The Performance of Critical Congenital Heart Disease Screening in Rural Versus Urban Locations in the Northwest United States.

Objectives: Pulse oximetry screening of newborns detects critical congenital heart disease (CCHD). Rural birth location is known to affect timing and management of when infants with CHD undergo surgery, but its association with CCHD screening is unknown. We assess the relationship between rural location and postnatal CCHD diagnosis and describe lesion-specific modes of diagnosis. Study design: Data were abstracted from medical records at 2 cardiac surgery centers in Washington state. Infants with CCHD, defined as CHD requiring either cardiac intervention or resulting in death at <1 month of age, born between July 2015 and June 2020, were included and classified by method of identification. Patient home ZIP codes were used to determine rural location. Results: We included 561 newborns with CCHD; 35% were diagnosed postnatally. Predominant postnatal mechanisms of identification (n = 194) included symptomatic before CCHD screening period (56%), CCHD screening (22%), and symptomatic after false-negative screen (15%). Postnatal diagnosis rate increased with degree of ruralness (48% in small rural/isolated regions vs 32% in urban regions; P = .01) and incidence of undiagnosed CCHD at birth increased with lower nursery levels (5.5/10 000 live births in nursery level 1 vs 2.1/10 000 live births in level 4). Conclusions: CCHD screening identifies 22% of postnatally diagnosed CCHD and 7% of cases overall in our region. Postnatal diagnosis is more common in rural regions. The incidence of undiagnosed CCHD at birth increases with decreasing nursery levels. This study supports the value of CCHD screening in routine newborn care, especially in rural areas and hospitals with lower nursery levels.

The impact of altitude on screening for critical congenital heart disease.

OBJECTIVES: The objectives were to determine the frequency with which pulse oximetry identifies critical congenital heart defects in asymptomatic full-term and late preterm newborns using the AAP expert panel algorithm in a variety of different hospital settings and to evaluate the impact of altitude on the rate of positive screens. METHODS: We conducted a prospective clinical study of implementation of a newborn pulse oximetry screening for congenital heart disease in 34 independent hospitals. Infants were eligible for enrollment if their gestational age was 35-44 weeks. RESULTS: Of the 34 sites which enrolled infants into our study, 24 were located at or below 2000 feet; 5 were located between 4700 and 6000 feet and 5 were located above 6000 feet in altitude. We screened 6109 infants; 65 (1.1%) had a positive screen. There were no differences in median gestational age, birth weight, mode of delivery or race/ethnicity for infants with a positive screen compared to infants with a negative screen. Infants with positive screens were more often male and more often born at sites located at high altitudes. The frequency of a positive screen increased from 0.2% for infants born at sites at or less than 2000 feet to 6% for sites located above 6000 feet. We stopped enrollment at the site located at 8163 feet after enrolling 65 infants because 23 (35%) were positive. CONCLUSIONS: Screening infants for critical cardiac defects at altitude is complicated by the increased false positive screens.

Quality improvement measures in pulse-oximetry newborn heart screening: a time series analysis.

BACKGROUND AND OBJECTIVES: The use of pulse-oximetry screening to detect critical congenital heart defects in newborns has gained national and international momentum in the past decade. Our hospital system began screening in 2008. Since then, our program has undergone leadership changes and multiple quality improvement interventions. The aims of this study are to evaluate the evolution of our pulse-oximetry program and to provide insights from lessons learned over the course of a long-standing program. METHODS: We reviewed 6 years of screening data and evaluated trends of missed screens, false-positives, protocol violations, and parental decline of screening. We implemented 3 quality improvement interventions (change in protocol, redesign of an electronic medical record documentation system to autocalculate results, and transition from research to standard-of-care) and reviewed the impact of a rigorous quality assurance review process. We used linear regression and statistical process control charts to evaluate the data. RESULTS: A total of 18,363 newborns were screened; we identified 5 critical cases. We observed a significant decrease in missed (P < .001) and false-positive (P = .03) screens over time but found no significant trend in the rate of percentage of protocol violations (P = .26) or decline of screening (P = .99). Each metric showed behavior attributable to at least 1 quality improvement intervention. CONCLUSIONS: We established a sustainable pulse-oximetry screening program in our community hospital system, and the screening has now become routine. The quality of our screening was influenced by choice of screening protocol, rigor of quality assurance reviews, and the process used to interpret screening results.

Leftward displacement of septum primum in hypoplastic left heart syndrome.

Embryologic development of atrial septum primum antedates formation of the atrioventricular and semilunar valves. Leftward displacement of the superior attachment of septum primum (LDSP) has only been described in hypoplastic left heart syndrome (HLHS). This study reports the frequency of LDSP in HLHS and correlates LDSP with other echocardiographic features of HLHS. Preoperative echocardiograms for 72 consecutive patients with classic HLHS from 1996 to 2002 at Children's Hospital of Wisconsin were reviewed. One patient was excluded for inadequate imaging. Data for the 71 patients included the following: size, location, and Doppler gradient across the atrial septal defect (ASD); location of attachment of septum primum; size and patency of the aortic valve (AoV) annulus; size and patency of the mitral valve (MV) annulus; ascending aorta diameter (AAD); and left-ventricular end diastolic dimension (LVEDD). Patients were categorized into three groups: aortic atresia/mitral atresia (AA/MA), aortic atresia/mitral stenosis (AA/MS), and aortic stenosis/mitral stenosis (AS/MS). LDSP was seen in 46 of 71 patients (64 %). By diagnostic group, 32 of 35 patients with AA/MA had LDSP (91 %) compared with 10 of 19 AA/MS patients (53 %) and 4 of 17 AS/MS patients (24 %), p < 0.05. AoV patency was seen in 4 of 46 (9 %) patients with LDSP compared with 13 of 25 (52 %) patients with normal atrial attachment, p < 0.005. Mean left heart dimensions in infants with LDSP compared with normal attachment were as follows: AoV annulus 2.24 versus 3.83 mm, AAD 2.34 versus 4.1 mm, MV annulus 3.21 versus 6.48 mm, and LVEDD 6.38 versus 13.83 mm. By two-way analysis of variance of diagnostic category versus atrial septal attachment with interaction, MV annulus and AAD were independently predicted smaller by LDSP versus normal atrial attachment, p < 0.05. Nonsignificant factors included AoV annulus, LVEDD, ASD size, and Doppler gradient. LDSP correlates with more severe maldevelopment of the left heart in patients with HLHS. Because formation of septum primum precedes development and growth of the intracardiac valves, we speculate that LDSP may be an initiating event in the development of HLHS. In addition, prenatal identification of LDSP may help direct planning of potential in utero therapies.