Clinical practice guidelines for pediatric lung transplantation in China

doi:10.3877/cma.j.issn.1674-3903.2026.01.001

Abstract

Abstract:

Pediatric lung transplantation is currently the only effective treatment for children with end-stage lung disease. In recent years, with the continuous advancement of organ donation and transplantation in China, the number of pediatric lung transplant procedures has gradually increased. However, given the relatively late initiation of pediatric lung transplantation in China, the development across centers remains uneven, clinical outcomes vary significantly, and unified professional consensus and management standards are lacking. To address these challenges, this guideline was developed through extensive discussion and evidence-based analysis by a multidisciplinary panel of experts. It focuses on key aspects of pediatric lung transplantation and proposes scientific and standardized principles for clinical practice, with the aim of improving both short- and long-term survival in pediatric recipients and promoting the normalization, standardization, and high-quality development of pediatric lung transplantation in China.

Key words: Pediatric lung transplantation, Indications, Contraindications, Preoperative evaluation and management, Surgical strategy, Postoperative management

Lung Transplantation Group, Chinese Society of Organ Transplantation, Chinese Medical Association, China Quality Management and Control Center for Lung Transplantation. Clinical practice guidelines for pediatric lung transplantation in China[J]. Chinese Journal of Transplantation(Electronic Edition), 2026, 20(01): 1-14.

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References 86

1	Rossano JW, Cherikh WS, Chambers DC, et al. The International Thoracic Organ Transplant Registry of the International Society for Heart and Lung Transplantation: twenty-first pediatric heart transplantation report-2018; focus theme: multiorgan transplantation [J]. J Heart Lung Transplant, 2018, 37(10): 1184-1195.
2	Singh TP, Hsich E, Cherikh WS, et al. The International Thoracic Organ Transplant Registry of the International Society for Heart and Lung Transplantation: 2025 annual report of heart and lung transplantation[J]. J Heart Lung Transplant, 2025, 44(12)：1857-1873.
3	黄洁夫. 中国器官捐献和移植发展报告(2023) [M]. 北京：清华大学出版社，2023.
4	Li XS, Wang ZT, Wu B, et al. Pediatric lung transplantation in China, 2019-2023[J]. World J Pediatr, 2025，21(6)：597-612.
5	陈耀龙，杨克虎，王小钦，等. 中国制订/修订临床诊疗指南的指导原则(2022版)[J]. 中华医学杂志，2022, 102(10): 697-703.
6	Howick Jeremy, Chalmers Iain, Glasziou Paul, et al. Explanation of the 2011 Oxford Centre for Evidence-Based Medicine (OCEBM) Levels of Evidence (Background Document). Oxford Centre for Evidence-Based Medicine[EB/OL]. [2025-11-05]. URL
7	Benoit TM, Benden C. Pediatric lung transplantation: supply and demand[J]. Curr Opin Organ Transplant, 2019, 24(3): 324-328.
8	Benden C． Pediatric lung transplantation[J]. J Thorac Dis, 2017, 9(8): 2675-2683.
9	Werner R, Benden C. Pediatric lung transplantation as standard of care[J]. Clin Transplant, 2021, 35(1): e14126.
10	Schmid FA, Benden C. Special considerations for the use of lung transplantation in pediatrics[J]. Expert Rev Respir Med, 2016, 10(6): 655-662.
11	Leard LE, Holm AM, Valapour M, et al. Consensus document for the selection of lung transplant candidates: an update from the International Society for Heart and Lung Transplantation[J]. J Heart Lung Transplant, 2021, 40(11): 1349-1379.
12	Hayes D Jr, Benden C, Sweet SC, et al. Current state of pediatric lung transplantation[J]. Lung, 2015, 193(5): 629-637.
13	Hansmann G, Koestenberger M, Alastalo TP, et al. 2019 updated consensus statement on the diagnosis and treatment of pediatric pulmonary hypertension: The European Pediatric Pulmonary Vascular Disease Network (EPPVDN), endorsed by AEPC, ESPR and ISHLT[J]. J Heart Lung Transplant, 2019, 38(9): 879-901.
14	广州医科大学附属第一医院，国家呼吸医学中心，广州呼吸健康研究院，等. 儿童肺移植中国专家建议[J]. 中华实用儿科临床杂志，2023, 38(11): 806-813.
15	Bos S, Murray J, Marchetti M, et al. ERS/EBMT clinical practice guidelines on treatment of pulmonary chronic graft-versus-host disease in adults[J]. Eur Respir J, 2024, 63(3)：2301727.
16	Zajacova A, Schoemans H, Greer M, et al. Lung transplantation for pulmonary chronic graft-versus-host disease: a missed opportunity？[J]. JHLT Open, 2025, 7: 100209.
17	Hakim A, Cooke KR, Pavletic SZ, et al. Diagnosis and treatment of bronchiolitis obliterans syndrome accessible universally[J]. Bone Marrow Transplant, 2019, 54(3): 383-392.
18	Bryant R 3rd, Morales D, Schecter M. Pediatric lung transplantation[J]. Semin Pediatr Surg, 2017, 26(4): 213-216.
19	Conrad C, Cornfield DN. Pediatric lung transplantation: promise being realized[J]. Curr Opin Pediatr, 2014, 26(3): 334-342.
20	陈静瑜，田东. 临床肺移植[M]. 上海：上海交通大学出版社，2023.
21	Goldfarb SB, Levvey BJ, Edwards LB, et al. The registry of the International Society for Heart and Lung Transplantation: nineteenth pediatric lung and heart-lung transplantation report-2016; focus theme: primary diagnostic indications for transplant[J]. J Heart Lung Transplant, 2016, 35(10): 1196-1205.
22	Sweet SC. Pediatric lung transplantation[J]. Respir Care, 2017, 62(6): 776-798.
23	中国医师协会血液科医师分会，中华医学会血液学分会. 造血干细胞移植后闭塞性细支气管炎综合征诊断与治疗中国专家共识(2022年版)[J]. 中华血液学杂志，2022, 43(6): 441-447.
24	Benden C． Specific aspects of children and adolescents undergoing lung transplantation[J]. Curr Opin Organ Transplant, 2012, 17(5): 509-514.
25	Kuklinski CA, Blatter JA. Interstitial lung disease as an indication for pediatric lung transplant[J]. Pediatr Pulmonol, 2024，59(9)：2313-2320.
26	Killian MO. Psychosocial predictors of medication adherence in pediatric heart and lung organ transplantation[J]. Pediatr Transplant, 2017, 21(4).
27	Cypel M, Yeung JC, Liu M, et al. Normothermic ex vivo lung perfusion in clinical lung transplantation[J]. N Engl J Med, 2011, 364(15): 1431-1440.
28	Inci I, Schuurmans MM, Kestenholz P, et al. Long-term outcomes of bilateral lobar lung transplantation[J]. Eur J Cardiothorac Surg, 2013, 43(6): 1220-1225.
29	Eberlein M, Reed RM, Maidaa M, et al. Donor-recipient size matching and survival after lung transplantation. a cohort study[J]. Ann Am Thorac Soc, 2013, 10(5): 418-425.
30	Eberlein M, Reed RM, Permutt S, et al. Parameters of donor-recipient size mismatch and survival after bilateral lung transplantation[J]. J Heart Lung Transplant, 2012, 31(11): 1207-1213.
31	Allen U, Green M. Prevention and treatment of infectious complications after solid organ transplantation in children[J]. Pediatr Clin North Am, 2010, 57(2): 459-479.
32	Kao CM, Michaels MG. Approach to vaccinating the pediatric solid organ transplant candidate and recipient[J]. Front Pediatr, 2023, 11: 1271065.
33	Danziger-Isakov L, Kumar D. Vaccination of solid organ transplant candidates and recipients: Guidelines from the American society of transplantation infectious diseases community of practice [J]. Clin Transplant, 2019, 33(9): e13563.
34	Rubin LG, Levin MJ, Ljungman P, et al. 2013 IDSA clinical practice guideline for vaccination of the immunocompromised host[J]. Clin Infect Dis, 2014, 58(3): 309-318.
35	Elgharably H, Javorski MJ, McCurry KR. Bilateral sequential lung transplantation: technical aspects[J]. J Thorac Dis, 2021, 13(11): 6564-6575.
36	Macchiarini P, Ladurie FL, Cerrina J, et al. Clamshell or sternotomy for double lung or heart-lung transplantation？[J]. Eur J Cardiothorac Surg, 1999, 15(3): 333-339.
37	Arndt G, Granger E, Glanville A, et al. Clamshell incision vs. sternal-sparing incision in lung transplantation [J]. J Heart Lung Transplant, 2013, 32(4, Supplement): S265.
38	Martens T, Kanakis M, Spencer H, et al. Pediatric lung transplantation: results of volume reduction in smaller children[J]. Pediatr Transplant, 2020, 24(5): e13752.
39	Santos Silva J, Olland A, Massard G, et al. Does lobar or size-reduced lung transplantation offer satisfactory early and late outcomes？[J]. Interact Cardiovasc Thorac Surg, 2020, 31(1): 93-97.
40	Weill D, Benden C, Corris PA, et al. A consensus document for the selection of lung transplant candidates: 2014-an update from the Pulmonary Transplantation Council of the International Society for Heart and Lung Transplantation[J]. J Heart Lung Transplant, 2015, 34(1): 1-15.
41	Harringer W, Haverich A. Heart and heart-lung transplantation: standards and improvements[J]. World J Surg, 2002, 26(2): 218-225.
42	Sweet SC. Pediatric living donor lobar lung transplantation[J]. Pediatr Transplant, 2006, 10(7): 861-868.
43	裘刚．新生儿重症监护室开展体外膜肺氧合的常见问题及对策[J]. 中华实用儿科临床杂志，2019, 34(23): 1761-1766.
44	儿童体外膜氧合专家共识撰写组，中华医学会儿科学分会急救学组. 体外膜氧合在儿童危重症应用的专家共识[J]. 中华儿科杂志，2022, 60(3): 183-191.
45	Koh W, Zang H, Ollberding NJ, et al. Extracorporeal membrane oxygenation bridge to pediatric lung transplantation: modern era analysis[J]. Pediatr Transplant, 2023, 27(6): e14570.
46	蔡子文，张泰隆，王寅，等. ECMO在儿童心脏移植中的应用[J]. 器官移植，2023, 14(1): 49-55.
47	Sharma NS, Hartwig MG, Hayes D JR. Extracorporeal membrane oxygenation in the pre and post lung transplant period[J]. Ann Transl Med, 2017, 5(4): 74.
48	Casswell GK, Pilcher DV, Martin RS, et al. Buying time: the use of extracorporeal membrane oxygenation as a bridge to lung transplantation in pediatric patients[J]. Pediatr Transplant, 2013, 17(8): E182-E188.
49	Toprak D, Midyat L, Freiberger D, et al. Outcomes of mechanical support in a pediatric lung transplant center[J]. Pediatr Pulmonol, 2017, 52(3): 360-366.
50	Thompson K, Staffa SJ, Nasr VG, et al. Mortality after lung transplantation for children bridged with extracorporeal membrane oxygenation[J]. Ann Am Thorac Soc, 2022, 19(3): 415-423.
51	Barbaro RP, MacLaren G, Annich GM, et al. Bridging children to lung transplantation using extracorporeal membrane oxygenation[J]. Ann Am Thorac Soc, 2022, 19(3): 357-359.
52	Chiel LE, Winthrop ZA, Fynn-Thompson F, et al. Extracorporeal membrane oxygenation and paracorporeal lung assist devices as a bridge to pediatric lung transplantation[J]. Pediatr Transplant, 2022, 26(5): e14289.
53	岳冰清，陈静瑜. 儿童肺移植国内外研究进展[J]. 中华实用儿科临床杂志，2021, 36(2): 154-157.
54	Pereszlenyi A, Lang G, Steltzer H, et al. Bilateral lung transplantation with intra- and postoperatively prolonged ECMO support in patients with pulmonary hypertension[J]. Eur J Cardiothorac Surg, 2002, 21(5): 858-863.
55	Bermudez CA, Shiose A, Esper SA, et al. Outcomes of intraoperative venoarterial extracorporeal membrane oxygenation versus cardiopulmonary bypass during lung transplantation[J]. Ann Thorac Surg, 2014, 98(6): 1936-1942.
56	Pettenuzzo T, Ocagli H, Sella N, et al. Intraoperative extracorporeal support for lung transplant: a systematic review and network meta-analysis[J]. J Anesth Analg Crit Care, 2024, 4(1): 81.
57	Parikh AN, Merritt TC, Ccrvajal HG, et al. A comparison of cardiopulmonary bypass versus extracorporeal membrane oxygenation: does intraoperative circulatory support strategy affect outcomes in pediatric lung transplantation？[J]. Clin Transplant, 2021, 35(6): e14289.
58	Olsen MC, Anderson MJ, Fehr JJ, et al. ECMO for pediatric lung transplantation[J]. ASAIO J, 2017, 63(6): e77-e80.
59	Yue B, Wu B, Zhang J, et al. Pediatric lung transplantation in the largest lung transplantation center of China: embarking on a long road[J]. Sci Rep, 2020, 10(1): 12471.
60	Koh W, Rao SB, Yasechko SM, et al. Postoperative management of children after lung transplantation[J]. Semin Pediatr Surg, 2022, 31(3): 151179.
61	Di Nardo M, Tikkanen J, Husain S, et al. Postoperative management of lung transplant recipients in the intensive care unit[J]. Anesthesiology, 2022, 136(3): 482-499.
62	Beer A, Reed RM, Bölükbas S, et al. Mechanical ventilation after lung transplantation. An international survey of practices and preferences[J]. Ann Am Thorac Soc, 2014, 11(4): 546-553.
63	Van Raemdonck D, Hartwig MG, Hertz MI, et al. Report of the ISHLT Working Group on primary lung graft dysfunction Part IV: prevention and treatment: a 2016 Consensus Group statement of the International Society for Heart and Lung Transplantation[J]. J Heart Lung Transplant, 2017, 36(10): 1121-1136.
64	Labarinas S, Coss-Bu JA, Onyearugbulem C, et al. Influence of early extubation on post-operative outcomes after pediatric lung transplantation[J]. Pediatr Transplant, 2021, 25(2): e13776.
65	Wong JY, Westall GP, Snell GI. Bronchoscopic procedures and lung biopsies in pediatric lung transplant recipients[J]. Pediatr Pulmonol, 2015, 50(12): 1406-1419.
66	Hayes D Jr, Kirkby S, Wehr AM, et al. A contemporary analysis of induction immunosuppression in pediatric lung transplant recipients[J]. Transpl Int, 2014, 27(2): 211-218.
67	Erdman J, Wolfram J, Nimke D, et al. Pediatric lung transplant outcomes based on immunosuppressive regimen at discharge: retrospective cohort study using real-world evidence from the US Scientific Registry of Transplant Recipients[J]. Transplant Proc, 2023, 55(7): 1692-1705.
68	Marquet P, Cros F, Micallef L, et al. Tacrolimus bayesian dose adjustment in pediatric renal transplant recipients[J]. Ther Drug Monit, 2021, 43(4): 472-480.
69	Smibert OC, Paraskeva MA, Westall G, et al. An update in antimicrobial therapies and infection prevention in pediatric lung transplant recipients[J]. Paediatr Drugs, 2018, 20(6): 539-553.
70	Solomon M, Grasemann H, Keshavjee S. Pediatric lung transplantation[J]. Pediatr Clin North Am, 2010, 57(2): 375-391.
71	Guilinger RA, Paradis IL, Dauber JH, et al. The importance of bronchoscopy with transbronchial biopsy and bronchoalveolar lavage in the management of lung transplant recipients[J]. Am J Respir Crit Care Med, 1995, 152(6 Pt 1): 2037-2043.
72	Perreas KG, McNeil K, Charman S, et al. Extended ganciclovir prophylaxis in lung transplantation[J]. J Heart Lung Transplant, 2005, 24(5): 583-587.
73	Mead L, Danziger-Isakov LA, Michaels MG, et al. Antifungal prophylaxis in pediatric lung transplantation: an international multicenter survey[J]. Pediatr Transplant, 2014, 18(4): 393-397.
74	Husain S, Camargo JF. Invasive Aspergillosis in solid-organ transplant recipients: guidelines from the American Society of Transplantation Infectious Diseases Community of Practice[J]. Clin Transplant, 2019, 33(9): e13544.
75	Jomphe V, Lands LC, Mailhot G. Nutritional requirements of lung transplant recipients: challenges and considerations[J]. Nutrients, 2018, 10(6)：790.
76	Kalnins D, Pencharz PB, Grasemann H, et al. Energy expenditure and nutritional status in pediatric patients before and after lung transplantation[J]. J Pediatr, 2013, 163(5): 1500-1502.
77	Faro A, Mallory GB, Visner GA, et al. American Society of Transplantation executive summary on pediatric lung transplantation[J]. Am J Transplant, 2007, 7(2): 285-292.
78	Schwerk N, Carlens J, Köditz H, et al. Post-operative management of children after lung transplantation[J]. JHLT Open, 2025, 9: 100301.
79	Aurora P, Edwards LB, Christie JD, et al. Registry of the International Society for Heart and Lung Transplantation: Twelfth Official Pediatric Lung and Heart/Lung Transplantation Report-2009[J]. J Heart Lung Transplant, 2009，28(10)：1023-1030.
80	Shapiro R, Sarwal MM, Raina R．，et al. Pediatric solid organ transplantation: a practical handbook[M]. 3rd ed. Berlin: Springer, 2023.
81	Brosig CL. Psychological functioning of pediatric lung transplant candidates/recipients: a review of the literature[J]. Pediatr Transplant, 2003, 7(5): 390-394.
82	Burton JH, Marshall JM, Munro P, et al. Rehabilitation and transition after lung transplantation in children[J]. Transplant Proc, 2009, 41(1): 296-299.
83	Tanaka S, Nakajima D, Sakamoto R, et al. Outcome and growth of lobar graft after pediatric living-donor lobar lung transplantation[J]. J Heart Lung Transplant, 2023, 42(5): 660-668.
84	Sritippayawan S, Keens TG, Horn MV, et al. Does lung growth occur when mature lobes are transplanted into children？[J]. Pediatr Transplant, 2002, 6(6): 500-504.
85	Hayes D Jr, Cherikh WS, Chambers DC, et al. The International Thoracic Organ Transplant Registry of the International Society for Heart and Lung Transplantation: twenty-second pediatric lung and heart-lung transplantation report-2019; focus theme: donor and recipient size match[J]. J Heart Lung Transplant, 2019, 38(10): 1015-1027.
86	Iablonskii P, Carlens J, Mueller C, et al. Indications and outcome after lung transplantation in children under 12 years of age: a 16-year single center experience [J]. J Heart Lung Transplant, 2022, 41(2): 226-236.

推荐强度	证据级别	描述
A	1a	RCTs的系统评价
	1b	结果可信区间小的RCT
	1c	显示"全或无效应"的任何证据
B	2a	队列研究的系统评价
	2b	单个的队列研究(包括低质量的RCT，如失访率>20%)
	2c	基于患者结局的研究
	3a	病例对照研究的系统评价
	3b	单个病例对照研究
C	4	病例系列报告、低质量队列研究和低质量病例对照研究
D	5	专家意见(即无临床研究支持的仅依据基础研究或临床经验的推测)

推荐强度	证据级别	描述
A	1a	RCTs的系统评价
	1b	结果可信区间小的RCT
	1c	显示"全或无效应"的任何证据
B	2a	队列研究的系统评价
	2b	单个的队列研究(包括低质量的RCT，如失访率>20%)
	2c	基于患者结局的研究
	3a	病例对照研究的系统评价
	3b	单个病例对照研究
C	4	病例系列报告、低质量队列研究和低质量病例对照研究
D	5	专家意见(即无临床研究支持的仅依据基础研究或临床经验的推测)

常见适应证	转诊时机
囊性纤维化	(1)FEV1<50%预测值，并伴有6 min步行速度慢(<400 m)、低氧血症(PaO₂<8 kPa或<60 mmHg)、高碳酸血症(PaCO₂>6.6 kPa或>50 mmHg)或肺动脉高压(mPAP>25 mmHg) (2)FEV1<50%预测值，伴FEV1快速下降 (3)FEV1<40%预测值
PAH	(1)符合EPPVDN发布的儿童PAH共识中评估结果为中或高风险等级的患儿 (2)尽管进行干预，但营养状况和生长仍继续恶化 (3)呼吸衰竭，长期无创通气 (4)右心衰竭的临床证据，生长落后 (5)WHO心功能分级Ⅲ~Ⅳ级(6)疾病相关的继发性肝或肾功能障碍的体征，反复咯血或晕厥 (7)需前列环素治疗 (8)BNP或NT-proBNP水平显著升高 (9)超声心动图检查：右心房和心室严重扩大，左、右心室收缩功能障碍 (10)侵入性血流动力学指标：心指数<2.5 L·min^－1·(m²)^－1, mPAP/mSAP>0.75, mRAP>14 mmHg, RVRi>15 WUm² (11)尽管进行适当治疗或近期因PAH恶化而住院治疗，但疾病仍进展 (12)正在考虑进行房间隔造口术或反向Potts分流术作为姑息性手术(移植可能是术后选择)
间质性肺疾病	(1)有间质性肺病的组织学证据和进行性肺功能下降 (2)进行性PAH (3)经组织病理学证实或X线影像学检查确诊为寻常型间质性肺炎 (4)任何形式的肺纤维化，且FVC<80%或DLCO<40% (5)任何形式的肺纤维化且过去2年内出现以下任一项：FVC≥10%、DLCO≥15%、FVC≥5%同时伴有呼吸道症状恶化或影像学进展 (6)需要辅助供氧 (7)尽管接受治疗，但炎症仍继续进展
肺表面活性物质缺乏	(1)SP-B和ABCA3功能障碍伴难治性呼吸衰竭 (2)SP-C、NKX2.1和不严重的ABCA3功能障碍伴疾病进展 (3)进行性PAH
闭塞性细支气管炎	(1)BODE评分5~6分，并有以下提示死亡风险增加的指标：①≥3次急性加重；②过去2年内BODE评分增加>1分；③CT显示肺动脉直径/主动脉直径>1；④FEV1占20%~25%预测值 (2)BODE评分≥7分 (3)FEV1<20%预测值 (4)中重度PAH (5)慢性高碳酸血症 (6)严重急性加重 (7)充分治疗后症状仍恶化 (8)生活质量差 (9)需接受肺减容术
支气管肺发育不良	(1)严重呼吸衰竭 (2)进行性PAH
艾森曼格综合征	(1)进行性PAH (2)运动耐量受损 (3)生活质量恶化

常见适应证	转诊时机
囊性纤维化	(1)FEV1<50%预测值，并伴有6 min步行速度慢(<400 m)、低氧血症(PaO₂<8 kPa或<60 mmHg)、高碳酸血症(PaCO₂>6.6 kPa或>50 mmHg)或肺动脉高压(mPAP>25 mmHg) (2)FEV1<50%预测值，伴FEV1快速下降 (3)FEV1<40%预测值
PAH	(1)符合EPPVDN发布的儿童PAH共识中评估结果为中或高风险等级的患儿 (2)尽管进行干预，但营养状况和生长仍继续恶化 (3)呼吸衰竭，长期无创通气 (4)右心衰竭的临床证据，生长落后 (5)WHO心功能分级Ⅲ~Ⅳ级(6)疾病相关的继发性肝或肾功能障碍的体征，反复咯血或晕厥 (7)需前列环素治疗 (8)BNP或NT-proBNP水平显著升高 (9)超声心动图检查：右心房和心室严重扩大，左、右心室收缩功能障碍 (10)侵入性血流动力学指标：心指数<2.5 L·min^－1·(m²)^－1, mPAP/mSAP>0.75, mRAP>14 mmHg, RVRi>15 WUm² (11)尽管进行适当治疗或近期因PAH恶化而住院治疗，但疾病仍进展 (12)正在考虑进行房间隔造口术或反向Potts分流术作为姑息性手术(移植可能是术后选择)
间质性肺疾病	(1)有间质性肺病的组织学证据和进行性肺功能下降 (2)进行性PAH (3)经组织病理学证实或X线影像学检查确诊为寻常型间质性肺炎 (4)任何形式的肺纤维化，且FVC<80%或DLCO<40% (5)任何形式的肺纤维化且过去2年内出现以下任一项：FVC≥10%、DLCO≥15%、FVC≥5%同时伴有呼吸道症状恶化或影像学进展 (6)需要辅助供氧 (7)尽管接受治疗，但炎症仍继续进展
肺表面活性物质缺乏	(1)SP-B和ABCA3功能障碍伴难治性呼吸衰竭 (2)SP-C、NKX2.1和不严重的ABCA3功能障碍伴疾病进展 (3)进行性PAH
闭塞性细支气管炎	(1)BODE评分5~6分，并有以下提示死亡风险增加的指标：①≥3次急性加重；②过去2年内BODE评分增加>1分；③CT显示肺动脉直径/主动脉直径>1；④FEV1占20%~25%预测值 (2)BODE评分≥7分 (3)FEV1<20%预测值 (4)中重度PAH (5)慢性高碳酸血症 (6)严重急性加重 (7)充分治疗后症状仍恶化 (8)生活质量差 (9)需接受肺减容术
支气管肺发育不良	(1)严重呼吸衰竭 (2)进行性PAH
艾森曼格综合征	(1)进行性PAH (2)运动耐量受损 (3)生活质量恶化

分类	类别诊断
先天性遗传性疾病	支气管肺发育不良，原发性纤毛运动障碍，α1-抗胰蛋白酶缺乏症，肺表面活性物质代谢障碍(如ABCA3突变、表面活性蛋白B/C缺乏症)等
囊性纤维化	囊性纤维化
间质性肺病	特发性肺纤维化，BO伴机化性肺炎/隐源性机化性肺炎，脱屑性间质性肺炎，淋巴细胞间质性肺炎，非特异性间质性肺炎，ARDS/肺炎，间质性肺炎，肺泡蛋白沉积症，肺淀粉样变，婴幼儿慢性肺炎，朗格汉斯细胞组织细胞增生症，过敏性肺炎，特发性肺含铁血黄素沉着症，吸入性烧伤/外伤，肺毛细血管扩张症，硬皮病等
肺动脉高压	特发性肺动脉高压
非特发性肺动脉高压	先天性畸形，其他先天性原因，艾森曼格综合征(房间隔缺损、多发性先天性畸形、动脉导管未闭、室间隔缺损等)，门脉性肺动脉高压，肺毛细血管瘤病，肺血管疾病，肺静脉闭塞症，肺动脉瓣狭窄，继发性肺动脉高压
BO(非再次移植)	BO(非再次移植)
二次移植	由于以下原因移植失败，再次移植：急性排斥反应，原发性移植物失功，BO，限制性移植物功能障碍，其他
其他	肉芽肿性肺病(结节病、Wegener肉芽肿)，免疫缺陷及免疫相关性肺病(支气管肺曲霉病、普通变异性免疫缺陷病、移植物抗宿主病)，Swyer-James综合征，其他罕见肺病

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