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Objectives Sudden death is common in patients with hypoplastic left heart syndrome and comparable lesions with parallel systemic and pulmonary circulation from a common ventricular chamber. It is hypothesized that unforeseen acute deterioration is preceded by subtle changes in physiologic dynamics prior to overt clinical extremis. Our objective is to develop a computer algorithm to automatically recognize precursors to deterioration in real-time, providing an early warning to care staff. Methods Continuous high-resolution physiologic recordings were obtained from 25 children with parallel systemic and pulmonary circulation who were admitted to the CVICU of Texas Children’s Hospital between their early neonatal palliation and stage 2 surgical palliation. Instances of cardiorespiratory deterioration (defined as the need for CPR or endotracheal intubation) were found via a chart review. A classification algorithm was applied to both primary and derived parameters that were significantly associated with deterioration. The algorithm was optimized to discriminate pre-deterioration physiology from stable physiology. Results Twenty cardiorespiratory deterioration events were identified in 13 of the 25 infants. The resulting algorithm was both sensitive and specific for detecting impending events, one to two hours in advance of overt extremis (ROC Area = 0.91, 95% CI = 0.88–0.94). Conclusion Automated, intelligent analysis of standard physiologic data in real time can detect signs of clinical deterioration too subtle for the clinician to observe without the aid of a computer. This metric may serve as an early warning indicator of critical deterioration in patients with parallel systemic and pulmonary circulation.

Background: The prevalence of obesity in long-term survivors with complex congenital heart disease may be increasing, and little is known about the timing and onset of weight gain and growth patterns in these high-risk patients. Methods and Results: Prevalence rates of overweight/obesity and longitudinal changes in body mass index (BMI) with age were determined in 606 patients with Fontan circulation seen at a tertiary care cardiology center from 1992 – 2012. The number of clinic encounters (n) was stratified by age group (n=401, 2–5y; n= 333, 6–11y; n=217, 12–19y; and n=129, >20y). Among adults, 39% were overweight/obese at last clinic visit; 22% overweight and 17% obese. Childhood anthropometric data were available for 82 adults; of which 15% (n=12/82) were overweight/obese in childhood. The likelihood of being overweight/ obese as an adult was 3 times higher if there was a BMI ≥ 85th percentile in childhood (CI 2.1–4.5, p <0.01). Overweight/obesity in adulthood was associated with lower heart failure rates (4% vs. 19%, P=0.03). Pediatric rates of overweight/obesity were comparable to national data (NHANES 2011–2012) in every age group: at 2–5y, (25 vs. 23%), 6–11y (26 vs. 34%), and 12–19y (15 vs. 35%). Systolic blood pressure was higher in overweight/obese children as young as 2–5 years of age. Conclusions: Childhood and adult survivors with Fontan circulation have high rates of overweight/obesity. Childhood obesity is a strong predictor of future adiposity and is linked to changes in systolic blood pressure at a very young age.