Applications of cardiac magnetic resonance imaging in sickle cell disease

Niss, Omar; Taylor, Michael D.

doi:10.1016/j.bcmd.2017.08.005

Cited by 8 publications

(5 citation statements)

References 89 publications

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“…Myocardial fibrosis is definitively detected in patients either during postmortem autopsy, echocardiography or CMR imaging. While echocardiography is the gold standard for clinical detection of diastolic dysfunction, CMR is superior at detecting myocardial interstitial fibrosis even before onset of diastolic dysfunction 38. Myocardial fibrosis occurs diffusively with ageing.…”

Section: Myocardial Fibrosis In Scdmentioning

confidence: 99%

Sickle cell disease: at the crossroads of pulmonary hypertension and diastolic heart failure

Wood

Gladwin

Straub

2019

Heart

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Sickle cell disease (SCD) is caused by a single point mutation in the gene that codes for beta globin synthesis, causing haemoglobin polymerisation, red blood cell stiffening and haemolysis under low oxygen and pH conditions. Downstream effects include widespread vasculopathy due to recurring vaso-occlusive events and haemolytic anaemia, affecting all organ systems. Cardiopulmonary complications are the leading cause of death in patients with SCD, primarily resulting from diastolic heart failure (HF) and/or pulmonary hypertension (PH). HF in SCD often features biventricular cardiac hypertrophy and left ventricular (LV) diastolic dysfunction. Among HF cases in the general population, approximately half occur with preserved ejection fraction (HFpEF). The insidious evolution of HFpEF differs from the relatively acute evolution of HF with reduced ejection fraction. The PH of SCD has diverse origins, which can be pulmonary arterial (precapillary), pulmonary venous (postcapillary) or pulmonary thromboembolic. It is also appreciated that patients with SCD can develop both precapillary and postcapillary PH, with elevations in LV diastolic pressures, as well as elevations in transpulmonary pressure gradient and pulmonary vascular resistance. Regardless of the cause of PH in SCD, its presence significantly reduces functional capacity and increases mortality. PH that occurs in the presence of HFpEF is usually of postcapillary origin. This review aims to assemble what has been learnt from clinical and animal studies about the manifestation of PH-HFpEF in SCD, specifically the contributions of LV diastolic dysfunction and myocardial fibrosis, in an attempt to gain an understanding of its evolution.

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Section: Myocardial Fibrosis In Scdmentioning

confidence: 99%

Sickle cell disease: at the crossroads of pulmonary hypertension and diastolic heart failure

Wood

Gladwin

Straub

2019

Heart

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“…47,65 Finally, cardiac magnetic resonance imaging is an emerging diagnostic tool for myocardial dysfunction in patients with SCD. 66 The use of hydroxyurea or chronic transfusion as well as targeted pulmonary arterial hypertension therapy is recommended as part of management therapy based on risk stratification and the etiology of PH, but data for randomized controlled studies are lacking. 59 Supplemental oxygen should be administered to maintain arterial oxyhemoglobin saturation of at least 90% at rest or with exertion.…”

Section: How Do Ahr and Parenchymal Lung Disease Contribute To Hypoximentioning

confidence: 99%

How I treat hypoxia in adults with hemoglobinopathies and hemolytic disorders

Machogu

Machado

2018

Blood

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Hemoglobinopathies are caused by genetic mutations that result in abnormal hemoglobin molecules, resulting in hemolytic anemia. Chronic complications involving the lung parenchyma, vasculature, and cardiac function in hemoglobinopathies result in impaired gas exchange, resulting in tissue hypoxia. Hypoxia is defined as the deficiency in the amount of oxygen reaching the tissues of the body and is prevalent in patients with hemoglobinopathies, and its cause is often multifactorial. Chronic hypoxia in hemoglobinopathies is often a sign of disease severity and is associated with increased morbidity and mortality. Therefore, a thorough understanding of the pathophysiology of hypoxia in these disease processes is important in order to appropriately treat the underlying cause and prevent complications. In this article, we discuss management of hypoxia based on three different cases: sickle cell disease, β-thalassemia, and hereditary spherocytosis. These cases are used to review the current understanding of the disease pathophysiology, demonstrate the importance of a thorough clinical history and physical examination, explore diagnostic pathways, and review the current management.

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“…Cardiac disease in SCD has been extensively described through a wide range of diagnostic modalities, such as Doppler echocardiography, scintigrafic techniques and, more recently, cardiac magnetic resonance (CMR) imaging [26][27][28]. Due to its multiparametric nature, CMR is a powerful method for in-depth evaluation and monitoring of myocardial structural and functional disorders in SCD patients [28,29].…”

Section: Introductionmentioning

confidence: 99%

Multiparametric Cardiac Magnetic Resonance Assessment in Sickle Beta Thalassemia

Pistoia,

Meloni,

Positano

et al. 2024

Diagnostics

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Cardiac involvement in sickle beta thalassemia (Sβ-thal) patients has been poorly investigated. We aimed to evaluate cardiac function and myocardial iron overload by cardiovascular magnetic resonance (CMR) in patients with Sβ-thal. One-hundred and eleven Sβ-thal patients consecutively enrolled in the Myocardial Iron Overload in Thalassemia (MIOT) network were studied and compared with 46 sickle cell anemia (SCA) patients and with 111 gender- and age- matched healthy volunteers. Cine images were acquired to quantify biventricular function. Myocardial iron overload (MIO) was assessed by the T2* technique, while macroscopic myocardial fibrosis was evaluated by the late gadolinium enhancement (LGE) technique. In Sβ-thal and SCA patients, the morphological and functional CMR parameters were not significantly different, except for the left atrial area and left ventricular (LV) stroke volume, indexed by body surface area (p = 0.023 and p = 0.048, respectively), which were significantly higher in SCA patients. No significant differences between the two groups were found in terms of myocardial iron overload and macroscopic myocardial fibrosis. When compared to healthy subjects, Sβ-thal patients showed significantly higher bi-atrial and biventricular parameters, except for LV ejection fraction, which was significantly lower. The CMR analysis confirmed that Sβ-thal and SCA patients are phenotypically similar. Since Sβ-thal patients showed markedly different morphological and functional indices from healthy subjects, it would be useful to identify Sβ-thal/SCA-specific bi-atrial and biventricular reference values.

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Applications of cardiac magnetic resonance imaging in sickle cell disease

Cited by 8 publications

References 89 publications

Sickle cell disease: at the crossroads of pulmonary hypertension and diastolic heart failure

Sickle cell disease: at the crossroads of pulmonary hypertension and diastolic heart failure

How I treat hypoxia in adults with hemoglobinopathies and hemolytic disorders

Multiparametric Cardiac Magnetic Resonance Assessment in Sickle Beta Thalassemia

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