A Prospective Management Strategy for Heterotaxy Syndrome with Intestinal Rotation Abnormalities: Imaging Does Not Predict Need for Surgery.

PURPOSE: Heterotaxy syndrome (HS) affects right-left anatomical development in 3% of children with congenital heart disease. Commonly, these patients have intestinal rotation abnormalities (IRA) that differ from typical malrotation. In this prospective study, we examine the development of a management pathway, imaging findings, and clinical course of patients with HS and IRA. METHODS: After literature review, a multispecialty focus group designed a pathway for HS. Participants from a single institution were prospectively enrolled from 2016 to 2019. They underwent an abdominal ultrasound and UGI, however timing was variable based on symptoms. Symptomatic IRA was defined as feeding intolerance, bilious or non-bilious vomiting, bloating, severe reflux, and intermittent pain or abdominal distention. Screening for symptoms occurred every three months for the first two years and then annually. RESULTS: 18 participants were followed for a median of 5.0 years. Three clinical categories were identified: 1) asymptomatic, not requiring intestinal surgery, 72.2%, 2) symptomatic with feeding intolerance or failure to thrive requiring gastrostomy placement and diagnostic laparoscopy with Ladd procedure, 16.7%, and 3) symptomatic requiring urgent exploration for suspicion of volvulus, 11.1%. Need for surgery did not necessarily correlate with US and/or UGI findings. CONCLUSIONS: In children with HS and IRA, postnatal imaging did not inform the need for intestinal surgery. Although rare, volvulus or other forms of proximal obstruction can be anticipated, and symptomatic patients should be offered surgical intervention. A multidisciplinary care pathway for patients with HS and IRA can coordinate care and help counsel families on the likelihood of needing intestinal surgery for IRA. LEVEL OF EVIDENCE: Level II. TYPE OF STUDY: Prospective Cohort Study.

Injectable oxygenation therapeutics: evaluating the oxygen delivery efficacy of artificial oxygen carriers and kosmotropes in vitro.

The aim of this paper was to utilise an existing in vitro setup to quantify the oxygen offloading capabilities of two different subsets of injectable oxygenation therapeutics: (1) artificial oxygen carriers (AOCs), which bind or dissolve oxygen and act as transport vectors, and (2) kosmotropes, which increase water hydrogen bonding and thereby decrease the resistance to oxygen movement caused by the blood plasma. Dodecafluoropentane emulsion (DDFPe) was chosen to represent the AOC subset while trans sodium crocetinate (TSC) was selected to represent the kosmotrope subset. PEG-Telomer-B (PTB), the surfactant utilised to encapsulate DDFP in emulsion form, was also tested to determine whether it affected the oxygen transport ability of DDFPe. The in vitro set-up was used to simulate a semi closed-loop circulatory system, in which oxygen could be delivered from the lungs to hypoxic tissues. Results of this study showed that (1) 0.5 ml of a PFC outperformed 6.25 ml of a kosmotrope in a controlled, in vitro setting and (2) that PTB and sucrose do not contribute to the overall oxygen transportation efficacy of DDFPe. These results could be therapeutically beneficial to ongoing and future pre-clinical and clinical studies involving various oxygenation agents.

In vitro model to compare the oxygen offloading behaviour of dodecafluoropentane emulsion (DDFPe).

The aim of this paper was to create an in vitro setup to quantify the oxygen offloading capabilities of dodecafluoropentane emulsion (DDFPe) in a hypoxic environment. Oxygen offloading from DDFPe was characterized under different gaseous environments and under pre-oxygenated conditions. Results of this study showed that (1) oxygen offloading is inversely related to the solubility of the selected sparging gas in saline and (2) both pre-oxygenated and simultaneously oxygenated DDFPe display similar magnitudes of oxygen transport. These results could be applicable to on-going and future studies involving a variety of hypoxic conditions where oxygen delivery might be therapeutically beneficial.