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[Left ventricule function and sickle-cell anemia. Echocardiographic study]
G Estrade1, O Poitrineau, F Bernasconi
1Service de cardiologie, centre hospitalier régional de Fort-de-France.
Insights
Patients with sickle cell disease (SS) show enlarged cardiac chambers and increased cardiac output, indicative of an "anemic heart." This contrasts with normal subjects, highlighting significant cardiovascular changes in SS disease.
Area of Science:
- Cardiology
- Hematology
- Medical Imaging
Context:
- Sickle cell disease (SS) is a genetic blood disorder with known systemic complications.
- Cardiac involvement in SS disease is increasingly recognized but requires detailed characterization.
- Echocardiography is a key non-invasive tool for assessing cardiac structure and function.
Purpose:
- To investigate cardiac chamber dimensions and left ventricular function in patients with homozygous sickle cell disease (SS) and sickle cell trait (AS, SC, BS).
- To compare echocardiographic parameters between SS disease patients, sickle cell trait patients, and healthy controls.
- To identify specific cardiac adaptations and potential pathologies associated with SS disease.
Summary:
- Echocardiography revealed significantly larger left atrial, left ventricular, and right ventricular dimensions in SS disease patients compared to controls.
- Patients with SS disease exhibited higher indexed myocardial surfaces, increased end-diastolic left ventricular volumes, and elevated cardiac index and stroke volume.
- Peripheral resistances were lower in SS disease patients, and velocities of circumferential fiber shortening were reduced, consistent with an "anemic heart" phenotype in two-thirds of patients.
Impact:
- Findings demonstrate significant cardiac remodeling and altered hemodynamics in sickle cell disease, contributing to the understanding of its cardiovascular burden.
- The identification of an "anemic heart" phenotype in a majority of SS patients underscores the need for cardiac monitoring and management.
- This study provides valuable echocardiographic data that can inform clinical practice and future research on cardiovascular complications in sickle cell disease.
Abstract:
The dimensions of the cardiac chambers and left ventricular function were studied by echocardiography in 40 patients with homozygous sickle cell disease (SS) and 25 patients with heterozygous sickle cell trait (12 AS, 10 SC, 3 BS) and compared with the same parameters in 30 normal subjects. Patients with homozygous SS disease had significantly larger left atrial (32.2 +/- 6.47 mm vs 26.62 +/- 5.64 mm; p less than 0.001), left ventricular (46.4 +/- 8.49 mm vs 39.97 +/- 5.29 mm; p less than 0.001) and right ventricular (17.95 +/- 5.08 mm vs 15.5 +/- 3.17 mm; p less than 0.02) dimensions: they also had higher indexed myocardial surfaces (11.48 +/- 2.36 cm2/m2 vs 8.21 +/- 1.97 cm2/m2; p less than 0.001) and indexed end diastolic left ventricular volumes (101.84 +/- 22.74 ml/m2 vs 65.05 +/- 10.81 ml/m2; p less than 0.001), a higher cardiac index (4.77 +/- 1.33 l/mn/m2 vs 3.58 +/- 1.08 l/mn/m2; p less than 0.001) and stroke volume (71.77 +/- 32.86 ml vs 49.56 +/- 13.39 ml; p less than 0.001). Peripheral resistances were significantly lower (1341 +/- 469 vs 1626 +/- 368 dynes/s/cm-5; p less than 0.01) as were the velocities of circumferential fibre shortening (VCF) (1.11 +/- 0.20 circ/s vs 1.28 +/- 0.24 circ/s; p less than 0.01). Two thirds of homozygous SS disease patients (27/40) had appearances of an anaemic heart with eccentric left ventricular hypertrophy, normal left ventricular systolic function, an increased cardiac index (5.01 +/- 1.19 l/mn/m2) and low peripheral arterial resistances.(ABSTRACT TRUNCATED AT 250 WORDS)