Cardiovascular Complications of Sickle Cell Disease

Cardiac magnetic resonance (CMR) has gained increasing clinical application in
cardiopulmonary diseases. Due to its 3-dimensional nature, CMR is considered the
gold-standard for quantifying left and right ventricular systolic function and size.
Additionally, its high tissue contrast allows for a detailed characterization of myocardial
tissue. Specifically, the use of techniques such as late gadolinium enhancement can be used
to detect the presence of tiny amounts of myocardial scar. Other techniques have been shown
to correlate strongly with myocardial iron content. Just as importantly, CMR perfusion
imaging can accurately quantify myocardial blood flow and can provide tremendous insight
into the function of the microcirculation. CMR's high spatial and temporal resolution, its
3-dimensional approach, its ability to characterize the tissue, and its ability to evaluate
the micro- and macro-circulation make it a comprehensive technique for the evaluation of
heart disease. Recently, one CMR study has already shown the presence of cardiac
microvascular disease in a subset of adult sickle cell disease (SCD) patients in the absence
of infarcted myocardium, myocardial iron overload, or coronary artery disease, increasing
the evidence for the contribution of left heart disease to pulmonary hypertension (PH)
development in these patients; unfortunately, strong conclusions could not be made because
the study was underpowered. Thus, this proposal will leverage the advantages offered by CMR
to better characterize and detect the PH and cardiopulmonary subphenotypes in the SCD
patient population.

Inclusion Criteria:

- Patients must be 18+

- Patients who were diagnosed with SCD confirmed by high-pressure liquid chromatography
or hemoglobin electrophoresis will be eligible for the study

- Only patients in stable condition will be included

- Patients receiving transfusions will not be excluded

Exclusion Criteria:

- Patients with vaso-occlusive crises or an episode of acute chest syndrome within the
previous four weeks (after 4 weeks have passed, the patients may be re-evaluated for
eligibility)

- Patients with high degree heart block; active, hemodynamically significant,
ventricular arrhythmias; unstable coronary syndromes; history of myocardial
infarction within 1 month of the study.

- Contraindications to gadolinium-enhanced magnetic resonance examination such as
severe claustrophobia, Pacemaker, defibrillators, cerebral aneurysm clips, or
neurostimulator.

- Pregnancy

- Patients with sinus node dysfunction