Cardiac angiosarcoma: an unusual presentation with cutaneous metastases.

Angiosarcoma is the most common primary malignant neoplasm of the heart. The incidence of metastatic disease is 66% to 89%; however, initial presentation with metastatic disease is rare. We report the case of a patient who presented initially with soft tissue and cutaneous metastases in the absence of cardiac symptoms.

Diagnosis of clinically unsuspected gallbladder rupture by peritoneal fluid cytology. A case report.

BACKGROUND: Rupture of the biliary system resulting in bile ascites may occur spontaneously or as a complication of inflammatory disease, biliary tract manipulation or trauma. Leakage of bile into the peritoneal cavity can cause severe chemical peritonitis, requiring rapid surgical intervention. CASE: A 64-year-old male status post total laryngectomy for squamous cell carcinoma developed abdominal pain and ascites. Diagnostic paracentesis was performed, and bile pigment was noted on cytologic examination, raising the possibility of biliary rupture. This was confirmed radiographically, and the patient underwent exploratory laparotomy, where a ruptured gallbladder was found and cholecystectomy performed. CONCLUSION: Recognition of bile pigment on cytologic examination of ascitic fluid followed by measurement of ascitic fluid bilirubin levels can alert clinicians to the presence of clinically unsuspected bile peritonitis in patients with rupture of the biliary system.

Changes in gene expression in the intact human heart. Downregulation of alpha-myosin heavy chain in hypertrophied, failing ventricular myocardium.

Using quantitative RT-PCR in RNA from right ventricular (RV) endomyocardial biopsies from intact nonfailing hearts, and subjects with moderate RV failure from primary pulmonary hypertension (PPH) or idiopathic dilated cardiomyopathy (IDC), we measured expression of genes involved in regulation of contractility or hypertrophy. Gene expression was also assessed in LV (left ventricular) and RV free wall and RV endomyocardium of hearts from end-stage IDC subjects undergoing heart transplantation or from nonfailing donors. In intact failing hearts, downregulation of beta1-receptor mRNA and protein, upregulation of atrial natriuretic peptide mRNA expression, and increased myocyte diameter indicated similar degrees of failure and hypertrophy in the IDC and PPH phenotypes. The only molecular phenotypic difference between PPH and IDC RVs was upregulation of beta2-receptor gene expression in PPH but not IDC. The major new findings were that (a) both nonfailing intact and explanted human ventricular myocardium expressed substantial amounts of alpha-myosin heavy chain mRNA (alpha-MHC, 23-34% of total), and (b) in heart failure alpha-MHC was downregulated (by 67-84%) and beta-MHC gene expression was upregulated. We conclude that at the mRNA level nonfailing human heart expresses substantial alpha-MHC. In myocardial failure this alteration in gene expression of MHC isoforms, if translated into protein expression, would decrease myosin ATPase enzyme velocity and slow speed of contraction.

Detection of Epstein-Barr virus in cardiac biopsies of heart transplant patients with lymphoproliferative disorders.

We investigated whether in situ hybridization for EBV RNA on routine cardiac biopsies could be used as a predictive test for the development of posttransplant lymphoproliferative disorder (PTLD) in cardiac transplant recipients. We examined the sensitivity of the test by determining the frequency of EBV-positive cells in cardiac biopsy specimens from patients with a known history of PTLD. Biopsy specimens obtained during routine monitoring for rejection before or shortly after the diagnosis of PTLD from 10 pediatric heart transplant patients were examined. Four of 74 specimens (5.4%) demonstrated EBV-positive lymphocytes in the cardiac biopsy rejection infiltrates. The four positive specimens were obtained from 3 different patients, all before the diagnosis of PTLD. Given the low number of cardiac biopsy specimens with EBV-positive lymphocytes, as well as the low incidence of PTLD in cardiac transplant patients, we conclude that a routine screening of all cardiac biopsy specimens using in situ hybridization for EBV with the intention of predicting PTLD is not warranted. However, in situ hybridization for EBV might be used in selected cases, such as those in which the transplant patient does not respond to immunosuppressive therapy for rejection. In these patients, the presence of EBV-positive lymphocytes in biopsy specimens initially interpreted as showing rejection might instead raise the suspicion of incipient PTLD.

Incidence of myocarditis in peripartum cardiomyopathy.

Peripartum cardiomyopathy (PC), an uncommon cause of peripartum heart failure, is defined as a cardiomyopathy presenting in the last trimester of pregnancy or the first 6 months postpartum, without evidence of preexisting cardiovascular disease. The etiology of PC and idiopathic dilated cardiomyopathy (IDC) remains uncertain. Several reports have addressed possible differences in clinical presentation and prognosis between these groups. A relatively high incidence of myocarditis has been recently reported in patients with PC, raising the possibility that this may represent a distinct difference between this condition and IDC. A retrospective review of endomyocardial biopsy specimens from 34 patients fulfilling the criteria for a diagnosis of PC was therefore performed to further evaluate this finding. Results indicate a lower incidence of myocarditis (8.8%, 3 of 34) than that reported in other studies. This incidence was comparable to that found in an age- and sex-matched control population undergoing transplantation for IDC (9.1%, 2 of 22). Factors that may influence the diverse range in the reported incidence of myocarditis are discussed.

Acute rejection: significance of elapsed time after transplantation.

In July 1990 the new standardized grading scheme for the International Society for Heart and Lung Transplantation was established. One of the purposes of "splitting" the grades was to learn the significance and outcome of the various grades proposed. Between January 1990 and November 1992, 263 grade 1A, 19 grade 1B, and 100 grade 2 "new" rejection episodes were identified at our institution. These episodes occurred in 86 adult recipients who underwent transplantation between January 1990 and August 1992 and 98 recipients who underwent transplantation before January 1990, in whom the episodes occurred more than 1 year from the date of transplantation. The outcome of the episode was determined on subsequent endomyocardial biopsy samples to be resolution or "progression" to a higher grade. The percentage of focal mild (grade 1A) rejection episodes progressing to a moderate (grade 3A) rejection in the first 6 months after transplantation was 24.4% (20 of 82) compared with a progression rate of 5.1% (3 of 59) and 2% (3 of 149) in episodes occurring beyond 6 months and 1 year after transplantation, respectively (p < 0.005). Of the focal moderate (grade 2) rejection episodes receiving no augmentation of immunosuppression, 35.7% (5 of 14) occurring within the initial 6 months and 7.3% (3 of 41) of all such episodes diagnosed beyond 1 year after transplantation progressed to a moderate rejection. A similar trend was seen in the focal moderate rejection episodes that received augmentation of immunosuppression: 26.3% (5 of 19) of episodes occurring within 6 months and 0 of 15 of all episodes occurring beyond 1 year after transplantation progressed to a moderate rejection.(ABSTRACT TRUNCATED AT 250 WORDS)

A comparative immunohistochemical study of uterine smooth muscle neoplasms with emphasis on the epithelioid variant.

We evaluated the immunophenotypes of 22 spindled and 36 epithelioid uterine smooth muscle neoplasms (SMNs) and 16 extrauterine nongastrointestinal spindled smooth-muscle neoplasms for various markers. The epithelioid neoplasms were subdivided into two histological groups designated true and intermediate, the former showing typical epithelioid features and the latter showing epithelioid features that could be explained by cross-sectioning of blunt spindled cells. Desmin, muscle-specific actin, and smooth muscle actin were equally sensitive in detecting muscle differentiation in all these neoplasms. The true epithelioid variants were more frequently keratin positive but less frequently positive for vimentin, CD34 or the muscle markers, compared with their spindled counterparts. The intermediate epithelioid variants more closely resembled the spindled neoplasms in their immunostaining for muscle markers, vimentin, and CD34 but like the true epithelioid variants were relatively frequently positive for keratin. CD34 was positive in 36% of the spindled and 6% of the true epithelioid uterine SMNs, in most cases faintly. Antikeratin AE1 was positive more frequently than CAM5.2, with 18% of the spindled and 35% of the true epithelioid neoplasms being AE1 positive. The immunophenotype of uterine SMNs, including the epithelioid variant, permits their distinction from carcinomas based on their frequent reactivity for muscle markers in spite of their high rate of keratin positivity. They show sufficient overlap in immunoreactivity with endometrial stromal sarcomas to preclude definitive differentiation from them on immunohistochemical features alone.

Long-term outcome after heart transplantation for peripartum cardiomyopathy.

To elucidate the long-term outcome and frequency of complications after heart transplantation for peripartum cardiomyopathy (PPCM), we compared the courses of eight consecutive patients undergoing transplantation for PPCM with those of nine female age-matched control subjects undergoing transplantation for idiopathic dilated cardiomyopathy (IDCM). No significant differences could be found in baseline variables between the two groups with the exception of the number of pregnancies (2.5 +/- 1.5 vs 0, p = 0.0002). Two patients in each group died during the first 6 months after transplantation, and one in each group died later. Actuarial survival rates were 75% +/- 15% and 78% +/- 14% (p = NS) at 1 year and 60% +/- 18% and 78% +/- 14% (p = NS) at 5 years in PPCM and IDCM patients, respectively. Linearized rejection rates during the first 3 months were 1.85 +/- 0.56 and 1.91 +/- 0.49 (p = NS) and during the second 3 months were 0.18 +/- 0.18 and 0.45 +/- 0.26 (p = NS), respectively. Similarly no significant differences in linearized infection rates were found. Among patients surviving more than 6 months after transplantation, after a mean follow-up period of 4.5 +/- 3.1 years for those with PPCM and 7.8 +/- 3.2 years for those with IDCM, 83% and 100%, respectively, were rehabilitated; hemodynamic findings were normal in all patients and the frequency of other transplant-associated complications was similar in both groups. In conclusion, heart transplantation is a valuable option for patients with PPCM and severe congestive heart failure that is unresponsive to conventional treatment.(ABSTRACT TRUNCATED AT 250 WORDS)

Graft coronary disease in pediatric heart and combined heart-lung transplant recipients: a study of fifteen cases.

Graft coronary disease (GCD) has emerged as the most important deterrent to long-term survival in adult heart transplant recipients. The incidence, natural history, and pathobiology of GCD is less well understood in the pediatric transplant population. This study evaluated the histopathologic and immunohistochemical features of GCD in the Stanford pediatric heart and heart-lung group. Eighty-eight patients, ages 1 week to 18 years, received heart transplants between 1974 and 1992, and 15 patients, ages 1 month to 18 years received heart-lung transplants between 1981 and 1992 at Stanford University Hospital. There were 50 males and 38 females in the heart transplant group; 39 (11%) had idiopathic cardiomyopathy, 26 (30%) had congenital heart disease, 13 (15%) had viral cardiomyopathy, seven (8%) had familial cardiomyopathy, two (2%) had cardiomyopathy resulting from doxorubicin therapy, and one other case was not further delineated. In the heart-lung transplant group, there were eight males and seven females; pretransplantation diagnoses included nine (60%) with congenital heart disease and Eisenmenger's physiology, three (20%) with primary pulmonary hypertension, and one (7%) each with cystic fibrosis, bronchopulmonary dysplasia, and congenital lymphangiectasia. Fifteen (17%) of the heart transplant recipients and three (20%) of the heart-lung transplant recipients had GCD on angiographic or pathologic examination. Histopathologic samples were available on 14 cases (11 heart transplants and three heart-lung transplants).(ABSTRACT TRUNCATED AT 250 WORDS)