Features of Cardiacvascular Abnormalities and Clinical Results in Patients with Fontan Operation for Heterotaxy Syndrome_Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Authors：

LIUWei , LIUJin-fen , ZHANGHai-bo , HONGHai-fa , SUNQi ,  CHENHui-wen

Department of Pediatric Cardiovascular and Thoracic Surgery, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai 200127, P. R. China;

Corresponding author：

CHENHui-wen, Email: hwhwhc@hotmail.com

Keywords：

Heterotaxy syndrome; Fontan procedure; Cardiacvascular abnormalities

DOI：

10.7507/1007-4848.20150183

Video：

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Objective To summarize the cardiac-vascular abnormalities and clinical results in patients with Fontan operation for heterotaxy syndrome. Method We retrospectively analyzed the medical records of 81 patients who underwent the Fontan operation with heterotaxy syndrome between September 2008 and September 2013. There were 49 male and 32 female patients at age of 3.79 (range 2.07-13.02) years with preoperative room air saturation of 81% (range 63%-97%) and weight of 14.8 (10.0-36.0) kg. Results There were 70 patients in the right atrial isomerism group and left in 11 patients. Dextrocardia was seen in 16 patients, and single atrium in 50 patients. Single ventricle was found in 44 patients, conotruncal defects in 40 patients, pulmonary stenosis in 72 patients, pulmonray atresia in 8 patients, common atrioventricular valve in 61 patients, and bilateral superior vena cava in 54 patients. The staged Fontan procedure was applied in 57 patients and one stage in 24 patients. Operation strategies were included intra/extracardiac conduit (n=17), extracardiac conduit (n=48), lateral tunnel (n=14) and direct cavopulmonray connection (n=2).There were 15 early deaths. Postoperative complications included low cardiac output in 15 patients, hepatic insufficiency in 35 patients, renal insufficiency in 55 patients required peritoneal dialysis and arrhythmia in 28 patients. The room air saturation was 89% (range 78%-98%) before discharge and time of follow-up was from 2 months to 6 years in 64 patients. Thromboembolic events were found in 2 patients who had accomplished conduit replacement operation. Conclusion Compared with reported literatures in western countries, heteraotaxia patients are mostly with right atrial isomerism. Fontan palliation is still the main treatment option and strict indication was needed for satisfactory clinical results.

Citation： LIUWei, LIUJin-fen, ZHANGHai-bo, HONGHai-fa, SUNQi, CHENHui-wen. Features of Cardiacvascular Abnormalities and Clinical Results in Patients with Fontan Operation for Heterotaxy Syndrome. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2015, 22(8): 729-733. doi: 10.7507/1007-4848.20150183 Copy

1.	Kim SJ. Heterotaxy syndrome. Korean Circ J, 2011, 41(5):227-232.
2.	Eronen MP, Aittomaki KA, Kajantie EO, et al. The outcome of patients with right atrial isomerism is poor. Pediatr Cardiol, 2013, 34(2):302-307.
3.	Eronen MP, Aittomaki KA, Kajantie EO, et al. Outcome of left atrial isomerism at a single institution. Pediatr Cardiol, 2012, 33(4):596-600.
4.	Cohen MS, Anderson RH, Cohen MI, et al. Controversies, genetics, diagnostic assessment, and outcomes relating to the heterotaxy syndrome. Cardiol Young, 2007, 17 (Suppl 2):29-43.
5.	Balan A, Lazoura O, Padley SP, et al. Atrial isomerism:a pictorial review. J Cardiovasc Comput Tomogr, 2012, 6(2):127-136.
6.	Qureshi AU, Kazmi U, Kazmi T, et al. Congenital heart defects associated with atrial heterotaxy. J Coll Physicians Surg Pak, 2012, 22(9):549-552.
7.	Nakayama Y, Hiramatsu T, Iwata Y, et al. Surgical results for functional univentricular heart with total anomalous pulmonary venous connection over a 25-year experience. Ann Thorac Surg, 2012, 93 (2):606-613.
8.	Anagnostopoulos PV, Pearl JM, Octave C, et al. Improved current era outcomes in patients with heterotaxy syndromes. Eur J Cardio-thorac Surg, 2009, 35(5):871-878.
9.	Bartz PJ, Driscoll DJ, Dearani JA, et al. Early and late results of the modified Fontan operation for heterotaxy syndrome 30 years of experience in 142 patients. J Am Coll Cardiol, 2006, 48(11):2301-2305.
10.	Kanter KR, Vincent RN. Management of aortopulmonary collateral arteries in Fontan patients:occlusion improves clinical outcome. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu, 2002, 5:48-54.
11.	Stewart RD, Pasquali SK, Jacobs JP, et al. Contemporary Fontan operation:association between early outcome and type of cavopulmonary connection. J Coll Physicians Surg Pak, 2012, 93(4):1254-1261.
12.	Ota N, Fujimoto Y, Murata M, et al. Improving outcomes of the surgical management of right atrial isomerism. Ann Thorac Surg, 2012, 93(3):832-839.
13.	Naito Y, Aoki M, Matsuo K, Nakajima H, et al. Intracardiac Fontan procedure for heterotaxy syndrome with complex systemic and pulmonary venous anomalies. Eur J Cardiothorac Surg, 2010, 37(1):197-203.
14.	Yildirim SV, Tokel K, Varan B, et al. Clinical investigations over.
15.	years to establish the nature of the cardiac defects in patients having abnormalities of lateralization. Cardiol Young, 2007, 17(3):275-282.
16.	De La Villeon B, Le Goudeveze S, Goudard Y, et al. Polysplenia syndrome. J Visc Surg, 2011, 148(5):e395-e396.
17.	沈蓉, 张玉奇, 蔡及明, 等. 心脾综合征的多普勒超声心电图诊断. 医学影像学杂志, 2007, 17(8):836-839.
18.	Yan YL, Tan KB, Yeo GS. Right atrial isomerism:preponderance in Asian fetuses. Using the stomach-distance ratio as a possible diagnostic tool for prediction of right atrial isomerism. Cardiol Young, 2008, 37(11):906-912.
19.	Lin JH, Chang CI, Wang JK, et al. Intrauterine diagnosis of heterotaxy syndrome. Am Heart J, 2002, 143(6):1002-1008.
20.	Berg C, Geipel A, Smrcek J, et al. Prenatal diagnosis of cardiosplenic syndromes:a 10-year experience. Ultrasound Obstet Gynecol, 2003, 22(5):451-459.
21.	Taketazu M, Lougheed J, Yoo SJ, et al. Spectrum of cardiovascular disease, accuracy of diagnosis, and outcome in fetal heterotaxy syndrome. Am J Cardiol, 2006, 97(5):720-724.
22.	张陈, 刘豫阳, 盛锋. Fontan 手术的危险因素. 国外医学科学分册, 2005, 32(2):127-129.
23.	Ota N, Fujimoto Y, Hirose K, et al. Improving results of atrioventricular valve repair in challenging patients with heterotaxy syndrome. Cardiol Young, 2010, 20(1):60-65.
24.	Foerster SR, Gauvreau K, McElhinney DB, et al. Importance of totally anomalous pulmonary venous connection and postoperative pulmonary vein stenosis in outcomes of heterotaxy syndrome. Pediatr Cardiol, 2008, 29(3):536-544.
25.	Lim DS, Graziano JN, Rocchini AP, et al. Transcatheter occlusion of aortopulmonary shunts during single-ventricle surgical palliation. Catheter Cardiovasc Interv, 2005, 65(3):427-433.

1. Kim SJ. Heterotaxy syndrome. Korean Circ J, 2011, 41(5):227-232.
2. Eronen MP, Aittomaki KA, Kajantie EO, et al. The outcome of patients with right atrial isomerism is poor. Pediatr Cardiol, 2013, 34(2):302-307.
3. Eronen MP, Aittomaki KA, Kajantie EO, et al. Outcome of left atrial isomerism at a single institution. Pediatr Cardiol, 2012, 33(4):596-600.
4. Cohen MS, Anderson RH, Cohen MI, et al. Controversies, genetics, diagnostic assessment, and outcomes relating to the heterotaxy syndrome. Cardiol Young, 2007, 17 (Suppl 2):29-43.
5. Balan A, Lazoura O, Padley SP, et al. Atrial isomerism:a pictorial review. J Cardiovasc Comput Tomogr, 2012, 6(2):127-136.
6. Qureshi AU, Kazmi U, Kazmi T, et al. Congenital heart defects associated with atrial heterotaxy. J Coll Physicians Surg Pak, 2012, 22(9):549-552.
7. Nakayama Y, Hiramatsu T, Iwata Y, et al. Surgical results for functional univentricular heart with total anomalous pulmonary venous connection over a 25-year experience. Ann Thorac Surg, 2012, 93 (2):606-613.
8. Anagnostopoulos PV, Pearl JM, Octave C, et al. Improved current era outcomes in patients with heterotaxy syndromes. Eur J Cardio-thorac Surg, 2009, 35(5):871-878.
9. Bartz PJ, Driscoll DJ, Dearani JA, et al. Early and late results of the modified Fontan operation for heterotaxy syndrome 30 years of experience in 142 patients. J Am Coll Cardiol, 2006, 48(11):2301-2305.
10. Kanter KR, Vincent RN. Management of aortopulmonary collateral arteries in Fontan patients:occlusion improves clinical outcome. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu, 2002, 5:48-54.
11. Stewart RD, Pasquali SK, Jacobs JP, et al. Contemporary Fontan operation:association between early outcome and type of cavopulmonary connection. J Coll Physicians Surg Pak, 2012, 93(4):1254-1261.
12. Ota N, Fujimoto Y, Murata M, et al. Improving outcomes of the surgical management of right atrial isomerism. Ann Thorac Surg, 2012, 93(3):832-839.
13. Naito Y, Aoki M, Matsuo K, Nakajima H, et al. Intracardiac Fontan procedure for heterotaxy syndrome with complex systemic and pulmonary venous anomalies. Eur J Cardiothorac Surg, 2010, 37(1):197-203.
14. Yildirim SV, Tokel K, Varan B, et al. Clinical investigations over.
15. years to establish the nature of the cardiac defects in patients having abnormalities of lateralization. Cardiol Young, 2007, 17(3):275-282.
16. De La Villeon B, Le Goudeveze S, Goudard Y, et al. Polysplenia syndrome. J Visc Surg, 2011, 148(5):e395-e396.
17. 沈蓉, 张玉奇, 蔡及明, 等. 心脾综合征的多普勒超声心电图诊断. 医学影像学杂志, 2007, 17(8):836-839.
18. Yan YL, Tan KB, Yeo GS. Right atrial isomerism:preponderance in Asian fetuses. Using the stomach-distance ratio as a possible diagnostic tool for prediction of right atrial isomerism. Cardiol Young, 2008, 37(11):906-912.
19. Lin JH, Chang CI, Wang JK, et al. Intrauterine diagnosis of heterotaxy syndrome. Am Heart J, 2002, 143(6):1002-1008.
20. Berg C, Geipel A, Smrcek J, et al. Prenatal diagnosis of cardiosplenic syndromes:a 10-year experience. Ultrasound Obstet Gynecol, 2003, 22(5):451-459.
21. Taketazu M, Lougheed J, Yoo SJ, et al. Spectrum of cardiovascular disease, accuracy of diagnosis, and outcome in fetal heterotaxy syndrome. Am J Cardiol, 2006, 97(5):720-724.
22. 张陈, 刘豫阳, 盛锋. Fontan 手术的危险因素. 国外医学科学分册, 2005, 32(2):127-129.
23. Ota N, Fujimoto Y, Hirose K, et al. Improving results of atrioventricular valve repair in challenging patients with heterotaxy syndrome. Cardiol Young, 2010, 20(1):60-65.
24. Foerster SR, Gauvreau K, McElhinney DB, et al. Importance of totally anomalous pulmonary venous connection and postoperative pulmonary vein stenosis in outcomes of heterotaxy syndrome. Pediatr Cardiol, 2008, 29(3):536-544.
25. Lim DS, Graziano JN, Rocchini AP, et al. Transcatheter occlusion of aortopulmonary shunts during single-ventricle surgical palliation. Catheter Cardiovasc Interv, 2005, 65(3):427-433.

Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Features of Cardiacvascular Abnormalities and Clinical Results in Patients with Fontan Operation for Heterotaxy Syndrome

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