What is the role of lab studies in the workup of pulmonary arterial hypertension (PAH)?

Updated: Apr 25, 2018
  • Author: Kristin E Schwab, MD; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
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A complete blood (CBC) count, biochemistry panel, prothrombin time (PT), and activated partial thromboplastin time (aPTT) should be obtained at baseline. Arterial blood gas determinations should be performed to assess for hypoxemia.

Collagen-vascular disease screening can be performed by measuring antinuclear antibody (ANA) levels, as well as checking for rheumatoid factor (RF) and antineutrophil cytoplasmic antibody (ANCA). When there is clinical suspicion for scleroderma, anti-Scl-70, anticentromere, and anti-U1-RNP antibodies can also be checked.

Liver function tests, as well as markers of synthetic function (ie, albumin, international normalized ratio [INR]), and platelet levels may indicate liver disease and/or portal hypertension.

Brain natriuretic peptide (BNP of NT-proBNP) should be performed on appropriate patients.

HIV testing, hepatitis serology tests, and urine toxicology screening should also be considered.

In patients at risk for heritable pulmonary arterial hypertension (PAH), screening for gene mutations such as BMPR2 also may be considered.

Iron studies may also be indicated in at risk patients with PAH, as a study by Soon et al found a high prevalence of iron deficiency in patients with PAH. [20] It was found to be significantly more common in patients with idiopathic PAH (IPAH) than in those with CTEPH.

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