Clinical and Laboratory Predictors of Elevated TRV In Sickle Cell Anaemia

Jane S Afriyie-Mensah,Martin Adadey,Yvonne A Dei-Adomakoh,Audrey Forson,Thomas A Ndanu,Joseph K Acquaye,Krzysztof Roszkowski

doi:10.14302/issn.2372-6601.jhor-18-2139

Abstract

Pulmonary hypertension (PH) has become an increasingly recognized complication in sickle cell anaemia (SCA) and is a major cause of morbidity and mortality. Though the burden of SCA in sub-Saharan Africa is high, there is paucity of data on SCA-associated PH with little or no attention given to it in routine patient care. The current study therefore sought to determine the prevalence of PH and its associated risk factors among adult patients with SCA. This was a cross-sectional study involving 76 clinically stable, hydroxyurea-naive participants. We obtained socio-demographic and clinical history. Measurement of Tricuspid Regurgitant jet Velocity (TRV) was obtained via transthoracic echocardiography and lung function was assessed using spirometry and pulse oximetry. Other investigations were complete blood counts, free plasma haemoglobin, serum urea and creatinine. Twenty-five (32.9%) of study participants had elevated TRV (≥ 2.5m/s) on Doppler echocardiography, which was suggestive of raised pulmonary artery systolic pressure. There were significant associations between elevated TRV and steady-state haemoglobin (p < 0.001), blood urea level (p = 0.030), presence of chronic leg ulcers (p = 0.043) and oxygen saturation (p < 0.001) and these may be identifiable and modifiable risk factors for selective screening with echocardiography in a resource poor setting.

Highlights

Pulmonary involvement in sickle cell disease (SCD) has been shown to be a major factor affecting morbidity and mortality, being recognized as an important determinant of survival [1,2].This has become apparent in recent times as increased numbers of SCD patients reach adulthood due to improved survival strategies in childhood 3
Pulmonary hypertension (PH) complicated by right heart failure has emerged as an independent risk factor for morbidity and mortality among SCD patients, especially in those with homozygous disease
Assessment of tricuspid regurgitant jet velocity (TRV) via trans-thoracic echocardiography has been shown to predict the presence of PH in SCD patients and has prognostic significance

Summary

Introduction

Pulmonary involvement in sickle cell disease (SCD) has been shown to be a major factor affecting morbidity and mortality, being recognized as an important determinant of survival [1,2].This has become apparent in recent times as increased numbers of SCD patients reach adulthood due to improved survival strategies in childhood 3. The elevation in pulmonary artery systolic pressures (PASP) has been shown to be mild to moderate in sickle cell patients compared to those with idiopathic or scleroderma associated PH, yet the former presents with equivalent symptoms of severe dyspnoea, exercise limitation, hypoxaemia and progressive right heart failure 10. This spells out the need for screening and early identification of elevations in PASP using non-invasive means such as trans-thoracic echocardiography (ECHO) instead of invasive right heart catheterization (RHC), which is the diagnostic procedure.

Methods

Results

Conclusion