Surgical management of subaortic obstruction in single left ventricle and tricuspid atresia

Subaortic obstruction caused by either a restrictive bulboventricular foramen in single left ventricle with an outflow chamber or by a restrictive ventricular septal defect in tricuspid atresia with transposition of the great arteries can lead to a hypertrophied, noncompliant ventricle and excessive pulmonary blood flow. This combination is disadvantageous to potential Fontan procedure candidates because they are dependent on good ventricular function and low pulmonary vascular resistance for survival. The results of surgical procedures to directly or indirectly relieve significant subaortic obstruction (gradient greater than 30 mm Hg) in 24 patients, 16 with single left ventricle and 8 with tricuspid atresia, were reviewed. Four patients had a left ventricular apex to descending aorta valved conduit; none survived. Seven patients had resection of subaortic tissue; four survived and four developed heart block at surgery. Adequate gradient relief was evident in only one of the four survivors. Thirteen patients had a main pulmonary artery to ascending aorta anastomosis or conduit; six survived. All survivors had adequate gradient relief. The overall survival was 42% (10 of 24). None of seven patients with a subaortic gradient greater than 75 mm Hg survived. These data show that: Surgical relief of established subaortic obstruction in patients with single left ventricle and tricuspid atresia carries a high mortality rate, especially if the subaortic gradient is greater than 75 mm Hg. The best procedure appears to be the pulmonary artery to ascending aorta anastomosis. A clearer understanding of the factors leading to the development of significant subaortic obstruction is necessary to prevent it or to devise improved therapeutic strategies.

This article has been cited by other articles:

				P. K. Mishra Management strategies for interrupted aortic arch with associated anomalies Eur J Cardiothorac Surg, April 1, 2009; 35(4): 569 - 576. [Abstract] [Full Text] [PDF]

				R. Sittiwangkul, A. Azakie, G. S. Van Arsdell, W. G. Williams, and B. W. McCrindle Outcomes of tricuspid atresia in the Fontan era Ann. Thorac. Surg., March 1, 2004; 77(3): 889 - 894. [Abstract] [Full Text] [PDF]

				Y.-T. Lan, R.-K. Chang, and H. Laks Outcome of patients with double-inlet left ventricle or tricuspid atresia with transposed great arteries J. Am. Coll. Cardiol., January 7, 2004; 43(1): 113 - 119. [Abstract] [Full Text] [PDF]

				S. M. Bradley, J. M. Simsic, A. M. Atz, and B. H. Dorman The infant with single ventricle and excessive pulmonary blood flow: results of a strategy of pulmonary artery division and shunt Ann. Thorac. Surg., September 1, 2002; 74(3): 805 - 810. [Abstract] [Full Text] [PDF]

				R. H. Pass, D. E. Solowiejczyk, J. M. Quaegebeur, L. Liberman, K. Altmann, W. M. Gersony, and A. J. Hordof Bulboventricular foramen resection: hemodynamic and electrophysiologic results Ann. Thorac. Surg., April 1, 2001; 71(4): 1251 - 1254. [Abstract] [Full Text] [PDF]

				Z. Amin, C. L. Backer, C. E. Duffy, and C. Mavroudis Does banding the pulmonary artery affect pulmonary valve function after the Damus-Kaye-Stansel operation? Ann. Thorac. Surg., September 1, 1998; 66(3): 836 - 841. [Abstract] [Full Text] [PDF]

				D. B. McElhinney, V. M. Reddy, N. H. Silverman, and F. L. Hanley MODIFIED DAMUS-KAYE-STANSEL PROCEDURE FOR SINGLE VENTRICLE, SUBAORTIC STENOSIS, AND ARCH OBSTRUCTION IN NEONATES AND INFANTS: MIDTERM RESULTS AND TECHNIQUES FOR AVOIDING CIRCULATORY ARREST J. Thorac. Cardiovasc. Surg., November 1, 1997; 114(5): 718 - 726. [Abstract] [Full Text]

				A. Serraf, S. Conte, F. Lacour-Gayet, J. Bruniaux, M. Sousa-Uva, R. Roussin, and C. Planche Systemic obstruction in univentricular hearts: Surgical options for neonates Ann. Thorac. Surg., October 1, 1995; 60(4): 970 - 977. [Abstract] [PDF]

				J. A. M. van Son, V. M. Reddy, G. S. Haas, and F. L. Hanley MODIFIED SURGICAL TECHNIQUES FOR RELIEF OF AORTIC OBSTRUCTION IN {S,L,L} HEARTS WITH RUDIMENTARY RIGHT VENTRICLE AND RESTRICTIVE BULBOVENTRICULAR FORAMEN J. Thorac. Cardiovasc. Surg., October 1, 1995; 110(4): 909 - 915. [Abstract] [Full Text]

				W. J. Brawn, B. Sethia, R. Jagtap, O. F. W. Stumper, J. G. C. Wright, J. V. De Giovanni, E. D. Silove, M. Jackson, and N. Sreeram Univentricular heart with systemic outflow obstruction: Palliation by primary Damus procedure Ann. Thorac. Surg., June 1, 1995; 59(6): 1441 - 1447. [Abstract] [PDF]

				R. N. Gates, H. Laks, A. Elami, D. C. Drinkwater Jr, J. M. Pearl, B. L. George, J. M. Jarmakani, and R. G. Williams Damus-Stansel-Kaye procedure: Current indications and results Ann. Thorac. Surg., July 1, 1993; 56(1): 111 - 119. [Abstract] [PDF]

				C. B. Huddleston, C. E. Canter, and T. L. Spray Damus-Kaye-Stansel with cavopulmonary connection for single ventricle and subaortic obstruction Ann. Thorac. Surg., February 1, 1993; 55(2): 339 - 346. [Abstract] [PDF]

				S. A. Webber, S. S. Sett, and J. G. LeBlanc Univentricular atrioventricular connection with subaortic stenosis: A staged surgical approach Ann. Thorac. Surg., August 1, 1992; 54(2): 344 - 347. [Abstract] [PDF]

				M. N. Ilbawi, S. Y. DeLeon, W. R. Wilson Jr, J. A. Quinones, D. A. Roberson, T. S. Husayni, O. G. Thilenius, and R. A. Arcilla Advantages of early relief of subaortic stenosis in single ventricle equivalents Ann. Thorac. Surg., October 1, 1991; 52(4): 842 - 849. [Abstract] [PDF]

				J. J. Lamberti, R. D. Mainwaring, J. D. Waldman, L. George, J. W. Mathewson, R. L. Spicer, and S. E. Kirkpatrick The Damus-Fontan procedure Ann. Thorac. Surg., September 1, 1991; 52(3): 676 - 679. [Abstract] [PDF]

				C. I. Tchervenkov, M. J. Beland, D. A. Latter, and A. R. C. Dobell Norwood operation for univentricular heart with subaortic stenosis in the neonate Ann. Thorac. Surg., November 1, 1990; 50(5): 822 - 825. [Abstract] [PDF]

				M. C. Bethea and J. L. Reynolds Treatment of bulboventricular foramen stenosis by ventricle-ascending aorta valved-conduit bypass Ann. Thorac. Surg., May 1, 1989; 47(5): 765 - 766. [Abstract] [PDF]