This is a case report of a successful single-lobe lung transplantation for pulmonary hypertension secondary to alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV). A 6-year-old boy underwent living-donor single-lobe transplantation with the right lower lobe from his 31-year-old mother. The pretransplantation graft size matching was acceptable: the estimated graft forced vital capacity (FVC) was 96.5% of the recipient's predicted FVC, and the graft size measured by computed tomography (CT) volumetry was 166% of the recipient's chest cavity volume. Right pneumonectomy followed by implantation was performed under cardiopulmonary bypass (CPB). The pulmonary arterial pressure was significantly decreased to 31/12 mm Hg immediately after transplantation, and the first PaO2 /FiO2 in the intensive-care unit (ICU) was 422 mm Hg. Lung perfusion scintigraphy showed 97.5% perfusion to the right implanted lung 3 months after transplantation. Chest CT showed a mass rapidly growing in the native left upper lobe 6 months after transplantation, which was diagnosed as posttransplant lymphoproliferative disorder (PTLD) by a CT-guided biopsy. After immunosuppressant reduction and six courses of chemotherapy with rituximab, he underwent native left upper lobectomy for salvage lung resection 13 months after transplantation. Seven months after lobectomy, he has returned to normal school life without any sign of tumor recurrence.

中文翻译：

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活体单叶肺移植治疗肺泡毛细血管发育不良伴肺静脉错位引起的肺动脉高压。

这是一例成功的单叶肺移植治疗继发于肺静脉错位的肺泡毛细血管发育不良 (ACD/MPV) 的肺动脉高压的病例报告。一名 6 岁男孩接受了活体单肺叶移植，右肺下叶来自他 31 岁的母亲。移植前移植物大小匹配是可以接受的：估计的移植物用力肺活量 (FVC) 是接受者预测 FVC 的 96.5%，并且通过计算机断层扫描 (CT) 体积测量法测量的移植物大小是接受者胸腔容积的 166%。在体外循环 (CPB) 下进行右肺切除术和植入术。肺动脉压在移植后立即显着降低至 31/12 mm Hg，重症监护病房（ICU）的第一 PaO2 /FiO2 为 422 mm Hg。肺灌注显像显示移植后 3 个月右侧植入肺的灌注率为 97.5%。胸部 CT 显示移植后 6 个月在原生左上叶迅速生长的肿块，通过 CT 引导活检诊断为移植后淋巴组织增生性疾病（PTLD）。在减少免疫抑制剂和 6 个疗程的利妥昔单抗化疗后，他在移植后 13 个月接受了自体左上肺叶切除术以进行挽救性肺切除术。肺叶切除术后七个月，他已恢复正常的学校生活，没有任何肿瘤复发的迹象。胸部 CT 显示移植后 6 个月在原生左上叶迅速生长的肿块，通过 CT 引导活检诊断为移植后淋巴组织增生性疾病（PTLD）。在减少免疫抑制剂和 6 个疗程的利妥昔单抗化疗后，他在移植后 13 个月接受了自体左上肺叶切除术以进行挽救性肺切除术。肺叶切除术后七个月，他已恢复正常的学校生活，没有任何肿瘤复发的迹象。胸部 CT 显示移植后 6 个月在原生左上叶迅速生长的肿块，通过 CT 引导活检诊断为移植后淋巴组织增生性疾病（PTLD）。在减少免疫抑制剂和 6 个疗程的利妥昔单抗化疗后，他在移植后 13 个月接受了自体左上肺叶切除术以进行挽救性肺切除术。肺叶切除术后七个月，他已恢复正常的学校生活，没有任何肿瘤复发的迹象。