Heart failure in beta thalassemia: a 5-year follow-up study.
OBJECTIVE
To evaluate the survival of patients with beta thalassemia and heart failure who were treated with iron chelation therapy.


METHODS
Fifty-two consecutive patients with beta thalassemia and heart failure were followed in a prospective 5-year study. All patients underwent a full clinical examination with chest radiograph, electrocardiogram, and echocardiographic investigation performed at 6-month intervals or when a new symptom developed.


RESULTS
Of the 52 patients (mean [+/- SD] age, 24 +/- 5 years), 25 (48%) survived 5 years after the onset of heart failure. Forty-three patients had left-sided heart failure, and 9 had right-sided heart failure. Those with left-sided heart failure were younger at presentation with heart failure (22 +/- 4 years vs. 31 +/- 6 years; P <0.001), had lower ejection fractions (36% +/- 9% vs. 64% +/- 10%; P <0.001), and had a lower mean serum ferritin level (3355 +/- 1241 ng/mL vs. 6,397 +/- 1,613 ng/mL; P <0.001).


CONCLUSIONS
The 5-year survival rate in patients with beta thalassemia with heart failure was greater than previously reported. There are clinical characteristics that may make patients more likely to develop left- or right-sided heart failure.
Kremastinos
D T
DT
2nd Department of Cardiology, Onassis Cardiac Surgery Center, Athens, Greece
Tsetsos
G A
GA
Tsiapras
D P
DP
Karavolias
G K
GK
Ladis
V A
VA
Kattamis
C A
CA
D000328:Adult; D000959:Antihypertensive Agents / Q000627:therapeutic use; D004232:Diuretics / Q000627:therapeutic use; D015150:Echocardiography, Doppler; D015716:Electrocardiography, Ambulatory; D004656:Enalapril / Q000627:therapeutic use; D005260:Female; D005500:Follow-Up Studies; D005665:Furosemide / Q000627:therapeutic use; D006333:Heart Failure / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology* / Q000401:mortality*; D006801:Humans; D007502:Iron Chelating Agents / Q000627:therapeutic use; D008297:Male; D011446:Prospective Studies; D018709:Statistics, Nonparametric; D016019:Survival Analysis; D016896:Treatment Outcome; D017086:beta-Thalassemia / Q000150:complications* / Q000188:drug therapy / Q000401:mortality*
D000959:Antihypertensive Agents; D004232:Diuretics; D007502:Iron Chelating Agents; D004656:Enalapril; D005665:Furosemide