Biomedical subjects
Publications and source records attributed to W D Edwards.
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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Coronary calcium determined by electron beam computed tomography (CT) has not been systematically evaluated regarding prediction of histopathologic atherosclerotic disease. Furthermore, gender specificity has not been examined. The 3 major epicardial arteries were dissected from 13 consecutive hearts (5 women and 8 men) after autopsy. Each artery was straightened and scanned using CT in contiguous 3 mm thick cross sections. After imaging, histologic sections were prepared at corresponding intervals and luminal area obstruction determined by planimetry. Electron beam CT scans were analyzed to determine coronary calcium area (i.e., tomographic area with CT density > 130 Hounsfield units). A total of 522 histologic specimens were examined and paired with corresponding CT scans (182 in women, 340 in men). Receiver-operating characteristic (ROC) analysis was used to define site specificity of calcium area for luminal area narrowing by atherosclerosis. ROC curve areas for segmental CT calcium and prediction of atherosclerosis representing mild, moderate, or severe disease were, respectively, 0.712, 0.843, and 0.857 for women and 0.732, 0.793, and 0.841 for men. Curves relating false-positive rate (1-specificity) to predefined degrees of atherosclerotic narrowing versus calcium area were curvilinear. In both women and men, calcium areas on the order of 1 mm2/coronary segment were necessary to predict at least mild atherosclerosis with a false-positive rate of 0% (i.e., 100% specificity), whereas a calcium area > 3 mm2 was necessary to predict the same result for severe disease. In conclusion, coronary artery calcium area as determined by electron beam CT has the potential to predict segmental histopathologic coronary disease.(ABSTRACT TRUNCATED AT 250 WORDS)
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Knowledge of the variations in coronary artery pattern is important in the arterial switch operation for complete transposition of the great arteries (TGA). As autopsy specimens provide the most definitive means of identifying the coronary anatomy, 255 hearts with complete TGA were reviewed by a single pathologist. The age of the patients ranged from 1 day to 34 years (mean, 2.9 years). The origin of the coronary arteries was defined as seen by an observer looking from the pulmonary artery toward the aorta. The usual pattern with the right coronary artery originating from the right hand sinus and the left coronary artery from the left hand sinus (184 cases) and the circumflex coronary artery arising from the right coronary artery (46 cases) accounted for 90% of the cases. Eleven other patterns were identified. The usual coronary artery pattern was more prevalent in TGA with the aorta in a right anterior or anterior position (74.8%) than in TGA with a side-by-side relationship of the great arteries (38.9%). In only 2 cases (0.8%) was an aortic intramural course of the left coronary artery identified. The latter 2 cases confirm our belief that an aortic intramural course of the left coronary artery or the left anterior descending coronary artery must be assumed when the vessel has an aberrant origin from the right sinus or when it is in intimate relationship with the commissure between the right and left sinuses and courses between the great arteries. In the vast majority of specimens a favorable coronary artery pattern with regard to feasibility of the arterial switch operation was encountered.
OBJECTIVES: In a porcine coronary model, fibrin film soaked for 3 h in heparin was used as a circumferential coating on a tantalum stent to assess the effect of this naturally occurring biopolymer on arterial healing. The results were compared with those obtained with medical grade polyurethane-coated stainless steel stents. BACKGROUND: Thrombus plays an important role in healing after arterial injury and may affect the development of neointimal hyperplasia. Manipulation of the initial thrombus may alter the healing response. To study this, we placed a template of fibrin in a porcine coronary artery restenosis model. METHODS: Thirty-four fibrin film stents were delivered in 20 swine. Oversizing was avoided, to prevent deep arterial injury, by placement of optimally sized stents. Initial patency of the stented vessel was confirmed by angiography. RESULTS: Three fibrin-stented swine died within 48 h; in each, the stent was occluded with a fibrin/red blood cell mass. In two of these three, a portion of the exogenous fibrin had become detached from the stent and partially occluded the lumen. Of the remaining 31 stents, all were patent at elective sacrifice at 28 days. Eighty-four percent had a diameter stenosis < 50%, and the mean (+/- SD) diameter stenosis was 32.3 +/- 13%. There was no evidence of significant foreign-body giant-cell reaction. These results contrasted with the medical grade polyurethane-coated stents placed according to the same protocol without oversizing. Twelve of these stents were placed; six swine died of thrombotic occlusion within the 1st 48 h. At elective sacrifice at 28 days, the remaining polyurethane-coated stents were occluded by marked neointimal hyperplasia. CONCLUSIONS: Fibrin film-coated stents seem promising as a template for modifying the local response to arterial injury and for potentially decreasing restenosis rates.
OBJECTIVE: We report the first case of restrictive cardiomyopathy occurring in a patient with the eosinophilia-myalgia syndrome. DESIGN: In this article, we discuss the various clinical manifestations of the eosinophilia-myalgia syndrome. MATERIAL AND METHODS: In a 46-year-old woman with the eosinophilia-myalgia syndrome, orthopnea, chronic persistent edema, and severe dyspnea on exertion developed 2 years after she had discontinued use of L-tryptophan. Doppler echocardiography showed ventricular filling confined to early diastole and no atrial filling during ventricular systole--the Doppler hallmarks of restrictive disease. Right-sided cardiac catheterization revealed that the pulmonary wedge pressure equaled the pulmonary artery diastolic pressure and the mean right atrial pressure. A myocardial biopsy specimen showed dense endocardial fibrosis. Special immunofluorescent stains for eosinophilic granule major basic protein showed substantial deposition along the endocardial myocardial interface, an indication that eosinophils were present some time in the past. RESULTS: A follow-up telephone call 14 months after the patient's initial assessment at the Mayo Clinic revealed that she had class III symptoms of congestive heart failure. She was receiving high doses of three diuretics daily, and her condition had improved considerably since her first examination at our institution. CONCLUSION: Restrictive cardiomyopathy may occur in the setting of the eosinophilia-myalgia syndrome and should be considered in patients with this disease in whom exertional dyspnea and peripheral edema occur.
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A unique case of trauma-related cartilage embolization to the lungs is reported. The patient had sustained a fracture of the left humerus, related to seizures from suspected homicidal strychnine poisoning, and had undergone an open-reduction procedure. Although embolization of extravascular material to the lungs is well recognized, to our knowledge, trauma-related cartilage embolization has not been previously described in humans. Such emboli may have the potential to contribute to mortality and morbidity in trauma patients.