Abstract
The incidence of the visceral heterotaxia syndrome in the congenital heart structure is about 3%. Atrioventricular valves regurgitation
is usually found in 69% of these patients. Biventricular correction in most cases is impossible because of complex intracardiac
lesions, and univentricular correction is more preferable. The associated intervention on the AV valve is considered to increase risk
of Fontan procedure This article is aimed to present a case of successful correction with good midterm result of the common AV valve
regurgitation together with modified Fontan operation in a patient with the visceral heterotaxia syndrome.
References
- Rubino M., Van Praagh S., Kadoba K., Pessotto R., Van
Praagh R. Systemic and pulmonary venous connections in
visceral heterotaxy with asplenia: Diagnostic and surgical considerations
based on seventy-two autopsied cases. J. Thorac.
Cardiovasc. Surg. 1995; 110: 641-50.
- Imai Y., Takanashi Y., Hoshino S., Terada M., Aoki M., Ohta J.
Modified Fontan procedure in ninety-nine cases of atrioventricular
valve regurgitation. J. Thorac. Cardiovasc. Surg. 1997;
113: 262-9.
- Peoples W.M., Moller J.N., Edwards J.E. Polysplenia: a rewiew
of 146 cases. Pediatr. Cardiol. 1983; 4: 129-37.
- Koh M., Yagihara T., Uemura H., Kagisaki K., Hagino I.,
Ishizaka T. et al. Biventricular repair for Right Atrial Isomerism.
Ann. Thorac. Surg. 2006; 8 1 : 1808-16.
- Stamm C., Friehs I., Duebener L.F., Zurakowski D., Mayer
J.E., Jonas R.A. et al. Improving results of the modified Fontan
operation in patients with heterotaxy syndrome. Ann. Thorac.
Surg. 2002; 74: 1967-78.
- Kim Soo-Jin, Kim Woong-Han, Lim H.G., Lee Chang-Ha, Lee J.Y.
Improving results of the Fontan procedure in patients with heterotaxy
syndrome. Ann. Thorac. Surg. 2006; 82: 1245-51.
- Cetta F., Feldt, P.W., O'Leary P.W, Mair D.D., Warnes C.A,
Driscoll D.G. et al. Improved early morbidity and mortality
after Fontan operation: the Mayo Clinic experience, 1987 to
1992. J. Am. Coll. Cardiol. 1996; 28: 480-6.
- Hashmi A., Abu-Sulaiman R., McCrindle B.W., Smallhorn
J.F., Williams W.G., Freedom R.M. Management and
outcomes of right atrial isomerism: a 26-year experience. J.
Am. Coll. Cardiol. 1998; 31: 1120-6.
- Bartz P.J., Driscoll D.J., Dearani J.A., Puga F.J., Danielson
G.K., O'Leary P.W. 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:
2301-5.
- Yurlov I.A., Podzolkov V.P., Zelenikin M.M., Kovalev D.V.,
Babaev G.K., Putiato N.A. еt all. Experience with bidirectional cavopulmonary anastomosis and modified Fontan operation
in patients with single ventricle and concomitant visceral heterotaxy.
Interact. Cardiovasc. Thorac. Surg. 2011; 12: 563-8.
- Culbertson C.B., George B.L., Day R.W., Laks H.,
Williams R.G. Factors influencing survival of patients with heterotaxy
syndrome undergoing the Fontan procedure. J. Am.
Coll. Cardiol. 1992; 20: 678-84.
- Misumi Y., Hoashia T., Kagisakia K., Kitanob M., Kurosakib
K., Shiraishib I., Yagiharaa T., Ichikawaa H. Long-term
outcomes of common atrioventricular valve plasty in patients
with functional single ventricle. Interact. Cardiovasc. Thorac.
Surg. 2014; 18: 259-65.
