JOURNAL OF SHANDONG UNIVERSITY (HEALTH SCIENCES) ›› 2015, Vol. 53 ›› Issue (11): 41-45.doi: 10.6040/j.issn.1671-7554.0.2015.404

• Clinical Medicine • Previous Articles     Next Articles

Initial palliative repair of pulmonary atresia with ventricular septal defect by rehabilitating right ventricle to pulmonary artery connection

FENG Zhiyu, GUO Rui, YANG Yanliang, LI Haiming, DONG Mingliang, XU Huihui, LIU Yimin, GUO Kai, WANG Guangzhen, WANG Hui   

  1. Department of Cardiac Surgery, Qilu Children s Hospital of Shandong University, Jinan 250022, Shandong, China
  • Received:2015-04-04 Online:2015-11-10 Published:2015-11-10

Abstract: Objective To assess the efficacy and safety of right ventricle to pulmonary artery connection as an initial procedure in patients with pulmonary atresia and ventricular septal defect. Methods Clinical data of 8 patients (mean age 3.2±4.7 months, range 10 days-11 months) with pulmonary atresia, ventricular septal defect and pulmonary artery hypoplasia (McGoon ratio 0.94±0.27) treated during Nov. 2013 and Jan. 2015 were retrospectively reviewed. Connection of right ventricle to pulmonary artery was constructed to rehabilitate the anterior blood flow. All patients were carefully followed up with echocardiography at 3-month intervals after the procedure. When pulmonary artery achieved satisfactory growth and McGoon index was considered adequate ratio reached the criterion of curative resection, a complete repair was performed. Results No early deaths occurred. During the follow-up of 3 to 10 months (mean 8.5±3.1 months), significant pulmonary artery growth was obtained (McGoon ratio 1.21±0.35, P=0.01) in all patients. One case whose McGoon ratio reached 1.63 after 13 months received complete repair. Another case with severe cyanotic spells 12 months after the initial palliation whose McGoon ratio was 1.27 also underwent anatomic repair. Postoperative recovery was uneventfully with satisfactory haemodynamics. At the last visit, the cardiac function was grade Ⅰ in New York Heart Association. The other 6 patients were still awaiting complete repair with a satisfactory pulmonarytree growth at last evaluation. Conclusion Early rehabilitating anterior blood flow by connecting right ventricle and pulmonary artery yields a relatively low morbidity and mortality, with good functional results in patients with pulmonary atresia, ventricular septal defect and hypoplastic native pulmonary arteries. Ultimately, significant pulmonary artery growth can be obtained and this strategy allows complete repair in majority of patients.

Key words: Treatment outcome, Palliative care, Pulmonary atresia with ventricular septal defect, Cardiac surgical procedures, Right ventricle to pulmonary artery connection

CLC Number: 

  • R654
[1] Fukui D, Kai H, Takeuchi T, et al. Longest survivor of pulmonary atresia with ventricular septal defect:well-developed major aortopulmonary collateral arteries demonstrated by multidetector computed tomography[J]. Circulation, 2011, 124(19):2155-2157.
[2] Rome JJ, Mayer JE, Castaneda AR, et al. Tetralogy of Fallot with pulmonary atresia. Rehabilitation of diminutive pulmonary arteries[J]. Circulation, 1993, 88(4 Pt 1):1691-1698.
[3] Reddy VM, Liddicoat JR, Hanley FL. Midline one-stage complete unifocalization and repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals[J]. J Thorac Cardiovasc Surg, 1995, 109(5):832-844.
[4] Watanabe N, Mainwaring RD, Reddy VM, et al. Early complete repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals[J]. Ann Thorac Surg, 2014,97(3): 909-915.
[5] Jacquemart C, Damry N, Massin MM. Long-term natural history of aorto-pulmonary collaterals in pulmonary atresia[J]. Acta Cardiol, 2014, 69(6):695-696.
[6] Norgaard MA, Alphonso N, Cochrane AD, et al. Major aorto-pulmonary collateral arteries of patients with pulmonary atresia and ventricular septal defect are dilated bronchial arteries[J]. Eur J Cardiothorac Surg, 2006, 29(5):653-658.
[7] Song SW, Park HK, Park YH, et al. Pulmonary atresia with ventricular septal defects and major aortopulmonary collateral arteries[J]. Circ J, 2009, 73(3):516-522.
[8] Gupta A, Odim J, Levi D, et al. Staged repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: experience with 104 patients[J]. J Thorac Cardiovasc Surg, 2003, 126(6):1746-1752.
[9] d'Udekem Y, Alphonso N, Norgaard MA, et al. Pulmonary atresia with ventricular septal defects and major aortopulmonary collateral arteries: unifocalization brings no long-term benefits[J]. J Thorac Cardiovasc Surg, 2005, 130(6):1496-1502.
[10] Liava'a M, Brizard CP, Konstantinov IE, et al. Pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals: neonatal pulmonary artery rehabilitation without unifocalization[J]. Ann Thorac Surg, 2012, 93(1):185-191.
[11] Gerelli S, van Steenberghe M, Murtuza B, et al. Neonatal right ventricle to pulmonary connection as a palliative procedure for pulmonary atresia with ventricular septal defect or severe tetralogy of Fallot[J]. Eur J Cardiothorac Surg, 2014, 45(2): 278-288.
[12] Fang M, Wang H, Jin Y, et al. Development of pulmonary arteries after a central end-to-side shunt in patients with pulmonary atresia, ventricular septal defect, and diminutive pulmonary arteries[J]. Thorac Cardiovasc Surg, 2014, 62(3):211-215.
[13] Fouilloux V, Bonello B, Kammache I, et al. Management of patients with pulmonary atresia, ventricular septal defect, hypoplastic pulmonary arteries and major aorto-pulmonary collaterals: Focus on the strategy of rehabilitation of the native pulmonary arteries[J]. Arch Cardiovasc Dis, 2012, 105(12):666-675.
[14] Dragulescu A, Kammache I, Fouilloux V, et al. Long-term results of pulmonary artery rehabilitation in patients with pulmonary atresia, ventricular septal defect, pulmonary artery hypoplasia, and major aortopulmonary collaterals[J]. J Thorac Cardiovasc Surg, 2011, 142(6):1374-1380.
[15] O'Connor MJ, Ravishankar C, Ballweg JA, et al. Early systemic-to-pulmonary artery shunt intervention in neonates with congenital heart disease[J]. J Thorac Cardiovasc Surg, 2011, 142(1):106-112.
[16] Williams JA, Bansal AK, Kim BJ, et al. Two thousand Blalock-Taussig shunts: a six-decade experience[J]. Ann Thorac Surg, 2007, 84(6):2070-2075.
[17] Kim H, Sung SC, Chang YH, et al. Outcome of staged sepair of tetralogy of Fallot with pulmonary atresia and a ductus-dependent pulmonary circulation: should primary repair be considered?[J]. Korean J Thorac Cardiovasc Surg, 2011, 44(6):392-398.
[18] Mumtaz MA, Rosenthal G, Qureshi A, et al. Melbourne shunt promotes growth of diminutive central pulmonary arteries in patients with pulmonary atresia, ventricular septal defect, and systemic-to-pulmonary collateral arteries[J]. Ann Thorac Surg, 2008, 85(6):2079-2083.
[19] Kim H, Sung SC, Choi KH, et al. A central shunt to rehabilitate diminutive pulmonary arteries in patients with pulmonary atresia with ventricular septal defect[J]. J Thorac Cardiovasc Surg, 2015, 149(2):515-520.
[20] Zhang Y, Hua Z, Yang K, et al. Outcomes of the rehabilitative procedure for patients with pulmonary atresia, ventricular septal defect and hypoplastic pulmonary arteries beyond the infant period[J]. Eur J Cardiothorac Surg, 2014, 46(2):297-303.
[21] 郑景浩, 徐志伟, 刘锦纷, 等. 肺动脉闭锁合并室间隔缺损和MAPCA手术理念探讨[J]. 中华胸心血管外科杂志, 2014, 30(5):257-260. ZHENG Jinghao, XU Zhiwei, LIU Jinfen, et al. The research of operation concept in pulmonary atresia with ventricular septai defect and major aortopulmonary collaterals[J]. Chin J Thorac Cardiovasc Surg, 2014, 30(5):257-260.
[1] FENG Zhiyu, YANG Yanliang, GUO Rui, XU Huihui, ZHANG Jian, GUO Kai, WANG Guangzhen, WANG Hui. Diagnosis and surgical treatment of pulmonary artery sling complicated with tracheal stenosis [J]. JOURNAL OF SHANDONG UNIVERSITY (HEALTH SCIENCES), 2017, 55(8): 48-51.
[2] GAO Jianbu, YANG Shouzhong, LI Yudong, QIAO Chong, ZHANG Songyu. Trend survey of inpatient use of drugs for acute myocardial infarction during 10 years in a hospital of Nanyang City [J]. JOURNAL OF SHANDONG UNIVERSITY (HEALTH SCIENCES), 2017, 55(5): 66-69.
[3] KANG Ma-fei, BU Qing, LIU Ying, LUO Mei-qing, LIAO Li-li, TU Jiang-jiang. Pemetrexed alone or in combination with nedaplatin in recurrence and carboplatin/paclitaxel resistance advanced epithelial ovarian carcinoma [J]. JOURNAL OF SHANDONG UNIVERSITY (HEALTH SCIENCES), 2011, 49(5): 122-124.
[4] Niyaz·Hurshidam, ZHAO Hua-rong, Abdulla·Mangnisha, BAO Yong-xing. A randomized clinical trial in comparison of concurrent single agent cisplatin, cisplatin in combination with 5-fluorouracil and cisplatin in combination  with taxel for locally advanced cervical squamous cell cancer [J]. JOURNAL OF SHANDONG UNIVERSITY (HEALTH SCIENCES), 2011, 49(5): 128-131.
[5] . CT cisternography in the classification and treatment of 
childhood intracranial arachnoid cyst 
[J]. JOURNAL OF SHANDONG UNIVERSITY (HEALTH SCIENCES), 2009, 47(9): 111-113.
[6] . PAR index in evaluation of the treatment outcome of different techniques [J]. JOURNAL OF SHANDONG UNIVERSITY (HEALTH SCIENCES), 2009, 47(7): 78-80.
[7] ZHANG Shuai,LI Jian-min,FENG Rong-jie,LI Zhen-feng,LIU Wen-guang,LIU Liang,LI Dong,YANG Jing-yan. Long-term effect of massive allograft bones implantation on defects: a more than 20 years observation [J]. JOURNAL OF SHANDONG UNIVERSITY (HEALTH SCIENCES), 2008, 46(3): 301-304.
[8] QU Yi,ZHANG Ying,ZHANG Xiao,ZHOU Fang,XU Xiao-yi. Effect of octreotide combined with transpupillary thermotherapy on experimental choroidal neovascularization [J]. JOURNAL OF SHANDONG UNIVERSITY (HEALTH SCIENCES), 2008, 46(10): 941-944.
[9] LIU Zhi-rong,WANG Lei,LU Hui,YU Shu-li. Changes of hepatic alpha-SMA and TIMP-1 induced by interferon-alpha-2b combined with ribavirin in patients with chronic hepatitis C [J]. JOURNAL OF SHANDONG UNIVERSITY (HEALTH SCIENCES), 2007, 45(4): 365-368.
[10] WANG Zhi-ang,DING Xua,SUN Peng/sup>,WANG Cheng-wei,HAO Xiao-guang,PAN Shun. Role of pre-operative cerebral angiography in cerebral artery stenosis stent angioplasty [J]. JOURNAL OF SHANDONG UNIVERSITY (HEALTH SCIENCES), 2007, 45(2): 146-148.
[11] LI Yue-ua,GUO Feng,GONG Xing-un,PANG Xin-an. Myocardioprotective effect of adenosine preconditioning on open heart surgery [J]. JOURNAL OF SHANDONG UNIVERSITY (HEALTH SCIENCES), 2006, 44(8): 798-801.
[12] ZHANG Zhe,PENG Zhong-min,CHEN Jing-han,MENG Long,DU Jia-jun.WANG Lei,WANG Xiao-hang,ZHANG Lin. [J]. JOURNAL OF SHANDONG UNIVERSITY (HEALTH SCIENCES), 2006, 44(10): 1075-1077.
Viewed
Full text


Abstract

Cited

  Shared   
  Discussed   
No Suggested Reading articles found!