Researchers recently discovered that patients with pulmonary arterial hypertension (PAH) related to congenital heart disease (CHD) and those with idiopathic PAH demonstrated a differential distribution pattern of serum lipids, as published in Respiratory Medicine.

Progressive narrowing and hardening of pulmonary blood vessels can occur in PAH. This increases vascular resistance, which hastens the arrival of right-sided heart failure.

In the last few years, the role of lipid dysregulation in the pathophysiology of PAH has gained increasing recognition, and metabolic syndrome has been identified as a risk factor for developing PAH. 

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“Although several basic and translational works highlight lipid abnormalities in PAH, no study has yet demonstrated a differential distribution of serum lipids in idiopathic PAH and CHD-PAH,” Chen and colleagues wrote and hence decided to investigate. 

Read more about PAH etiology 

They conducted a single-center retrospective study from June 2014 to December 2019 on patients with PAH (n=170) who were seen from the Department of Cardiovascular Medicine at the Second Xiangya Hospital of Central South University in Changsha, China. Among the 170 patients, 101 had CHD-PAH and 69 had idiopathic PAH. 

Blood samples were taken to test for the patients’ lipid biochemistry. The researchers also performed hemodynamic assessments by right heart catheterization. 

The results demonstrated that all types of cholesterol, including high-density lipoprotein cholesterol (HDL-C), low-density lipoprotein cholesterol (LDL-C), and total cholesterol (CHOL) were significantly lower in patients with idiopathic PAH in association with right heart function.

The researchers also discovered that LDL-C and CHOL were lower in patients with CHD-PAH, but these were not associated with heart failure or disease severity. 

“Based on our investigation, we conclude that HDL-C, LDL-C and CHOL correlate weak to moderate with heart function in [idiopathic PAH], while no such association was observed in CHD-PAH,” they said. 


Chen J, Rathinasabapathy A, Luo J, et al. Differential serum lipid distribution in IPAH and CHD-PAH patientsRespir Med. 2021;191. doi:10.1016/j.rmed.2021.106711