1Cardiovascular Research Center, Mashhad University of Medical Sciences, Mashhad, Iran
2Cardiologist,17 Shahrivar Hospital,Mashhad,Iran
3Cardiologist ,Yasuj University of Medical Sciences, Yasuj, Iran
Inroduction: Heart failure (HF) is an important cause of morbidity and mortality in the cases of Beta-thalassemia major. The purpose of this study was to estimate HF prevalence in these patients and to assess the survivability of those who were treated with intensive chelating therapy.
Design and methods: This cross sectional study included 72 beta-thalassemia major cases, the mean age at the time of referral was 15.7±6.2 years (range 6-35 years) and were followed in a prospective 2 year study. A self-reporting symptom questionnaire was administered, a 12-lead ECG was taken and an echocardiography was obtained from all participants. Echocardiography was performed at 6 month intervals or when a new symptom developed. Results: Risk factors (except for iron overload) in the study population were hypothyroidism and diabetes mellitus. The male to female ratio was0.75.Twelve patients had left ventricular (LV) systolic dysfunction and 57,79% had LV diastolic dysfunction whereas 11,15% had RV failure. Fifty-nine (81%) patients had cardiac disease of which diastolic dysfunction was the most common manifestation .Those with systolic dysfunction were older at presentation (22 ± 6 years versus 31 ± 4 years; P <0.001), and had the highest mean serum ferritin level (3,355 ± 1241 ng/mL versus 6,397 ± 1,613 ng/mL; P <0.001). The 2 year survival rate in patients with beta thalassemia in this study was 98%. Conclusions: Diastolic dysfunction is highly prevalent in even asymptomatic beta-thalassemia major patients. The high prevalence of diastolic dysfunction is indicative of a significant amount of the population who are at a high risk for HF