Giorgio Derchi

TL;DR: Data indicate that sildenafil citrate is effective in the treatment of PH in hemoglobinopathies that cannot be treated with alternative oral drugs and is well tolerated long-term at a daily dose of 100 mg, though studies including more patients may uncover toxicities and limitations of efficacy.

TL;DR: A multicenter cross-sectional study of 1309 Italian β-thalassemia patients with tricuspid-valve regurgitant jet velocity ≥ 32 m/s on transthoracic echocardiography further underwent right heart catheterization to confirm the diagnosis of pulmonary arterial hypertension.

TL;DR: These recommendations outline the definition, the follow-up and the treatment of the main heart complications in this group of patients and perform according to the American Cardiology College (ACC) and American Heart Association (AHA) guidelines.

TL;DR: In patients with thalassaemia the significant presence of myocardial fibrosis/necrosis seems to be a time-dependent process correlating with cardiovascular risk factors and cardiac complications.

TL;DR: Pulmonary hypertension (PHT), defined as Doppler peak systolic tricuspid gradient (TG) higher than 30 mmHg, develops in a high percentage of patients with β-thalassemia (10% in thalassemia major and greater than 50% in THA intermedia [TI]).