Assessment of survival without cardiac disease of thalassemic patients of Shiraz, Iran, 2005

Background: Thalassemia is the most common hemolytic anemia in the world. Absence or
irregular chelation therapy in these patients leads to cardiac complications such as heart failure,
precarditis and arrhythmia. These complications are among the most common cause of death in
thalassemic patients. The purpose of this study was investigation of survival without cardiac
disease and effective factors in these patients.
Methods and materials: In a retrospective study (with enumeration method) the survival without
cardiac disease was studied in 806 thalassemic patients (406 male and 400 female) referring to
Cooley’s ward of Dastgheib hospital in Shiraz using a data collection form. In addition to
determination of age of cardiac disease incidence to survival analysis, demographic information
and age at onset and type of transfusion and age at onset and method of chelation therapy also
specified. Our data analyzed using descriptive and analytical (Kaplan-Meier and Log Rank test)
statistic.
Results: The age range of the subjects was from 1 to 43 years (mean ± SD: 15.34 years ± 6.82).
The prevalence of cardiac disease was 15.9%. The mean age of onset of cardiac disease and mean
age of patients with complications were 16.93 ± 5.44 and 19.6 ± 5.8 respectively and the estimated
average years of survival with cardiac disease was 2.67 years. The median of survival without
cardiac disease was 31.5 years and the estimated rates of survival without cardiac disease of 20
and 25 years were 75% and 69% respectively. There was a significant association between
cumulative probability of occurrence of cardiac disease and age, education of parents and age of
chelation therapy onset.
Conclusions: Early onset of blood transfusion with late onset of chelating therapy increases the
occurrence probability of cardiac disease. Educational level of parents and education of parents
and patients about thalassemia and its complication would be very helpful in prevention of
complications.