Undetected Critical Congenital Heart Disease

Years Active: 2011-2012

Congenital heart disease occurs in 7 to 9 of 1000 live births in the United States. One-fourth of these infants have critical congenital heart disease (CCHD) which is defined as severe and life-threatening disease requiring surgical or catheter intervention in the first year of life. Diagnosis places the infant at risk for hemodynamic compromise secondary to hypoxia, acidosis, and shock.

The goal of the Undetected Critical Congenital Heart Disease (CCHD) Registry was to identify infants discharged from the nursery who return with a diagnosis of critical congenital heart disease (congenital heart disease requiring surgical or catheter based intervention within the first month of life). In 2011, 11 infants were identified, and in 2012, 8 infants were initially discharged from the hospital without a diagnosis of CCHD.

The Tennessee pulse oximetry-screening program for CCHD was officially begun in January of 2013, by direction of the Tennessee State Legislature. With hospitals across the state initiating CCHD screening programs, the first case of CCHD detected via pulse oximetry in Tennessee was identified. The infant failed the screen and an echocardiogram diagnosed pulmonary atresia with ventricular septal defect and multiple aorto-pulmonary collateral arteries.

Tennessee pediatric cardiologists partnered with TIPQC and the Tennessee Department of Health to track undetected infants with CCHD by creating an Undetected Critical Congenital Heart Disease Registry. This registry serves to monitor the implementation of statewide implementation of pulse oximetry screening, particularly to collect data on infants with false negative screens (babies who pass the screen, but subsequently present with CCHD).

This project was led by Dr. Bill Walsh, and includes: Memphis-Jean Ballweg, Marilyn Robinson-Lebonheur, ETSU-Mike Devoe, Rajani Anand, Ehrlanger-Nisha Surenderanath, Knoxville-Jeff Jennings, Mike Liske, Vanderbilt-Jessica Mouledoux,and the State Birth defects registry-David Law.

UCCHD Before and After SaO2 Screening

Project Aim

The aim of this project is to improve the health of infants in Tennessee by providing a statewide registry of infants with CCHD whose heart disease goes undetected until after discharge from the hospital. The immediate goal was, to monitor the implementation impact of mandatory discharge SaO2 screening. Subsequently, the project aims to provide a statewide repository of cases that would provide a data set for further refinement of a CCHD screening system. Provisional preliminary data through 2013 is shown above.