Osti Nicola, Racanelli Vito, Susca Nicola, Martinelli Nicola, Maino Alberto
Unit of Internal Medicine, Valli del Noce Regional Hospital, Azienda Provinciale per i Servizi Sanitari-APSS, Trento, Italy.
Unit of Internal Medicine, Santa Chiara Regional Hospital, Azienda Provinciale per i Servizi Sanitari-APSS, Trento, Italy.
Int J Cardiol Heart Vasc. 2024 Dec 5;56:101565. doi: 10.1016/j.ijcha.2024.101565. eCollection 2025 Feb.
Inferior vena cava agenesis (IVCA) is a rare vascular abnormality characterised by the absence of one or more segments of the inferior vena cava and represents an underestimated cause of deep vein thrombosis (DVT). Given the very low prevalence of this condition and the lack of clinical trials, there is no consensus about the optimal anticoagulation strategy in IVCA-associated DVT.
To investigate efficacy and safety of direct oral anticoagulants (DOACs) in IVCA-associated DVT.
We described three patients with IVCA-associated DVT followed at our Institution and treated with DOACs. Then, we performed a systematic review of the literature for ICVA-associated DVT treated with DOACs.
In addition to our 3 cases, we found data from 19 publications for a total of 30 patients with IVCA-associated DVT treated with DOACs (24 subjects treated with rivaroxaban, 8 with apixaban, and one with dabigatran). Most patients were males (72.7 %) with a median age at DVT onset of 26.0 years (min-max range 13-64 years). The majority of DVT events were unprovoked (76.0 %). The standard thrombophilia tests were mainly negative. The median follow-up period during DOAC therapy was 1.0 years (min-max range 0-10 years), with one recurrent splanchnic vein thrombosis reported and no haemorrhagic events.
IVCA is a rare cause of DVT, which should be suspected in young adults with unprovoked DVT. Although future studies are needed, available data may support the use of DOACs in IVCA-associated DVT, with a reassuring profile of both efficacy and safety.
下腔静脉发育不全（IVCA）是一种罕见的血管异常，其特征为下腔静脉的一个或多个节段缺失，是深静脉血栓形成（DVT）的一个未得到充分认识的病因。鉴于这种疾病的患病率极低且缺乏临床试验，对于IVCA相关DVT的最佳抗凝策略尚无共识。
研究直接口服抗凝剂（DOACs）治疗IVCA相关DVT的有效性和安全性。
我们描述了在我们机构接受治疗并使用DOACs的3例IVCA相关DVT患者。然后，我们对使用DOACs治疗ICVA相关DVT的文献进行了系统综述。
除了我们的3例病例外，我们还从19篇出版物中找到了数据，共有30例使用DOACs治疗的IVCA相关DVT患者（24例接受利伐沙班治疗，8例接受阿哌沙班治疗，1例接受达比加群治疗）。大多数患者为男性（72.7%），DVT发病的中位年龄为26.0岁（最小-最大范围13-64岁）。大多数DVT事件是自发性的（76.0%）。标准的血栓形成倾向检测主要为阴性。DOAC治疗期间的中位随访期为1.0年（最小-最大范围0-10年），报告了1例复发性内脏静脉血栓形成，无出血事件。
IVCA是DVT的罕见病因，在患有自发性DVT的年轻成年人中应怀疑此病。尽管需要未来的研究，但现有数据可能支持在IVCA相关DVT中使用DOACs，其有效性和安全性令人放心。
