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Concealed pulmonary venous obstruction in right atrial isomerism with pulmonary outflow tract obstruction—surgical management following modified Blalock-Taussig shunt

Published online by Cambridge University Press:  19 August 2008

Ing-Sh Chiu ,
Nan-Koong Wang ,
Mei-Hwan Wu ,
Fen-Fen Wu  and
Chi-Ren Hung
Ing-Sh Chiu*
Affiliation:
From the Departments of Surgery and Pediatrics, National Taiwan University Hospital, Taipei
Nan-Koong Wang
Affiliation:
Department of Pediatrics, Cathay General Hospital, Taipei
Mei-Hwan Wu
Affiliation:
From the Departments of Surgery and Pediatrics, National Taiwan University Hospital, Taipei
Fen-Fen Wu
Affiliation:
From the Departments of Surgery and Pediatrics, National Taiwan University Hospital, Taipei
Chi-Ren Hung
Affiliation:
From the Departments of Surgery and Pediatrics, National Taiwan University Hospital, Taipei
*
Dr. Ing-Sh Chiu, Department of Surgery, National Taiwan University Hospital, No. 7. Chung-San Road, Taipei, Taiwan100
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Summary

Obstruction to the pulmonary venous return is a frequent associated anomaly in patients with isomerism of the right atrial appendages. Yet, preoperative diagnosis by means of either cross-sectional echocardiography or cardiac catheterization can be intriguing. Indeed, the presence of two morphologically right lungs reduce considerably the size of window for precordial echocardiography. Also, in the presence of severe pulmonary stenosis or atresia, it can be difficult at cardiac catheterization to enter the pulmonary trunk. In these patients, construction of a systemic-to-pulmonary artery anastomosis will almost inevitably result in pulmonary edema. Between May 1984 and December 1988, five patients with isomerism of the right atrial appendages, severely decreased pulmonary blood flow and concealed obstruction to the pulmonary venous return were admitted to our hospital. A modified Blalock Taussig shunt by interposition of a polytetrafluoroethylene prosthesis was performed in each patients and all of them developed pulmonary edema. Three patients died despite appropriate medical treatment. The remaining two patients were successfully treated by banding of the Blalock shunt. This was performed in the first patient at the time of the initial surgery, when prior to closure of chest, pulmonary edema became manifest. The second patient who developed pulmonary edema early postoperatively, underwent cardiac catheterization to confirm the clinical diagnosis of obstruction to the pulmonary venous return. Reduction of blood flow through the Blalock shunt with resolution of edema was initially achieved by means of a partially occluding balloon catheter. Pulmonary edema recurred one week later because of rupture of the balloon and the patient eventually underwent a successful banding of the Blalock shunt through a left thoracotomy. We conclude that preoperative assessment of the pulmonary venous return is mandatory in patients with right isomerism and reduced pulmonary blood flow. Construction of a modified Blalock-Taussig shunt in the presence of concealed obstruction to the pulmonary venous return will almost inevitably cause pulmonary edema. Banding of the Blalock shunt can be successful, as observed in our experience, for the management of this serious complication.

Keywords

Obstruction to pulmonary venous returnright atrial isomerism of the right atrial appendagesBlalock-Taussig shunt
Type
Original Articles
Information
Cardiology in the Young , Volume 2 , Issue 1 , January 1992 , pp. 95 - 99
Copyright
Copyright © Cambridge University Press 1992

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References

1.Ivemark, BI. Implications of agenesis of the spleen on the pathogenesis of cono-truncus anomalies in childhood: an analysis of the heart malformations in the splenic agenesis syndrome, with fourteen new cases. Acta Paediatr Scand 1958; 44 [Suppl]: 1–110.Google Scholar
2.Delisle, G, Ando, M, Calder, AL, Zuberbuhler, JR, Rochenmacher, S, Alday, LE, Mangini, O, Van Praagh, SV, Van Praagh, R. Total anomalous pulmonary venous connection: Report of 93 autopsied cases with emphasis on diagnostic and surgical considerations. Am Heart J 1976; 91: 99–122.Google Scholar
3.Chiu, IS, How, SW, Wang, JK, Wu, MH, Chu, SH, Lue, HC, Hung, CR. Clinical implications of atrial isomerism. Br Heart J 1988; 60: 72–77.Google Scholar
4.Sapire, DW, Ho, SY, Anderson, RH, Rigby, ML. Diagnosis and significance of atrial isomerism. Am J Cardiol 1986; 58: 342–346.Google Scholar
5.Gersony, WM. Obstruction to pulmonary venous return obscured by decreased pulmonary blood flow. Chest 1973; 64: 283–287.CrossRefGoogle ScholarPubMed
6.Muster, AJ, Paul, MH, Nikaidoh, H. Tetralogy of Fallot associated with total anomalous pulmonary venous drainage. Chest 1973; 64: 323–326.CrossRefGoogle ScholarPubMed
7.Freedom, RM, Olley, PM, Coceani, F, Rowe, RD. The prostaglandin challenge. Test to unmask obstructed total anomalous pulmonary venous connections in asplenia syndrome. Br Heart J 1978; 40: 91–94.CrossRefGoogle ScholarPubMed
8.DeLeon, SY, Gidding, SS, Ilbawi, MN, Idriss, FS, Muster, AJ, Cole, RB, Paul, MH. Surgical management of infants with complex cardiac anomalies associated with reduced pulmonary blood flow and total anomalous pulmonary venous drainage. Ann Thorac Surg 1987; 43: 207–211.CrossRefGoogle ScholarPubMed
9.Di Donato, R, di Carlo, D, Squitieri, C, Rossi, E, Ammirati, A, Marino, B, Marcelletti, C. Palliation of cardiac malformations associated with right isomerism (asplenia syndrome) in infancy. Ann Thorac Surg 1987; 44: 35–39.CrossRefGoogle ScholarPubMed
10.Ueda, K, Nojima, K, Saito, A, Nakano, H, Yokota, M, Muraoka, R. Modified Blalock-Taussig shunt operation without cardiac catheterization: two-dimensional echocardiographic preoperative assessment in cyanotic infants. Am J Cardiol 1984; 54: 1296–1299.CrossRefGoogle ScholarPubMed
11.Lamberti, JJ, Waldman, JD, Mathewson, JW, Kirkpatrick, SE. Repair of subdiaphragmatic total anomalous pulmonary venous connection without cardiopulmonary bypass. J Thorac Cardiovasc Surg 1984; 88: 627–630.CrossRefGoogle ScholarPubMed