Rightward shift of the QRS axis in idiopathic pleuroparenchymal fibroelastosis

Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare interstitial lung disease characterized by upper-lobe–predominant fibrosis and thoracic cage deformity. However, its electrocardiographic (ECG) features remain poorly defined. We retrospectively analyzed ECG, echocardiography, and chest computed tomography (CT) findings in 20 patients with IPPFE and 18 with idiopathic pulmonary fibrosis (IPF). Patients with IPPFE showed a more rightward QRS axis than those with IPF (median +70.5° [interquartile range (IQR) 55.3–77.3] vs. +32.5° [IQR 1.3–63.5], p = 0.007). The PR interval was shorter in IPPFE but within normal limits. CT showed greater upward displacement of the pulmonary hila. No significant correlations were found between QRS axis and hilar elevation, flat chest index, or echocardiographic indices. These findings suggest that the rightward QRS axis shift may reflect altered cardiac orientation associated with thoracic deformity rather than overt pulmonary hypertension. ECG abnormalities may aid recognition of IPPFE, particularly when radiological findings are subtle.