Living‐donor single‐lobe lung transplantation for pulmonary hypertension due to alveolar capillary dysplasia with misalignment of pulmonary veins. Issue 6 (27th January 2020)

• Record Type: Journal Article
• Authors: Nakajima, Daisuke; Oda, Hiromi; Mineura, Katsutaka; Goto, Tatsuya; Kato, Itaru; Baba, Shiro; Ikeda, Tadashi; Chen‐Yoshikawa, Toyofumi F.; Date, Hiroshi
• Is Part Of: American journal of transplantation. Volume 20:Issue 6(2020)
• Page Start: 1739
• Page End: 1743
• Publication Date: 2020-01-27
• Subjects: clinical research/practice -- donors and donation: living -- lung transplantation/pulmonology -- lung transplantation: living donor; Transplantation of organs, tissues, etc -- Periodicals; 617.95
• DOI: 10.1111/ajt.15762
• Languages: English
• ISSNs: 1600-6135

Abstract

Abstract : 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. Abstract : Living‐donor single‐lobe lung transplantation can be a viable option for pulmonary hypertension secondary to alveolar capillary dysplasia with misalignment of pulmonary veins.