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Sickle Cell Disease-Induced Pulmonary Hypertension: A Review of Pathophysiology, Management, and Current Literature.

ABSTRACT: Sickle cell disease is an inherited hemoglobinopathy leading to the synthesis of hemoglobin S. Hemoglobin S results in the formation of abnormal sickle-shaped erythrocytes that lead to hematologic abnormalities such as hemolytic anemia and increased risks of thrombosis. Another particular problem encountered with the disease is pulmonary hypertension. The objective of this narrative review is to discuss the prevalence, pathophysiology mechanisms, diagnostic techniques, treatment options, and prognostic indicators in the setting of sickle cell disease with pulmonary hypertension. Additionally, the review also highlights other advancements that are being investigated. Considering the significant morbidity, mortality, and prevalence of pulmonary hypertension in patients with sickle cell disease, it is important to account for the aforementioned domains in the future guidelines to provide optimal and individualized care to the high-risk individuals as well as reduce the progression of disease, morbidity, and mortality rates.

SUBMITTER: Sheikh AB

PROVIDER: S-EPMC8740209 | biostudies-literature | 2021 Dec

REPOSITORIES: biostudies-literature

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Sickle Cell Disease-Induced Pulmonary Hypertension: A Review of Pathophysiology, Management, and Current Literature.

Sheikh Abu Baker AB Nasrullah Adeel A Lopez Erick Daniel ED Tanveer Ud Din Mian M Sagheer Shazib S Shah Ishan I Javed Nismat N Shekhar Rahul R

Pulse (Basel, Switzerland) 20210923 3-4

Sickle cell disease is an inherited hemoglobinopathy leading to the synthesis of hemoglobin S. Hemoglobin S results in the formation of abnormal sickle-shaped erythrocytes that lead to hematologic abnormalities such as hemolytic anemia and increased risks of thrombosis. Another particular problem encountered with the disease is pulmonary hypertension. The objective of this narrative review is to discuss the prevalence, pathophysiology mechanisms, diagnostic techniques, treatment options, and pro ...[more]

PMID: 35083171

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Project description:Pulmonary hypertension affects ∼10% of adult patients with sickle cell disease (SCD), particularly those with the homozygous genotype. An increase in pulmonary artery systolic pressure, estimated noninvasively by echocardiography, helps identify SCD patients at risk for pulmonary hypertension, but definitive diagnosis requires right-heart catheterization. About half of SCD-related pulmonary hypertension patients have precapillary pulmonary hypertension with potential etiologies of (1) a nitric oxide deficiency state and vasculopathy consequent to intravascular hemolysis, (2) chronic pulmonary thromboembolism, or (3) upregulated hypoxic responses secondary to anemia, low O2 saturation, and microvascular obstruction. The remainder have postcapillary pulmonary hypertension secondary to left ventricular dysfunction. Although the pulmonary artery pressure in SCD patients with pulmonary hypertension is only moderately elevated, they have a markedly higher risk of death than patients without pulmonary hypertension. Guidelines for diagnosis and management of SCD-related pulmonary hypertension were published recently by the American Thoracic Society. Management of adults with sickle-related pulmonary hypertension is based on anticoagulation for those with thromboembolism; oxygen therapy for those with low oxygen saturation; treatment of left ventricular failure in those with postcapillary pulmonary hypertension; and hydroxyurea or transfusions to raise the hemoglobin concentration, reduce hemolysis, and prevent vaso-occlusive events that cause additional increases in pulmonary pressure. Randomized trials have not identified drugs to lower pulmonary pressure in SCD patients with precapillary pulmonary hypertension. Patients with hemodynamics of pulmonary arterial hypertension should be referred to specialized centers and considered for treatments known to be effective in other forms of pulmonary arterial hypertension. There have been reports that some of these treatments improve SCD-related pulmonary hypertension.

Dataset Information

Sickle Cell Disease-Induced Pulmonary Hypertension: A Review of Pathophysiology, Management, and Current Literature.

Publications

Sickle Cell Disease-Induced Pulmonary Hypertension: A Review of Pathophysiology, Management, and Current Literature.

Similar Datasets

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