Frequently asked question: How does sickle cell disease lead to pulmonary hypertension?

High blood pressure in the lungs, which is known as pulmonary hypertension, occurs when there is a shortage of nitric oxide (NO). The breakdown of sickle cells leads to more nitric oxide (NO) being bound to the increased amount of free hemoglobin in the bloodstream. This means you will have less active NO in your system. Normally, NO relaxes the wall of blood vessels, widening them to allow more blood to flow through. If NO levels are low, you could develop high blood pressure in the lungs, because the blood vessels in the lungs are unable to relax properly, leading to reduced blood flow through the lungs. This is called pulmonary hypertension. Other processes are also affected by NO shortage, causing extra complications in SCD patients.

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