Chronic cyanosis and vascular function: implications for patients with cyanotic congenital heart disease.
In patients with cyanotic congenital heart disease, chronic hypoxaemia leads to important changes in blood vessel function and structure. Some of these alterations are maladaptive and probably contribute to impaired cardiopulmonary performance and an increased incidence of thrombotic and embolic events. Recent evidence suggests that deranged endothelial function, a sequel of chronic cyanosis, could be an important factor in the pathogenesis of cyanosis-associated cardiovascular risk. In this article, we discuss the physiological and mechanical consequences of compensatory erythrocytosis and possible pathophysiological mechanisms of vascular dysfunction in chronic cyanosis.
Cordina
Rachael L
RL
Department of Cardiology, Royal Prince Alfred Hospital and University of Sydney, Australia.
Celermajer
David S
DS
D001809:Blood Viscosity; D002908:Chronic Disease; D003490:Cyanosis / Q000209:etiology* / Q000503:physiopathology; D018450:Disease Progression; D004730:Endothelium, Vascular / Q000503:physiopathology*; D006330:Heart Defects, Congenital / Q000150:complications* / Q000503:physiopathology; D006801:Humans; D000860:Hypoxia / Q000097:blood / Q000150:complications* / Q000503:physiopathology; D008833:Microcirculation; D009131:Muscle, Smooth, Vascular / Q000503:physiopathology*; D011086:Polycythemia / Q000097:blood / Q000150:complications / Q000503:physiopathology; D014652:Vascular Diseases / Q000150:complications / Q000503:physiopathology*; D014661:Vasoconstriction / Q000502:physiology*