Abstract 13329: The Natural and Un-Natural History of Patients with Scimitar Syndrome: An Italian Multicentric Study
Purpose: To evaluate the natural and un-natural history of patients with scimitar syndrome within an Italian multi-centric data collection.
Methods: Medical records of all patients with a diagnosis of scimitar syndrome from 11 Italian centers were reviewed. Eighty-five patients aged from 1.2 months to 72 years were included. Group1 (n=42) included patients who underwent surgical repair, Group 2 (n=43) included patients followed in their natural history. The primary outcome was patient’s clinical status at last follow-up.
Results: Symptoms were present in 52 patients at diagnosis, including recurrent respiratory tract infections (n=43), congestive heart failure (CHF)(n=17) and cyanosis (n=4))(Group1 versus Group2, p=ns). Patients in Group1 showed a higher prevalence of associated cardiac defects (ACD)(27/42 vs 17/43, p<0.05), moderate/severe right ventricular dilation (42/42 vs 12/43; p<0.0001), pulmonary artery hypertension (PAH, as mean pulmonary arterial pressure>25 mmHg)(28/42 vs 11/43; p=0.0004) when compared to Group2. Median age at surgery was 9 years (range 1 months-64 years); 31 patients were treated by an intra-atrial baffle repair, and 11 patients by a re-implantation of the scimitar vein onto left atrium. There were 4 in-hospital and 3 late deaths (5 in Group1 and 2 in Group2, p=ns). At the last follow-up, 36 patients had a clinical improvement (21 in Group 1 and 15 in Group 2, p=ns) mainly justified by a decreasing prevalence of respiratory symptoms with age. Eleven patients were still symptomatic, 8 with CHF (3 patients in Group1 and 5 in Group2, p=ns), 3 with respiratory symptoms, (1 patient in Group1 and 2 in Group2, p=ns). Variables associated with cardiac symptoms at follow-up were: CHF at diagnosis (p=0.0001), ACD (p=0.0003), moderate/severe right ventricle dilation (p=0.007) and PAH (p=0.0005). Patients who presented the association of ≥3 of these variables and underwent surgical repair (Group1) had a better long-term outcome (p<0.05).
Conclusions: Scimitar syndrome is not a benign disease. Predictors of poor clinical outcomes are CHF at diagnosis, ACD, moderate/severe right ventricle dilation and PAH. Surgical treatment was associated to a clinical benefit over medical treatment when ≥3 predictors were present simultaneously
- © 2012 by American Heart Association, Inc.