Cardiovascular Manifestations of Sickle Cell Disease

Nadjib Hammoudi; François Lionnet; Alban Redheuil; Gilles Montalescot


Eur Heart J. 2020;41(13):1365-1373. 

In This Article

Adaptive Cardiac Remodelling

Reduced blood oxygen-carrying capacity caused by decreased haemoglobin level induces an increased cardiac output as a compensatory mechanism in order to fulfil the body metabolic demand.[16] In an acute setting of haemoglobin loss, no structural heart remodelling is generally observed and tachycardia represents the main mechanism leading to cardiac output increase. In contrast, high-output state in SCD patients is related to a major increase in stroke volume associated with only a mild increase of heart rate.[16–18] Chronic anaemia induces an important peripheral vascular dilation which leads to decreased systemic resistances. The consequent low blood pressure[19] induces reflex stimulation of the sympathetic nervous and renin–angiotensin–aldosterone system and kidney salt and water retention thus leading to increase in plasma volume.[20] Volume overload leads to substantial modifications of LV structure as chronic increased cardiac preload and LV cavity dilation associated with compensatory eccentric myocardial hypertrophy in order to normalize LV wall systolic stress.[18,21] Cardiac remodelling, which involves also the left atrium and right cavities,[17,22] begins early in childhood and advances with age.[15,17]