Surgical Treatment of Rarely Absent Pulmonary Valve Syndrome_Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Authors：

靳永強 , 李洪銀 ,  吳清玉

清華大學醫學院清華大學第一附屬醫院心臟外科(北京 100016);

Corresponding?author：

吳清玉, Email: wuqingyu@mail.tsinghua.edu.cn

Keywords：

肺動脈瓣缺如綜合征; 右室雙出口; 外科手術

DOI：

10.7507/1007-4848.20160122

Video：

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Abstract Full text Figures/Tables Video References Cited by

目的探討罕見肺動脈瓣缺如綜合征（absent pulmonary valve syndrome，APVS）的外科治療效果。方法回顧性分析3例APVS患者在低溫體外循環下行根治手術，其中女2例、男1例，年齡分別為9個月、14歲、10歲，其中2例合并右室雙出口（double outlet right ventricle，DORV）。3例均采用自體心包片重建肺動脈瓣，1例行肺動脈成形術，2例未處理肺動脈。結果 3例均存活，術中食管超聲及術后超聲復查肺動脈瓣功能良好，隨訪8～24個月，均恢復良好、生活質量明顯改善，復查心臟彩色超聲心動圖肺動脈瓣功能良好，無明顯反流。結論 APVS是一種少見的先天性心臟病，合并DORV或心內其他結構正常的病例非常罕見，手術治療可獲得良好效果。

Citation： 靳永強, 李洪銀, 吳清玉. Surgical Treatment of Rarely Absent Pulmonary Valve Syndrome. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2016, 23(5): 518-520. doi: 10.7507/1007-4848.20160122 Copy

1.	Matthew SY, Deane Y, Christian PB, et al. Long-term outcomes of patients with absent pulmonary valve syndrome:38 years of experience. Ann Thorac Surg, 2014, 97(5):1671-1677.
2.	Chevers N. Recherches sur les maladies de I'artere pulmonaire. Arch Gen Mde, 1847(15):489-510.
3.	Brown JW, Ruzmetov M, Vijay P, et al. Surgical treatment of absent pulmonary valve syndrome associated with bronchial obstruction. Ann Thorac Surg, 2006, 82(6):2221-2226.
4.	Kreutzer C, Schlichter A, Kreutzer G, et al. Tetralogy of Fallot with absent pulmonary valve:A surgical technique for complete repair. Thorac Cardiovasc Surg, 1999, 117(1):192-194.
5.	Staffor EG, Mair DD, McGoon DC, et al. Tetralogy of Fallot with absent pulmonary valve:surgical considerations and results. Circulation, 1973, 48(suppl 1):Ⅲ 24-30.
6.	Godart F, Houyel L, Lacour-Gayet F, et al. Absent pulmonary valve syndrome:surgical treatment and considerations. Ann Thorac Surg, 1996, 62(1):136-142.
7.	McDonnell BE, Raff GW, Gaynor JW, et al. Outcome after repair of tetralogy of Fallot with absent pulmonary valve. Ann Thorac Surg, 1999, 67(5):1391-1395.
8.	張懷軍, 許建屏, 吳清玉, 等. 法樂四聯癥合并肺動脈瓣缺如的矯治. 中國體外循環雜志, 2000, 15(5):296-297.
9.	Watterson KG, Malm TK, Karl TR, et al. Absent pulmonary valve syndrome (operation in infants with airway obstruction). Ann Thorac Surg, 1992, 54(6):1116-1119.
10.	Lars N, Anthony A, Petrous VA, et al. The lecompte maneuver for relief of airway compression in absent pulmonary valve syndrome. Ann Thorac Surg, 2006, 81(6):1802-1807.
11.	Alsoufi B, Williams WG, Hua Z, et al. Surgical outcomes in the treatment of patients with tetralogy of Fallot and absent pulmonary valve. Eur J Cardiothorac Surg, 2007, 31(3):354-359.
12.	凌雁, 郭少先, 沈向東, 等. 法洛四聯癥合并肺動脈瓣缺如患者手術矯治的近中期結果. 中國胸心血管外科臨床雜志, 2011, 18(2):114-116.
13.	Karl TR, Pincott JR, Taylor FN, et al. Surgical treatment of absent pulmonary valve syndrome. Thorac Cardiovasc Surg, 1986, 91: 590-597.
14.	丁以群, 陳寄梅, 岑堅定, 等. 肺動脈瓣缺如綜合癥的外科治療. 中華心血管外科雜志, 2013, 29(2):65-68.
15.	Chen JM, Glickstein JS, Margossian R, et al. Superior outcomes for repair in infants and neonates with tetralogy of Fallot with absent pulmonary valve syndrome. Thorac Cardiovasc Surg, 2006, 132(5):1099-1104.

1. Matthew SY, Deane Y, Christian PB, et al. Long-term outcomes of patients with absent pulmonary valve syndrome:38 years of experience. Ann Thorac Surg, 2014, 97(5):1671-1677.
2. Chevers N. Recherches sur les maladies de I'artere pulmonaire. Arch Gen Mde, 1847(15):489-510.
3. Brown JW, Ruzmetov M, Vijay P, et al. Surgical treatment of absent pulmonary valve syndrome associated with bronchial obstruction. Ann Thorac Surg, 2006, 82(6):2221-2226.
4. Kreutzer C, Schlichter A, Kreutzer G, et al. Tetralogy of Fallot with absent pulmonary valve:A surgical technique for complete repair. Thorac Cardiovasc Surg, 1999, 117(1):192-194.
5. Staffor EG, Mair DD, McGoon DC, et al. Tetralogy of Fallot with absent pulmonary valve:surgical considerations and results. Circulation, 1973, 48(suppl 1):Ⅲ 24-30.
6. Godart F, Houyel L, Lacour-Gayet F, et al. Absent pulmonary valve syndrome:surgical treatment and considerations. Ann Thorac Surg, 1996, 62(1):136-142.
7. McDonnell BE, Raff GW, Gaynor JW, et al. Outcome after repair of tetralogy of Fallot with absent pulmonary valve. Ann Thorac Surg, 1999, 67(5):1391-1395.
8. 張懷軍, 許建屏, 吳清玉, 等. 法樂四聯癥合并肺動脈瓣缺如的矯治. 中國體外循環雜志, 2000, 15(5):296-297.
9. Watterson KG, Malm TK, Karl TR, et al. Absent pulmonary valve syndrome (operation in infants with airway obstruction). Ann Thorac Surg, 1992, 54(6):1116-1119.
10. Lars N, Anthony A, Petrous VA, et al. The lecompte maneuver for relief of airway compression in absent pulmonary valve syndrome. Ann Thorac Surg, 2006, 81(6):1802-1807.
11. Alsoufi B, Williams WG, Hua Z, et al. Surgical outcomes in the treatment of patients with tetralogy of Fallot and absent pulmonary valve. Eur J Cardiothorac Surg, 2007, 31(3):354-359.
12. 凌雁, 郭少先, 沈向東, 等. 法洛四聯癥合并肺動脈瓣缺如患者手術矯治的近中期結果. 中國胸心血管外科臨床雜志, 2011, 18(2):114-116.
13. Karl TR, Pincott JR, Taylor FN, et al. Surgical treatment of absent pulmonary valve syndrome. Thorac Cardiovasc Surg, 1986, 91: 590-597.
14. 丁以群, 陳寄梅, 岑堅定, 等. 肺動脈瓣缺如綜合癥的外科治療. 中華心血管外科雜志, 2013, 29(2):65-68.
15. Chen JM, Glickstein JS, Margossian R, et al. Superior outcomes for repair in infants and neonates with tetralogy of Fallot with absent pulmonary valve syndrome. Thorac Cardiovasc Surg, 2006, 132(5):1099-1104.

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