Key result
Pulmonary hypertension is linked to ~11-fold higher mortality in sickle cell disease.
Why the study?
With prolonged survival in sickle cell disease, cardiovascular complications including systemic vasculopathy, pulmonary hypertension, left ventricular diastolic dysfunction, and sudden death are increasingly evident and require further cardiac investigation to recognize and treat high-risk patients.
As survival improves in sickle cell disease, progressive pulmonary hypertension and left ventricular diastolic dysfunction have emerged as major independent predictors of reduced exercise capacity and mortality, necessitating targeted screening with echocardiography, NT-proBNP, and confirmatory right heart catheterization.
Supports echocardiographic screening in sickle cell disease; leaves open whether targeted interventions improve survival.
Sickle cell disease is characterized by recurrent episodes of ischemia-reperfusion injury to multiple vital organ systems and a chronic hemolytic anemia, both contributing to progressive organ dysfunction. The introduction of treatments that induce protective fetal hemoglobin and reduce infectious complications has greatly prolonged survival. However, with increased longevity, cardiovascular complications are increasingly evident, with the notable development of a progressive proliferative systemic vasculopathy, pulmonary hypertension (PH) and left ventricular diastolic dysfunction. Pulmonary hypertension is reported in autopsy studies and numerous clinical studies have shown that increased pulmonary pressures are an important risk marker for mortality in these patients. In epidemiological studies, the development of PH is associated with intravascular hemolysis, cutaneous leg ulceration, renal insufficiency, iron overload and liver dysfunction. Chronic anemia in sickle cell disease results in cardiac chamber dilation and a compensatory increase in left ventricular mass. This is often accompanied by left ventricular diastolic dysfunction which has also been a strong independent predictor of mortality patients with sickle cell disease. Both PH and diastolic dysfunction are associated with marked abnormalities in exercise capacity in these patients. Sudden death is an increasingly recognized problem and further cardiac investigations are necessary to recognize and treat high-risk patients.
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Gladwin et al. (2012) conducted a review in Sickle cell disease. Pulmonary hypertension and left ventricular diastolic dysfunction in sickle cell disease vs. Sickle cell disease without pulmonary hypertension or diastolic dysfunction was evaluated on All-cause mortality. Pulmonary hypertension (TRV ≥3.0 m/s, RR 10.6) and left ventricular diastolic dysfunction (RR 4.8) are common in sickle cell disease and serve as strong independent predictors of mortality.
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