Pulmonary Artery Hypertension in Children Living with Sickle Cell Anaemia

Abstract

Pulmonary artery hypertension (PAH) ultimately leads to straining of the right ventricle and increases the risk of heart failure in
affected patients. Its clinical presentation is similar to that of many other diseases thus delaying the diagnosis until the disease is far
advanced. It remains one of the leading causes of death in adults with sickle cell anaemia (SCA) worldwide. It confers a high risk
of death with two-year mortality rates as high as 40-50% even at modest elevation of pulmonary artery pressure. Median survival
age after detection of the disease is said to be 25.6-months. Early detection of elevated pulmonary artery pressure in childhood and
appropriate intervention by optimization of anti-haemolytic therapy may prevent the progression of this complication. The current
writes up is a review of literatures on pulmonary artery hypertension among children with sickle cell anaemia. This will give information
which will aid early diagnosis and treatment of pulmonary artery hypertension among children with sickle cell anaemia. This
will ultimately improve the quality of life of children with sickle cell anaemia and reduce morbidity and mortality from the disease
in adults and children living with sickle cell anaemia.