Derchi Giorgio, Lai Maria E, Marcaccini Paolo, Carta Maria P, Vacquer Stefania
Ente Ospedaliero Ospedali Galliera, Cardiologia, Genova, Italia.
Hemoglobin. 2010;34(1):61-6. doi: 10.3109/03630260903547765.
We report a case of a 43-year-old woman, affected by human immunodeficiency virus (HIV) and beta-thalassemia major (beta-TM), adequately treated with antiretroviral and transfusion-chelation therapy, that develops progressive right ventricular dysfunction due to severe pulmonary arterial hypertension (PAH), in absence of symptoms. The existence of both HIV and beta-TM cardiomiopathy has recently been reported, but how these two diseases have a "competition of guilt" for creating PAH is still to be understood. The main physiopathological principles regarding HIV and beta-TM associated PAH are reviewed. The possible interplay between these two different pathologies is discussed.
我们报告了一例43岁女性病例,该患者感染人类免疫缺陷病毒(HIV)并患有重型β地中海贫血(β-TM),接受抗逆转录病毒和输血螯合治疗后病情得到充分控制,但在无症状的情况下因严重肺动脉高压(PAH)出现进行性右心室功能障碍。近期已有关于HIV和β-TM心肌病并存的报道,但这两种疾病如何在导致PAH方面存在“罪责竞争”仍有待明确。本文对HIV和β-TM相关PAH的主要生理病理机制进行了综述,并讨论了这两种不同病理情况之间可能的相互作用。