713: FATAL MIS-C IN A CHILD WITH PALLIATED SINGLE-VENTRICLE PHYSIOLOGY

Introduction: Children with single ventricle physiology (SV) are at high risk of in-hospital morbidity and mortality, with much of that increased risk coming in the first year of life. Understanding which children are at the highest risk for clinical deterioration may allow for increased monitoring and earlier escalation of care, with associated decreased mortality. Methods: We conducted a retrospective chart review of all admissions to the pediatric cardiology non-ICU inpatient service from 2014 - 2018 for children < 18 years old. Clinical deterioration was defined as an unplanned transfer to the ICU or inpatient mortality. Children with SV were selected by diagnosis codes. Results: From the entire cohort of 1612 pediatric cardiology admissions (56 % male, 25% SV), 288 admissions had a deterioration event including 26 deaths. Infants less than one year with SV (n = 197 admissions) were significantly more likely to have a deterioration event (107 events over 62 admissions with an event) than the overall pediatric cardiology cohort (OR 2.11, 95% CI 1.52-2.93). Among infants with SV, those with a deterioration event were significantly younger (median 1.7 v 4.3 months, p < 0.001). Further, at baseline they had significantly lower oxygen saturation (84% v 87%, p < 0.01), lower systolic blood pressure (85mmHg v 90mmHg, p< 0.02), higher respiration rate (48 v 44, p < 0.01), and higher hematocrit (44.0 v 40.2, p < 0.005) compared to those who remained stable. Mean Pediatric Early Warning Scores (PEWS) were significantly higher for infants with SV who had a deterioration event (1.4 v 0.9, p < 0.001) and PEWS scores significantly increased in the 48 hours prior to an event (p < 0.001). Of the 104 non-death events, 61 required an increase in oxygen support and 51 required a fluid bolus prior to the event (p < 0.001). Conclusions: Infants with SV are at high risk for clinical deterioration. There are baseline differences in vital signs and lab work between those that remain stable and those that have a deterioration event. PEWS scores and oxygen and fluid treatment significantly increase prior to deterioration events. Leveraging data from the Electronic Medical Record to identify the highest risk patients may allow for earlier detection and intervention to prevent clinical deterioration.

Download Full-text

Single Ventricle Physiology

The Heart and Circulation ◽

10.1007/978-3-030-25062-1_20 ◽

2019 ◽

pp. 235-244

Author(s):

Branko Furst

Keyword(s):

Single Ventricle ◽

Single Ventricle Physiology

Download Full-text

Single-centre experience in stenting arterial shunts for adult CHD patients with single-ventricle physiology and pulmonary blood flow dependent on arterial shunts

Cardiology in the Young ◽

10.1017/s1047951118001464 ◽

2018 ◽

Vol 28 (12) ◽

pp. 1431-1435

Author(s):

Mohamed Kasem ◽

James Bentham ◽

John Thomson

Keyword(s):

Blood Flow ◽

Pulmonary Blood Flow ◽

Single Ventricle ◽

Well Being ◽

Adult Patients ◽

P Value ◽

Intermediate Step ◽

Medium Term ◽

Single Ventricle Physiology ◽

Post Procedure

AbstractObjectivesThe objectives of this study were to describe the approach to stenting arterial shunts in adult congenital patients with single-ventricle physiology and to assess the medium-term clinical and haemodynamic outcomes following stent insertion.BackgroundAdult patients with single-ventricle physiology and pulmonary blood flow dependent on a surgically placed arterial shunt who did not progress to venous palliation are extremely challenging to manage. Progressive cyanosis secondary to narrowing of the shunt has a marked impact on exercise tolerance and results in intolerable well-being for these patients. Stenting arterial shunts in adult patients is one method that can help improve pulmonary blood flow. There is very limited information in the literature about this patient.MethodsThis is a retrospective study for arterial shunts stenting conducted between 2008 and 2016. The peripheral oxygen saturations, the NYHA status, the haemoglobin, and the degree of atrio-ventricular valve regurgitation as assessed on transthoracic echo were compared before and 6–12 months after procedures.ResultsThere was a short-term improvement in oxygen saturations; the pre-procedure mean was 75.8 (SD 2.55)% (range 70–85%) and post-procedure mean was 83 (SD 2.52)% (range 78–87%), with a p value of 0.04. Haemoglobin level decreased from a pre-procedure mean of 22.06–20.28 g/L 6 months post procedure (range 18.1–24.4 to 13–23.3 g/L), with a p value of 0.44. NYHA class decreased from a mean of 3.2–2.2 post procedure. Left atrial volume for four of the cases did not change (22.6–76.6 ml [mean 48.4 ml] to 29.6–72.9 ml [mean 52 ml], p value: 0.83).ConclusionsStenting stenotic arterial shunts is a useful method to gain a medium-term improvement on the oxygen saturation and clinical symptoms, and may act as a useful intermediate step for further management plans.

Download Full-text