Modified intra/extracardiac conduit total cavopulmonary connection for the treatment of complex congenital heart disease_Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Authors：

HUANG Jianbing , MEI Ju , ZHANG Junwen , ZHANG Yunjiao , ZHANG Li ,  DING Fangbao

Department of Cardiothoracic Surgery, Xinhua Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, 200092, P.R.China;

Corresponding author：

DING Fangbao, Email: 40821247@qq.com

Keywords：

Complex congenital heart disease; total cavopulmonary connection; intra/extracardiac conduit

DOI：

10.7507/1007-4848.201703052

Video：

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Objective To investigate the surgery experience of modified intra/extracardiac conduit total cavopulmonary connection (TCPC). Methods We retrospectively analyzed clinical data of 47 patients of complex congenital heart disease undergoing intra/extracardiac conduit total cavopulmonary connection in our hospital between January 2008 and December 2015. There were 29 males and 18 females with a median age of 7 years (range 4 to 9 years) and median body weight of 22 kg (range 14 to 38 kg). The heart echocardiography and cardiac imaging confirmed diagnosis suitable for TCPC surgery. Results There was no early death in the whole group. The mean pulmonary arterial pressure was 16 (12–20) mm Hg and the ventilation time was 14 (7–97) h. The main complications were intractable pleural effusion in 7 patients, low cardiac output syndrome in 3 patients, repeated supraventricular tachycardia in 1 patient. All the patients recovered after treatment. At the end of discharge, the percutaneous oxygen saturation was 85%–96% (92.6%±3.3%). The echocardiography showed the conduit pressure was 0–2 mm Hg. Patients were followed up for 1 to 7 years. Three patients were lost. One patient had intestinal nutrition loss, receving repeated pleural effusion, the treatment was ineffective, died after 4 years. Four patients of repeated pleural effusion improved after treatment. One patient repeated attacks supraventricular tachycardia within 1 year, controlled by amiodaronum, already stopped about 28 months. No recurrence occurred. All survivors were in New York Heart Association (NYHA) functional class Ⅰ or Ⅱ, with good activity tolerance. Conclusion The modified intra/extracardiac conduit TCPC combines the advantages of both the lateral tunnel and the extracardiac conduit. The operation is simple, used in the treatment of complex congenital heart disease. The short-term and mid-term results are encouraging.

Citation： HUANG Jianbing, MEI Ju, ZHANG Junwen, ZHANG Yunjiao, ZHANG Li, DING Fangbao. Modified intra/extracardiac conduit total cavopulmonary connection for the treatment of complex congenital heart disease. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2018, 25(4): 303-306. doi: 10.7507/1007-4848.201703052 Copy

1.	Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax, 1971, 26(3): 240-248.
2.	Puga FJ, Chiavarelli M, Hagler DJ. Modifications of the Fontan operation applicable to patients with left atrioventricular valve atresia or single atrioventricular valve. Circulation, 1987, 76(2): 53-60.
3.	DeLeval MR, Kilner P, Gewillig M, et al. Total cavopulmonary connection: a logical ahemative to atriopulmonary connection for eomplex Fontan operations. J Thorac Cardiovasc Surg, 1988, 96(5): 682-695.
4.	Humes RA, Feldt RH, Porter CJ, et al. The modified Fontan operation for asplenia and polysplenia syndromes. J Thoraci Cardiovasc Surg, 1988, 96(2): 212-218.
5.	Marcelletti C, Como A, Giannico S, et al. Inferior vena cava-pulmonary artery extracardiac conduit. A new form of right heart bypass. J Thorac Cardiovasc Surg, 1990, 100(2): 228-232.
6.	Gewillig M. The Fontan circulation. Heart, 2005, 91(6): 839-846.
7.	d’Udekem Y, Iyengar AJ, Cochrane AD, et al. The Fontan procedure: contemporary techniques have improved long-term outcomes. Circulation, 2007, 116(11 Suppl): I157-I164.
8.	Robbersvisser D, Miedema M, Nijveld A, et al. Results of staged total cavopulmonary connection for functionally univentricular hearts; comparison of intra-atrial lateral tunnel and extracardiac conduit. Eur J Cardiothorac Surg, 2010, 37(4): 934-941.
9.	Mondésert B, Marcotte F, Mongeon FP, et al. Fontan circulation: success or failure? Canadian J Cardiol, 2013, 29(7): 811-820.
10.	Gewillig M, Goldberg DJ. Failure of the Fontan circulation. Heart Fail Clin, 2014, 10(1): 105-116.
11.	Robbers VD, Miedema MA, Boersma E, et al. Results of staged total cavopulmonary connection for functionally univentricular hearts; comparison of intra-atrial lateral tunnel and extracardiac conduit. Eur J Cardiothorac Surg, 2010, 37(4): 934-941.
12.	Backer CL, Deal BJ, Kaushal S, et al. Extracardiac versus intra-atrial lateral tunnel Fontan: extracardiac is better. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu, 2011, 14(1): 4-10.
13.	Kim SJ, Kim WH, Lim HG, et al. Outcome of 200 patients after an extracardiac Fontan procedure. J Thorac Cardiovasc Surg, 2008, 136(1): 108-116.
14.	Davies RR, Chen JM, Mosca RS. The Fontan procedure: evolution in technique; attendant imperfections and transplantation for “failure”. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu, 2011, 14(1): 55-66.
15.	Walsh EP. Interventional electrophysiology in patients with congenital heart disease. Circulation, 2007, 115(25): 3224-3234.
16.	Michielon G, Gharagozloo F, Julsrud PR, et al. Modified Fontan operation in the presence of anomalies of systemic and pulmonary venous connection. Circulation, 1993, 88(5 Pt 2): II141-II148.
17.	Jonas RA. The intra/extracardiac conduit fenestrated Fontan. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu, 2011, 14(1): 11-18.

1. Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax, 1971, 26(3): 240-248.
2. Puga FJ, Chiavarelli M, Hagler DJ. Modifications of the Fontan operation applicable to patients with left atrioventricular valve atresia or single atrioventricular valve. Circulation, 1987, 76(2): 53-60.
3. DeLeval MR, Kilner P, Gewillig M, et al. Total cavopulmonary connection: a logical ahemative to atriopulmonary connection for eomplex Fontan operations. J Thorac Cardiovasc Surg, 1988, 96(5): 682-695.
4. Humes RA, Feldt RH, Porter CJ, et al. The modified Fontan operation for asplenia and polysplenia syndromes. J Thoraci Cardiovasc Surg, 1988, 96(2): 212-218.
5. Marcelletti C, Como A, Giannico S, et al. Inferior vena cava-pulmonary artery extracardiac conduit. A new form of right heart bypass. J Thorac Cardiovasc Surg, 1990, 100(2): 228-232.
6. Gewillig M. The Fontan circulation. Heart, 2005, 91(6): 839-846.
7. d’Udekem Y, Iyengar AJ, Cochrane AD, et al. The Fontan procedure: contemporary techniques have improved long-term outcomes. Circulation, 2007, 116(11 Suppl): I157-I164.
8. Robbersvisser D, Miedema M, Nijveld A, et al. Results of staged total cavopulmonary connection for functionally univentricular hearts; comparison of intra-atrial lateral tunnel and extracardiac conduit. Eur J Cardiothorac Surg, 2010, 37(4): 934-941.
9. Mondésert B, Marcotte F, Mongeon FP, et al. Fontan circulation: success or failure? Canadian J Cardiol, 2013, 29(7): 811-820.
10. Gewillig M, Goldberg DJ. Failure of the Fontan circulation. Heart Fail Clin, 2014, 10(1): 105-116.
11. Robbers VD, Miedema MA, Boersma E, et al. Results of staged total cavopulmonary connection for functionally univentricular hearts; comparison of intra-atrial lateral tunnel and extracardiac conduit. Eur J Cardiothorac Surg, 2010, 37(4): 934-941.
12. Backer CL, Deal BJ, Kaushal S, et al. Extracardiac versus intra-atrial lateral tunnel Fontan: extracardiac is better. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu, 2011, 14(1): 4-10.
13. Kim SJ, Kim WH, Lim HG, et al. Outcome of 200 patients after an extracardiac Fontan procedure. J Thorac Cardiovasc Surg, 2008, 136(1): 108-116.
14. Davies RR, Chen JM, Mosca RS. The Fontan procedure: evolution in technique; attendant imperfections and transplantation for “failure”. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu, 2011, 14(1): 55-66.
15. Walsh EP. Interventional electrophysiology in patients with congenital heart disease. Circulation, 2007, 115(25): 3224-3234.
16. Michielon G, Gharagozloo F, Julsrud PR, et al. Modified Fontan operation in the presence of anomalies of systemic and pulmonary venous connection. Circulation, 1993, 88(5 Pt 2): II141-II148.
17. Jonas RA. The intra/extracardiac conduit fenestrated Fontan. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu, 2011, 14(1): 11-18.

Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Modified intra/extracardiac conduit total cavopulmonary connection for the treatment of complex congenital heart disease

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