Standardized Approach To Intervention For Intestinal Malrotation In Single Ventricle Patients With Heterotaxy Syndrome: Impact On Interstage Attrition And Time To Superior Cavopulmonary Connection
ANASTASIOS C POLIMENAKOS1, BRENDAN SHAFER1, LAUREN MATHIS1, VINAYAK KAMATH2
1CHILDREN'S HOSPITAL OF GEORGIA, AUGUSTA, GA;2MEDICAL COLLEGE OF GEORGIA, AUGUSTA, GA
Single ventricle physiology(SV) and heterotaxy syndrome(HS) carry significant early and interstage morbidity/mortality especially associated with extracardiac related anomalies.Attrition rate and time-related events associated with intestinal malrotation (IM) are, yet, to be determined.We sought to evaluate hospital and interstage outcomes in relation with symptoms-driven compared to elective deployment of IM interventions
Twelve patients with SV and HS to whom IM procedures performed from January 2004 to December 2016 were studied.Early shunt failure, time to superior cavopulmonary connection(SCPC) and interstage attrition were assessed and outcome factors analyzed. Protocol included predetermined intraoperative anesthesia and cardiopumonary monitoring expectations and ICU care. Since September 2014 based on protocol all IM interventions were performed at the time of hospitalization for the stage-I palliation irrespective of clinical manifestations.Based on indications for IM intervention patients were assigned to GroupA(n-8)=expectant symptoms-driven versus GroupB(n-4)=protocol-driven.
Median gestational age was 37.5 weeks(range, 36-39 GroupA vs GroupB; p=0.6).Stage-I palliation was systemic-to-pulmonary shunt(SPS) only(n-9), SPS and total anomalous pulmonary venous connection repair(n-2) and Norwood-like operation(n-1).Median duration from stage I palliation to IM intervention was 82 days(range, 57-336) compared to 14 days(range, 11-31); p<0.05. At Stage I operation 7 had SPS, 1 SPS with TAPVR repair(GroupA) compared to 2 SPS, 1 SPS with TAPVR and 1 Norwood (GroupB). In GroupA 5 patients exhibited feeding intolerance prior to IM intervention, 2 had early signs of intestinal obstruction and 1 presented with volvulus and intestinal ischemia.Median age at IM intervention for the entire cohort was 87 days(range, 8-345) - GroupA 99 days(range, 68-345) vs GroupB 25 days(range, 8-39); p<0.05. Early SPS failure occurred in 25%(2 of 8) for Group A compared to none in GroupB (p<0.05).Hospital mortality following IM intervention for the entire cohort was 25% [GroupA 37.5% (3 of 8) versus GroupB: 0; p < 0.05]. Interstage survival for the entire cohort was 67% [GroupA 50%(4 of 8) versus GroupB: 100%; p < 0.05].Time to SCPC after Stage-I palliation was 186 days (range, 169-218) for GroupA compared to 118 days (range, 97-161)(GroupB); p<0.05.Elective IM intervention based on standardized protocol at the time of Stage-I palliation is protective of early and interstage attrition(p<0.05).
IM interventions in SV with HS are associated with significant interstage attrition and might impact the time to SCPC.SPS are at risk for early failure after IM interventions.Risk of early and interstage morbidity might be mitigated when a comprehensive standardized approach of IM interventions is favored.
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