Abstract 1946: Predictors of Active Clinical Progression in Shone’s Syndrome
Background: Shone’s syndrome involves multiple levels of left sided cardiac obstructions including supramitral ring, mitral stenosis, subaortic stenosis, aortic stenosis and coarctation, and can demonstrate active progression in some patients.
Objective: To identify predictors of disease progression in patients with Shone’s syndrome.
Methods: We identified patients with an echocardiographic diagnosis of Shone’s syndrome, defined as the presence of two or more left cardiac obstructive lesions, from 1988 to 2003, with at least 5 years of clinical and echocardiographic follow-up at Children’s Hospital Boston. Patients with surgical palliation for hypoplasic left heart syndrome were excluded. Demographical, clinical and anatomical data were collected. A judgment-based evaluation of the echocardiographic studies was performed by two pediatric cardiologists to identify progression of the disease. Progression was defined as either worsening of a previous left sided obstruction (related or not to lack of growth), or the presence of a new one. Univariate and multivariate logistic regression analysis was performed to identify predictors of progression.
Results: One hundred and fifty five patients were identified, and 105 meet the inclusion criteria. The most common combinations of lesions at diagnosis were aortic coarctation with mitral stenosis 41%, aortic coarctation with aortic stenosis 30%, and mitral stenosis with aortic stenosis 20%. The most frequent associated conditions were atrial septal defect 34%, ventricular septal defect 29% and left superior vena cava 14%. Progression was found in 55% of the patients. Univariate analysis demonstrated that age < 1 year, the presence aortic coarctation and ventricular septal defect were related with progression. In multivariate analysis, only age < 1 year (OR: 3.8, p = 0.002), and aortic coarctation (OR: 3.0, p = 0.03) were related. The most common lesions that showed progression were subaortic stenosis 28%, aortic stenosis 24% and mitral stenosis 23%.
Conclusions: Age and the presence of aortic coarctation were associated with disease progression. Among patients with Shone’s syndrome, the most common sites of progression were the aortic and mitral valves, and the subaortic area.