Background: Multilevel obstruction of left-sided heart structures was originally characterized by Shone and colleagues. The formulation of an appropriate operative strategy remains problematic. Surgical outcomes have generally been poor. This review examines operative strategies and mid-term outcomes.
Methods: A retrospective review was done of 28 patients who presented with the diagnosis of Shone complex at a median age of 14 days, with 46% presenting within the first week of life. At presentation, 5 patients were in cardiovascular collapse, and 10 were at New York Heart Association functional class III or IV. All patients had a structurally abnormal mitral complex. A mean transvalvular pressure gradient of 8 mm or greater existed in 15 patients, 6 had a supraannular mitral ring, and 26 had a coarctation of the aorta. Twelve mitral valve operations were done, with an average time from presentation to operative intervention of 15 months.
Results: Two patients were lost to follow-up. Of 26 remaining patients, 2 died after a second operative intervention. The average follow-up of 24 patients was 6.3 years (range, 1 to 16 years). Overall survival was 93%. All surviving patients are in class I or II congestive heart failure.
Conclusions: Long-term survival of patients diagnosed with Shone complex is excellent. Operative strategies for this complex group should be individualized. Mitral interventions may generally be deferred.