OBJECTIVE: Shone's syndrome is a complex consisting of mitral valve stenosis in 
addition to left ventricle outflow obstruction. There are a few studies 
evaluating the long-term outcomes in this population. We sought to determine the 
long-term outcomes in our paediatric population with Shone's syndrome and the 
factors associated with left heart growth.
METHODS: All patients diagnosed with Shone's syndrome with biventricular 
circulation treated between 1978 and 2010 were reviewed. Baseline 
echocardiograms and data from catheterisations were also reviewed. Number of 
interventions (surgical+transcatheter), incidence of mitral valve replacement, 
and incidence of heart transplantation were tracked. Survival of the population 
and left heart structural growth were also reviewed.
RESULTS: A total of 121 patients with Shone's syndrome presented at a median age 
of 28 days (0-17.3 years) and were followed-up for 7.2 years (0.01-35.5 years). 
These patients underwent 258 interventions during the study period, and the 
presence of coarctation was associated with repeat left heart interventions. The 
10-year, transplant-free survival was 86%. Presence of pulmonary hypertension 
was associated with mortality. Left heart structural growth was seen for mitral 
and aortic valve annuli and left ventricular end-diastolic dimension over time.
CONCLUSIONS: Shone's syndrome patients undergo a number of left heart 
interventions. Coarctation of the aorta is associated with an increased 
likelihood for repeat interventions. Survival appears to be more favourable than 
expected. Significant left heart growth will occur in the population. Pulmonary 
hypertension is associated with an increased risk of mortality.