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Pulmonary Hypertension in ß-Thalassemia
First Department of Internal Medicine, University of Athens Medical School, "Laiko" General Hospital, Athens, Greece
Address for correspondence: Athanasios Aessopos, M.D., Ph.D., First Department of Internal Medicine, "Laiko" General Hospital, 17 Aghiou Thoma St., Athens 115 27, Greece. Voice: +30210-777-1161; fax: +30210-777-1161. aaisopos{at}cc.uoa.gr; dfarm1{at}panafonet.gr
Cardiac involvement represents the leading cause of mortality in both forms of ß-thalassemia, namely, thalassemia major (TM) and thalassemia intermedia (TI), and pulmonary hypertension (PHT) is part of the cardiopulmonary complications of the disease. PHT was initially documented in a small group of TI patients with right heart failure. In a subsequent study of a large 110-patient series, aged 32.5 ± 11.4 years, age-related PHT was encountered in nearly 60% of cases, having caused right heart failure in six of them; interestingly, all patients had preserved left ventricular systolic function. Conflicted evidence, however, existed with respect to the development of PHT in heterogeneously treated and young TM populations. To resolve this discrepancy, a recent study compared cardiac disease between two large aged-matched groups of TM (n = 131) and TI (n = 74) patients, both treated uniformly in the currently accepted manner (regular transfusion and chelation therapy in TM, absence of any particular treatment in TI); well-treated TM patients, in contrast to TI patients, did not develop PHT, while systolic left ventricular dysfunction was present only in TM cases. PHT in ß-thalassemia results from a rather complex pathophysiology, in which chronic tissue hypoxia seems to hold a key role. Although both forms of the disease share a common molecular background, the diverse severity of the genetic defect and of the resulting clinical phenotype require a different therapeutic approach. Regular lifelong therapy in TM patients eliminates chronic hypoxia, thereby preventing PHT, whereas the absence of systematic treatment in TI leads to a cascade of reactions that compensate for chronic anemia, but at the same time allow the development of PHT.
Key Words: thalassemia • pulmonary hypertension • heart failure • transfusions • iron chelation This article has been cited by other articles:
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