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Cardiac manifestations in sickle cell disease varies with patient genotype
Author(s) -
Guedeney Paul,
Lionnet François,
Ceccaldi Alexandre,
Stankovic Stojanovic Katia,
Cohen Ariel,
Mattioni Sarah,
Montalescot Gilles,
Bachmeyer Claude,
Isnard Richard,
Haymann JeanPhilippe,
Hammoudi Nadjib
Publication year - 2018
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/bjh.15238
Subject(s) - medicine , cardiology , ejection fraction , ventricle , diastole , cardiac index , left ventricular hypertrophy , cardiac output , blood pressure , heart failure
Summary Cardiac involvement is well characterized in sickle cell anaemia ( SCA ) but cardiac features associated with Haemoglobin SC (Hb SC ) disease are mostly unknown. We compared 60 patients with Hb SC disease (median age 31 years, 25 men) to 60 SCA patients and 60 controls matched for age and gender. Left ventricular ejection fraction ( LVEF ), left ventricle ( LV ) mass index ( LVM i), cardiac index and peak tricuspid regurgitation velocity ( TRV ) were measured using echocardiography. LV filling pressures were assessed using the ratio of early diastolic transmitral velocity to tissue velocity (E/e’ ratio). The LVM i was higher in both genotypes compared to controls. However, whereas LV hypertrophy was observed only in 3(5%) Hb SC patients, this condition was diagnosed in 27(45%) SCA patients ( P < 0·0001). While cardiac index and TRV were similar in Hb SC compared to controls, SCA patients exhibited elevated cardiac output and TRV . LVEF was similar in the 3 groups. However, both genotypes had a higher E/e’ ratio compared to controls. Cardiac involvement in SCA was related to anaemia and haemolysis, while LV diastolic dysfunction and TRV in Hb SC disease patients were related to arterial hypertension and overweight comorbidities. In summary, cardiac involvement and its determinants are different in Hb SC disease and SCA . Patient's genotype should be considered with regard to the echocardiographic indications and findings.