Interstitial Lung Disease–Associated Cardiac Dysfunction: Beyond Pulmonary Hypertension

Interstitial lung diseases (ILDs) are a heterogeneous group of fibrotic and inflammatory lung disorders characterized by restrictive physiology and impaired gas exchange. However, emerging evidence suggests that ILD is a multisystem disorder with significant cardiovascular involvement extending beyond pulmonary hypertension (PH). Cardiac complications, including right ventricular (RV) remodeling, left ventricular (LV) dysfunction, heart failure with preserved ejection fraction (HFpEF), ischemic heart disease, and arrhythmias contribute substantially to morbidity and mortality. Pulmonary hypertension remains the most studied cardiac complication, with prevalence estimates ranging from 15% to 80% depending on disease severity and diagnostic modality. Chronic hypoxic vasoconstriction, capillary bed destruction, and endothelial dysfunction drive pulmonary vascular remodeling, leading to progressive RV pressure overload and maladaptive remodeling. However, recent data demonstrate a growing burden of LV dysfunction and heart failure. Large cohort studies show that heart failure independently predicts mortality in both IPF and non-IPF ILD populations. Global Burden of Disease analyses indicate a fivefold rise in ILD-associated heart failure prevalence over the past three decades, particularly among older adults. ILD has also been associated with increased risks of ischemic heart disease and myocardial infarction, independent of traditional cardiovascular risk factors. Arrhythmias—particularly atrial fibrillation and other atrial tachyarrhythmias—occur frequently and are associated with higher mortality, longer hospital stays, and increased healthcare costs. CTD-ILD populations demonstrate particularly elevated risks of heart failure, arrhythmias, and pulmonary arterial hypertension. Cardiac dysfunction—both right- and left-sided—substantially influences prognosis. Integrated cardiopulmonary assessment and early cardiovascular surveillance are essential to improve outcomes in ILD.