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Surgical repair of right aortic arch and aberrant left subclavian artery has traditionally involved ligamentum division. Such patients can have stenosis at the origin of the aberrant subclavian artery either at the time of presentation or later. The more recently popularized repair involving resection of Kommerell diverticulum with transfer of the subclavian artery to the left carotid artery allows resection of the stenotic segment and serves as an effective treatment for subclavian stenosis as well. We present three cases of early repair of this arch anomaly with associated subclavian stenosis repaired successfully in that manner., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

We hypothesize that there are post-operative, non-surgical risk factors that could be modified to prevent the occurrence of chylothorax, and we seek to determine those factors. Retrospective chart review of 285 consecutive patients < 18 years who underwent cardiac surgery from 2015 to 2017 at a single institution pediatric intensive care unit. Data was collected on patient demographics, cardiac lesion, surgical and post-operative characteristics. Primary outcome was development of chylothorax. Of 285 patients, median age was 189 days, median weight was 6.6 kg, 48% were female, and 10% had trisomy 21. 3.5% of patients developed upper extremity DVTs, and 8% developed chylothorax. At 24 h following surgery, a majority were in the 0-10% fluid overload category or had a negative fluid balance (63% and 34%, respectively), and a positive fluid balance was rare at 72 h (16%). In univariate analysis, age, weight, bypass time, DVT, arrhythmia, and trisomy 21 were significantly associated with chylothorax and adjusted for in logistic regression. Presence of an upper extremity DVT (OR 49.8, p < 0.001) and trisomy 21 (OR 5.8, p < 0.001) remained associated with chylothorax on regression modeling. The presence of an upper extremity DVT and trisomy 21 were associated with the development of chylothorax. Fluid overload was rare in our population. The presence of positive fluid balance, fluid overload, elevated central venous pressure, and early initiation of fat containing feeds were not associated with chylothorax.

Background: Regional cerebral perfusion (RCP) is used as an adjunct or alternative to deep hypothermic circulatory arrest (DHCA) for neonates and infants undergoing aortic arch repair. Clinical studies have not demonstrated clear superiority of either strategy, and multicenter data regarding current use of these strategies are lacking. We sought to describe the variability in contemporary practice patterns for use of these techniques., Methods: The Society of Thoracic Surgeons Congenital Heart Surgery Database (2010-2013) was queried to identify neonates and infants whose index operation involved aortic arch repair with cardiopulmonary bypass. Perfusion strategy was classified as isolated DHCA, RCP (with less than or equal to ten minutes of DHCA), or mixed (RCP with more than ten minutes of DHCA). Data were analyzed for the entire cohort and stratified by operation subgroups., Results: Overall, 4,523 patients (105 centers) were identified; median age seven days (interquartile range: 5.0-13.0). The most prevalent perfusion strategy was RCP (43%). Deep hypothermic circulatory arrest and mixed perfusion accounted for 32% and 16% of cases, respectively. In all, 59% of operations involved some period of RCP. Regional cerebral perfusion was the most prevalent perfusion strategy for each operation subgroup. Neither age nor weight was associated with perfusion strategy, but reoperations were less likely to use RCP (31% vs 45%, P < .001). The combined duration of RCP and DHCA in the RCP group was longer than the DHCA time in the DHCA group (45 vs 36 minutes, P < .001)., Conclusion: There is considerable variability in practice regarding perfusion strategies for arch repair in neonates and infants. In contemporary practice, RCP is the most prevalent perfusion strategy for these procedures. Use of DHCA is also common. Further investigation is warranted to ascertain possible relative merits of the various perfusion techniques., (© The Author(s) 2016.)

An unusual case of total anomalous pulmonary venous connection surviving to adulthood without surgical correction is presented. Transthoracic echocardiography first led to this diagnosis and magnetic resonance imaging refined the anatomic diagnosis leading to successful surgical correction.