Liver disease in chelated transfusion-dependent thalassemics: the role of iron overload and chronic hepatitis C.

Abstract Iron overload and hepatitis virus C infection cause liver fibrosis in thalassemics. In a monocentric retrospective analysis of liver disease in a cohort of 191 transfusion-dependent thalassemics, in 126 patients who had undergone liver biopsy (mean age 17.2 years; 58 hepatitis virus C-RNA positive and 68 hepatitis virus C-RNA negative) the liver iron concentration (median 2.4 mg/gr dry liver weight) was closely related to serum ferritin levels (R = 0.58; p<0.0001). Male gender (OR 4.12) and serum hepatitis virus C-RNA positivity (OR 11.04) were independent risk factors for advanced liver fibrosis. The majority of hepatitis virus C-RNA negative patients with low iron load did not develop liver fibrosis, while hepatitis virus C-RNA positive patients infected with genotype 1 or 4 and iron overload more frequently developed advanced fibrosis. Hepatitis virus C infection is the main risk factor for liver fibrosis in transfusion-dependent thalassemics. Adequate chelation therapy usually prevents the development of liver fibrosis in thalassemics free of hepatitis virus C-infection and reduces the risk of developing severe fibrosis in thalassemics with chronic hepatitis C

Long-term treatment with deferiprone enhances left ventricular ejection function when compared to deferoxamine in patients with thalassemia major

Transfusion and iron chelation treatment have significantly reduced morbidity and improved survival of patients with thalassemia major. However, cardiac disease continues to be the most common cause of death. We report the left-ventricular ejection fraction, determined by echocardiography, in one hundred sixtyeight patients with thalassemia major followed for at least 5 years who received continuous monotherapy with deferoxamine (N = 108) or deferiprone (N = 60). The statistical analysis, using the generalized estimating equations model, indicated that the group treated with deferiprone had a significantly better left-ventricular ejection fraction than did those treated with deferoxamine (coefficient 0.97; 95% CI 0.37; 1.6, p = 0.002). The heart may be particularly sensitive to iron-induced mitochondrial damage because of the large number of mitochondria and its low level of antioxidants. Deferiprone, because of its lower molecular weight, might cross into heart mitochondria more efficiently, improving their activity and, thereby, myocardial cell function. Our findings indicate that the long-term administration of deferiprone significantly enhances left-ventricular function over time in comparison with deferoxamine treatment. However, because of limitations related to the design of this study, these findings should be confirmed in a prospective, randomized clinical trial

Tricuspid Valve Replacement with Mechanical Prostheses: Long-Term Results

Background and aim of the study: Historically, tricuspid valve replacement (TVR) has been associated with high mortality and morbidity, and current knowledge in the long-term outcome of TVR is limited. The study aim was to review the authors' experience from a consecutive series of patients. Methods: Between January 1990 and December 2005, a total of 43 patients (seven males, 36 females; mean age 52 14 years) underwent TVR. The etiology was rheumatic in 33 patients (77%) and degenerative disease in 10 (22%). Thirty-six patients (84%) were in NYHA class III or IV. Thirty-four patients (79%) underwent redo procedures; all patients underwent TVR with a mechanical prosthesis. Results: The overall operative mortality was 16% (n = 7). Of the 36 survivors, nine (25%) died during follow up. The Kaplan-Meier survival at 2.5, 5, and 10 years was 78%, 70%, and 58%, respectively. Five patients (14%) underwent reoperation during follow up (three for tricuspid valve thrombosis) and all five survived the reoperation. Freedom from reoperation at five and 10 years was 90% and 74%, respectively. On permutation test analysis, older age, liver congestion and redo surgery were found to be the major determinants of long-term mortality. Conclusion: TVR carries a higher short- and long-term mortality when compared to left-heart valve surgery. A timely referral before the development of end-stage cardiac impairment might determine a further improvement in outcome

Long-term results of sequential vein coronary artery bypass grafting compared with totally arterial myocardial revascularization: a propensity score-matched follow-up study

OBJECTIVES: The aim of the study was to analyse the early and long-term outcomes of a consecutive series of patients who underwent sequential coronary artery bypass grafting (CABG) and to compare them with a matched population of totally arterial revascularized patients. METHODS: From January 1994 to December 1996, 209 patients underwent total arterial myocardial revascularization at our institution [arterial (ART) group]. In the same period, 2097 patients underwent CABG with left internal thoracic artery on left anterior descending and great saphenous vein on the right and circumflex branches sequentially [sequential vein (SV) group]. The propensity score methodology was used to obtain risk-adjusted outcome comparisons between the two groups (209 vs 243 patients in the ART group and SV group, respectively). RESULTS: In-hospital mortality was 1% in the ART group and 0.4% in the SV group (P = 0.86). Mean follow-up was 14 \ub1 4 years. Long-term survival was comparable among the two study groups [actuarial 5- and 15-year survival rates were 97 vs 93% and 82 vs 79% in the ART group and the SV group, respectively (P = 0.29)]. At follow-up, recurrence of angina (17 vs 18%; P = 0.99), acute myocardial infarction (MI) (3 vs 5%; P = 0.72) and repeated percutaneous coronary intervention (19 vs 21%; P = 0.69) were similar in the ART group compared with the SV group. In the Cox regression analysis, type of revascularization was not an independent predictor of any long-term outcomes (death or major adverse cardiac events). In asymptomatic patients, exercise stress test at follow-up was comparable between the two groups (P = 0.14). CONCLUSIONS: Sequential vein CABG appears to have good early and long-term clinical outcomes. Also, early and long-term incidence of acute MI was not significantly higher in the SV group. However, further studies with a larger population are warranted in order to confirm the present results