JUNIPER PUBLISHERS - OPEN ACCESS JOURNAL OF CARDIOLOGY & CARDIOVASCULAR THERAPY Abstract Congenital heart disease is the most frequent malformation, many cases are complex cyanotic congenital heart defects characterized by low arterial oxygen saturation. Unrepaired intracardiac or extracardiac congenital heart defects which causes a persistent right to left shunt, they are responsible for hypoxemic syndrome, this is a multisystemic disorder affecting various organs and symstems: hematopoietic, central nervous, gastrointestinal, urinary, cardiovascular, Immune, musculoskeletal and endocrine. The objective this review is to explain the pathophysiological mechanisms adapted to the chronic hypoxia, the consequences derived from the affectation in the different systems. The diagnostic approach and management in centers specialized in adults with congenital heart disease. This review was made with articles search sources in databases PubMed, ScienceDirect, OVID, HINARI, S