Definition of inseparably fused ventricular myocardium in thoracopagus: fetal echocardiographic utility and pathologic refinement.

Correlative echocardiographic and pathological findings in a thoracopagus with conjoined hearts are reported. One twin had tricuspid atresia with discordant atrioventricular connections and concordant ventriculoarterial connections. The morphologic right ventricle was hypoplastic and there was a large muscular ventricular septal defect.

Manpower deficiencies in cardiovascular pathology. Implications for medical care of cardiovascular diseases.

The general shortage of pathology manpower has been well documented, but virtually no published data are available on cardiovascular pathologists. To address this situation, the Society for Cardiovascular Pathology surveyed all chairpersons of academic pathology departments, chiefs of academic clinical cardiovascular programs, including adult and pediatric cardiology and cardiovascular surgery, in the United States and Canada, regarding their perceptions of cardiovascular pathology manpower and the training and areas of expertise necessary for a competent cardiovascular pathologist. Of total respondents, 41% stated that their institution's department of pathology needed to add a cardiovascular pathologist, 39% of respondents replied that money and funding were available to hire an additional cardiovascular pathologist, yet only 19% stated that their institution intended to add a cardiovascular pathologist.

Acute rheumatic carditis: diagnostic and therapeutic challenges in the era of heart transplantation.

Heart transplantation as a treatment for end-stage heart failure has spawned numerous important challenges in patient care. A heart transplant patient with clinically unsuspected acute rheumatic carditis had an ultimately fatal course marked by refractory rejection and early death after transplantation. The patient had several immune abnormalities.

Endomyocardial biopsy findings after photopheresis treatment of cardiac transplant rejection.

Photopheresis is a potential therapy for allograft rejection in which reinfusion of mononuclear cells exposed to ultraviolet-A irradiation after pretreatment with 8-methoxypsoralen may initiate immunosuppressive responses. Endomyocardial biopsies (EMBs) of cardiac transplant recipients with moderate acute rejection (IHSLT grades 2 and 3) treated with photopheresis (7 patients/9 treatments) and followed for six months or more were evaluated and compared with biopsies of patients treated with corticosteroids (7 patients/8 treatments) and followed for a similar time period. The first posttreatment EMB showed improvement in 100% of corticosteroid-treated patients, compared with 56% of photopheresis-treated patients (p < 0.

A decade of acceptable autopsy rates. Does concordance of clinician and pathologist views explain relative success?

In an attempt to better understand the basis and significance of an annual autopsy rate consistently over 45% for the past decade, we recently investigated the attitudes and practices of 36 pathologists and 176 clinicians in our institution with respect to the function of the autopsy service and the utility of the autopsy. The autopsy report was "not used in a consistent manner" by 57% of clinical respondents. Several clinicians thought that autopsy reports were too long (20%) and too slow (38%), but not with the frequency that pathologists did, 73% and 58%, respectively.

Photopheresis versus corticosteroids in the therapy of heart transplant rejection. Preliminary clinical report.

Background: Photopheresis is a technique in which reinfusion of mononuclear cells exposed to UV-A light ex vivo after in vivo treatment with 8-methoxypsoralen initiates host-immunosuppressive responses.

Methods And Results: To determine if photopheresis safely reverses International Society for Heart and Lung Transplantation (ISHLT) rejection grades 2, 3A, and 3B without hemodynamic compromise, 16 heart transplant patients with ISHLT rejection grades 2, 3A, and 3B were randomized to photopheresis or corticosteroid therapy. The average number of mononuclear cells treated with each photopheresis procedure was 9.

The clinical problem of occult cardiac amyloidosis. Forensic implications.

A 68-year-old man with known coronary heart disease experienced rapidly progressive cardiac dysfunction and was found to have occult cardiac amyloidosis at autopsy. The amyloidosis was undiagnosed during life and initially at autopsy. Marked diffuse involvement of the intramural coronary arteries by amyloid deposits resulted in severe luminal compromise of numerous medium and small vessels.

Ischemic myocardial injury in rapidly paced dogs: Contribution to ventricular dysfunction.

Chronic rapid ventricular pacing in the dog has been shown to provide a predictable and stable model of heart failure for which the operative pathomechanism remains in question. In a group of 20 dogs with congestive failure induced by rapid ventricular pacing for a mean duration of 29.5 ± 13.

Successful treatment of heart transplant rejection with photopheresis.

Photopheresis is a potential therapy for rejection in which reinfusion of mononuclear cells exposed to ultraviolet-A light ex vivo, after treatment with 8-methoxypsoralen in vivo, initiates host immune responses that specifically inhibit the cytotoxicity of the photomodulated mononuclear cells. Between May 1990 and January 1991, 7 heart transplant (HT) patients (age 42.2 +/- 16.

Progress in blood lipid reporting practices by clinical laboratories in North America. Changes from 1985 to 1990.

In an attempt to assess the impact of the National Cholesterol Education Program recommendations and to provide follow-up to a 1985 survey of blood lipid reporting practices in 152 academic clinical laboratories in North America, the same laboratories were resurveyed regarding current blood lipid-reporting practices. All 97 laboratories that responded to our survey routinely measured serum total cholesterol, while 94% measured high-density lipoprotein cholesterol, 99% measured triglycerides, 26% apolipoproteins A1 and B, and a single laboratory measured apolipoprotein E. There was a marked downward shift in reference ranges for serum total cholesterol used by laboratories in 1990 compared with 1985.

Cytotoxic and viral neutralizing antibodies crossreact with streptococcal M protein, enteroviruses, and human cardiac myosin.

The development of autoimmunity in certain instances is related to infectious agents. In this report, cytotoxic monoclonal antibodies (mAbs) that recognize epitopes on both enteroviruses and the bacterium Streptococcus pyogenes are described. Murine anti-streptococcal mAbs that were crossreactive with streptococcal M protein, human cardiac myosin, and other alpha-helical coiled-coil molecules were found to neutralize coxsackieviruses B3 and B4 or poliovirus type 1.

Enteroviral infection of mice with severe combined immunodeficiency. Evidence for direct viral pathogenesis of myocardial injury.

Inbred mice with genetically determined severe combined immunodeficiency (SCID) lack mature T and B lymphocyte functions. To distinguish direct viral effects in the pathogenesis of myocarditis from those mediated by antigen-specific and histocompatibility-complex-restricted host immunity, we inoculated coxsackievirus B3 into homozygous young adult SCID mice. We found that infected SCID mice invariably developed extensive myocarditis between 7 and 14 days postinoculation with high subsequent mortality.

Preferential endocardial residence of B-cells in the "Quilty effect" of human heart allografts: immunohistochemical distinction from rejection.

Endocardial infiltrates (EI) are a common and often problematic observation in endomyocardial biopsy specimens (EMBs) from patients receiving cyclosporine immunosuppression following cardiac transplant. Histologic and immunohistologic findings in 23 EMBs from 19 patients and 15 autopsy or explanted allografts demonstrated EIs to be rich in B lymphocytes (871/mm2) compared to T-lymphocytes (803/mm2). Macrophages also demonstrated an endocardial preference over deeper myocardium.

Progress and challenges in the pathological diagnosis of myocarditis.

The pathological diagnosis of myocarditis rests on well-described histopathological criteria. Appreciation for the disease-specific sensitivity of the endomyocardial biopsy, as well as the phenotypical and functional nature of inflammatory infiltrates, will enhance the clinical utility of the biopsy technique. New information regarding the role of enteroviruses in immunological sensitization and the study of enteroviruses in murine models and human patients are fostering new perspectives and routes of investigation of appropriate therapeutic interventions for myocarditis.

Associated occurrence of persistent truncus arteriosus and asplenia.

An infant with persistent truncus arteriosus associated with splenic agenesis and the asplenia syndrome is reported, including clinical, echocardiographic and autopsy findings. To the authors' knowledge this association has not been previously reported.

Progressive cardiac fibrosis and myocyte injury in v-fps transgenic mice. A model for primary disorders of connective tissue in the heart?

Transgenic mice that express v-fps protein-tyrosine kinase have severe cardiac or neurologic abnormalities and a high incidence of lymphoid or mesenchymal tumors. The cardiac lesions of v-fps transgenic mice were examined at less than 1, 2, 3, 6, 14, 26, and 43 weeks of age (total N = 19) and compared with nontransgenic littermate controls (N = 34). Three of eight transgenic animals 1 to 4 days old showed moderate proliferation of connective tissue elements most evident along the septal endocardium of the right ventricle.

Susceptibility to Coxsackievirus B3-induced chronic myocarditis maps near the murine Tcr alpha and Myhc alpha loci on chromosome 14.

This study was undertaken to determine the genetic control of host susceptibility to coxsackievirus B3 (CVB3)-induced chronic myocarditis in a mouse model. An autosomal recessive autoimmune myocardial disease (amd) gene (possibly more than one gene), which determined susceptibility to CVB3-induced chronic myocarditis in the A/J and DBA/2J inbred mouse strains, was mapped to a segment of chromosome 14. Data from both the AXB/BXA recombinant inbred (RI) strains and the B10.

Immunoreactivity of anti-streptococcal monoclonal antibodies to human heart valves. Evidence for multiple cross-reactive epitopes.

Association of group A streptococci with acute rheumatic fever and valvular heart disease is well established; however the basis of valve injury remains unclear. In this study, anti-streptococcal monoclonal antibodies (MAbs) cross-reactive with myocardium were reacted with sections from 22 rheumatic valves, nine normal, five endocarditic, one 'floppy,' and one Marfan valve. In immunohistochemical studies, MAb reactivity was observed with cardiac myocytes, smooth muscle cells, cell surface and cytoplasm of endothelial cells lining valves, and valvular interstitial cells.