Biomedical subjects
Publications and source records attributed to W D Edwards.
We conclude that echocardiographically detected cardiac rhabdomyomata are common in patients with tuberous sclerosis complex, and are more prevalent and prominent in the younger patient. Tumors regress in size or number, or both, in most patients aged < 4 years, and less so in older patients. Cardiac rhabdomyomata are associated with a higher incidence of preexcitation and may increase the risk for arrhythmia.
The objective of this study was to evaluate the incidence and correlates of sudden unexpected nontraumatic death among young adults in a well-surveyed population. The incidence and pathogenesis of sudden unexpected nontraumatic death in a young adult population (aged 20 to 40 years old) have not been well defined. All residents 20 to 40 years old from Olmsted County, Minnesota, who had nontraumatic sudden death between 1960 and 1989 were included. Histologic and gross cardiac specimens were examined. The incidence of sudden death was estimated based on the ratio of number of observed events to relative census data for the Olmsted County population from the last 3 decades. Statistical comparisons between age decades were obtained with the chi-square test. Incidence trends were tested using Poisson regression. Of the 54 subjects, 19 were women (4.1/10(5) population annually) and 35 were men (8.7/10(5) population annually). An increase in incidence of sudden death was evident in men. Causes of death included coronary artery disease, noncardiovascular disease, suspected primary arrhythmia, vascular disease, myocarditis, hypertrophic cardiomyopathy, and unknown causes. Gross and histologic features suggestive of right ventricular dysplasia were found in 9 subjects (17%), but 6 of these 9 had other established causes of death. Of the 27 sudden deaths between 1980 and 1989, 9 (33%) had a history of cocaine abuse. A trend in increasing incidence of sudden death in young men is noted. A high prevalence of cocaine abuse was observed in young adults who died suddenly. Histologic features of right ventricular dysplasia were prevalent but were not necessarily the primary cause of death.
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A 23-year-old man with Wiskott-Aldrich syndrome, chronic aortitis, and severe aneurysmal dilatation of the thoracic aorta successfully underwent two-stage graft replacement of the ascending and descending thoracic aorta. Nine years postoperatively, he is asymptomatic and employed full time.
This investigation was performed to provide a comprehensive physicochemical characterization of calcific deposits (CDs) that form on human heart valves under various pathological conditions. We examined and characterized CDs associated with aortic stenosis on congenitally bicuspid valves (n = 10), degenerative aortic stenosis on valves with previously normal anatomy (n = 10), and rheumatic aortic (n = 10) and mitral (n = 10) stenosis. Native and deproteinated CDs underwent chemical analysis and structural characterization, whereas deproteinated CDs were measured for thermodynamic solubility. The CDs in valvular heart disease were microcrystalline apatitic products containing substantial amounts of sodium, magnesium, carbonate, fluoride, and organic fraction. The properties of natural heart valve CDs were compared with those of previously measured CDs that form on or in heart valve bioprostheses. Compared with bioprosthetic valve CDs, natural valve CDs have a higher ratio of calcium to phosphorus, higher crystallinity, and lower solubility. These differences indicate that natural heart valve CDs appear to comprise a more mature biomineral. If the formation of mature CDs proceeds through transient stages involving unstable precursors, then the main strategy for prevention of calcific deterioration of bioprosthetic heart valves would be the development of locally applied long-term inhibitors that both (1) suppress nucleation and growth of more soluble precursors and (2) inhibit subsequent augmentation of less soluble CDs.
BACKGROUND: Congenital pulmonary vein stenosis is a rare cause of obstruction of pulmonary venous blood flow with a high mortality. Acquired pulmonary vein stenosis is an equally serious condition. METHODS: Eight patients (age range, 3 months to 43 years; median age, 1.5 years) underwent surgical relief of pulmonary vein stenosis. Two had congenital pulmonary vein stenosis, 5 had pulmonary vein stenosis that was acquired after surgical treatment of total anomalous pulmonary venous connection, and 1 had pulmonary vein stenosis associated with idiopathic mediastinal fibrosis and calcification. RESULTS: One infant died 2 months after correction of acquired pulmonary vein stenosis. At follow-up extending to 16 years (median follow-up, 6.5 years), 6 patients are in New York Heart Association functional class I, and 1 patient is in class II. CONCLUSIONS: In view of the dismal natural history of untreated pulmonary vein stenosis, prompt surgical relief of the stenosis may be a rewarding undertaking.
Two broad-based masses arising along the right atrial septum in a 31-year-old man were detected by transesophageal echocardiography and were thought to represent partially calcified myxomas. However, at operation they were cystic structures filled with blood and calcific nodules. Microscopically, they represented varices with phleboliths. Angiography and ultrafast computed tomography may help distinguish this entity from myxomas and other right atrial intracavitary masses.
OBJECTIVES: The study objectives were 1) to assess the long-term outcome of patients with biopsy-proved lymphocytic myocarditis (Dallas criteria), and 2) to compare the outcome of these patients with that of patients with idiopathic dilated cardiomyopathy. BACKGROUND: Endomyocardial biopsy is frequently performed in patients presenting with dilated cardiomyopathy to identify lymphocytic myocarditis. Most previous studies of the natural history of myocarditis were performed before the establishment of the Dallas criteria. Thus, it is important to evaluate the prognostic value of positive endomyocardial biopsy findings in patients presenting with dilated cardiomyopathy, using standardized criteria for lymphocytic myocarditis. METHODS: All endomyocardial biopsy results from the Mayo Clinic (October 1979 to April 1988) with a diagnosis of myocarditis were reclassified according to the Dallas criteria. Patients whose biopsy specimens showed borderline or lymphocytic myocarditis were included in the study group; those with systemic inflammatory diseases known to be associated with myocardial involvement were excluded. Study group survival was compared with that for a cohort of patients with idiopathic dilated cardiomyopathy seen at the Mayo Clinic from 1976 to 1987 who had endomyocardial biopsy findings negative for myocarditis. RESULTS: Biopsy specimens from 41 patients met the Dallas criteria for a diagnosis of myocarditis (n = 28) or borderline myocarditis (n = 13). Of these 41 patients, 9 were excluded because of the presence of systemic diseases known to be associated with myocarditis, and 5 patients were excluded because of lack of available follow-up data. The myocarditis study group therefore included 27 patients (10 with borderline myocarditis, 17 with myocarditis). Fifty-eight patients with a diagnosis of idiopathic dilated cardiomyopathy who underwent endomyocardial biopsy served as the comparison cohort. Ejection fraction was lower in patients with idiopathic dilated cardiomyopathy ([mean +/- SD] 25 +/- 11%) than in those with myocarditis (38 +/- 19%, p = 0.001), even though a higher proportion of myocarditis group patients were in New York Heart Association functional class III or IV (63%) than patients in the dilated cardiomyopathy group (30%, p = 0.005). There was no difference in 5-year survival rate between the myocarditis and idiopathic dilated cardiomyopathy groups (56% vs. 54%, respectively). CONCLUSIONS: This study demonstrates that the long-term outcome of patients with biopsy-proved myocarditis seen in a referral setting is poor, although no different from that of patients with idiopathic dilated cardiomyopathy. With the current lack of proved effective treatment for lymphocytic myocarditis and no demonstration of survival benefit for patients with myocarditis, these data suggest that endomyocardial biopsy performed to exclude myocarditis is of limited prognostic value in the routine evaluation of dilated cardiomyopathy.
From December 1964 to October 1993, 40 patients (aged 5 months to 70 years, mean 21.8 years, median 13.6 years) with corrected transposition and systemic atrioventricular valve insufficiency underwent replacement (n = 39) or repair (n = 1) of the systemic atrioventricular valve. Thirty-nine patients had situs solitus and 1 had situs inversus. Associated anomalies included Ebstein's malformation of the systemic atrioventricular valve (n = 22), ventricular septal defect (n = 19), and pulmonary stenosis (n = 14). Preoperatively, 16 patients (40.0%) had complete heart block and 27 patients (67.5%) were in New York Heart Association functional classes III and IV. The early mortality was 10.0% (n = 4) and 8 patients died subsequently. The principal cause of death in all 12 patients was systemic ventricular failure. Overall survival including early mortality was 78.0% at 5 years and 60.7% at 10 years; survival excluding early mortality was 86.7% at 5 years and 67.5% at 10 years. Survivorship correlated with preoperative systemic ventricular ejection fraction of 44% or more (p < 0.001) and later interval of operation (9 deaths in 15 patients before 1981 versus 3 deaths in 25 patients subsequently) (p = 0.06). There were no cases of surgically induced complete heart block. Two patients underwent late reoperations related to the systemic atrioventricular valve prosthesis. Follow-up extended to 26.0 years (median 4.7 years). At last follow-up, 18 of the 28 survivors were in New York Heart Association functional class I, 9 were in class II, and 1 was in class III. We conclude that the results of systemic atrioventricular valve replacement in corrected transposition have improved significantly during the past decade. To preserve systemic ventricular function, we suggest operation be considered at the earliest sign of progressive ventricular dysfunction as assessed by serial clinical evaluation and echocardiography.
Transesophageal echocardiography (TEE) is used frequently in patients with cerebrovascular ischemia. On TEE, a typical appearance of a cardiac fibroelastoma is that of a pedunculated, mobile mass attached to a leaflet of a valve. Surgical excision of the lesion may lead to resolution of the symptoms and prevent further cerebrovascular ischemic events; valve replacement is seldom necessary. Herein we describe three patients with cerebral or ocular ischemia in whom histologic study confirmed a cardiac papillary fibroelastoma after initial detection by TEE. Cardiac papillary fibroelastomas should be considered in the differential diagnosis of transient ischemic attack and stroke, even in cases of recurrent events in the same vascular distribution. Although the use of echocardiography in the evaluation of stroke and transient ischemic attack is controversial, TEE must be considered in patients in whom the cause of cerebrovascular ischemia is unclear after noninvasive neurovascular studies or transthoracic echocardiography, even if the patient's cardiac history and the findings on physical examination are normal.
OBJECTIVE: To determine the initial clinical manifestations and echocardiographic features of right ventricular dysplasia as encountered in a major cardiovascular referral center in the United States. DESIGN: We conducted a retrospective study of cases of right ventricular dysplasia diagnosed at the Mayo Clinic between January 1978 and January 1993. MATERIAL AND METHODS: In an institutional data-base search, we identified 20 patients with right ventricular dysplasia. Echocardiographic, electrophysiologic, Holter monitoring, cardiac catheterization, and endomyocardial biopsy results were analyzed. The mean duration of follow-up was 7 years. RESULTS: In the 12 female and 8 male patients (mean age, 30 years; range, 3 to 60), the initial manifestations of right ventricular dysplasia included ventricular arrhythmia (45%), congestive heart failure (25%), heart murmur (10%), asymptomatic (10%), complete heart block (5%), and sudden death (5%). First-order relatives were affected in 30% of the patients. Ventricular tachycardia with morphologic features of left bundle branch block was inducible in seven of nine patients. On Holter monitoring, all but 2 of 15 patients studied had frequent ventricular ectopic activity (Lown grade 2 or more). Characteristic fatty infiltration of the myocardium was present in 7 of 13 right ventricular biopsy specimens. Inordinate right ventricular enlargement was present in 60% of the patients at first echocardiographic assessment and in two other patients on follow-up assessment. Variable left ventricular involvement was noted in 50% of the cases. During the follow-up period, four patients died: two died suddenly, one died of congestive heart failure, and one died of respiratory failure after a coronary artery bypass operation. Of the 16 living patients, 8 are doing well, 3 have an implanted cardiac defibrillator, 3 are receiving antiarrhythmic agents, and 2 have undergone cardiac transplantation because of progressive biventricular failure. CONCLUSION: Patients with right ventricular dysplasia have varied initial manifestations and a high frequency of serious cardiovascular symptoms and complications.
An unusual echocardiographic appearance of a cardiac echinococcal lesion confirmed intraoperatively is described in a patient with nonspecific cardiac complaints. Although previous reports have emphasized the echolucent, often multiseptated nature of echinococcal lesions, such characteristics were absent in this case. Thus, in the appropriate clinical setting, echinococcal infection should be included in the differential diagnosis of solid mass lesions of the heart because the surgical approach may need to be altered.
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OBJECTIVES: The aim of this study was to determine the relation between coronary artery calcification detected by ultrafast computed tomographic scanning and histopathologic coronary artery disease. BACKGROUND: Recent studies suggest that discrete coronary artery calcification as visualized by ultrafast computed tomographic scanning may facilitate the noninvasive detection or estimation, or both, of the in situ extent of coronary disease. Such quantitative relations have not been established. METHODS: Thirteen consecutive perfusion-fixed autopsy hearts (from eight male and five female patients aged 17 to 83 years) were scanned by ultrafast computed tomographic scanning in contiguous 3-mm tomographic sections. The major epicardial arteries were dissected free, positioned longitudinally and scanned again in cross section. Coronary artery calcification in a coronary segment was defined as the presence of one or more voxels with a computed tomographic density > 130 Hounsfield units. Each epicardial artery was sectioned longitudinally, stained and measured with a planimeter for quantification of cross-sectional and atherosclerotic plaque areas at 3-mm intervals, corresponding to the computed tomographic scans. A total of 522 paired coronary computed tomographic and histologic sections were studied. RESULTS: Direct relations were found between ultrafast computed tomographic scanning coronary artery calcium burden and atherosclerotic plaque area and percent lumen area stenosis. However, the range for plaque area or percent lumen stenosis, or both, associated with a given calcium burden was broad. Three hundred thirty-one coronary segments showed no calcification by computed tomography. Although atherosclerotic disease was found in several corresponding pathologic specimens, > 97% of these noncalcified segments were associated with nonobstructive disease (< 75% area stenosis); if no calcification was determined in an entire coronary vessel, all corresponding coronary disease was found to be nonobstructive. To determine the relation between arterial calcification and any atheromatous disease, computed tomographic calcium burden for each segment was paired with the histologic absence or presence of disease. Ultrafast computed tomographic scanning had a sensitivity and specificity of 59% and 90% and a negative and positive predictive value of 65% and 87%, respectively. A direct correlation was found (r = 0.99) between total calcium burden calculated from tomographic scans of the heart as a whole and scans of the arteries obtained in cross section. CONCLUSIONS: The detection of coronary calcification by ultrafast computed tomographic scanning is highly predictive of the presence of histopathologic coronary disease, but the use of this technique to define the extent of coronary disease may be limited. However, the absence of coronary calcification at any site is highly specific for the absence of obstructive disease.
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