true incidence of myocardial sarcoid may be even greater than suggested here. Rhythm and conduction disturbances are more common in the cardiac sarcoid group, but the findings suggest that only the small subset of patients with severe, grossly evident myocardial sarcoid are at increased risk for sudden death.

Long-Term Outcome of Fulminant Myocarditis as Compared with Acute (Nonfulminant) Myocarditis

BACKGROUND Lymphocytic myocarditis causes left ventricular dysfunction that may be persistent or reversible. There are no clinical criteria that predict which patients will recover ventricular function and which cases will progress to dilated cardiomyopathy. We hypothesized that patients with fulminant myocarditis may have a better long-term prognosis than those with acute (nonfulminant) myocarditis. METHODS We identified 147 patients considered to have myocarditis according to the findings on endomyocardial biopsy and the Dallas histopathological criteria. Fulminant myocarditis was diagnosed on the basis of clinical features at presentation, including the presence of severe hemodynamic compromise, rapid onset of symptoms, and fever. Patients with acute myocarditis did not have these features. The incidence of the end point of this study, death or heart transplantation, was ascertained by contact with the patient or the patients family or by a search of the National Death Index. The average period of follow-up was 5.6 years. RESULTS A total of 15 patients met the criteria for fulminant myocarditis, and 132 met the criteria for acute myocarditis. Among the patients with fulminant myocarditis, 93 percent were alive without having received a heart transplant 11 years after biopsy (95 percent confidence interval, 59 to 99 percent), as compared with only 45 percent of those with acute myocarditis (95 percent confidence interval, 30 to 58 percent; P=0.05 by the log-rank test). Fulminant myocarditis was an independent predictor of survival after adjustments were made for age, histopathological findings, and hemodynamic variables. The rate of transplantation-free survival did not differ significantly between the patients considered to have borderline myocarditis and those considered to have active myocarditis according to the Dallas histopathological criteria. CONCLUSIONS Fulminant myocarditis is a distinct clinical entity with an excellent long-term prognosis. Aggressive hemodynamic support is warranted for patients with this condition.

Infarct Expansion Versus Extension: Two Different Complications of Acute Myocardial Infarction

Abstract Precordial S-T segment mapping studies have suggested that extension of acute transmural myocardial infarcts occurs in up to 80 percent of patients within 6 days. To determine the morphologic nature of extension 76 consecutive acute myocardial infarcts aged 30 days or less were studied. All infarcts had been clinically diagnosed and proved at autopsy. Extension (histologically more recent foci of contraction band necrosis around an infarct) was found in only 13 infarcts (17 percent). However, “expansion” (acute dilatation and thinning of the area of infarction not explained by additional myocardial necrosis) was present in 45 infarcts (59 percent). Severe expansion did not develop until 5 days after infarction and was greater with transmural and first infarcts. Clinically diagnosed extension manifested by new pain, S-T segment elevation, rise in serum creatine kinase level and increased congestive heart failure occurred in 14 of the 76 patients (18 percent). At autopsy these clinical extensions were associated with expansion alone in three patients, with extension alone in two and with both in nine. The study shows that expansion is a common complication of acute myocardial infarction that can worsen cardiac function through left ventricular dilatation and can mimic or possibly cause infarct extension. In contrast, extension with additional myocardial necrosis is an infrequent accompaniment of acute myocardial infarction and is usually a result of hypoperfusion.

Evaluation of a QRS scoring system for estimating myocardial infarct size

This study correlated the location and size of posterolateral myocardial infarcts (MIs) measured anatomically with that estimated by quantitative criteria derived from the standard 12-lead ECG. Twenty patients were studied who had autopsy-proved, single, posterolateral MIs and no confounding factors of ventricular hypertrophy or bundle branch block in their ECG. Left ventricular anatomic MI size ranged from 1 to 46%. No patient had a greater than or equal to 0.04-second Q wave in any electrocardiographic lead and only 55% had a 0.03-second Q wave. A 29-point, simplified QRS scoring system consisting of 37 weighted criteria was applied to the ECG. Points were scored by the ECG in 85% of the patients (range 1 to 8 points). MI was indicated by a wide variety of QRS criteria; 19 of the 37 criteria from 8 different electrocardiographic leads were met. The correlation coefficient between MI size measured anatomically and that estimated by the QRS score was 0.72. Each point represented approximately 4% MI of the left ventricular wall.

Myocardial lesions of progressive systemic sclerosis. A cause of cardiac dysfunction.

The nature, prevalence, functional significance, and indeed existence of myocardial disease in progressive systemic sclerosis (PSS) has been debated. In this study the clinical and pathological features of 52 autopsied patients were analyzed in an attempt to resolve these questions. A distinctive focal myocardial lesion ranging from contraction band necrosis to replacement fibrosis throughout both ventricular walls was present in 23 patients who had widely patent extramural coronary arteries. There were no morphologic abnormalities of the intramyocardial coronary arteries to account for these lesions. Comparing those patients having severe (13), mild (10), or no (24) PSS myocardial lesions, and patent extramural coronary arteries, there were no major differences in age, sex, frequency and severity of pulmonary, renal or hypertensive disease which could account for the myocardial necrosis and fibrosis. The three groups did differ, however, with regard to clinical cardiac abnormalities: ventricular arrhythmias and conduction disturbances were six and two times as frequent, respectively, in those with severe myocardial PSS compared to the other two groups. A pattern of primary myocardial disease with intractable congestive heart failure resulted from severe myocardial PSS in four patients, angina pectoris with normal coronary arteries was associated with the severe myocardial lesion in three patients, and sudden death in five. The occurrence of contraction band necrosis suggests that the myocardial damage in PSS might be due to intermittent vascular spasm of the type recognized in the digits and possibly kidneys and lungs, i.e., an intramyocardial Raynauds phenomenon. The findings in our patients clearly show that myocardial progressive systemic sclerosis is a distinct entity with relatively frequent occurrence which may lead to arrhythmias, congestive heart failure, angina pectoris with normal coronary arteries and sudden deat.

Clinicopathoiogic description of myocarditis

Histologic evidence of myocarditis was demonstrated in 35 of 348 patients submitted to endomyocardial biopsy over 5 years. Analysis of the histologic findings and clinical course of these patients resulted in a new clinicopathologic classification of myocarditis in which four distinct subgroups are identified. Patients with fulminant myocarditis become acutely ill after a distinct viral prodrome, have severe cardiovascular compromise, multiple foci of active myocarditis by histologic study and ventricular dysfunction that either resolves spontaneously or results in death. Patients with acute, chronic active and chronic persistent myocarditis have a less distinct onset of illness. Patients with acute myocarditis present with established ventricular dysfunction and may respond to immunosuppressive therapy or their condition may progress to dilated cardiomyopathy. Those with chronic active myocarditis initially respond to immunosuppressive therapy, but they have clinical and histologic relapses and develop ventricular dysfunction associated with chronic inflammatory changes including giant cells on histologic study. Chronic persistent myocarditis is characterized by a persistent histologic infiltrate, often with foci of myocyte necrosis but without ventricular dysfunction despite other cardiovascular symptoms such as chest pain or palpitation.

Two-dimensional echocardiographic recognition of myocardial injury in man: comparison with postmortem studies.

To assess the accuracy of phased-array two-dimensional echocardiography in detecting, localizing and quantifying myocardial injury in man, the relationship of two-dimensional echocardiographic wall motion abnormalities to morphologic evidence of myocardial infarction was evaluated in 20 autopsied patients. Comparisons were made between independent two-dimensional echocardiographic readings of left ventricular segmental wall motion and morphologic evidence of myocardial injury. Of 15 infarcts, 14 were detected by regional akinesis, dyskinesis or hypokinesis. The relationship between abnormal segmental wall motion and morphologic evidence of myocardial necrosis or fibrosis was significant. Seventy-nine of 88 (90%) of infarcted segments showed abnormal wall motion, although 38 of 82 (46%) of morphologically normal segments also demonstrated wall motion abnormalities. Fifty-eight of 65 segments that showed regional akinesis or dyskinesis were transmurally infarcted. Twenty-five of 38 pathologically normal segments seen by two-dimensional echocardiography as akinetic or dyskinetic were adjacent to scar. Hypokinesis was nonspecific (31 segments normal, 21 subendocardial infarction). Normal wall motion excluded transmural infarction (0 of 46 segments), but in one patient was associated with subendocardial injury (nine/42 segments). We assessed circumferential extent of regional akinesis or dyskinesis in blind fashion with a light-pen system, expressed as a percentage of end-diastolic circumference, and compared this with the corresponding cross section of the left ventricle examined pathologically. By linear regression, extent of two-dimensional echocardiographic akinesis or dyskinesis and extent of left ventricular circumference demonstrating morphologic evidence of transmural infarction correlated well: pathologic percent circumference infarcted = 1.14 (two-dimensional echocardiographic percent circumference akinetic/dyskinetic) −14.48; r = 0.90. Wall motion abnormalities therefore overestimated the amount of myocardial circumference infarcted, possibly because of the proximity of morphologically normal segments to scar or because segments adjacent to the lesions were reversibly ischemic. The results suggest that two-dimensional echocardiographic evidence of regional wall motion abnormality is sensitive in detecting and localizing segmental pathologic myocardial lesions, but overestimates their extent.

The causes of dilated cardiomyopathy: A clinicopathologic review of 673 consecutive patients

OBJECTIVES The purpose of this study was to document the various causes of dilated cardiomyopathy in a large group of adult patients with congestive heart failure. BACKGROUND Previous reports of the causes of dilated cardiomyopathy have usually been case reports of a single specific etiology or review articles. The frequency of any single specific heart muscle disease is largely unknown. METHODS We evaluated 673 patients referred for congestive heart failure due to dilated cardiomyopathy. The evaluation included medical history, physical examination, routine blood chemistry and hematologic measurements, electrocardiography and echocardiography. Thyroid function tests, antinuclear antibody tests and urinary vanillylmandelic acid and metanephrine levels were also obtained. Endomyocardial biopsy with right heart catheterization was performed in every patient. Coronary arteriography was performed in patients who had at least two standard cardiovascular risk factors or a history suggestive of myocardial ischemia. The cases were retrospectively reviewed, and a final cause for dilated cardiomyopathy was listed for each patient. RESULTS The most common causes of dilated cardiomyopathy were idiopathic origin (47%), idiopathic myocarditis (12%) and coronary artery disease (11%). The other identifiable causes of dilated cardiomyopathy made up 31% of the total cases. CONCLUSIONS Idiopathic dilated cardiomyopathy is a common cause of congestive heart failure. Specific heart muscle diseases occur with much less frequency.

The relationship between coronary artery lesions and myocardial infarcts: Ulceration of atherosclerotic plaques precipitating coronary thrombosis

A review of 494 myocardial lesions at least 3 cm. in one dimension reveal 418 (85 per cent) related to atherosclerotic coronary lesions, 55 (11 per cent) related to coronary emboli of various types, 18(3.5 per cent) without specific coronary lesions but related to clinical events associated with coronary hypoperfusion, and 3 (0.5 per cent) associated with miscellaneous coronary lesions. In 399 of 418 (96 per cent) atherosclerotic coronary lesions of all ages complete occlusion (remote of fresh) or histological evidence of lumenal recanalization was present. These coronary lesions were situated within extramural coronary artery segments one to several centimeters proximal to the myocardial lesions which were confined to the distribution of the respective partially or totally occluded coronary segments. In the atherosclerotic cornary lesions less than 2 weeks of age partially or totally occlusive thrombus was found in 67 of 69 (97 per cent) cases and a underlying plaque ulceration, erosion, or rupture was present in 64 of 69 (93 per cent) instances. These endothelial and intimal injuries were generally focal in nature, often extending over a length of only 100 to 200 mu. In no instance could it be stated with certainty that the oldest portion of the atherosclerotic ulceration-thrombus complex was younger in age than its associated myocardial lesion. On the contrary, in 10 of 69 (14 per cent) of the cases portion of the coronary thrombus, usually at the site of plaque ulceration, were histologically older than the myocardial lesion. In addition, the presence of thrombus and plaque debris admixtures further suggested the antecedent nature of the coronary lesion in relation to the myocardial lesion. Atherosclerotic coronary lesions associated with myocardial lesions of 2 to 8 weeks of age had identifiable thromboses in all instances and underlying plaque ulceration, erosions, or ruptures in 17 of 21 (80 per cent). Endothelial injuries were more difficult to assess due to the obscuring features of organizing lumenal thrombus. Interface i.e., plaque ulceration, erosions, or ruptures, were reliably detectable up to approximately on month of age. Coronary arter thromboemboli accounted for a significant percentage of myocardial lesions, were usually associated with normal or minimal coronary artery disease, and frequently involved smaller intramural coronary vessels of the heart. Organization and recanalization of thromboemboemboli tended to be rapid and complete so that in the late stages the residual intimal plaque was sometimes difficult to identify. Myocardial lesions related to clinical events associated with coronary artery hypoperfusion centric, and not confined to the distribution of a single coronary artery. They were unassociated with acute coronary lesions and histologically displayed contraction band necrosis more frequently than the embolic and atherosclerotic related lesions. An explanation was found for the overwhelming majority of myocardial lesions...

Echocardiographic Findings in Fulminant and Acute Myocarditis

OBJECTIVES We sought to use echocardiography to assess the presentation and potential for recovery of left ventricular (LV) function of patients with fulminant myocarditis compared with those with acute myocarditis. BACKGROUND The clinical course of patients with myocarditis remains poorly defined. We have previously proposed a classification that provides prognostic information in myocarditis patients. Fulminant myocarditis causes a distinct onset of illness and severe hemodynamic compromise, whereas acute myocarditis has an indistinct presentation, less severe hemodynamic compromise and a greater likelihood of progression to dilated cardiomyopathy. METHODS Echocardiography was performed at presentation and at six months to test the hypothesis that fulminant (n = 11) or acute (n = 43) myocarditis could be distinguished morphologically. RESULTS Patients with both fulminant (fractional shortening 19 +/- 4%) and acute myocarditis (17 +/- 7%) had LV systolic dysfunction. Patients with fulminant myocarditis had near normal LV diastolic dimensions (5.3 +/- 0.9 cm) but increased septal thickness (1.2 +/- 0.2 cm) at presentation, while those with acute myocarditis had increased diastolic dimensions (6.1 +/- 0.8 cm, p < 0.01 vs. fulminant) but normal septal thickness (1.0 +/- 0.1 cm, p = 0.01 vs. fulminant). At six months, patients with fulminant myocarditis had dramatic improvement in fractional shortening (30 +/- 8%) compared with no improvement in patients with acute myocarditis (19 +/- 7%, p < 0.01 for interaction between time and type of myocarditis). CONCLUSIONS Fulminant myocarditis is distinguishable from acute myocarditis by echocardiography. Patients with fulminant myocarditis exhibit a substantial improvement in ventricular function at six months compared with those with acute myocarditis. Echocardiography has value in classifying patients with myocarditis and may provide prognostic information.