What are the complications of pulmonary arterial hypertension (PAH)?

Updated: Aug 06, 2021
  • Author: Kristin E Schwab, MD; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
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The most common—and feared—complication from pulmonary hypertension is right-sided heart failure. Progression to right-sided heart failure is part of the natural history of pulmonary arterial hypertension (PAH), and it is often present to some degree at the time of diagnosis. Registry and institutional data further implicate this as the most common cause of death in PAH patients, with data suggesting that 44-73% of PAH patients who die do so because of right-sided heart failure or sudden cardiac death. [16, 17]

In addition to right-sided heart failure, other causes of death include complications that arise because of dilatation of the pulmonary artery. These include pulmonary artery dissection and rupture, massive hemoptysis, and left main compression syndrome, where the left main coronary artery is compressed by the pulmonary artery trunk. [18] Hemoptysis is often secondary to a bronchial arterial source, as hypoxic vasoconstriction in the pulmonary arteries leads to collateralization and proliferation of the bronchial arteries. Supraventricular and, less commonly ventricular, arrhythmias may also occur, presumably triggered by right-sided heart disease.

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