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

Other Cardiovascular Complications

Myocardial Infarction

The prevalence of myocardial infarction (MI) in patients with SCD is low, ranging from 0% to 3% according to CMR studies (Table 1). In these patients, MI is mostly not related to atherosclerotic stenosis and myocardial necrosis is commonly attributed to microvascular alteration.[29] However, in the setting of acute sickling episode one could not exclude Type 2 MI in anaemic patients.[58] Finally, an increase of cardiac troponin in these SCD patients may have other causes such as right ventricular failure, renal insufficiency, and pulmonary embolism.[59]

The MI presentation is atypical occurring at a younger age in low cardiovascular risk profile population than classic atherosclerotic infarction.[58] The chest pain is usually diffuse and can be overshadowed by other musculoskeletal symptoms. Moreover, the non-ST-segment elevation syndrome is the predominant manifestation and the electrocardiogram modifications are non-specific. Thus, an acute MI can be missed easily with inappropriate decisions taken due to delays in the diagnosis. When a MI is clinically suspected, a coronary angiogram, a computed tomography angiography or a CMR can be used to confirm the working diagnosis (Figure 4, Supplementary material online, Videos S6–S9).[35] The management of these patients requires hospitalization in an intensive care unit with all the facilities to rule-in the correct diagnosis and treat accordingly.[58]

Venous Thromboembolism

SCD patients are at increased risk of venous thrombosis due to multiple factors including a chronic decrease in anticoagulant proteins of haemostasis and platelet activation.[60] In a large study involving 6237 patients with long-term follow-up, the incidence of venous thromboembolism was 11%, including pulmonary embolism in half of the cases.[45] This rate was about three times higher than in matched controls. Importantly, incident venous thromboembolism is associated with increased mortality. The rate of recurrence is high affecting one-third of the patients within 5 years.[45] Thereby, long-term anticoagulation should be systematically discussed in SCD patients with unprovoked venous thromboembolism.[60]

In a patient from an endemic area suffering from unprovoked venous thromboembolism the haemoglobin SC disease should be sought. In fact, one-third of patients are diagnosed with haemoglobin SC disease at an adult age following an acute clinical complication.[14] This genotype is known to confer a higher hypercoagulable state than homozygous S genotype.[61] Importantly, D-Dimer level is chronically elevated in SCD patients.[62] This biomarker should not be used in the diagnosis algorithm of venous thromboembolism in this population.