Manifestaciones cardiovasculares de anemia de células falciformes
Open Access
- 1 July 2015
- journal article
- Published by Publicidad Permanyer, SLU in Revista Colombiana de Cardiología
- Vol. 22 (4), 187-196
- https://doi.org/10.1016/j.rccar.2015.03.011
Abstract
ResumenLa anemia de células falciformes es una hemoglobinopatía hereditaria producida por la presencia de hemoglobinaS, que disminuye la solubilidad y a través del proceso de polimerización da lugar a hematíes en forma de hoz que obstruyen la red microvascular. Se caracteriza por episodios de daño por isquemia-reperfusión que contribuyen a la disfunción de órganos vitales. El advenimiento de la terapia inductora de hemoglobina fetal, asociada a la profilaxis antibiótica, ha permitido prolongar la supervivencia. Sin embargo, al incrementar la longevidad, las complicaciones cardiovasculares se hacen evidentes con el desarrollo de vasculopatía sistémica, infarto agudo de miocardio, hipertensión arterial pulmonar y disfunción ventricular. El objetivo de este artículo es revisar la fisiopatología y el tratamiento de las principales manifestaciones cardiovasculares en pacientes con anemia de células falciformes.AbstractSickle cell anemia is an inherited hemoglobinopathy caused by the presence of hemoglobinS, which lowers the solubility and through the process of polymerization results in sickle erythrocytes obstructing the microvascular network. This entity is characterized by episodes of ischemia-reperfusion injury to vital organs, contributing to its dysfunction. The advent of inducing fetal hemoglobin therapy associated with antibiotic prophylaxis has allowed prolonged survival. However, by increasing longevity, cardiovascular complications are evident, with the development of systemic vascular disease, acute myocardial infarction, pulmonary arterial hypertension and left ventricular dysfunction. The aim of this paper is to review the pathophysiology and treatment of major cardiovascular events in patients with sickle cell anemiaKeywords
This publication has 60 references indexed in Scilit:
- The relationship between the severity of hemolysis, clinical manifestations and risk of death in 415 patients with sickle cell anemia in the US and EuropeHaematologica, 2012
- Mortality in Adults With Sickle Cell Disease and Pulmonary HypertensionJAMA, 2012
- Hospitalization for pain in patients with sickle cell disease treated with sildenafil for elevated TRV and low exercise capacityBlood, 2011
- Endothelin receptor antagonists for pulmonary hypertension in adult patients with sickle cell diseaseBritish Journal of Haematology, 2009
- Prospective Echocardiography Assessment of Pulmonary Hypertension and Its Potential Etiologies in Children With Sickle Cell DiseaseThe American Journal of Cardiology, 2009
- Relative systemic hypertension in patients with sickle cell disease is associated with risk of pulmonary hypertension and renal insufficiencyAmerican Journal of Hematology, 2007
- Hemodynamic and Functional Assessment of Patients with Sickle Cell Disease and Pulmonary HypertensionAmerican Journal of Respiratory and Critical Care Medicine, 2007
- Deconstructing sickle cell disease: Reappraisal of the role of hemolysis in the development of clinical subphenotypesBlood Reviews, 2007
- Diastolic Dysfunction Is an Independent Risk Factor for Death in Patients With Sickle Cell DiseaseJournal of Invasive Cardiology, 2007
- Trial of low doses of aspirin as prophylaxis insickle cell diseaseThe Journal of Pediatrics, 1983