Echocardiographic findings in patients with sickle cell disease

Abstract

Pulmonary hypertension is a complication of sickle cell disease that is associated with increased mortality. Whether this complication is associated with hemolysis has been questioned. Systolic pulmonary artery blood pressure can be estimated from echocardiography-determined tricuspid regurgitation velocity (TRV). A velocity of 2.5 m/s or higher suggests possible pulmonary hypertension. A retrospective review of hospital records from adult patients with sickle cell disease undergoing echocardiography in 2006 and 2007 was performed at a tertiary level hospital. Echocardiographic, demographic, and clinical laboratory data were collected. Echocardiographic results were available for 105 adult sickle cell patients. Of these, 62 (59%) had a TRV ≥2.5 m/s and 24 (22.8%) had a TRV ≥3.0 m/s. Mitral valve regurgitation was observed in 44% and left ventricular abnormalities (defined by either hypertrophy or dilation) in 28% of cases. Elevated TRV had independent and significant associations with greater age, higher serum lactate dehydrogenase (LDH) concentration, and lower hemoglobin concentration. We confirmed that elevated TRV is common among hospital-based adults with sickle cell disease. Significant, independent associations were found with both elevated LDH concentration and degree of anemia, suggesting that hemolytic and other mechanisms contribute to pulmonary hypertension in patients with sickle cell disease.

Altered Left Ventricular Twist Is Associated with Clinical Severity in Adults and Adolescents with Homozygous Sickle Cell Anemia

“…This is consistent with previous work in this population. 24,25 However, contrary to our hypothesis, there was no difference between subjects with and without SCA when myocardial strain was evaluated using twodimensional strain. Ejection fraction was preserved in all patients, and there was no significant difference in two-dimensional strain A lateral RV (cm/sec) 9.8 6 0.7 9.5 6 0.5 .05…”

Intravascular hemolysis and the pathophysiology of sickle cell disease

“…Compounding the effects of these NO-and arginine-scavenging pathways, lysed red cells release asymmetric dimethylarginine, an endogenous inhibitor of NOS (18). A role for intravascular hemolysis in promoting endothelial dysfunction was bolstered by epidemiological cohort studies linking laboratory biomarkers of the intensity of hemolytic anemia and risk of developing specific complications of SCD, including PH (19), cutaneous leg ulceration (20,21), priapism (22), stroke (23), and, recently, pro- hyperhemolysis (19,39,44,(48)(49)(50)(51)(52)(53)(54)(55)(56). PH, either screened for with TRV or definitively ascertained by right heart catheterization, is closely associated with the intensity of hemolysis and mortality (discussed below).…”

“…Many of these studies also evaluated the relationship between estimated or directly measured pulmonary artery pressures and reduced exercise capacity and/or risk of death. These studies include the NIH-PH (19), Duke (60), UNC (49), MSH (39), CSSCD (54), PUSH (50,61), and Walk-PHASST (51) cohorts, a Greek cohort (62), and a recent 656-SCD-patient echocardiographic screening study in Créteil, France (63). The analysis of more than 600 screening patients in both the Walk-PHASST and Créteil cohorts found similar associations between indices of hemolytic anemia, high TRV, and risk of death (51,63).…”

Assessment of Ventricular Function in Adults with Sickle Cell Disease: Role of Two-Dimensional Speckle-Tracking Strain

“…[13][14][15] Both left ventricular (LV) systolic and diastolic dysfunction may occur in adults and children with SCD, but LV systolic dysfunction is not common and is usually observed in older patients and in those with associated disorders such as systemic hypertension and renal failure. 13,16 Although most studies in adults show abnormalities of LV diastolic function, 3,[17][18][19] LV ejection fraction (LVEF) and/or other conventional indices of systolic function have usually been reported to remain normal. 20 It is well known that conventional indices of LV systolic function are limited in identifying and measuring early ventricular dysfunction because of the volume-expanded highcardiac output state present in chronic anemia.…”