Cardiolipin (CL), the signature phospholipid of mitochondrial membranes, is 
crucial for both mitochondrial function and cellular processes outside of the 
mitochondria. The importance of CL in cardiovascular health is underscored by 
the life-threatening genetic disorder Barth syndrome (BTHS), which manifests 
clinically as cardiomyopathy, skeletal myopathy, neutropenia, and growth 
retardation. BTHS is caused by mutations in the gene encoding tafazzin, the 
transacylase that carries out the second CL remodeling step. In addition to 
BTHS, CL is linked to other cardiovascular diseases (CVDs), including 
cardiomyopathy, atherosclerosis, myocardial ischemia-reperfusion injury, heart 
failure, and Tangier disease. The link between CL and CVD may possibly be 
explained by the physiological roles of CL in pathways that are 
cardioprotective, including mitochondrial bioenergetics, autophagy/mitophagy, 
and mitogen activated protein kinase (MAPK) pathways. In this review, we focus 
on the role of CL in the pathogenesis of CVD as well as the molecular mechanisms 
that may link CL functions to cardiovascular health.