Pathophysiology and treatment of pulmonary hypertension in sickle cell disease

VR Gordeuk, OL Castro… - Blood, The Journal of the …, 2016 - ashpublications.org
Pulmonary hypertension affects∼ 10% of adult patients with sickle cell disease (SCD),
particularly those with the homozygous genotype. An increase in pulmonary artery systolic …

Cardiomyopathy with restrictive physiology in sickle cell disease

O Niss, CT Quinn, A Lane, J Daily, PR Khoury… - JACC: Cardiovascular …, 2016 - jacc.org
Objectives: The aim of this study was to identify a unifying cardiac pathophysiology that
explains the cardiac pathological features in sickle cell disease (SCD). Background …

Association between diffuse myocardial fibrosis and diastolic dysfunction in sickle cell anemia

O Niss, R Fleck, F Makue, T Alsaied… - Blood, The Journal …, 2017 - ashpublications.org
Sickle cell anemia (SCA)–related cardiomyopathy is characterized by diastolic dysfunction
and hyperdynamic features. Diastolic dysfunction portends early mortality in SCA. Diastolic …

Cardiovascular complications of sickle cell disease

V Sachdev, DR Rosing, SL Thein - Trends in cardiovascular medicine, 2021 - Elsevier
Sickle cell disease (SCD) is the most common inherited blood disorder in the United States,
and a global health problem. Pathological features of the abnormal hemoglobin (HbS) result …

Sickle cell anemia mice develop a unique cardiomyopathy with restrictive physiology

N Bakeer, J James, S Roy, J Wansapura… - Proceedings of the …, 2016 - pnas.org
Cardiopulmonary complications are the leading cause of mortality in sickle cell anemia
(SCA). Elevated tricuspid regurgitant jet velocity, pulmonary hypertension, diastolic, and …

Cardiovascular manifestations of sickle cell disease

N Hammoudi, F Lionnet, A Redheuil… - European heart …, 2020 - academic.oup.com
Sickle cell disease (SCD) is the most frequent genetic haemoglobinopathy worldwide. Early
childhood mortality has dramatically decreased in high-income countries, and most patients …

Early initiation of disease-modifying therapy can impede or prevent diffuse myocardial fibrosis in sickle cell anemia

O Niss, J Detterich, JC Wood, TD Coates… - Blood, The Journal …, 2022 - ashpublications.org
Cardiovascular disease is a major cause of mortality in patients with sickle cell disease
(SCD). Niss et al previously reported that cardiac magnetic resonance in 25 patients showed …