ABSTRACT
Accumulation of excess iron in heart can lead to cardiac dysfunction, which is the most common cause of death in thalassemia major patients. Biopsy is an invasive procedure and therefore not an ideal option to assess iron load. However, standard/usual non-invasive methods, such as ferritin measurement, have some limitations and the results show poor correlations with iron load. Magnetic Response Imaging (MRI-T2*), as a non-invasive and reliable method for iron load assessment in organs such as liver and heart, can be suggested as a favorable alternative. This cross-sectional study was implemented in Thalassemia and Hemophilia Clinic Center (Sarvar) affiliated with Mashhad University of Medical Sciences, Mashhad, Iran, from 2012 to 2013. After the approval of the research protocol by the local ethic committee, laboratory tests, including CBC and serum ferritin, were carried out, and echocardiography and heart and liver MRI-T2* were performed. All statistical analysis was done through SPSS software (version 11.5), using independent sample t test and Pearson's correlation coefficient test. A P value ≤0.05 was considered to be significant. 88 patients with the mean (±SD) age of 21.2 (±5.6) years, (range 11-37 years) were observed. Iron load was assessed using MRI-T2* with the following results: Out of 88 patients, 48.9 % had mild to severe cardiac siderosis, and 75.2 % had mild to severe liver siderosis. We demonstrated a correlation between liver MRI-T2* and serum ferritin, and heart MRI-T2* and ejection fraction. However, no correlation between liver and heart MRI-T2* was observed. Heart and liver siderosis is a common and serious problem in thalassemia major patients, and MRI-T2* as a sensitive and non-invasive technique can be used for early/timely detection of siderosis and good therapeutic monitoring in these patients.
ABSTRACT
BACKGROUND AND OBJECTIVES: The rate of consanguineous marriage is high in Middle Eastern countries such as Iran. The relationship between consanguineous marriage and congenital heart disease is discussed in some studies, but there is not much data for relationship between atrial septal defect (ASD) and consanguineous marriage. The aim of this study was to evaluate the relationship between consanguineous marriage and ASD echocardiographic characteristics. DESIGN AND SETTINGS: This was a cross-sectional study approved by Mashhad University of Medical Sciences ethics committee and took place in Mashhad, Iran, for a period of 3 years from August 2008 till September 2011. METHODS: In this cross-sectional study, 113 ASD patients participated and they were categorized into 3 groups on the basis of family relationship between their parents: first group-"no relationship," second group- "third degree relationship," and third group- "far relationship." RESULTS: Among the 54 male and 59 female ASD patients, the most prevalent type of ASD was ASD secundum (85.0%) followed by sinus venosus (8.8%). A total of 56% patients were present in the first group and 15% and 29% in the second group and the third group, respectively." The relationship between consanguinity and type of ASD (P < .001) and gender (P < .001 each) was observed. The relationship between the age of onset of disease and consanguinity (P=.003) was also observed. CONCLUSION: Considering the fact that there is a high prevalence of ASD and consanguineous marriage in Iran and bearing in mind the results of the present study, we recommend educating couples about the outcomes of consanguineous marriage in pre-marriage counseling.
