Modified Fontan Procedure for Complex Congenital Heart Diseases_Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Authors：

ZOUMing-hui , CUIHu-jun , MALi , XIAYuan-sheng , YANGSheng-chun , CHENWei-dan , CUIYan-qin ,  CHENXin-xin

Heart Center, Guangzhou Women and Children's Medical Center, Guangzhou 510623, P. R. China;

Corresponding?author：

CHENXin-xin, Email: zingerchen@163.com

Keywords：

Fontan procedure; Congenital heart disease; Fenestration

DOI：

10.7507/1007-4848.20150012

Video：

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Objective To review our clinical experience of modified Fontan procedure for complex congenital heart disease (CHD) in children. Methods Clinical data of 62 children with complex CHD who underwent modified Fontan procedure in Guangzhou Women and Children's Medical Center from May 2008 to December 2013 were retrospectively analyzed. There were 41 male and 21 female patients with their median age of 4 years(range, 16 months to 14 years) and body weight of 12.5 (8.9-49.5) kg. Diagnosis included functional single ventricle in 45 patients, transposition of the great arteries (TGA) with ventricular septal defect (VSD) and left ventricular outflow tract obstruction (LVOTO) in 6 patients, corrected TGA with VSD and LVOTO in 6 patients, double outlet right ventricle with severe pulmonary stenosis in 4 patients, and right ventricular dysplasia in 1 patient. Previous procedure included pulmonary artery banding in 10 patients, unilateral bidirectional Glenn shunt in 37 patients, and bilateral bidirectional Glenn shunt in 8 patients. Seventeen patients received single-stage modified Fontan procedure, and 45 patients received two-stage modified Fontan procedure. Extracardiac conduit was used in 56 patients, and lateral tunnel was used in 6 patients. Concomitant procedures included fenestration in 41 patients, atrioventricular valvuloplasty in 6 patients, and pulmonary artery angioplasty in 3 patients. Results Two patients in both single-stage and two-stage groups died postoperatively with mortality of 11.8% and 4.4% respectively (P=0.299). Postoperative mechanical ventilation time, length of ICU stay, chestdrainage duration, postoperative hospital stay and morbidity were not statistically different between single-stage and two-stage group. Mean follow-up was 2.0 ±0.5 years (range, 3 months to 5 years). There were 2 late death in the singlestage group but no late death in the two-stage group. Growth of all survival children was good, and their exercise capacity significantly improved. Percutaneous oxygen saturation was higher than 90%. Echocardiography showed patent superior and inferior vena cava anastomosis without thrombosis, stenosis, atrioventricular valve regurgitation aggravation or pulmonary venous return obstruction. All survival patients were in New York Heart Association class Ⅰ or Ⅱ. None of the patients had arrhythmia, chronic effusion or protein losing enteropathy. Conclusion Early and mid-term results of modified Fontan procedure were satisfactory for children with complex CHD. For children with high risk factors, staged Fontan procedure can reduce surgical mortality.

Citation： ZOUMing-hui, CUIHu-jun, MALi, XIAYuan-sheng, YANGSheng-chun, CHENWei-dan, CUIYan-qin, CHENXin-xin. Modified Fontan Procedure for Complex Congenital Heart Diseases. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2015, 22(1): 39-43. doi: 10.7507/1007-4848.20150012 Copy

1.	?Stern HJ. Fontan "Ten Commandments" revisited and revised. Pediatr Cardiol, 2010, 31(8):1131-1134.
2.	Hosein RB, Clarke AJ, McGuirk SP, et al. Factors influencing early and late outcome following the Fontan procedure in the current era. The ‘Two Commandments’? Eur J Cardiothoracic Surg, 2007, 31(3):344-353.
3.	Petit CJ. Staged Single-ventricle palliation in 2011:outcomes and expectations. Congenit Heart Dis, 2011, 6(5):406-416.
4.	Stern HJ. Aggressive coiling of aortopulmonary collaterals in single-ventricle patients is warranted. Pediatr Cardiol, 2010, 31(4):449-453.
5.	吳清玉, 張明奎, 李洪銀, 等. 分期全腔靜脈-肺動脈連接術治療復雜先天性心臟病. 中華外科雜志, 2009, 47(7):530-533.
6.	Bridges ND, Jonas RA, Mayer JE, et al. Bidirectional cavopulmonary anastomosis as interim palliation for high-risk Fontan candidates. Early results. Circulation, 1990, 82 (5 Suppl):IV170-IV176.
7.	Trusler GA, Williams WG, Cohen AJ, et al. The cavopulmonary shunt. Evolution of a concept. Circulation, 1990, 82 (suppl IV):IV131-IV138.
8.	Pridjian AK, Mendelsohn AM, Lupinetti FM, et al. Usefulness of the bidirectional Glenn procedure as staged reconstruction for the functional single ventricle. Am J Cardiol, 1993, 71(11):959-962.
9.	Masuda M, Kado H, Shiokawa Y, et al. Clinical results of the staged Fontan procedure in high-risk patients. Ann Thorac Surg, 1998, 65(6):1721-1725.
10.	鄭景浩, 徐志偉, 蘇肇伉, 等. 二期Fontan 術治療小兒復雜先天性心臟病. 中華胸心血管外科雜志, 2007, 23(2):97-99.
11.	Backer CL, Deal BJ, Mavroudis C, et al. Extracardiac versus intraatrial lateral tunnel Fontan:extracardiac is better. Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann, 2011, 14(1):4-10.
12.	Jonas RA.The intra/extracardiac conduit fenestrated Fontan. Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann, 2011, 14(1):11-18.
13.	Kogon B. Is the extracardiac conduit the preferred Fontan approach for patients with univentricular hearts? The extracardiac conduit is the preferred Fontan approach for patients with univentricular hearts. Circulation, 2012, 126(21):2511-2515.
14.	Khairy P, Poirier N. Is the extracardiac conduit the preferred Fontan approach for patients with univentricular hearts? The extracardiac conduit is not the preferred Fontan approach for patients with univentricular hearts. Circulation, 2012, 126(21):2516-2525.
15.	Kreutzer C, Schlichter AJ, Simon JL, et al. A new method for reliable fenestration in extracardiac conduit Fontan operations. Ann Thorac Surg, 2003, 75(5):1657-1659.
16.	Ocello S, Salviato N, Marcelletti CF. Results of 100 consecutive extracardiac conduit Fontan operations. Pediatr Cardiol, 2007, 28(6):433-437.
17.	O'Brien JE Jr, Marshall JA, Young AR, et al. The nonfenestrated extracardiac Fontan procedure:a cohort of 145 patients. Ann Thorac Surg, 2010, 89(6):1815-1820.
18.	Thompson LD, Petrossian E, McElhinney DB, et al. Is it necessary to routinely fenestrate an Extracardiac Fontan? J Am Coll Cardiol, 1999, 34(2):539-544.
19.	Lemler MS, Scott WA, Leonard SR, et al. Fenestration improves clinical outcome of the fontan procedure:a prospective, randomized study. Circulation, 2002, 105(2):207-212.
20.	朱宏斌, 鄭景浩, 劉錦紛, 等. Fontan 術后頑固性胸腔積液相關因素分析及處理. 中華胸心血管外科雜志, 2005, 21(3):173-174.
21.	Salazar JD, Zafar F, Siddiqui K, et al. Fenestration during Fontan palliation:Now the exception instead of the rule. J Thorac Cardiovasc Surg, 2010, 140(1):129-136.
22.	Jonas RA, Castanada AR. Modified Fontan procedure:atrial buffle and systemic venous to pulmonary artery anastomotic techniques. J Card Surg, 1988, 3(2):91-96.
23.	李世國, 蔣世良, 徐仲英, 等. 經導管封堵全腔靜脈肺動脈連接術外管道開窗的初步臨床應用. 中華心血管病雜志, 2008, 36(6):489-492.

1. ?Stern HJ. Fontan "Ten Commandments" revisited and revised. Pediatr Cardiol, 2010, 31(8):1131-1134.
2. Hosein RB, Clarke AJ, McGuirk SP, et al. Factors influencing early and late outcome following the Fontan procedure in the current era. The ‘Two Commandments’? Eur J Cardiothoracic Surg, 2007, 31(3):344-353.
3. Petit CJ. Staged Single-ventricle palliation in 2011:outcomes and expectations. Congenit Heart Dis, 2011, 6(5):406-416.
4. Stern HJ. Aggressive coiling of aortopulmonary collaterals in single-ventricle patients is warranted. Pediatr Cardiol, 2010, 31(4):449-453.
5. 吳清玉, 張明奎, 李洪銀, 等. 分期全腔靜脈-肺動脈連接術治療復雜先天性心臟病. 中華外科雜志, 2009, 47(7):530-533.
6. Bridges ND, Jonas RA, Mayer JE, et al. Bidirectional cavopulmonary anastomosis as interim palliation for high-risk Fontan candidates. Early results. Circulation, 1990, 82 (5 Suppl):IV170-IV176.
7. Trusler GA, Williams WG, Cohen AJ, et al. The cavopulmonary shunt. Evolution of a concept. Circulation, 1990, 82 (suppl IV):IV131-IV138.
8. Pridjian AK, Mendelsohn AM, Lupinetti FM, et al. Usefulness of the bidirectional Glenn procedure as staged reconstruction for the functional single ventricle. Am J Cardiol, 1993, 71(11):959-962.
9. Masuda M, Kado H, Shiokawa Y, et al. Clinical results of the staged Fontan procedure in high-risk patients. Ann Thorac Surg, 1998, 65(6):1721-1725.
10. 鄭景浩, 徐志偉, 蘇肇伉, 等. 二期Fontan 術治療小兒復雜先天性心臟病. 中華胸心血管外科雜志, 2007, 23(2):97-99.
11. Backer CL, Deal BJ, Mavroudis C, et al. Extracardiac versus intraatrial lateral tunnel Fontan:extracardiac is better. Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann, 2011, 14(1):4-10.
12. Jonas RA.The intra/extracardiac conduit fenestrated Fontan. Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann, 2011, 14(1):11-18.
13. Kogon B. Is the extracardiac conduit the preferred Fontan approach for patients with univentricular hearts? The extracardiac conduit is the preferred Fontan approach for patients with univentricular hearts. Circulation, 2012, 126(21):2511-2515.
14. Khairy P, Poirier N. Is the extracardiac conduit the preferred Fontan approach for patients with univentricular hearts? The extracardiac conduit is not the preferred Fontan approach for patients with univentricular hearts. Circulation, 2012, 126(21):2516-2525.
15. Kreutzer C, Schlichter AJ, Simon JL, et al. A new method for reliable fenestration in extracardiac conduit Fontan operations. Ann Thorac Surg, 2003, 75(5):1657-1659.
16. Ocello S, Salviato N, Marcelletti CF. Results of 100 consecutive extracardiac conduit Fontan operations. Pediatr Cardiol, 2007, 28(6):433-437.
17. O'Brien JE Jr, Marshall JA, Young AR, et al. The nonfenestrated extracardiac Fontan procedure:a cohort of 145 patients. Ann Thorac Surg, 2010, 89(6):1815-1820.
18. Thompson LD, Petrossian E, McElhinney DB, et al. Is it necessary to routinely fenestrate an Extracardiac Fontan? J Am Coll Cardiol, 1999, 34(2):539-544.
19. Lemler MS, Scott WA, Leonard SR, et al. Fenestration improves clinical outcome of the fontan procedure:a prospective, randomized study. Circulation, 2002, 105(2):207-212.
20. 朱宏斌, 鄭景浩, 劉錦紛, 等. Fontan 術后頑固性胸腔積液相關因素分析及處理. 中華胸心血管外科雜志, 2005, 21(3):173-174.
21. Salazar JD, Zafar F, Siddiqui K, et al. Fenestration during Fontan palliation:Now the exception instead of the rule. J Thorac Cardiovasc Surg, 2010, 140(1):129-136.
22. Jonas RA, Castanada AR. Modified Fontan procedure:atrial buffle and systemic venous to pulmonary artery anastomotic techniques. J Card Surg, 1988, 3(2):91-96.
23. 李世國, 蔣世良, 徐仲英, 等. 經導管封堵全腔靜脈肺動脈連接術外管道開窗的初步臨床應用. 中華心血管病雜志, 2008, 36(6):489-492.

Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Modified Fontan Procedure for Complex Congenital Heart Diseases

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