@article{3093967,
    title = "Thalassernia heart disease - A comparative evaluation of Thalassemia
major and Thalassemia intermedia",
    author = "Aessopos, A and Farmakis, D and Deftereos, S and Tsironi, A and and Tassiopoulos, S and Moyssakis, I and Karagiorga, M",
    journal = "Chest",
    year = "2005",
    volume = "127",
    number = "5",
    pages = "1523-1530",
    publisher = "ELSEVIER SCIENCE BV",
    issn = "0012-3692, 1931-3543",
    doi = "10.1378/chest.127.5.1523",
    keywords = "deferoxamine; heart failure; iron chelation; pulmonary hypertension;
thalassemia; transfusions",
    abstract = "Background: Heart disease represents the main determinant of survival in
beta-thalassemia, but its particular features in the two clinical forms
of the disease, thalassemia major (TM) and thalassemia intermedia (TI),
are not completely clarified.
Methods: We compared clinical and echocardiographic global parameters in
131 TM patients who received regular chelation transfusions and were
highly compliant with treatment (mean age, 28 +/- 6 years [+/- SD%
and 74 age-matched, TI patients who did not receive chelation
transfusions.
Results: Congestive heart failure was encountered in five patients with
TM (3.8%; age range, 25 to 29 years) and in two patients with TI
(2.7%; age range, 37 to 40 years). Systolic left ventiricular (LV)
dysfunction (ejection fraction < 55% or shortening fraction < 35%) was
only encountered in patients with TM (8.4%). Considerable pulmonary
hypertension (systolic tricuspid gradient > 35 mm Hg) was only present
in TI (23.0%). In the remaining patients without evident heart disease,
cardiac dimensions, LV mass, LV shortening and ejection fractions, and
cardiac output were significantly higher in patients with TI. LV
afterload was higher in patients with TM. LV diastolic early transmitral
diastolic peak flow velocity (E)/late transmitral diastolic peak flow
velocity (A) ratio was also higher in TM. Systolic and mean pulmonary
artery pressures and total pulmonary resistance were higher in both
young and old TI patients.
Conclusion: Regular lifelong transfusion and chelation therapy in TM
prevented premature heart disease and pulmonary hypertension, although
LV dysfunction still occurred and led to heart failure. The absence of
regular therapy in TI, in contrast, preserved systolic LV function but
allowed pulmonary hypertension development, which also led to heart
failure, starting within the fourth decade of fife, a decade later
compared to TM."
}