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It is unclear how the process of myxomatous disease leads to mitral valve regurgitation. We performed mechanical and biochemical tests to measure the differences between leaflets and chordae from myxomatous and normal human mitral valves. Uniaxial tensile tests and assays for uronic acid (glycosaminoglycans or GAGs) indicated that the myxoid tissues were more extensible, had lower stiffness and failure strength, and increased GAG concentration. Additional DNA assays suggest that the cells in myxoid valves are producing higher levels of GAGs than normal. Furthermore, all differences between the myxoid and normal tissues were far more pronounced in the chordae than in the leaflets. These findings support reported observations that myxoid-related chordal rupture is the leading cause of mitral valve regurgitation.

Previous studies of the association between acute cellular rejection and cardiac allograft vasculopathy (CAV) have yielded conflicting conclusions. We explored a possible association between acute cellular rejection and the extent of CAV, and we found a potential confounding variable that may obscure such an association.We investigated 140 patients (mean age, 51 +/- 11 years) who underwent serial intravascular ultrasound examinations at baseline and at 1 year after heart transplantation to assess CAV as change in maximal intimal thickness (CMIT). Patients were classified according to the presence or absence of biopsy-proven myocardial fibrosis. We used a standard biopsy-scoring system and a novel biopsy-scoring system, developed in our institution, to assess acute cellular rejection. Using univariate analysis, we found that CMIT was not associated with acute cellular rejection in the overall patient population (n = 140). However, we observed a correlation between CMIT and acute cellular rejection (standard method, r = 0.30, p = 0.01; novel method, r = 0.51, p0.0001) in patients who had no evidence of ischemic injury or fibrosis in their biopsy specimens (n = 57). Step-wise multiple regression showed that the rejection score derived from our novel method was associated more closely with the CMIT than was that derived from the traditional method.This data indicate that the presence of myocardial fibrosis masks an actuarial association between acute cellular rejection and the development of de novo allograft vasculopathy. As previously suspected, myocardial fibrosis is a marker for non-immune-mediated graft injury independently associated with an increased incidence of CAV.

A cascade of inflammatory reactions characterize acute vascular rejection after heart transplantation. This study was undertaken to test the hypothesis that acute vascular rejection is associated with up-regulation of vitronectin receptor (alphavbeta3), increased expression of tissue factor, and activation of the extracellular matrix metalloproteinase induction system.Acute vascular rejection developed in 14 heart transplant recipients within 2 weeks of transplantation, confirmed by immunofluorescence (AVR group). We compared these patients with 10 transplant recipients who had no evidence of acute vascular rejection or peritransplant ischemic injury (control group). We evaluated endomyocardial biopsy specimens for alphavbeta3, tissue factor, and extracellular matrix metalloproteinase inducer (EMMPRIN).Compared with the control group, the AVR group demonstrated evidence of significantly increased expression of alphavbeta3 (1.9-fold, p0.001), tissue factor (1.8-fold, p0.001), and EMMPRIN (1.5-fold, p0.001). All patients in the AVR group received plasmapheresis; 11 of 14 patients had evidence of ischemic necrosis on biopsy specimens, and 3 of 14 patients experienced hemodynamic compromise and graft dysfunction and died within 3 weeks of transplant. Another patient died at 10 months after transplant.Acute vascular rejection is associated with up-regulation of alphavbeta3, tissue factor, and activation of the matrix metalloproteinase induction system, which may contribute to the lethal morbidity associated with this disease.

Background— Cardiac allograft vasculopathy (CAV) limits the long-term success of cardiac transplantation. The incidence of CAV is increased in patients with elevated plasma levels of oxidized lipids or fibrin deposition within right heart biopsy (RHB) specimens. The present study investigated whether tissue factor (TF), the expression of which is regulated by oxidized lipids, is upregulated in patients with CAV. Methods and Results— A TF score was developed to quantify TF expression in RHB specimens from 63 consecutive patients undergoing routine annual posttransplantation RHB and coronary angiography. In patients >2 years (3.0±0.8 years) posttransplantation (n=35), a high TF score was observed with greater frequency (75% versus 26%, P Conclusions— These data demonstrate that early TF expression predicts subsequent development of CAV. Increased TF expression could link the elevated levels of oxidized LDL and fibrin deposition known to precede CAV. These findings suggest that TF may play a role in the pathophysiology of CAV and could offer a potential prognostic tool and a novel target for the prevention of CAV, possibly with antioxidants or inhibitors of the TF pathway.

Background Allograft coronary vasculopathy results from a complex interplay between immunologic and non-immunologic factors. We devised a computerized biopsy scoring method based on histopathology to predict the development of coronary vasculopathy. Methods One hundred forty heart transplant recipients underwent serial intravascular ultrasound analysis at baseline (within 1 month) and at 1 year after transplantation and were evaluated for development of coronary vasculopathy (change in coronary maximal intimal thickness, CMIT). We evaluated serial endomyocardial biopsy specimens for cellular rejection, vascular rejection, ischemia, and fibrosis. In a mathematical model, we computed a biopsy score in each patient based on the duration and severity of histopathology. Results We found a significant correlation between biopsy score (RY) and progression of coronary vasculopathy ( r = 0.54, p = 0.001). Using a sensitivity analysis method, an RY value of ≥560 predicted development of coronary vasculopathy with a sensitivity of 86%, specificity of 62%, and diagnostic accuracy of 80%. Compared with patients with low-risk biopsy scores (RY n = 37), patients with high-risk biopsy scores (RY ≥ 560, n = 103) had increased progression of coronary vasculopathy (CMIT, 0.59 ± 0.29 vs 0.19 ± 0.10 mm, p p = 0.01). Conclusion The biopsy score is an effective method for predicting the development of coronary vasculopathy and for predicting outcome in cardiac transplant recipients.

Mitral valve prolapse (MVP) is the most common cause of severe mitral regurgitation necessitating surgical correction. Unileaflet prolapse (ULP), usually involving the posterior leaflet, is more common than bileaflet prolapse (BLP), which is more difficult to repair. Little is known about clinical, echocardiographic, and biomechanical differences between ULP and BLP. In this study, biomechanical testing was performed on mitral valve leaflets and chordae obtained at operation for severe mitral regurgitation. Preoperative clinical characteristics and echocardiographic measurements were obtained on surgical patients (ULP = 88, BLP = 37). Men outnumbered women by a factor of 4:1 in ULP, and by 3:1 in BLP. Patients with BLP were younger (53.2 +/- 1.7 vs 59.5 +/- 1.1 years) than those with ULP, and this difference was greater in women (48.9 +/- 2.5 vs 62.9 +/- 2.2 years). BLP patients were less likely to be hypertensive, and more likely to undergo valve replacement rather than repair. Echocardiography showed that BLP leaflets were longer and thicker than ULP leaflets. The severity of mitral regurgitation was similar in both groups, although ULP patients had a much higher incidence of flail leaflets (45% vs 5% in BLP). Mechanical strength of chordae was greater in BLP than in ULP, although leaflet strength was similar. The increased chordal strength in BLP may be responsible for less flail. In patients with MVP and severe mitral regurgitation requiring surgery, ULP and BLP are distinct entities with substantial differences in the population affected, in echocardiographic manifestations including prevalence of flail, in chordal mechanics, and in the likelihood of surgical repair.

Myocardial ischemic injury after heart transplantation is associated with subsequent development of graft vasculopathy. Both vitronectin receptor (integrin alpha(v)beta3) and tissue factor play key roles in vascular endothelial cell injury. Matrix metalloproteinases (MMPs) are activated in ischemic injury models.Thirteen patients developed myocardial ischemic injury within 2 weeks of cardiac transplantation (ischemia group). These were compared with 10 transplantation patients who had no evidence of ischemia (control group). Endomyocardial biopsies were evaluated within 2 weeks of transplantation for alpha(v)beta3, tissue factor, and extracellular MMP inducer (EMMPRIN). At 1 year, MMPs were evaluated, and interstitial myocardial fibrosis was quantified. All patients underwent intravascular ultrasound at 1 month and 1 year after transplantation. Compared with control, the ischemia group demonstrated evidence of significant increased expression of alpha(v)beta3 (3.2-fold, P0.001), tissue factor (2.5-fold, P0.001), and EMMPRIN (1.9-fold, P=0.01). At 1 year, the ischemia group had a significant increase in myocardial fibrosis (24+/-1.8% versus 14+/-1.1%, P0.001) and zymographic activity of MMP-2 (1.4-fold, P0.001), MMP-3 (1.2-fold, P0.001), and MMP-9 (1.3-fold, P=0.01). Coronary vasculopathy progression was also more advanced in the ischemia group (change in coronary maximal intimal thickness over 1 year 0.54+/-0.1 versus 0.26+/-0.06 mm; P=0.031).Myocardial ischemic injury after cardiac transplantation is associated with upregulation of alpha(v)beta3, tissue factor, and activation of the MMP induction system, which may contribute to the subsequent development of allograft remodeling and vasculopathy.

Background — Endothelin-1 (ET-1), a potent vasoconstrictor, is released in response to several inflammatory cytokines after heart transplantation. The present study correlated patterns of myocardial ET-1 expression in heart biopsies with acute rejection, post-transplantation ischemic injury, and subsequent development of coronary vasculopathy. Methods and Results — Patterns of myocardial ET-1 expression were evaluated in 47 heart transplant recipients at 3 months after transplant. Transplant vasculopathy was documented by coronary angiography at 2 years after transplant. Expression of ET-1 was tabulated for both blood vessels and the interstitium. Vascular ET-1 expression was positive in 7/17 (41%) of patients with greater than grade 2 (International Society Heart Lung Transplant) rejection compared with 3/30 (10%) of patients with grade 0 and grade 1A rejection ( P =0.02). Compared with patients with negative interstitial ET-1 expression (n=22), patients with positive interstitial ET-1 expression (n=25) had higher incidence of post-transplantation ischemic injury (52% versus 9%, P =0.002), lower mean episodes of acute rejection (≥ grade 2) during the first 3 months of transplant (1.09±0.66 versus 1.86±1.6, P =0.048), and more common vasculopathy at 2 years (50% versus 15%, P =0.02), and they tended to have worse survival (83.2% versus 100%, P =0.058). Conclusions — Vascular ET-1 expression is likely to be associated with acute rejection. Interstitial ET-1 expression, however, is more likely to be associated with post-transplantation ischemic injury and subsequent development of coronary vasculopathy.

Background: Giant cell myocarditis causes essentially irreversible fulminant left ventricular dysfunction with associated conduction abnormalities and congestive failure. Response to immunosuppressive therapy is poor and cardiac transplantation is the only viable treatment option. The histologic hallmarks of giant cell myocarditis include a polymorphous inflammatory response with numerous multinucleated giant cells and extensive myocyte necrosis in a geographic pattern. There were 38 patients who received a cardiac transplant for giant cell myocarditis in the Giant Cell Myocarditis Registry. Among these patients, there were 9 recurrences of disease in the allograft. Concern has been expressed that recurrence of giant cell myocarditis in the allograft might be a contraindication for cardiac transplantation in the future. Methods In our single-center analysis we describe the clinical and histologic findings of 5 patients transplanted for giant cell myocarditis at the Cleveland Clinic. Results All but 1 of the patients were New York Heart Association (NYHA) class 4 with an average cardiac index (CI) of 1.52 liters/min · m 2 . Of the 5 patients transplanted, 1 developed recurrent giant cell myocarditis. Routine right ventricular endomyocardial biopsy at 1 week exhibited severe multifocal myocardial fibrosis in addition to mild acute vascular rejection and mild grade 1A cellular rejection. Follow-up biopsy in this patient indicated grade IIIA moderate acute rejection in addition to multinucleated giant cells. Two distinct inflammatory processes were noted consisting of foci of T-cell inflammation identified by immunohistochemistry to be consistent with rejection, and a second inflammatory process with few mononuclear cells staining for macrophage or T-cell markers with eosinophils and myocyte necrosis consistent with giant cell myocarditis. Follow-up right ventricular endomyocardial biopsies (RVBXs) in this patient have subsequently demonstrated improvement in the degree of inflammatory infiltrate without vascular or significant cellular rejection. Vascular rejection was noted in 1 of the remaining 4 patients and was treated successfully with muramab-CD3 and plasmapheresis. Conclusions Giant cell myocarditis should be expected to recur in the allograft and often does so concurrently with rejection. However, the disease in the allograft responds to therapy in a favorable manner, which differs dramatically from that in the native heart. This might be the result of detection of the disease at an earlier stage than in the native heart, or the immunosuppression milieu in the allograft. The favorable response to therapy suggests that the likelihood of recurrence of giant cell myocarditis should not be considered a barrier to transplantation.

Background A significant correlation between autofluorescence spectroscopy and heart allograft rejection has been described in the rat heterotropic allograft model. However, the use of this technique in human heart transplants has not been validate. Methods We obtained fluorescence and reflectance spectra on 37 human endomyocardial biopsy specimens and correlated the spectra with International Society Heart and Lung Transplantation grade for histologic rejection. Results Using different excitation wavelengths (ultraviolet, λ = 337 nm; blue, λ = 440 nm, and green, λ = 486 nm), we found no significant difference in the fluorescence spectra among the different grades of rejection. Conclusions Fluorescence spectroscopy is not a sensitive method for detecting rejection in human heart transplant recipients.

Porcine bioprosthetic valves are commonly used to replace the native cardiac valves when they become diseased and dysfunctional. One class of bioprosthesis consists of whole pig aortic valves, tanned in glutaraldehyde and mounted on plastic or wire frames. These devices work very well in the short term (< 5 years) but eventually begin to fail gradually and typically do not last more than 15 years [1]. Many have tried to elucidate a mechanism of bioprosthetic valve failure, but little progress has been made, until recently. Historically, calcification has been thought to be the primary mechanism of bioprosthetic valve failure [2]. Recently, however, it has become recognized that mechanical damage to bioprosthetic valves can occur independently from calcification [3]. This shift in opinion has been supported by a number of observations. For example, Purinya et. al. [4] have shown that after four years of implantation, bioprosthetic valve explants have lower failure stresses and fail at greater strains than fresh porcine bioprostheses. Similar observations have been made in valves fatigued in vitro [5]. Others have shown that these mechanical changes are accompanied by biochemical changes in the collagen fibers [6]. We have shown previously in preliminary studies that glycosaminoglycans (GAGs) are lost from implanted bioprosthetic valves [7]. Since GAGs are responsible for maintaining tissue viscoelasticity, loss of GAGs would have detrimental effects on tissue mechanics. In separate studies, we have demonstrated that damage to elastin can also have detrimental effects on tissue mechanics, and on valve durability as a whole [8].

Although myocarditis has been implicated in the pathogenesis of heart failure, a definitive relationship between myocardial inflammation, cardiac dysfunction, and changes in myocyte gene expression has not been established. In this study, we examined the hypothesis that myocardial inflammation and replacement fibrosis following an autoimmune response can progress to cardiac dysfunction and may result in progression to the heart failure phenotype. SWXJ mice were immunized with cardiac myosin on day 0 and day 7, in order to induce an autoimmune response to the myosin protein. Cardiac catheterization via the right carotid artery was performed on days 14, 21, 28, 35, and 42, using a 1.4F Millar transducer-tipped catheter. Hearts were weighed, and cross-sections were cut and stained with either haematoxylin and eosin or Masson's trichrome, in order to identify areas of inflammation and/or fibrosis. Myocardial gene expression was determined by Northern blot analysis. In mice with histological evidence of myocarditis, the heart weight/body weight ratio increased beginning on day 14, and cardiac function decreased beginning on day 21. Myocardial inflammation was accompanied by significant fibrosis beginning on day 21. Quantitation of mRNA showed expression of ventricular atrial naturietic factor, as well as a decrease in myosin heavy chain alpha, beginning on day 21. These data demonstrate that autoimmune inflammation of the heart results in significant cardiac dysfunction, leading to phenotypic alterations similar to those demonstrated in human heart failure and animal models of heart failure.

Primary synovial sarcoma of the heart is extremely rare. This report adds another case to the literature and emphasizes that this tumor, although rare, should be considered in the differential diagnosis of left atrial tumors.

The most frequently administered treatment for asymptomatic ISHLT Grade 3A cardiac allograft rejection is intravenous steroids or oral steroid pulse with a taper. This study analyzes the efficacy of 3-day 100-mg course of prednisone without a tapered regimen for the treatment of asymptomatic moderate cardiac allograft rejection.All new episodes of asymptomatic ISHLT Grade 3A rejections were treated with oral steroid pulse without taper, consisting of 100 mg of prednisone for 3 consecutive days followed by resuming the pre-rejection steroid dose on the fourth day. We retrospectively reviewed the histologic response of all treated episodes among all cardiac transplant recipients transplanted between January 1995 through December 1997 who were treated with triple therapy consisting of cyclosporine, azathioprine and steroids. Patients receiving additional or alternative immunosuppressives were excluded from the study. The treated episodes were analyzed as responders if the follow-up biopsy were Grade 0, 1A, 1B, or 2; treatment was counted as non-responders if the follow-up biopsy showed Grade 3A or higher.Of 230 cardiac transplant recipients, 100 patients received a 3-day 100 mg course of prednisone without taper for 174 new episodes of asymptomatic ISHLT Grade 3A rejection. The overall response rate was 75% (130/174 rejection episodes). A significant difference in the response rate was observed depending on the number of days post transplant. A comparison of the success rates among rejections which occurred90 days post transplant versus30 days revealed responses to be 88% versus 70% (p = 0.02); for rejections treated60 days post transplant versus30 days showed success rates of 84% versus 70% (p = 0.04). The mean age of the recipient revealed a trend to be lower among the non-responder group (49+/-12 years versus 53+/-9 years, p = 0.07). Having left ventricular assist device as a bridge to transplant did not significantly affect the treatment outcome. The response rates were 69% for the patients who required the assist device versus 77% for those not bridged (p = ns). There was no significant difference in the gender or the baseline immunosuppressive doses between the responders and non-responders. The cost of a 3-day outpatient, visiting nurse supervised intravenous steroid therapy versus 3 days of oral prednisone was $861 vs $6.88.Oral steroid pulse without taper is an effective and economical way to treat asymptomatic moderate grade cardiac allograft rejection. A 3-day course of 100 mg of prednisone without taper should be considered as first line of therapy for clinically stable form of moderate cardiac allograft rejection occurring60 days post transplant.

We report a case of a large epicardial lipoma associated with an unusual rupture of an infarcted interventricular septum. The proximity of the attachment of the lipoma to the rupture site, together with the unusual shape and location of the rupture, suggest that the lipoma perpetrated the rupture.

Objectives: We sought to determine whether cardiac transplant recipients who required a bridge to transplantation with an implantable left ventricular assist device had a different outcome than patients who underwent transplantation without such a bridge.Methods: A retrospective study of 256 cardiac transplants from 1992 to 1996 included 53 patients who received the HeartMate left ventricular assist device and 203 patients who had no left ventricular assist device support.Results: Left ventricular assist device transplants increased from 8% of all transplants in 1992 (n = 63) to 32% in 1995 (n = 65) and 43% in 1996 (n = 14 year to date). Patients with and without left ventricular assist device had similar age and sex distributions. Left ventricular assist device recipients were larger (body surface area 1.96 vs 1.86 m2, p = 0.004). They were more likely to have ischemic cardiomyopathy (70% vs 45%, p = 0.001) and type O blood group (51% vs 34%, p = 0.06). All patients with left ventricular assist device and 42% of those without had undergone previous cardiac operations by the time of transplantation (mean number per patient 1.5 vs 0.3, p < 0.001). More patients in the left ventricular assist device group had anti-HLA antibodies before transplantation (T-cell panel reactive antibody level >10% in 66% of left ventricular assist device group vs 15% of control group, p < 0.0001). Waiting time was longer for the left ventricular assist device than for patients in status I without a left ventricular assist device (median 88 vs 37 days, p = 0.002). There was no difference in length of posttransplantation hospital stay (median 15 days for each) or operative mortality (3.8% vs 4.4%). Mean follow-up averaged 22 months. No significant difference was found in Kaplan-Meier survival estimates. One-year survival was 94% in the left ventricular assist device group and 88% in the control group (difference not significant). Comparison of posttransplantation events showed no significant difference in actuarial rates of cytomegalovirus infection (20% vs 17%) or vascular rejection (15% vs 12%) at 1 year of follow-up. Similar percentages of patients were free from cellular rejection at 1 year of follow-up (12% vs 22%, p = 0.36).Conclusions: Left ventricular assist device support intensified the donor shortage by including recipients who otherwise would not have survived to transplantation. Bridging affected transplant demographics, favoring patients who are larger, have ischemic cardiomyopathy, have had multiple blood transfusions and complex cardiac operations, and are HLA sensitized. Successfully bridged patients wait longer for a transplant than do UNOS status I patients without such a bridge, but they have similar posttransplantation hospital stay, operative mortality, and survival to those of patients not requiring left ventricular assist device support. (J THORAC CARDIOVASC SURG 1996;112:1275-83)

Purpose To report the treatment of symptomatic conjunctival lymphangiectasis with conjunctival resection. Design Interventional case series. Methods Retrospective review of three cases of patients with symptomatic unilateral conjunctival lymphangiectasis who had conjunctival resection of the involved tissue down to bare sclera. Results The surgical area reepithelialized without recurrence of the lymphatics. In all cases the patients have remained asymptomatic for at least 14 months. Conclusion Conjunctival resection for symptomatic lymphangiectasis can be a successful therapeutic option for this condition.

Pulsed-wave Doppler echocardiography of left ventricular (LV) inflows was performed in 30 consecutive patients with biopsy-proven lymphocytic myocarditis. There were 21 men and 9 women (mean age 50 +/- 15 years). LV ejection fraction was < or = 30% in 73% of the patients. Sixty-six percent were in New York Heart Association functional class III to IV. Peak early (E) velocity, late (A) velocity, deceleration time and filling pattern were assessed. These values were compared with a control population. E velocity in lymphocytic myocarditis was significantly higher than in control subjects (79 +/- 34 vs 67 +/- 14 cm/s, p = 0.0034). A velocity was lower in patients with myocarditis than in control subjects (38 +/- 20 vs 49 +/- 12 cm/s, p = 0.0001). Correspondingly, the E/A ratio was greater in the myocarditis group (2.5 +/- 1.3 vs 1.5 +/- 0.5, p < 0.0001). In particular, mean deceleration time in patients with myocarditis was significantly lower than that of control subjects (151 +/- 52 vs 194 +/- 30 ms, p < 0.0001). Diastolic filling patterns were abnormal in 29 of 30 patients (97%) with lymphocytic myocarditis, revealing a restrictive pattern in 25, abnormal relaxation in 4 and a normal pattern in 1. Lymphocytic myo-carditis is therefore associated with LV diastolic dysfunction of a predominantly restrictive pattern.

Unstained frozen sections of normal and atherosclerotic human aorta and coronary artery were examined using histochemical and fluorescence microscopic techniques to identify the structures responsible for autofluorescence under 351 to 364 nm laser excitation. These structures included elastin and collagen in normal and atherosclerotic specimens, calcium deposits in calcified plaques, and granular or ring-shaped deposits histochemically identified as ceroid found in both calcified and non-calcified plaques. Qualitatively, both the color and intensity of ceroid autofluorescence differed greatly from that of elastin or collagen. The emission spectra of elastin, collagen, and ceroid were examined by microscopic spectrofluorimetry, and were found to differ significantly as well. When compared with spectra of elastin and collagen, spectra of ceroid were broader, shifted to the red, and were somewhat resistant to bleaching. We conclude that detection of laser-induced ceroid autofluorescence may aid in identifying plaques for laser ablation.

Two physicians who successfully motivated their patients to make lifestyle changes were true believers.

We reviewed autopsies in 50 consecutive cases of congenital heart disease between 1980 and 1988. Autopsy results were compared to premortem clinical diagnosis. The patients ranged in age from two days to 66.5 years with a mean age of 6.42 years; 68% were less than one year of age. The most common diagnoses were hypoplastic left heart syndrome in 16 (32%) and ventricular septal defect in five (10%). Other common diagnoses included coarctation of the aorta, hypoplastic right heart syndrome, tetralogy of Fallot, transposition of the great vessels and tricuspid atresia. No patient had a missed diagnosis that would have changed survival (Class 1). Four (8%) of the patients had one missed major diagnosis that did not change their survival because they were already being symptomatically treated or because no clinical treatment was available (Class 2). The majority of autopsy derived data were missed minor diagnoses that were related [14 (28%) - Class 3] or unrelated [12 (24%) - Class 4] to the terminal disease process. However, despite the small percentage of autopsy findings that would have directly affected survival, the autopsy provided important additional clinical information in 30 (60%) patients. This information could be used to correct management errors and to develop protocols for prophylaxis against known complications. The pediatric autopsy can provide useful clinical data that may be a guide to altering clinical therapy and is essential in providing genetic counseling.

Carney's complex is characterized by cardiac and cutaneous myxomas, mammary myxoid fibroadenomas, spotty mucocutaneous pigmentation, primary pigmented adrenocortical disease, large-cell calcifying Sertoli cell tumors of the testis, growth hormone-secreting pituitary adenomas, and psammomatous melanotic schwannomas. We present an illustrative case of Carney's complex in a 26-year-old woman who at age 20 underwent bilateral adrenalectomy for Cushing's syndrome. Six years later, she suffered multiple central nervous system embolic episodes secondary to three cardiac myxomas, which led to her death. Appropriate evaluation and management of patients with Carney's complex is outlined.

Thrombotic thrombocytopenic purpura (TTP) has only recently been documented in association with human immunodeficiency virus infection. The authors present the clinical, laboratory, and pathologic features of a unique case of secondary thrombotic thrombocytopenic purpura occurring in a patient with AIDS, including the immunohistologic characterization of the vascular thrombi. They also review 12 previously reported cases of thrombotic thrombocytopenic purpura associated with human immunodeficiency virus infection.

THE IMPACT OF CYTOGAM ON CARDIAC TRANSPLANT RECIPIENTS WITH MODERATE HYPOGAMMAGLOBULINEMIA M.H. Yamani, R.C. Starling, R. Avery, S. Mawhorter, A. McNeil, N.B. Ratliff, D. Cook, D. Pelegrin, P. Colosimo, K. Kiefer, R. Hobbs, D. Taylor, P. McCarthy, J.B. Young, Cardiovascular Medicine; Infectious Disease; Anatomic Pathology; Allogen Laboratory; CardioThoracic Surgery, Cleveland Clinic Foundation, Cleveland, OH

Objective To determine 1) the frequency of idiopathic aortitis in a large surgical cohort, 2) how often aortitis was associated with a systemic disease, and 3) whether the findings of aortitis in resected specimens predicted future occurrences of clinically apparent vascular injury due to vasculitis. Methods Retrospective chart and pathology review of 1,204 aortic surgical specimens that were gathered over a period of 20 years at a tertiary care medical center. A standardized database was used to compare features of aortitis patients with those of controls in whom inflammation was not present. Results Among 1,204 aortic specimens, 52 (4.3%) were clinically and pathologically classified as idiopathic aortitis. Sixty-seven percent of patients with idiopathic aortitis were women. In 96% of idiopathic aortitis patients with aneurysm formation, aortitis was present only in the thoracic aorta. Among 383 thoracic aortic aneurysms, 12% had idiopathic inflammatory features. In 96% of patients with idiopathic aortitis, symptoms of systemic illness had not been present at the time of surgery. In 31%, aortitis was associated with a remote history of vasculitis and a variety of other systemic disorders. During a mean followup period of 41.2 months, new aneurysms were identified among 6 of 25 patients who were not treated with glucocorticoids. None were identified among 11 patients who were treated with glucocorticoids (mean followup 35.5 months). Conclusion The frequency of idiopathic aortitis in a large surgical cohort was found to be 4.3%. Thoracic aorta aneurysm formation, in the absence of systemic illness, was the most common manifestation. In the setting of a cardiovascular surgery practice, aortitis may first become apparent only after pathologic evaluation of excised specimens. The appropriate medical treatment for patients with incidentally discovered aortitis is not known. Because 17% of our patients subsequently developed new aneurysms, we suggest that it would be prudent for patients with idiopathic aortitis identified at the time of surgery to be periodically evaluated for recurrent or persistent disease.

A case of congenital mitral stenosis with pulmonary hypertension is presented. The severity and rapid development of the pulmonary vascular changes are unusual, especially for a young child. The case underscores the importance of early detection of correctable congenital heart disease and emphasizes the unpredictability of the time course and severity of irreversible pulmonary hypertension.