Abstract

Pulmonary hypertension is an emerging complication of sickle cell anaemia with associated increased risk of mortality. In order to evaluate the clinical and electrocardiographic findings in adult sickle-cell patients with pulmonary hypertension, a cross sectional study was conducted on sixty two sickle cell anaemia patients and sixty two age and sex matched normal controls. Elevated pulmonary artery pressures (PAP), defined by PAP ≥ 30 mm Hg on echocardiography, was demonstrated in 41.9% of patients with sickle cell anaemia and in 3.2% of the controls; χ 2 = 26.571, P < 0.001. Right ventricular hypertrophy, increased P-wave duration, QTc interval, and QTc dispersion were significantly associated with pulmonary hypertension. Significant correlation was found between mean PAP and (1) Frequency of crisis (Spearman correlation = 0.320; P = 0.011), (2) body mass index (Pearson's correlation = −0.297; P = 0.019), and (3) QTc interval (Pearson's correlation 0.261; P = 0.040). Pulmonary hypertension in adult sickle anaemia patients is associated with electrocardiographic evidence of right ventricular hypertrophy, and correlates significantly with frequency of vaso-occlusive crisis, and QTc interval. The observations by this study tend to suggest that these parameters could be useful for early detection and prevention of pulmonary hypertension in patients with sickle cell anaemia.

Highlights

  • Pulmonary hypertension is an increasingly recognized complication of sickle-cell anaemia and a risk factor for early death [1,2,3,4]

  • An initial study in Howard University, USA, using echocardiographic assessment of tricuspid valve regurgitant jet velocity ≥2.5 m/sec as diagnostic criteria, demonstrated pulmonary hypertension in 32% of adult sickle-cell patients, and the prevalence appeared to increase with age of the patients [7]

  • Mean pulmonary artery pressure was significantly higher in patients compared with controls

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Summary

Introduction

Pulmonary hypertension is an increasingly recognized complication of sickle-cell anaemia and a risk factor for early death [1,2,3,4]. An initial study in Howard University, USA, using echocardiographic assessment of tricuspid valve regurgitant jet velocity ≥2.5 m/sec as diagnostic criteria, demonstrated pulmonary hypertension in 32% of adult sickle-cell patients, and the prevalence appeared to increase with age of the patients [7]. In patients between 40 and years old, the prevalence was 40% and increased to 55–60% by age and above. Other studies have documented prevalence rates between 20 and 40% [4, 8,9,10]

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