Report of the National Heart, Lung, and Blood Institute Working Group: An Integrated Network for Congenital Heart Disease Research.

The National Heart, Lung, and Blood Institute convened a working group in January 2015 to explore issues related to an integrated data network for congenital heart disease research. The overall goal was to develop a common vision for how the rapidly increasing volumes of data captured across numerous sources can be managed, integrated, and analyzed to improve care and outcomes. This report summarizes the current landscape of congenital heart disease data, data integration methodologies used across other fields, key considerations for data integration models in congenital heart disease, and the short- and long-term vision and recommendations made by the working group.

Automated prediction of cardiorespiratory deterioration in patients with single-ventricle parallel circulation: A multicenter validation study.

Objectives: Patients with single-ventricle physiology have a significant risk of cardiorespiratory deterioration between their first- and second-stage palliation surgeries. Detection of deterioration episodes may allow for early intervention and improved outcomes. Methods: A prospective study was executed at Nationwide Children's Hospital, Children's Hospital of Philadelphia, and Children's Hospital Colorado to collect physiologic data of subjects with single ventricle physiology during all hospitalizations between neonatal palliation and II surgeries using the Sickbay software platform (Medical Informatics Corp). Timing of cardiorespiratory deterioration events was captured via chart review. The predictive algorithm previously developed and validated at Texas Children's Hospital was applied to these data without retraining. Standard metrics such as receiver operating curve area, positive and negative likelihood ratio, and alert rates were calculated to establish clinical performance of the predictive algorithm. Results: Our cohort consisted of 58 subjects admitted to the cardiac intensive care unit and stepdown units of participating centers over 14 months. Approximately 28,991 hours of high-resolution physiologic waveform and vital sign data were collected using the Sickbay. A total of 30 cardiorespiratory deterioration events were observed. the risk index metric generated by our algorithm was found to be both sensitive and specific for detecting impending events one to two hours in advance of overt extremis (receiver operating curve = 0.927). Conclusions: Our algorithm can provide a 1- to 2-hour advanced warning for 53.6% of all cardiorespiratory deterioration events in children with single ventricle physiology during their initial postop course as well as interstage hospitalizations after stage I palliation with only 2.5 alarms being generated per patient per day.

Extracorporeal membrane oxygenation after stage I reconstruction for hypoplastic left heart syndrome.

OBJECTIVE: Although extracorporeal membrane oxygenation (ECMO) is an acceptable strategy for children with refractory cardiac dysfunction after cardiac surgery, its role after stage I reconstruction for hypoplastic left heart syndrome and its variants is controversial. Our objective is to describe the outcome of "nonelective" ECMO after stage I reconstruction. DESIGN: Retrospective case series. SETTING: Pediatric cardiac intensive care unit. PATIENTS: Infants placed on ECMO after stage I reconstruction from January 1998 to May 2005. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Of the 382 infants who underwent stage I reconstruction during the study period, 36 (9.4%) required ECMO in the postoperative period. There were 22 infants with hypoplastic left heart syndrome. Indications for ECMO included inability to separate from cardiopulmonary bypass in 14 and cardiac arrest in 22. Fourteen infants (38.8%) survived to hospital discharge. Nonsurvivors had longer cardiopulmonary bypass time (150.1 +/- 70.0 mins vs. 103.9 +/- 30.0 mins, p =. 01). 9/14 infants (64%) supported with ECMO> than 24 hrs after stage I reconstruction survived while only 5/22 infants (22%) requiring ECMO< 24 hrs of stage I reconstruction survived (p =. 02). Of note, all five infants diagnosed with an acute shunt thrombosis were early survivors. Mean duration of ECMO was 50.1 +/- 12.5 hrs for survivors and 125.2 +/- 25.0 for nonsurvivors (p =. 01). 7/14 early survivors are alive at a median follow-up of 20 months (2-78 months). CONCLUSIONS: In our experience, ECMO after stage I reconstruction can be life saving in about a third of infants with otherwise fatal conditions. It is particularly useful in potentially reversible conditions such as acute shunt thrombosis and transient depression of ventricular function.