Relationship between pulmonary and cardiac abnormalities in sickle cell disease: implications for the management of patients

Abstract

ObjectiveTo evaluate the association between clinical, pulmonary, and cardiovascular findings in patients with sickle cell disease and, secondarily, to compare these findings between sickle cell anemia patients and those with other sickle cell diseases.MethodsFifty-nine adults were included in this cross-sectional study; 47 had sickle cell anemia, and 12 had other sickle cell diseases. All patients underwent pulmonary function tests, chest computed tomography, and echocardiography.ResultsAbnormalities on computed tomography, echocardiography, and pulmonary function tests were observed in 93.5%, 75.0%; and 70.2% of patients, respectively. A higher frequency of restrictive abnormalities was observed in patients with a history of acute chest syndrome (85% vs. 21.6%; p-value < 0.0001) and among patients with increased left ventricle size (48.2% vs. 22.2%; p-value = 0.036), and a higher frequency of reduced respiratory muscle strength was observed in patients with a ground-glass pattern (33.3% vs. 4.3%; p-value = 0.016). Moreover, a higher frequency of mosaic attenuation was observed in patients with elevated tricuspid regurgitation velocity (61.1% vs. 24%; p-value = 0.014). Compared to patients with other sickle cell diseases, sickle cell anemia patients had suffered increased frequencies of acute pain episodes, and acute chest syndrome, and exhibited mosaic attenuation on computed tomography, and abnormalities on echocardiography.ConclusionA significant interrelation between abnormalities of the pulmonary and cardiovascular systems was observed in sickle cell disease patients. Furthermore, the severity of the cardiopulmonary parameters among patients with sickle cell anemia was greater than that of patients with other sickle cell diseases.

Changes seen on computed tomography of the chest in mildly symptomatic adult patients with sickle cell disease

“…The pathogenesis of vascular involvement in sickle cell disease has been attributed primarily to hemolysis caused by a reduction in the bioavailability of nitric oxide, a potent endogenous vasodilator; there is also a reduction in the activity of the arginine/nitric oxide synthase pathway. Those changes lead to significant vasoconstriction ( 6 , 7 ) . In sickle cell disease, there is also thrombin production caused by a chronic inflammatory response, which activates the coagulation cascade and abnormally increases phosphatidylserine exposure on the surface of erythrocytes ( 8 ) .…”

Anemia de Células Falciformes: Correlación Clínico-Patológica

“…Se estima que aproximadamente el 90% de las personas con ACF tienen una función pulmonar anormal, debido a que se ha relacionado la presencia de enfermedad crónica del pulmón con descompensaciones de la ACF ocasionadas por infecciones, émbolos grasos e infartos del órgano en cuestión; se cree que la hipertensión pulmonar es de hecho, una causa importante de muerte en pacientes adultos [46]. La cardiomegalia, hiperactividad torácica y los soplos cardíacos a menudo están presentes, secundarios a un estado de anemia crónica, hipertensión pulmonar y obstrucción microcirculatoria comúnmente asociada a hemoglobinopatías [46].…”

“…Se estima que aproximadamente el 90% de las personas con ACF tienen una función pulmonar anormal, debido a que se ha relacionado la presencia de enfermedad crónica del pulmón con descompensaciones de la ACF ocasionadas por infecciones, émbolos grasos e infartos del órgano en cuestión; se cree que la hipertensión pulmonar es de hecho, una causa importante de muerte en pacientes adultos [46]. La cardiomegalia, hiperactividad torácica y los soplos cardíacos a menudo están presentes, secundarios a un estado de anemia crónica, hipertensión pulmonar y obstrucción microcirculatoria comúnmente asociada a hemoglobinopatías [46]. Así, es evidente entonces que la fisiología vascular en la ACF se ve significativamente perturbada, como lo confirma el estudio llevado a cabo por Paixão et al en el que encontraron un incremento del diagnóstico de anormalidades cardiopulmonares en pacientes con ACF, con base en los hallazgos detectados en tomografías computarizadas, ecocardiografías y pruebas de funcionalidad pulmonar [46].…”

“…La cardiomegalia, hiperactividad torácica y los soplos cardíacos a menudo están presentes, secundarios a un estado de anemia crónica, hipertensión pulmonar y obstrucción microcirculatoria comúnmente asociada a hemoglobinopatías [46]. Así, es evidente entonces que la fisiología vascular en la ACF se ve significativamente perturbada, como lo confirma el estudio llevado a cabo por Paixão et al en el que encontraron un incremento del diagnóstico de anormalidades cardiopulmonares en pacientes con ACF, con base en los hallazgos detectados en tomografías computarizadas, ecocardiografías y pruebas de funcionalidad pulmonar [46].…”

Research Article <i>GSTT1</i> null genotype in sickle cell anemia and blood transfusion recurrence – a case report

“…These physiopathological events of SCA lead to a variety of morbidities such as pain, acute thoracic syndrome, stroke, aseptic necrosis of the bones, leg ulcers and proliferative retinopathy (Liu et al, 1994;Chevalier et al, 2016). Individuals with SCA are predisposed to chronic hemolytic anemia, inflammation, cell adhesion, tissue hypoxia, organ ischemia and tissue infarction (Pierrot-Gallo et al, 2015;Maioli et al, 2016). In addition, SCA patients are predisposed to oxidative stress with continuous production of ROS that can lead to hemoglobin self-oxidation .…”