Longitudinal analysis of heart and liver iron in thalassemia major.

High hepatic iron concentration (HIC) is associated with cardiac iron overload. However, simultaneous measurements of heart and liver iron often demonstrate no significant linear association. We postulated that slower rates of cardiac iron accumulation and clearance could reconcile these differences. To test this hypothesis, we examined the longitudinal evolution of cardiac and liver iron in 38 thalassemia major patients, using previously validated magnetic resonance imaging (MRI) techniques. On cross-sectional evaluation, cardiac iron was uncorrelated with liver iron, similar to previous studies. However, relative changes in heart and liver iron were compared with one another using a metric representing the temporal delay between them. Cardiac iron significantly lagged liver iron changes in almost half of the patients, implying a functional but delayed association. The degree of time lag correlated with initial HIC (r = 0.47, P < .003) and initial cardiac R2* (r = 0.57, P < .001), but not with patient age. Thus, longitudinal analysis confirms a lag in the loading and unloading of cardiac iron with respect to liver iron, and partially explains the weak cross-sectional association between these parameters. These data reconcile several prior studies and provide both mechanical and clinical insight into cardiac iron accumulation.

Nutritional deficiencies in iron overloaded patients with hemoglobinopathies.

One of the hallmarks of both sickle cell disease (SCD) and thalassemia major (TM) is accelerated oxidative damage. Decreased antioxidant levels and increased oxidant stress biomarkers are found in both diseases. Although isolated vitamin deficiencies have been reported in TM and nontransfused SCD patients, a comprehensive evaluation of vitamin and trace mineral levels has never been performed in chronically transfused SCD or TM patients. As vitamins and trace minerals may be consumed as a result of chronic oxidative stress; we hypothesized that levels of these compounds would correlate with surrogates of iron overload, hemolysis, and inflammation in chronically transfused patients. Using a convenience sample of our group of chronically transfused patients we studied 43 patients with SCD (17 male, 26 female) and 24 patients with TM (13 male and 11 female). The age range for our patients varied from 1.5 to 31.4 years. Levels of vitamins A, thiamin, B6, B12, C, D, E as well as selenium, zinc, copper, and ceruloplasmin were measured. We found that 40-75% of the patients were deficient in A, C, D and selenium and 28-38% of the patients had low levels of B vitamins and folate. There was little association with iron overload, hemolysis, or inflammation. Although the precise mechanism of these deficiencies is unclear, they may contribute to the morbidity of chronically transfused hemoglobinopathy patients.

Effective iron chelation practice for patients with ß-thalassemia major.

Chronic blood transfusion is the only treatment for severe anemia in patients with ß-thalassemia major. However, red blood cell transfusions lead to iron overload and subsequent organ damage because of the toxic effects of iron. The heart is particularly vulnerable to iron toxicity, and heart failure is the leading cause of death among these patients. Iron chelation therapy prevents or reverses iron loading, thereby reducing the risk of complications from excess iron. Serum ferritin and liver iron concentration often are used to gauge the risk of organ iron overload, but these measurements may not correlate well with cardiac iron load. Magnetic resonance imaging (MRI) is a noninvasive diagnostic tool that can provide a more direct measure of iron concentration in both the heart and liver. Cardiac iron determined by MRI is expressed as a function of T2*, in which higher values represent lower concentrations. Changes in T2* are used to assess the effectiveness of iron chelation and to adjust therapy. Early treatment and compliance are keys to successful therapy. Nursing strategies to optimize chelation therapy include identifying patients who are at risk for developing organ damage, developing chelation plans, promoting compliance, and educating patients. The efficacy and safety of iron chelators, as well as nursing best practices, are reviewed.

Stentless aortic valves are hemodynamically superior to stented valves during mid-term follow-up: a large retrospective study.

BACKGROUND: Several studies have compared left venticular mass (LVM) regression and hemodynamic data for stentless versus stented aortic bioprostheses with conflicting results. The major limitations of these studies are their small sample size and short-term follow-up. We therefore compared midterm LVM regression, hemodynamic data, and survival in a large population of tissue aortic valve replacement (AVR) patients. METHODS: All patients undergoing tissue AVR at our institution between 1998 and 2001 were included (n = 737). Patients were divided into two groups according to type of bioprosthetic implanted: stentless patients (total n = 310) (Toronto SPV [St Jude Medical, St Paul, MN], n = 146 and Freestyle [Medtronic, Minneapolis, MN], n = 164) and stented patients (total n = 427) (Perimount [Edwards Life Sciences Inc, Irvine, CA], n = 291 and Mosaic [Medtronic], n = 136). RESULTS: The two groups of patients had similar preoperative transvalvular gradients and LVM index (130 +/- 47 vs 130 +/- 42 g/m2 for stentless versus stented valves, respectively). Predischarge echos revealed that stentless patients had significantly lower mean transvalvular gradients (11 +/- 5 vs 15 +/- 6 mm Hg, p < 0.001) and larger effective orifice areas (1.32 +/- 0.52 vs 1.22 +/- 0.48 cm2, p = 0.01). Follow-up echocardiograms were obtained in 99% of surviving patients 28 +/- 22 (range, 0-79) months postoperatively. Stentless patients had significantly lower LVM index during follow-up (100 +/- 32 vs 107 +/- 32 g/m2, p = 0.005) and stentless valves were an independent predictor of LVM regression. Furthermore, a higher proportion of stented patients had residual LV hypertrophy during follow-up (28% vs 18%, p = 0.001). Stentless valves were associated with improved midterm survival by univariate analysis, but not by multivariable analysis. CONCLUSIONS: Midterm follow-up in a large number of patients reveals that stentless bioprostheses are hemodynamically superior to stented valves.