TY - JOUR
T1 - Clinical management of cardiovascular complications in patients with thalassaemia major
T2 - A large observational multicenter study
AU - Derchi, Giorgio
AU - Formisano, Francesco
AU - Balocco, Manuela
AU - Galanello, Renzo
AU - Bina, Patrizio
AU - Dess, Carlo
AU - Piga, Antonio
AU - Donato, Guido
AU - Cappellini, Maria Domenica
AU - Cassinerio, Elena
AU - Quarta, Gianni
AU - Melpignano, Angela
AU - Forni, Gian Luca
PY - 2011/3
Y1 - 2011/3
N2 - Aims To determine the clinical management of cardiovascular complications, and the extent of cardiac left ventricular (LV) involvement, in a large cohort of homogenously treated patients with thalassaemia major. Methods and results Participants were ®, an Internet-shared database. Patients were considered to have evidence of cardiovascular disease if at least one cardiovascular drug was recorded in their file. The majority of patients (422 of 524; 80.5) had not taken any cardiovascular drug. Among those who had angiotensin-converting enzyme-inhibitors were the most commonly used (81 patients) and these were used by significantly more males than females (P <0.01). Patients in whom cardiovascular drugs were prescribed showed evidence of cardiac structural and/or functional abnormalities, inasmuch as fractional shortening and ejection fraction were significantly lower (31.3 vs. 35 and 54.4 vs. 60.6; both P <0.001) and LV end-diastolic diameter index was significantly higher (32.9 vs. 31.8; P 0.004). Interestingly, when compared with patients in whom cardiovascular drug therapy was not deemed necessary, transfusion period was longer in treated patients (26.2 vs. 24.5 years; P 0.002). Conclusion Approximately 19 of regularly transfused and chelated thalassaemia major patients need cardiovascular drug therapy. This subgroup is characterized by a dilated and mildly hypokinetic left ventricle when compared with the majority of thalassaemia major patients, who do not need any cardioactive drug. These data underscore the importance of careful evaluation of cardiac functional status in patients with thalassaemia major. Moreover, this database may serve as a clinically useful reference grid for echocardiograph values in this patient population.
AB - Aims To determine the clinical management of cardiovascular complications, and the extent of cardiac left ventricular (LV) involvement, in a large cohort of homogenously treated patients with thalassaemia major. Methods and results Participants were ®, an Internet-shared database. Patients were considered to have evidence of cardiovascular disease if at least one cardiovascular drug was recorded in their file. The majority of patients (422 of 524; 80.5) had not taken any cardiovascular drug. Among those who had angiotensin-converting enzyme-inhibitors were the most commonly used (81 patients) and these were used by significantly more males than females (P <0.01). Patients in whom cardiovascular drugs were prescribed showed evidence of cardiac structural and/or functional abnormalities, inasmuch as fractional shortening and ejection fraction were significantly lower (31.3 vs. 35 and 54.4 vs. 60.6; both P <0.001) and LV end-diastolic diameter index was significantly higher (32.9 vs. 31.8; P 0.004). Interestingly, when compared with patients in whom cardiovascular drug therapy was not deemed necessary, transfusion period was longer in treated patients (26.2 vs. 24.5 years; P 0.002). Conclusion Approximately 19 of regularly transfused and chelated thalassaemia major patients need cardiovascular drug therapy. This subgroup is characterized by a dilated and mildly hypokinetic left ventricle when compared with the majority of thalassaemia major patients, who do not need any cardioactive drug. These data underscore the importance of careful evaluation of cardiac functional status in patients with thalassaemia major. Moreover, this database may serve as a clinically useful reference grid for echocardiograph values in this patient population.
KW - Cardiac dysfunction
KW - LV ejection fraction
KW - Thalassaemia major
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U2 - 10.1093/ejechocard/jeq190
DO - 10.1093/ejechocard/jeq190
M3 - Article
C2 - 21278193
AN - SCOPUS:79952586839
VL - 12
SP - 242
EP - 246
JO - European Journal of Echocardiography
JF - European Journal of Echocardiography
SN - 1525-2167
IS - 3
ER -