Vida VL, et al. - Using a large multi-institutional registry of scimitar syndrome (SS) patients, this study assessed the natural history of this condition and, survival and clinical status following surgical treatment. A high overall survival was reported for these patients but those with associated congenital heart diseases (CHDs) and with pulmonary hypertension had lower survival probability. A reduction in symptoms resulted from surgical treatment, however, given the significant risk of post-operative scimitar drainage stenosis/occlusion, it should be tailored to a comprehensive haemodynamic assessment and to the patient’s age.

Methods

• Researchers collected data on 485 SS patients from 51 institutions; 279 (57%) patients were treated surgically (STPs) and 206 (43%) were clinically monitored (CMPs).

Results

• As per data, the median age at last follow-up was 11.6 years (interquartile range 4–22 years).
• Researchers observed that at 30 years of age, overall survival probability was 88% [85–92% confidence intervals (CI)].
• They also found that overall survival probability was lower in patients with associated congenital heart disease (CHD) (P < 0.001) and pulmonary hypertension (P < 0.001).
• Findings revealed that at last follow-up, most patients were asymptomatic (279/451, 62%); STPs were more frequently asymptomatic than CMPs (73% vs. 47%, P < 0.001), with fewer cardiac [odds ratio (OR) 0.42, 95% CI 0.22–0.82] and respiratory symptoms (OR 0.08, 95% CI 0.02–0.28).
• Additionally, stenosis/occlusion of the scimitar drainage was detected in many STPs (63/254, 25%), was associated with a younger age at surgery (OR 0.4, CI 0.21–0.78).