Approximately 11% of patients with sickle cell anemia will have both pulmonary hypertension and diastolic dysfucnction. The presence of either complication can increase the risk of mortality for the patient.
Parameters:
(1) tricuspid regurgitant jet velocity (TRJV) in m/s
(2) E/A ratio on echocardiography
A tricuspid regurgitant jet velocity >= 2.5 m/s was used to diagnose pulmonary hypertension. Machado noted that a TRJV >= 3.0 m/s was associate with a faster decline.
A low E/A ratio (< 1.0) was used to define diastolic dysfunction.
Parameter
Finding
Points
tricuspid regurgitant velocity
< 2.5 m/s
0
>= 2.5 m/s
1
E/A ratio
>= 1.0
0
< 1.0
1
total risk score =
= SUM(points for the 2 parameters)
Interpretation:
• minimum risk score: 0
• maximum risk score: 2
• A risk score of 1 or 2 indicates a high risk for mortality (50% at 4 years).
• A risk score of 1 indicates a low risk for mortality (about 4% at 4 years).
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