Cardiac determinants of survival in beta-thalassemia.

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haematologica online 2006
haematologica/the hematology journal | 2006; 91(online) | 123 |
Cardiac determinants of survival in
beta-thalassemia
Haematologica 2006; 91:(9)e123
concerning survival of medically treated thalassemia
patients in Cyprus.1Although the authors have analyzed
data on a large cohort of patients with beta-thalassemia
and presented very encouraging data on survival for the
patients who were followed-up after the year 2000, there
are some issues to be addressed. It is well known that
congestive heart failure is the main cause of death in
transfusion-dependent patients with beta-thalassemia.
Cardiac function in these patients remains normal for
many years, but once symptoms of heart failure become
evident, death usually occurs within 1 year.2,3This is in
accordance with the results reported by Telfer et al,1
where the 53.4% of deceased patients died due to cardiac
causes. Although cardiac death is the most frequent cause
of death in patients with beta-thalassemia, early markers
of cardiac function with prognostic impact on survival
have not yet been adequately defined. In a recently pub-
lished study we investigated the impact of the Doppler-
demonstrated left ventricular restrictive filling pattern on
survival in a cohort of 45 asymptomatic adult patients
with beta-thalassemia and normal left ventricular systolic
function throughout a 15-year long observation period.4
Our data indicates that the presence of left ventricular
restrictive filling pattern predicts worse prognosis in
patients with beta-thalassemia. The Kaplan – Meier sur-
vival analysis showed that the mean survival in patients
with restrictive filling pattern was 11.1±1.2 years (95%
CI: 8.7 to 13.4 years), compared to 14.3±0.5 years (95%
CI: 13.3 to 15.2 years) in patients with non-restrictive fill-
We read with great interest the article by Telfer et al
ing pattern. The 15-year cumulative survival rate was
58% in patients with restrictive filling pattern and 88% in
patients with non-restrictive filling pattern (log-rank sta-
tistic=6.02, p=0.014). We also found that patients’ poor
compliance with chelation therapy was significantly
associated with restrictive filling pattern (p=0.007). In
conclusion, Doppler-echocardiography is the main tool in
the era of long-term follow-up of patients with beta-tha-
lassemia and should be performed routinely in these
patients. It would be very interesting if the authors of this
study, which includes more than 500 patients with beta-
thalassemia, provided the Doppler-echocardiographic
data of the patients who died due to cardiac causes.
Efthimiadis GK,1Hassapopoulou HP,2Tsikaderis DD,1Karvounis HI,1
Giannakoulas G,1Parharidis GE1
1Department of Cardiology, Aristotle University, AHEPA Hospital,
Thessaloniki, Greece
2Thalassemia Unit, AHEPA Hospital, Thessaloniki, Greece
References
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Pangalou E, et al. Survival of medically treated thalassemia
patients in Cyprus. Trends and risk factors over the period
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