Key result
Pediatric patients with Shone's syndrome had an 86% 10-year transplant-free survival, with pulmonary hypertension associated with increased mortality.
Cohort (n=121)
No
Children with Shone's syndrome have a favorable 10-year transplant-free survival of 86%, though pulmonary hypertension increases mortality risk and coarctation is associated with repeat interventions.
Pulmonary hypertension signals higher mortality risk in Shone syndrome; extends sparse paediatric outcome data but leaves causal links open.
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.
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Nicholson et al. (2016) conducted a cohort in Shone's syndrome (n=121). Shone's syndrome was evaluated on 10-year transplant-free survival. Pediatric patients with Shone's syndrome had an 86% 10-year transplant-free survival, with pulmonary hypertension associated with increased mortality.
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