Pulmonary arterial hypertension in thalassaemia patients- does splenectomy and disease severity increase the risk?

Abstract

INTRODUCTION AND OBJECTIVES: Pulmonary arterial hypertension (PAH) has been described in patients with thalassaemia. A causative association with splenectomy has been postulated. Our previous observations differed from this. We aimed to study these factors in our patients. METHODS: Pulmonary artery pressure (PAP) and other data of thaiassaemics attending the Thalassaemia Unit, Ragarna were studied using clinic records. PAP was measured using trans-thoracic 2D echocardiography. Four categories were separately analysed: thalassaemia major (TM) with splenectomy (A) and without (B), thalassaemia intermedia (TI) with splenectomy (C) and without (D). PAH was defined as PAP over 25 mm Hg. RESULTS: A total of 74 patients were studied, 60 (81%) with TM and 14 (19%) with TI. 25 of TMs (41%) and 10 of TIs (71%) had splenectomy (p <0.05). Mean ages (SD) of TM and TI were 20.71(8.4) and 32.6 (13.3) years respectively. Those of categories ABCD were 20.9 (7.4), 20.7 (9.1), 27 (9.6) and 46 (12.3) years. A total of 16 (21.6%) of all patients had PAH (95% CI13.8-32.3). This included 13 (21.7%) TM patients and three (21.4%) with TI (p-0.98). The prevalence of PAH was 17.1% (95 CI 8.4-33.5) in splenectomised thalassaemia patients and 25.6% (95% CI 16.5-43.8) in non splenectomised patients (p= 0.37), and in categories A,B,C,D were 16%, 23%, 20% and 25% respectively. Mean of PAP of splenectomised with PAH is 39 mmHg (SD=4.5) and in non splenectomised with PAH was 34.9 mmHg (SD=5.3) (p=0.8). CONCLUSIONS: PAH is a significant complication in patients with thalassaemia. However the severity of disease nor the splenectomy status were found to be causatively linked in our study.