Correlation between plasma biochemical parameters and cardio-hepatic iron deposition in thalassemia major patients.

INTRODUCTION: Major Thalassemia patients suffer from iron overload and organ damage, especially heart and liver damage. Early diagnosis and treatment with a chelator can reduce the complications and mortality of iron overload. Therefore, we aimed to investigate the biochemical and hematological predictors as an alternative and indirect indicator of iron deposition in heart and liver cells in comparison with the MRI T2* method as the gold standard. MATERIAL AND METHOD: MRI T2* was evaluated in the heart and liver tissues of 62 major beta-thalassemia patients undergoing regular transfusion and chelator therapy. Biochemical and hematological factors were also measured, including serum ferritin, serum electrolytes, liver enzymes, hemoglobin, blood glucose, and serum magnesium. The correlation between these factors was assessed using statistical evaluations. RESULT: Serum ferritin had a positive and significant correlation with liver siderosis based on MRI T2* (p-value = .015), and no significant association was observed with cardiac siderosis (p-value = .79). However, there was a significant positive correlation between cardiac iron deposition and fasting blood sugar level (p-value = -.049), and plasma level of liver enzymes (alanine aminotransferase (ALT) (p-value = .001), aspartate aminotransferase (AST ((p-value = .01)). Moreover, there was a significant negative correlation between cardiac iron overload and plasma magnesium level (p-value = .014). According to MRI T2*, there was no significant correlation between cardiac and hepatic iron overload (p value = .36). CONCLUSION: An increase in blood sugar or liver enzymes and a decrease in serum magnesium was associated with an increase in cardiac iron overload based on MRI T2*. Liver iron overload based on MRI T2* had a significant correlation with serum ferritin.

The correlation of cardiac biomarkers and myocardial iron overload based on T2* MRI in major beta-thalassemia.

Cardiac hemosiderosis is the primary factor to derive the pathogenesis of cardiac dysfunction in patients with transfusion dependent thalassemia. Biomarkers assessment along with T2 * MRI study could be employed to evaluate the severity of iron deposition-related damage and determination of the diagnostic and prognostic value of these inflammatory factors. The study was conducted on 62 patients (12-44 years old) with major thalassemia. The patients were under regular blood transfusion and they had no signs of cardiac defects, and chronic diseases. The serum levels of inflammatory factors (NT-proBNP, CRP, Copeptin HS) were determined before routine transfusion. Cardiac iron overload was assessed by T2* MRI (within the last three months), and T2* lower than 20 ms was considered as cardiac siderosis. The obtained results were analyzed using statistical methods. 92% of patients showed an increased level of hs-CRP (> 2 µg/dL). All cases showed increased levels of NT-proBNP (> 150 pg/mL). Only 29% of subjects showed high level of Copeptin, 25.8% of patients demonstrated cardiac siderosis based on the T2* MRI (< 20 ms) results. The serum levels of inflammatory factors were not significantly correlated with cardiac siderosis. Given the obtained results, it could be deduced that the serum levels of inflammatory factors could not be exploited for early detection of cardiac siderosis in major beta-thalassemia patients.

Takotsubo Cardiomyopathy With Significant Coronary Stenosis and Atrioventricular Conduction Block: A Rare Case Report With 3 Year Follow-Up.

INTRODUCTION: Takotsubo cardiomyopathy (TCMP) is a rare acute cardiomyopathy characterized by acute chest pain syndrome, similar to myocardial infarction, except that no significant stenosis is observed on coronary angiography in patients with this condition; these findings aid the diagnosis of TCMP. CASE PRESENTATION: We discuss an unusual case of TCMP in a 45-year-old woman with complete heart block and significant coronary artery stenosis. Maximal exercise test and perfusion scan after 1 month from the acute event did not show any ischemia; therefore, revascularization was not recommended. Her follow-up with normal echocardiographic data 3 years after the first event showed no recurrence. CONCLUSIONS: The present case and a few previous cases have showed that severe coronary artery disease may be occur in patients with TCMP and that TCMP may be associated with a high-degree atrioventricular block. The association between atrioventricular conduction block and TCMP as well as significant coronary stenosis is rarely reported; therefore, coronary angiography should be performed in all patients with clinical TCMP and the previous definition should be reconsidered. The occurrence of arrhythmia and later recovery is expected in these patients (due to a catecholamine surge).

Doppler echocardiographic assessment of pulmonary prostheses: a comprehensive assessment including velocity time integral ratio and prosthesis effective orifice area.

OBJECTIVE: Few reports have been published on the Doppler-derived echocardiographic data for pulmonary valve prostheses (PVPs). The aim of this study was to provide a comprehensive Doppler echocardiographic assessment of PVPs. METHODS: We studied 40 patients (mean age 24.2) with PVPs: 13 (32.5%) mechanical and 27 (67.5%) bioprosthetic valves. After clinical evaluation, all patients underwent complete, two-dimensional and Doppler studies. RESULTS: In 30 patients with normally functioning PVPs, the mean (SD) peak velocity was 2.33 (0.36) m/s with an average peak pressure gradient of 22.69 (6.7) mm Hg and an average mean pressure gradient of 12.5 (4.1) mm Hg. The mean PVPs velocity time integral (VTI) was 47.49 (12.78) cm with mean right ventricle outflow tract/peak velocity (PV) VTI ratio 0.43 (0.14), mean PVPs effective orifice area was 1.63 (0.36) cm(2). Metallic PVPs had significantly better hemodynamic Doppler study compared with biologic PVPs. In 9 patients with PVP malfunction, average peak PVPs velocity, average peak pressure gradient, mean pressure gradient, PV VTI, PV/left ventricle outflow tract VTI ratio was significantly increased (P < 0.05). CONCLUSION: This study contributes to establishing the normal range for Doppler hemodynamics in various PVPs.