Minaev Anton V, Gushchin Dmitry K, Kovalev Dmitry V, Mwela Bupe M
Congenital Heart Diseases Department, A.N. Bakoulev National Medical Research Center of Cardiovascular Surgery, Moscow, Russia.
Peoples' Friendship University of Russia, Moscow, Russia.
Egypt Heart J. 2023 May 8;75(1):39. doi: 10.1186/s43044-023-00365-2.
Thromboembolic events are a well-known risk for Fontan patients and often lead to morbidity and mortality in cyanotic patients and patients with a single ventricle. Coagulopathy and thrombophilia, in addition to disturbed Fontan blood flow and endothelial injury, are major contributors to thromboembolic complications. However, there is currently no consensus regarding the optimal medication to prevent or treat these events. Identification of coagulation disorders is therefore crucial for selecting appropriate management strategies and evaluating long-term outcomes in these patients.
We present the case of a 35-year-old male who underwent the Fontan procedure with a total cavopulmonary modification during childhood due to tricuspid atresia. He was admitted with complaints of headaches and visual disturbances, but no clear cardiovascular cause was identified. Standard coagulation test parameters were normal, but the thrombodynamics test indicated severe hypercoagulation and spontaneous clot formation. Anticoagulation therapy was initiated, and a subsequent thrombodynamics assay showed normalization of the coagulation parameters. The patient remained asymptomatic during the six-month follow-up period.
The thrombodynamics test is a valuable tool for the diagnosis of coagulation disorders, as it can assess coagulation parameters and clot growth in vitro. This method can also aid in the optimization of antithrombotic therapy. The presented clinical case highlights the potential use of the thrombodynamics test in Fontan patients to diagnose coagulation disorders and improve long-term outcomes.
血栓栓塞事件是Fontan手术患者众所周知的风险，常导致青紫型患者和单心室患者发病和死亡。除了Fontan血流紊乱和内皮损伤外，凝血功能障碍和易栓症是血栓栓塞并发症的主要促成因素。然而，目前对于预防或治疗这些事件的最佳药物尚无共识。因此，识别凝血障碍对于选择合适的管理策略和评估这些患者的长期预后至关重要。
我们报告一例35岁男性病例，该患者童年时因三尖瓣闭锁接受了全腔肺改良Fontan手术。他因头痛和视觉障碍入院，但未发现明确的心血管病因。标准凝血试验参数正常，但血栓动力学试验显示严重高凝和自发血栓形成。开始抗凝治疗，随后的血栓动力学检测显示凝血参数恢复正常。在六个月的随访期内，患者保持无症状。
血栓动力学试验是诊断凝血障碍的有价值工具，因为它可以在体外评估凝血参数和血栓生长。该方法还可有助于优化抗栓治疗。所呈现的临床病例突出了血栓动力学试验在Fontan患者中诊断凝血障碍和改善长期预后的潜在用途。
