Abnormal right ventricular to pulmonary artery coupling in sickle cell disease

What is it about?

Introduction: Sickle cell disease (SCD) is associated with an increased risk of pulmonary hypertension which can be suspected by echocardiography. The latter provides information on right ventriclular (RV) function and the RV to pulmonary artery (PA) coupling, which can be approached non-invasively by the ratio between the Tricuspid Annular Plane Systolic Excursion (TAPSE) and systolic Pulmonary Arterial Pressure (PAPs). Currently, there are no data on the TAPSE/PAPs ratio in SCD. Objective: The aim of this study is to describe the RV/PA coupling in homozygous sickle cell patients, compared with healthy subjects. Methods: This retrospective study was carried out on the cohort of SCD followed at our institution. We analyzed anthropometric, biological, clinical, hematological and echocardiographic data in a group of healthy subjects versus homozygous sickle cell patients. Results: Compared with normal subjects, patients with SCD had higher PAPs (p<0.001), larger right atrial surface (p=0.003) and a larger S-wave (p=0.02) at similar TAPSE (p=0.23). The TAPSE/PAPs ratio is lower in patients with SCD (p=0.002) (Figure 1). Conclusions: RV to PA coupling is impaired in patients with homozygous sickle cell, due to a higher PAPs compared with normal subjects, suggesting a maladaptation of the RV performance to an increase in afterload. This may suggest the presence of an intrinsic myocardial damage in homozygous sickle cell disease. Figure 1

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