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

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Date

2011

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Sri Lanka Medical Association

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.

Description

Oral Presentation Abstract (OP36), 124th Annual Scientific Sessions, Sri Lanka Medical Association, 2011 Colombo, Sri Lanka

Keywords

thalassaemia

Citation

The Ceylon Medical Journal. 2011; 56(Supplement 1):33

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