肺朗格汉斯细胞组织细胞增生症并发中央肺动脉广泛血栓的活体供体大叶肺移植手术。

IF 0.7 Q4 SURGERY
Naoki Date, Akihiro Ohsumi, Kenji Minatoya, Hiroshi Date
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引用次数: 0

摘要

背景:肺朗格汉斯细胞组织细胞增生症(PLCH肺朗格汉斯细胞组织细胞增生症(PLCH)是一种罕见疾病,其特点是朗格汉斯细胞沿小气管增生,导致肺实质结节性和囊性变。对于严重呼吸衰竭或肺动脉高压患者来说,肺移植是挽救生命的选择。在此,我们介绍一例成功进行肺移植的 PLCH 患者,该患者的中央肺动脉出现异常大的血栓:一名 47 岁的女性患者,有 16 年的 PLCH 病史,并迅速发展为呼吸衰竭。增强计算机断层扫描显示,扩张的中央肺动脉中有大量血栓。右心导管检查发现严重肺动脉高压,肺动脉平均压力为 48 mmHg。经过 3 个月的抗凝治疗,血栓明显缩小。然而,患者的呼吸状况并未改善。我们进行了双侧活体供体大叶肺移植手术,并在体外膜氧合下切除了主肺动脉中残留的血栓。受体扩张的主肺动脉因大小不匹配而需要直接移植。患者存活两年多,情况良好,血栓未复发:结论:对肺动脉内的大量血栓进行术前抗凝治疗是有效的,可确保肺移植手术的安全。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Living-donor lobar lung transplantation for pulmonary Langerhans cell histiocytosis complicated by extensive thrombi in central pulmonary arteries.

Background: Pulmonary Langerhans cell histiocytosis (PLCH) is a rare disorder characterized by the proliferation of Langerhans cells along the small airways, which causes nodular and cystic changes in the lung parenchyma. Lung transplantation can be a life-saving option for patients with severe respiratory failure or pulmonary hypertension. Herein, we present a case of successful lung transplantation in a patient with PLCH who developed unusually large thrombi in the central pulmonary artery.

Case presentation: A 47-year-old woman with 16-year history of PLCH with rapidly developing respiratory failure was admitted to our hospital for the evaluation of a lung transplant. Enhanced computed tomography revealed large thrombi in dilated central pulmonary arteries. Right heart catheterization revealed severe pulmonary hypertension, with a mean pulmonary artery pressure of 48 mmHg. The thrombi shrank markedly after 3 months of anticoagulation therapy. However, the respiratory status of the patient did not improve. We performed bilateral living-donor lobar lung transplantation with thrombectomy under extracorporeal membrane oxygenation for the remaining thrombi in the main pulmonary arteries. The dilated main pulmonary arteries of the recipient required direct plication for size mismatch. The patient survived in good condition for more than 2 years with no recurrence of thrombosis.

Conclusion: Preoperative anticoagulation therapy for massive thrombi in the pulmonary arteries was effective and led to safe lung transplantation.

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