enalapril and beta-Thalassemia

Cardiac complications are the major cause of death in patients with beta-thalassemia major. The purpose of this study was to assess the impact of long-term treatment with the angiotensin-converting enzyme inhibitor enalapril on left ventricular (LV) performance, with an emphasis on diastolic LV function because diastolic dysfunction has been found to be an early event in an asymptomatic thalassemic population with only mild impairment of LV systolic function.. We used echocardiography to study the impact of treatment with oral enalapril on the evolution of standard M-mode and Doppler indices, along with a recently introduced Doppler index of combined systolic and diastolic LV performance.. Patients were found to have significantly increased LV end-diastolic dimensions (LVEDD), LV end-systolic dimensions (LVESD), and left atrial dimensions and decreased LV fractional shortening (LVFS) compared with controls. After treatment with enalapril, LVESD decreased from 3.58 +/- 0.3 cm to 3.23 +/- 0.4 cm (P <.01) and LVFS increased from 32.6% +/- 4.0% to 38.0% +/- 3.1% (P <.001). Patients at baseline were found to have a significantly higher E-wave velocity, E/A ratio, and Doppler index compared with controls. The E-wave deceleration time was significantly shorter compared with that of controls. After treatment with enalapril, the E/A ratio decreased from 2.10 +/- 0.42 to 1.50 +/- 0.30 (P <.05), E-wave deceleration time increased from 0.12 +/- 0.02 seconds to 0.15 +/- 0.03 seconds (P <.01), and the Doppler index decreased from 0.46 +/- 0.10 to 0.37 +/- 0.14 (P <.05).. Enalapril was well tolerated in asymptomatic or minimally symptomatic patients with LV dysfunction resulting from beta-thalassemia major. Echocardiographically we demonstrated significant improvement in LV systolic and diastolic function. Whether this translates to improved long-term prognosis and survival remains to be further evaluated.

Topics: Adult; Angiotensin-Converting Enzyme Inhibitors; beta-Thalassemia; Blood Transfusion; Diastole; Echocardiography, Doppler; Enalapril; Female; Heart Ventricles; Humans; Hypertrophy, Left Ventricular; Male; Myocardial Contraction; Prognosis; Systole; Ventricular Function, Left

To evaluate the survival of patients with beta thalassemia and heart failure who were treated with iron chelation therapy.. 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.. 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).. 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.

Topics: Adult; Antihypertensive Agents; beta-Thalassemia; Diuretics; Echocardiography, Doppler; Electrocardiography, Ambulatory; Enalapril; Female; Follow-Up Studies; Furosemide; Heart Failure; Humans; Iron Chelating Agents; Male; Prospective Studies; Statistics, Nonparametric; Survival Analysis; Treatment Outcome