Modified Fontan Procedure for Patients with Heterotaxy Syndrome_Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Authors：

CHENWei-dan ^1,2 , CHENXin-xin ² ,  WANGWu-jun ¹

1. Department of Cardiovascular and Thoracic Surgery, Nanfang Hospital, Southern Medical University, Guangzhou 510515, P.R.China;
2. Department of Cardiovascular Surgery, Guangzhou Women and Children’s Medical Center, Guangzhou Medical University, Guangzhou 510623, P.R.China;

Corresponding author：

WANGWu-jun, Email: wang_wujun@126.com

Keywords：

Heterotaxy; Right atrial isomerism; Left atrial isomerism

DOI：

10.7507/1007-4848.20160084

Video：

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Objective To summarize the result and experience of modified Fontan procedure for patients with heterotaxy syndrome. Methods We retrospectively analyzed the clinical data of 17 patients with heterotaxy syndrome underwent cardiac operations in our hospital from September 2008 to November 2014. There were 11 males and 6 females at mean age of 4.7±2.5 years ranging from 3 to 10 years and at mean weight of 16.6±4.9 kilogram ranging from 10.6 to 27.0 kilogram. Two patients accepted modified Fontan surgery of stage one. The rest 15 patients accepted staging operation. They accepted modified Fontan surgery after they accepted a series of surgery like bidirectional Glenn procedure. Results There was no mortality in hospital. The peripheral blood oxygen saturation raised from 73%±12% to 91%±5%. There was no complications existing like thrombosis or severe arrhythmia. The follow-up duration was from 4 months to 6.5 years. There was 1 death during the time. The early and middle term mortality was 5.9% (1/17). Conclusions Single ventricle treatment remains the preferred procedure for patients with heterotaxy syndrome and its early and middle term results are satisfied.

Citation： CHENWei-dan, CHENXin-xin, WANGWu-jun. Modified Fontan Procedure for Patients with Heterotaxy Syndrome. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2016, 23(4): 366-368. doi: 10.7507/1007-4848.20160084 Copy

1.	Loomba RS, Hlavacek AM, Spicer DE, et al. Isomerism or heterotaxy: Which term leads to better understanding? Cardiology, 2015, 5(1): 1-7.
2.	Karadeniz A, Semiz Oysu A, Sahin S. Polysplenia/heterotaxy syndrome associated with aortic coarctation and multiple venous anomalies: Multidetector computed tomography findings. Vasc Endovascular Surg, 2010, 44(5): 381-384.
3.	Shiraishi I, Ichikawa H. Human heterotaxy syndrome - from molecular genetics to clinical features, management, and prognosis. Circ J, 2012, 76(9): 2066-2075.
4.	Serraf A, Bensari N, Houyel L, et al. Surgical management of congenital heart defects associated with heterotaxy syndrome. Eur J Cardiothoracic Surg, 2010, 38(6): 721-727.
5.	Reller MD, Strickland MJ, Riehle-Colarusso T, et al. Prevalence of congenital heart defects in metropolitan atlanta, 1998-2005. J Pediatr, 2008, 153(6): 807-813.
6.	Lin AE, Ticho BS, Houde K, et al. Heterotaxy: Associated conditions and hospital-based prevalence in newborns. J Am Coll Med Genet, 2000, 2(4): 157-172.
7.	Culbertson CB, George BL, Day RW, et al. Factors influencing survival of patients with heterotaxy syndrome undergoing the fontan procedure. J Am Coll Cardiol, 1992, 20(7): 678-684.
8.	Kim SJ. Heterotaxy syndrome. Kor Circ J, 2011, 41(7): 227-232.
9.	Eronen MP, Aittomaki KA, Kajantie EO, et al. Outcome of left atrial isomerism at a single institution. Pediatr Cardiol, 2012, 33(4): 596-600.
10.	Makhija Z, Marwah A, Mishra S, et al. Biventricular repair in heterotaxy patients. World J Pediatr Congenit Heart Surg, 2015, 6(2): 195-202.
11.	Kanemoto S, Abe M, Ban Y, et al. Staged Fontan operation for children with heterotaxy syndrome, bilateral ductus arteriosus, and nonconfluent pulmonary artery. Gen Thora Cardiovasc Surg, 2010, 58(1):336-340.
12.	Kutty S, Frommelt MA, Danford DA, et al. Medium-term outcomes of kawashima and completion fontan palliation in single-ventricle heart disease with heterotaxy and interrupted inferior vena cava. Ann thorac Surg, 2010, 90(8): 1609-1613.
13.	Sen S, Duchon J, Lampl B, et al. Heterotaxy syndrome infants are at risk for early shunt failure after ladd procedure. Ann thorac Surg, 2015, 99(3): 918-925.
14.	Eronen MP, Aittomaki KA, Kajantie EO, et al. The outcome of patients with right atrial isomerism is poor. Pediatr cardiol, 2013, 34(5): 302-307.
15.	Azakie A, Merklinger SL, Williams WG, et al. Improving outcomes of the fontan operation in children with atrial isomerism and heterotaxy syndromes. Ann thorac Surg, 2001, 72(5): 1636-1640.
16.	Stamm C, Friehs I, Duebener LF, et al. Improving results of the modified fontan operation in patients with heterotaxy syndrome. Ann thorac Surg, 2002, 74(3): 1967-1977; discussion: 1978.
17.	Gentille Lorente DI. Abnormal atrial rhythm in a heterotaxy syndrome. Arch Cardiovasc Dis, 2014, 107(3): 701-703.
18.	Sano S, Fujii Y, Arai S, et al. Atrioventricular valve repair for patient with heterotaxy syndrome and a functional single ventricle. Seminars in thoracic and cardiovascular surgery. Pediatr Cardiac Surg Ann, 2012, 15(6): 88-95.
19.	Hancock Friesen CL, Sherwood MC, Gauvreau K, et al. Forbess JM. Intermediate outcomes of atrioventricular valvuloplasty in lateral tunnel fontan patients. J Heart Val Dis, 2004, 13(2): 962-971; discussion: 971.
20.	Friedman KG, Salvin JW, Wypij D, et al. Risk factors for failed staged palliation after bidirectional glenn in infants who have undergone stage one palliation. Eur J Cardiothoracic Surg, 2011, 40(1):1000-1006.
21.	Sallehuddin A, Bulbul Z, Otero F, et al. Repair of atrioventricular valve regurgitation in the modified fontan operation. Eur J Cardiothoracic Surg, 2004, 26(6): 54-59.

1. Loomba RS, Hlavacek AM, Spicer DE, et al. Isomerism or heterotaxy: Which term leads to better understanding? Cardiology, 2015, 5(1): 1-7.
2. Karadeniz A, Semiz Oysu A, Sahin S. Polysplenia/heterotaxy syndrome associated with aortic coarctation and multiple venous anomalies: Multidetector computed tomography findings. Vasc Endovascular Surg, 2010, 44(5): 381-384.
3. Shiraishi I, Ichikawa H. Human heterotaxy syndrome - from molecular genetics to clinical features, management, and prognosis. Circ J, 2012, 76(9): 2066-2075.
4. Serraf A, Bensari N, Houyel L, et al. Surgical management of congenital heart defects associated with heterotaxy syndrome. Eur J Cardiothoracic Surg, 2010, 38(6): 721-727.
5. Reller MD, Strickland MJ, Riehle-Colarusso T, et al. Prevalence of congenital heart defects in metropolitan atlanta, 1998-2005. J Pediatr, 2008, 153(6): 807-813.
6. Lin AE, Ticho BS, Houde K, et al. Heterotaxy: Associated conditions and hospital-based prevalence in newborns. J Am Coll Med Genet, 2000, 2(4): 157-172.
7. Culbertson CB, George BL, Day RW, et al. Factors influencing survival of patients with heterotaxy syndrome undergoing the fontan procedure. J Am Coll Cardiol, 1992, 20(7): 678-684.
8. Kim SJ. Heterotaxy syndrome. Kor Circ J, 2011, 41(7): 227-232.
9. Eronen MP, Aittomaki KA, Kajantie EO, et al. Outcome of left atrial isomerism at a single institution. Pediatr Cardiol, 2012, 33(4): 596-600.
10. Makhija Z, Marwah A, Mishra S, et al. Biventricular repair in heterotaxy patients. World J Pediatr Congenit Heart Surg, 2015, 6(2): 195-202.
11. Kanemoto S, Abe M, Ban Y, et al. Staged Fontan operation for children with heterotaxy syndrome, bilateral ductus arteriosus, and nonconfluent pulmonary artery. Gen Thora Cardiovasc Surg, 2010, 58(1):336-340.
12. Kutty S, Frommelt MA, Danford DA, et al. Medium-term outcomes of kawashima and completion fontan palliation in single-ventricle heart disease with heterotaxy and interrupted inferior vena cava. Ann thorac Surg, 2010, 90(8): 1609-1613.
13. Sen S, Duchon J, Lampl B, et al. Heterotaxy syndrome infants are at risk for early shunt failure after ladd procedure. Ann thorac Surg, 2015, 99(3): 918-925.
14. Eronen MP, Aittomaki KA, Kajantie EO, et al. The outcome of patients with right atrial isomerism is poor. Pediatr cardiol, 2013, 34(5): 302-307.
15. Azakie A, Merklinger SL, Williams WG, et al. Improving outcomes of the fontan operation in children with atrial isomerism and heterotaxy syndromes. Ann thorac Surg, 2001, 72(5): 1636-1640.
16. Stamm C, Friehs I, Duebener LF, et al. Improving results of the modified fontan operation in patients with heterotaxy syndrome. Ann thorac Surg, 2002, 74(3): 1967-1977; discussion: 1978.
17. Gentille Lorente DI. Abnormal atrial rhythm in a heterotaxy syndrome. Arch Cardiovasc Dis, 2014, 107(3): 701-703.
18. Sano S, Fujii Y, Arai S, et al. Atrioventricular valve repair for patient with heterotaxy syndrome and a functional single ventricle. Seminars in thoracic and cardiovascular surgery. Pediatr Cardiac Surg Ann, 2012, 15(6): 88-95.
19. Hancock Friesen CL, Sherwood MC, Gauvreau K, et al. Forbess JM. Intermediate outcomes of atrioventricular valvuloplasty in lateral tunnel fontan patients. J Heart Val Dis, 2004, 13(2): 962-971; discussion: 971.
20. Friedman KG, Salvin JW, Wypij D, et al. Risk factors for failed staged palliation after bidirectional glenn in infants who have undergone stage one palliation. Eur J Cardiothoracic Surg, 2011, 40(1):1000-1006.
21. Sallehuddin A, Bulbul Z, Otero F, et al. Repair of atrioventricular valve regurgitation in the modified fontan operation. Eur J Cardiothoracic Surg, 2004, 26(6): 54-59.

Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Modified Fontan Procedure for Patients with Heterotaxy Syndrome

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