Presence of Circulating Anti-Myosin Antibodies in Endomyocardial Fibrosis

Endomyocardial Fibrosis is a tropical disease in which the heart cannot open properly to receive blood due to a scar that covers its inner layer. It affects mainly children and adolescents, and has a poor prognosis because the cause and mechanisms of scarring are unknown. The conventional treatment is frustrating and does not alter the natural history of the disease. Despite affecting several million people worldwide there has been little investigation on the mechanisms of the disease or drug development to improve its prognosis. In this study we investigate the presence of antibodies against the myocardial cells of African patients with severe and advanced EMF aiming at uncovering new pathways for the disease. Our results reveal that EMF patients have anti-myocardial antibodies in their blood. The reaction of these antibodies with the heart may be one of the mechanisms involved in the genesis of the fibrotic lesions. This knowledge may help in diagnosing the condition and provide alternatives for its management, using drugs that reduce the impact of the circulating antibodies in the cardiac tissue. The significance of these results needs confirmation on studies involving larger number of subjects due to frequent finding of antiheart antibodies in African populations with heart failure of any cause

The Aswan Rheumatic heart disease reGIstry: rationale and preliminary results of the ARGI database

BackgroundRheumatic Heart Disease (RHD) remains a major cause of valvular heart disease related mortality and morbidity in low- and middle-income countries, with significant variation in characteristics and course of the disease across different regions. However, despite the high disease burden, there is sparse region-specific data on demographics, disease characteristics and course in treated and untreated patients to guide policy.MethodsThe ARGI database is a hospital-based registry in a tertiary referral national centre (Aswan Heart Centre, AHC) in which all patients with the diagnosis of RHD are being included. The mode of presentation, including baseline clinical and echocardiographic characteristics (as well as other imaging modalities), biomarkers and genetics are being documented. Treatment modalities and adherence to treatment is being recorded and patients are followed up regularly every 6 and/or 12 months, or more frequently if needed.DiscussionThis study shows for the first time an in-depth analysis of the severity and phenotype of disease in Egyptian patients presenting with RHD as well as the progression with time and provides a platform for further comparisons of regional differences in these details as well as their causes. The ARGI database will be of help in achieving the objectives of the Cairo Accord aiming at eradication of RF and RHD

Patterns of Disease Progression in Hypertrophic Cardiomyopathy

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The heterotopic tracheal allograft as an animal model of obliterative bronchiolitis

Heterotopic tracheal allografts in small rodents have been shown to share many characteristics with the development of obliterative bronchiolitis (OB) in the clinic and therefore provide a suitable animal model for the study of OB. The model facilitates the examination of the pathogenesis of the disease and the elucidation of the cellular and molecular mechanisms involved in its development. The model provides a less technically demanding alternative to whole lung transplantation in small rodents and should lead to a speedier identification of new treatments that might prevent the development of post-transplantation OB in the clinic

Valve interstitial cells induce donor-specific T-cell anergy

AbstractObjectives: Valve allografts produce an immune response, which can influence their performance. The exact role of the interaction between recipient T cells and the different cellular components of the donor valve in stimulating an immune response is not known. Therefore the T-cell response to valve endothelial and interstitial cells was investigated in vitro. Methods: Valve endothelial and interstitial cells were characterized for cell-surface molecules before and after interferon γ treatment by means of a panel of specific monoclonal antibodies and flow cytometry. The proliferative response of highly purified T lymphocytes was used to assess the immunogenicity of cultured valve endothelial and interstitial cells. This was further investigated by using a 2-step tolerance-induction protocol. Results: Valve endothelial and interstitial cells express similar levels of human leukocyte antigens and adhesion and costimulatory molecules, which are either induced or upregulated after interferon γ treatment. T-cell responses to endothelial cells were detected after interferon γ treatment, but responses to interferon γ–treated interstitial cells were not detected. This lack of response resulted in the induction of T-cell anergy, which was reversed by the presence of the costimulatory molecule B7-1. Conclusions: Although valve endothelial and interstitial cells express a similar range of cell-surface molecules, it is only the endothelial cells that are immunogenic. In addition, we have shown that these 2 cell types interact in a donorspecific manner to orchestrate the immune response and therefore may have clinical relevance in the allogeneic response of the heart valve recipients.J Thorac Cardiovasc Surg 2001;122:129-3

Pulmonary autograft versus homograft replacement of the aortic valve: A prospective randomized trial

AbstractBackground: Pulmonary autografts offer many theoretical advantages. However, the operation is complex, may interfere with right ventricular and pulmonary outflow function, and requires a longer operative time than does the homograft operation. The effects of these potential disadvantages are unknown. Methods: To clarify these issues we randomized 70 patients undergoing aortic valve replacement to an aortic homograft group (group A = 37 patients; 53%; 34 male, 3 female) or a pulmonary autograft group (group B = 33 patients; 47%; 28 male, 5 female). Ages varied from 12 to 65 years (mean 39 ± 15 years) for group A and from 3 to 54 years (mean 29 ± 15 years) for group B (p = not significant). Eleven patients in group A (30%) and eight in group B (24%) had previous aortic valve surgery. All patients were operated on by the same surgeon. The mean cardiopulmonary bypass time was 113 ± 29 minutes (range 66 to 175 minutes) for group A and 151 ± 31 minutes (range 115 to 226 minutes) for group B (p < 0.002). Mean aortic crossclamp time was 85 ± 19 minutes (range 45 to 140 minutes) for group A and 109 ± 20 minutes (range 74 to 164 minutes) for group B (p = 0.02). In 32 patients (86.5%) the aortic homograft was implanted as a root with coronary reimplantation. All pulmonary autografts were implanted as a root. Results: No early or late deaths had occurred in this series at a mean follow-up time of 16 months (range 3 to 21 months). Two patients (one in each group) required reexploration for bleeding. No statistically significant differences were observed between the two groups with regard to ventilatory support (group A, mean 10 ± 8.5 hours; group B, mean 29 ± 85 hours), total blood loss (group A, mean 471 ± 347 ml; group B, mean 543 ± 404 ml), intensive care unit stay (group A, mean 1.2 ± 0.6 days; group B, mean 2 ± 3.7 days), and hospital stay (group A, mean 9.5 = 3.2 days; group B, mean 12 ± 6 days). Postoperatively, all patients are in New York Heart Association class I (93%) or II (7%) (p = not significant). Ejection fraction for the two groups did not change significantly over the follow-up period. Left ventricular mass and diastolic diameter showed progressive regression, with no apparent difference between the two treatment groups to date. Echocardiographic evalu- ation of aortic valve function at 6 months showed good valve function in all patients with no evidence of aortic regurgitation in 80% of both groups. In group B the right ventricular outflow gradient was below 15 mm Hg over the follow-up period. Holter monitoring, available only in 44 patients (63%), showed most of the arrhythmias to be grade 0 to 1 of the modified Lown grading system. Conclusion: Although the pulmonary autograft requires a significantly longer operating time, this does not seem to affect early and medium-term outcome when compared with results obtained with aortic homografts. Continued patient evaluation is warranted, particularly with regard to evidence of valve degeneration and right ventricular function and arrhythmias in the long term. (J Thorac Cardiovasc Surg 1997;113:894-900