Hipertensão pulmonar associada à anemia falciforme

A hipertensao pulmonar e uma complicacao comum em pacientes com anemia falciforme. A despeito das elevacoes leves das pressoes pulmonares, a morbimortalidade desses pacientes e alta e a hipertensao pulmonar e um fator de risco muito importante nestes pacientes. A patogenese da hipertensao pulmonar relacionada a anemia falciforme e multifatorial e inclui a hemolise, baixos niveis de oxido nitrico, hipoxia cronica, tromboembolismo, doenca hepatica cronica e asplenia. Na maioria dos pacientes, hipertensao arterial pulmonar e a causa principal para elevacoes na pressao arterial pulmonar, mas hipertensao pulmonar venosa tambem e um fator contribuinte em alguns pacientes. Existem poucos estudos especificos avaliando os efeitos de tratamento para a hipertensao pulmonar em pacientes com anemia falciforme. E provavel que a intensificacao da terapia para a anemia hemolitica em todos os pacientes e tratamento especifico para a hipertensao pulmonar em pacientes com hipertensao pulmonar severa seja benefica... Pulmonary hypertension is a common complication of sickle cell disease. In spite of the mild elevations in pulmonary artery pressures in these patients, morbidity and mortality is high and in adult patients with sickle cell disease, pulmonary hypertension is emerging as a major risk factor for death. The pathogenesis of pulmonary hypertension is likely multifactorial, including hemolysis, impaired nitric oxide bioavailability, chronic hypoxemia, thromboembolism, chronic liver disease and asplenia. In the majority of patients with sickle cell disease, pulmonary arterial hypertension is the cause of elevated pulmonary artery pressures, but also that pulmonary venous hypertension does contribute to pulmonary hypertension in a subgroup of patients. There are currently limited specific data on the effects of any treatment modality for pulmonary hypertension in patients with sickle cell disease. It is likely that maximization of sickle cell disease therapy, in all patients, and treatment with selective pulmonary vasodilators and antiproliferative agents, in patients with severe disease, would be beneficial...