Chinese expert consensus on surgical treatment of congenital heart disease: Unilateral absence of a pulmonary artery_Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Authors：

LI Wenlei ¹ , MA Li ¹ , WEN Shusheng ² ,  CHEN Xinxin ¹ ,  LI Shoujun ³ ,  ZHENG Jinghao ⁴

1. Heart Center, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, Guangdong Provincial Clinical Research Center for Child Health, Guangzhou, 510180, P. R. China;
2. Guangdong Cardiovascular Institute, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences, Guangzhou Medical University, Guangzhou, 510180, P. R. China;
3. Chinese Academy of Medical Sciences & Peking Union Medical College National Center for Cardiovascular Diseases, Fuwai Hospital Pediatric Surgery Center, Beijing, 100037, P. R. China;
4. Department of Cardiothoracic Surgery, Shanghai Children’s Medical Center, School of Medicine, Shanghai Jiao Tong University, National Children’s Medical Center, Shanghai, 200127, P. R. China;

Corresponding author：

CHEN Xinxin, Email: zingerchen@163.com; LI Shoujun, Email: zhengjh210@163.com; ZHENG Jinghao, Email: drlishoujunfw@163.com

Keywords：

Unilateral absence of a pulmonary artery; congenital heart disease; surgical treatment; expert consensus

DOI：

10.7507/1007-4848.202503091

Video：

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Unilateral absence of a pulmonary artery (UAPA) is a rare congenital malformation resulting from the failed development or premature involution of the sixth aortic arch during embryogenesis, leading to a failure to establish a connection with the main pulmonary artery. Currently, there is a notable lack of consensus regarding the surgical management of UAPA in China. Drawing upon the latest clinical research, this consensus aims to summarize surgical approaches and techniques to improve the clinical management of UAPA patients and serve as a scientific reference for physicians specializing in pediatric cardiology and structural heart disease. This consensus aims to promote the standardization of UAPA diagnosis and treatment, thereby facilitating improved patient outcomes and long-term management, and stimulating the continuous development and innovation of surgical treatment for this condition in China.

Citation： LI Wenlei, MA Li, WEN Shusheng, CHEN Xinxin, LI Shoujun, ZHENG Jinghao. Chinese expert consensus on surgical treatment of congenital heart disease: Unilateral absence of a pulmonary artery. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2025, 32(7): 905-908. doi: 10.7507/1007-4848.202503091 Copy

1.	Pfefferkorn JR, Löser H, Pech G, et al. Absent pulmonary artery. A hint to its embryogenesis. Pediatr Cardiol, 1982, 3(4): 283-286.
2.	Frentzel O. Ein fall von anormer communicationder aorta mit der arteria pulmonalis. Virchow's arch path. Anat, 1868, 43: 420-422.
3.	Bouros D, Pare P, Panagou P, et al. The varied manifestation of pulmonary artery agenesis in adulthood. Chest, 1995, 108(3): 670-676.
4.	Bockeria LA, Makhachev OA, Khiriev TKh, et al. Repair of congenital heart defects associated with single pulmonary artery. Asian Cardiovasc Thorac Ann, 2015, 23(2): 157-163.
5.	Bockeria LA, Makhachev OA, Khiriev TKh, et al. Congenital isolated unilateral absence of pulmonary artery and variants of collateral blood supply of the ipsilateral lung. Interact Cardiovasc Thorac Surg, 2011, 12(3): 509-510.
6.	Ten Harkel AD, Blom NA, Ottenkamp J. Isolated unilateral absence of a pulmonary artery: A case report and review of the literature. Chest, 2002, 122(4): 1471-1477.
7.	Kruzliak P, Syamasundar RP, Novak M, et al. Unilateral absence of pulmonary artery: Pathophysiology, symptoms, diagnosis and current treatment. Arch Cardiovasc Dis, 2013, 106(8-9): 448-454.
8.	Apostolopoulou SC, Kelekis NL, Brountzos EN, et al. "Absent" pulmonary artery in one adult and five pediatric patients: Imaging, embryology, and therapeutic implications. AJR Am J Roentgenol, 2002, 179(5): 1253-1260.
9.	Mery CM, Molina KM, Krishnamurthy R, et al. Pulmonary artery resuscitation for isolated ductal origin of a pulmonary artery. J Thorac Cardiovasc Surg, 2014, 148(5): 2235-2244.
10.	Santoro G, Capozzi G, Giordano M, et al. Fate of duct-dependent, discontinuous pulmonary arteries after arterial duct stenting. Pediatr Cardiol, 2017, 38(7): 1370-1376.
11.	Ishii Y, Miyamoto T, Kobayashi T, et al. Isolated unilateral agenesis of the pulmonary artery: Surgical repair with an artificial graft. Pediatr Cardiol, 2011, 32(4): 509-511.
12.	Yan X, Cen J, Luo X, et al. Surgical repair of unilateral absence of pulmonary artery in children with pulmonary hypertension: A single-center retrospective study. Transl Pediatr, 2022, 11(11): 1813-1822.
13.	Green GE, Reppert EH, Cohlan SQ, et al. Surgical correction of absence of proximal segment of left pulmonary artery. Circulation, 1968, 37(1): 62-69.
14.	Welch K, Hanley F, Johnston T, et al. Isolated unilateral absence of right proximal pulmonary artery: Surgical repair and follow-up. Ann Thorac Surg, 2005, 79(4): 1399-1402.
15.	Kosaka Y, Kurosawa H, Hoshino S, et al. Surgery for unilateral absence of pulmonary artery using autologous tissue. Ann Thorac Surg, 2003, 76(4): 1281-1283.
16.	Agrawal H, Petit CJ, Miro J, et al. Contralateral pulmonary hypertension following resuscitation of unilateral ductal origin of a pulmonary artery: A multi-institutional review. Pediatr Cardiol, 2018, 39(1): 71-78.
17.	Kim GB, Ban JE, Bae EJ, et al. Rehabilitation of pulmonary artery in congenital unilateral absence of intrapericardial pulmonary artery. J Thorac Cardiovasc Surg, 2011, 141(1): 171-178.

1. Pfefferkorn JR, Löser H, Pech G, et al. Absent pulmonary artery. A hint to its embryogenesis. Pediatr Cardiol, 1982, 3(4): 283-286.
2. Frentzel O. Ein fall von anormer communicationder aorta mit der arteria pulmonalis. Virchow's arch path. Anat, 1868, 43: 420-422.
3. Bouros D, Pare P, Panagou P, et al. The varied manifestation of pulmonary artery agenesis in adulthood. Chest, 1995, 108(3): 670-676.
4. Bockeria LA, Makhachev OA, Khiriev TKh, et al. Repair of congenital heart defects associated with single pulmonary artery. Asian Cardiovasc Thorac Ann, 2015, 23(2): 157-163.
5. Bockeria LA, Makhachev OA, Khiriev TKh, et al. Congenital isolated unilateral absence of pulmonary artery and variants of collateral blood supply of the ipsilateral lung. Interact Cardiovasc Thorac Surg, 2011, 12(3): 509-510.
6. Ten Harkel AD, Blom NA, Ottenkamp J. Isolated unilateral absence of a pulmonary artery: A case report and review of the literature. Chest, 2002, 122(4): 1471-1477.
7. Kruzliak P, Syamasundar RP, Novak M, et al. Unilateral absence of pulmonary artery: Pathophysiology, symptoms, diagnosis and current treatment. Arch Cardiovasc Dis, 2013, 106(8-9): 448-454.
8. Apostolopoulou SC, Kelekis NL, Brountzos EN, et al. "Absent" pulmonary artery in one adult and five pediatric patients: Imaging, embryology, and therapeutic implications. AJR Am J Roentgenol, 2002, 179(5): 1253-1260.
9. Mery CM, Molina KM, Krishnamurthy R, et al. Pulmonary artery resuscitation for isolated ductal origin of a pulmonary artery. J Thorac Cardiovasc Surg, 2014, 148(5): 2235-2244.
10. Santoro G, Capozzi G, Giordano M, et al. Fate of duct-dependent, discontinuous pulmonary arteries after arterial duct stenting. Pediatr Cardiol, 2017, 38(7): 1370-1376.
11. Ishii Y, Miyamoto T, Kobayashi T, et al. Isolated unilateral agenesis of the pulmonary artery: Surgical repair with an artificial graft. Pediatr Cardiol, 2011, 32(4): 509-511.
12. Yan X, Cen J, Luo X, et al. Surgical repair of unilateral absence of pulmonary artery in children with pulmonary hypertension: A single-center retrospective study. Transl Pediatr, 2022, 11(11): 1813-1822.
13. Green GE, Reppert EH, Cohlan SQ, et al. Surgical correction of absence of proximal segment of left pulmonary artery. Circulation, 1968, 37(1): 62-69.
14. Welch K, Hanley F, Johnston T, et al. Isolated unilateral absence of right proximal pulmonary artery: Surgical repair and follow-up. Ann Thorac Surg, 2005, 79(4): 1399-1402.
15. Kosaka Y, Kurosawa H, Hoshino S, et al. Surgery for unilateral absence of pulmonary artery using autologous tissue. Ann Thorac Surg, 2003, 76(4): 1281-1283.
16. Agrawal H, Petit CJ, Miro J, et al. Contralateral pulmonary hypertension following resuscitation of unilateral ductal origin of a pulmonary artery: A multi-institutional review. Pediatr Cardiol, 2018, 39(1): 71-78.
17. Kim GB, Ban JE, Bae EJ, et al. Rehabilitation of pulmonary artery in congenital unilateral absence of intrapericardial pulmonary artery. J Thorac Cardiovasc Surg, 2011, 141(1): 171-178.

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Chinese expert consensus on surgical treatment of congenital heart disease: Unilateral absence of a pulmonary artery

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