Congestive pulmonary arterial hypertension (PAH) is one of the most common complications of left to right shunt congenital heart disease. With the pulmonary artery pressure increasing, the shunt direction will reverse, eventually develop into Eisenmenger syndrome, and affect the patients' life. Studies in recent years have found that angiotensin -(1-7) and brain natriuretic peptide can adversely affect renin-angiotensin aldosterone system (RAAS), stromal cell derived factor can delay the pulmonary vascular remodeling, von Willebrand factor marks the pulmonary vascular endothelial function impaired, microRNA causes damage and homocysteine play a protective role in pulmonary vascular endothelial function. The RAAS activation, pulmonary vascular remodeling and endothelial dysfunction are related to the formation and development of PAH. We produced a comprehensive literature review about serological indexes in congestive PAH in this review.
