Pulmonary Hypertention Association With Sickle Cell Anaemia

PULMONARY HYPERTENTION ASSOCIATION WITH SICKLE CELL ANAEMIA
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PULMONARY HYPERTENSION ASSOCIATION WITH SICKLE CELL ANAEMIA
Introduction
Pulmonary Hypertension also abbreviated as PH is very rare but equally fatal. The disease is characterized by excessive blood pressure in the lungs after damage to the pulmonary arteries. The pulmonary arteries carry blood to the lungs from the right heart ventricle. In PH the condition causes the arteries to be narrowed and subsequently blocked. As a result of this narrow and blocked pulmonary arteries, the heart must operate under stress to ensure that blood is pumped. The indirect effect of this is an enlarged and weak organ – which may sometimes lead to the failure or death of the right heart. Many of the PH cases are, however, associated with other underlying conditions. Any disease which interferes with the normal blood flow is a predisposing factor to pulmonary hypertension (PH). These factors include Sickle Cell Anemia. Sickle Cell Disease (SCD) is a condition that encompasses a group of inherited blood cell disorders. SCD is characterized by abnormal hemoglobin – a protein that is responsible for the transportation of oxygen throughout the body. Hemoglobin is found explicitly in the red blood cells.
Method
The research to establish pulmonary hypertension associated with sickle cell disease was carried out through a critical analysis of the underlying causes of the disease in children. A careful review of other studies and literature on the subject was considered, and the below results obtained.
Results
TABLE 1
Summary of Reports of Echocardiographic Screening in Children and Adults with Sickle Cell Disease
TRV ≥ 2.5
TRV ≥ 3
Reported significant correlations
Author
Location
Ages (years)
Screened n
n
Percentage
n
Percentage
Morris et al. [58]
CA
7–17
60
15
25
n.r.
Young et al. [50]
MI
0–18
36
24a
66
n.r.
Low Hb, Plt; high retic, ACS
Nelson et al....