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Pulmonary hypertension in sickle cell disease:

Pulmonary hypertension is one of those chronic complications of sickle cell disease that you only hear on ward rounds or clinic visits headed by senior clinicians. The diagnostic criterion is itself complex; we shall gain an insight into this elusive, but sometimes fatal complication.

Upon right heart catheterization (RHC) for whatever indication, in a patient with sickle cell disease, if there’s an elevation of the resting mean pulmonary arterial pressure, that is, ≥25 mm Hg, we make a diagnosis of pulmonary hypertension.

An increase in the resting mean pulmonary arterial pressure can be due to various causes: sickle cell disease is one of them.

Sickle cell disease leads to chronic haemolytic anaemia. It leads to various vascular changes in the pulmonary vasculature leading to pulmonary artery hypertension, and this occurs in up to 10% of patients with sickle cell anaemia. Pulmonary artery hypertension per se accounts for about 40% to 50% of all cases of hypertension in the pulmonary vasculature.

The changes in the pulmonary vasculature may in one way or the other, also result in pulmonary venous hypertension, which is always associated with elevation of the pulmonary capillary wedge pressure ≥15 mm Hg and left ventricular dysfunction.

Sickle cell disease can increase the risk of developing chronic lung disease, chronic thromboembolic disease, both of which can be precursors for pulmonary hypertension. In many cases, however, the exact cause cannot be reliably identified.

Patients with sickle cell disease who have such hypertension may present with shortness of breath on routine activity, easy tiredness, chest pain, palpitations, lower limb swelling; syncope and lethargy. But as you can observe, these symptoms can be due to severe anaemia or heart failure. History taking and physical examination that is focused is crucial.

When there’s a suspicion of such hypertension, it is advisable to do echocardiography. It estimates pulmonary artery pressure using the tricuspid regurgitant jet velocity. Then confirm the diagnosis by right heart catheterization where the pulmonary arterial pressure and vaso-reactivity are measured.

Related article:

A brief look at multisystem organ failure as an acute complication in sickle cell disease; recommendations included.

Pulmonary hypertension increases the mortality risk among people with sickle cell disease. Read more about the condition here. Let’s look at what the expert panel had to say about the recommendations of its management.

  1. If people with sickle cell disease have symptoms or signs suggestive of pulmonary hypertension, refer them for echocardiography.
  2. For people with an elevated tricuspid regurgitant jet velocity ≥2.5m/sec, consult an expert in pulmonary hypertension to guide further assessment and management. It includes right heart catheterization and consideration of specific hypertension therapy.

Management of sickle cell disease requires a multidisciplinary approach, but first, it requires a primary care provider who pays attention to details. It is not just filling folic acid, Pen V and analgesics at every clinic visit.

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MBChB (MUK), Graduate Fellow, Department of Physiology, Makerere University Founder and Content Creator Peer reviewer, Associate Editor

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