Intractable Rare Dis Res. 2016;5(2):70-75. (DOI: 10.5582/irdr.2016.01023)

Cardiac manifestations of idiopathic pulmonary fibrosis.

Agrawal A, Verma I, Shah V, Agarwal A, Sikachi RR


Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, parenchymal disease of the lung with an estimated prevalence of 14-43 per 100,000. Patient usually presents with coughing and exertional dyspnea, which can lead to acute respiratory failure. IPF has been associated with various co-morbidities such as lung cancer, emphysema, obstructive sleep apnea (OSA), GERD and multiple cardiovascular consequences. The cardiovascular manifestations of IPF include pulmonary hypertension, heart failure, coronary artery disease, cardiac arrhythmias & cardiac manifestations of drugs used to treat IPF. This review will outline evidence of the association between IPF and cardiovascular conditions and attempt to provide insights into the underlying pathophysiology. We also discuss the impact of these cardiovascular diseases on patients with IPF including increased morbidity and mortality.

KEYWORDS: Idiopathic pulmonary fibrosis, pulmonary hypertension, right heart failure, atrial fibrillation, arrhythmias, coronary artery disease

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