Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)

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Rothman, A. et al.

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.[1]

References

Surgical management of subaortic obstruction in single left ventricle and tricuspid atresia. Rothman, A., Lang, P., Lock, J.E., Jonas, R.A., Mayer, J.E., Castaneda, A.R. J. Am. Coll. Cardiol. (1987) [Pubmed]

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