Surgical strategies and outcomes in patients with Shone complex: a retrospective review
James D St Louis1, Mary M Bannan, William A Lutin
1The Children's Medical Center at the Medical College of Georgia, Augusta, Georgia 30912-4040, USA. jstlouis@mcg.edu
Insights
Shone complex, a rare heart condition, shows excellent long-term survival with individualized surgical approaches. Mitral interventions can often be delayed, improving outcomes for patients with multilevel left-sided heart obstruction.
Area of Science:
- Cardiology
- Pediatric Cardiac Surgery
- Congenital Heart Disease
Background:
- Shone complex involves multilevel obstruction of left-sided heart structures.
- Developing effective surgical strategies for Shone complex has historically been challenging.
- Previous surgical outcomes for Shone complex have been generally poor.
Purpose of the Study:
- To review operative strategies for Shone complex.
- To examine mid-term outcomes of surgical interventions for Shone complex.
- To assess the long-term survival and functional status of patients with Shone complex.
Main Methods:
- Retrospective review of 28 patients diagnosed with Shone complex.
- Analysis of patient demographics, clinical presentation, and cardiac anatomy.
- Evaluation of surgical interventions, timing, and patient follow-up data.
Main Results:
- Overall survival rate of 93% among 26 evaluated patients.
- Average follow-up of 6.3 years for 24 patients.
- All surviving patients presented with New York Heart Association functional class I or II congestive heart failure.
Conclusions:
- Long-term survival for Shone complex is excellent.
- Individualized operative strategies are crucial for managing Shone complex.
- Mitral valve interventions can often be deferred in the management of Shone complex.
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.
