Scott B. Yeager

Distribution of and Mortality From Serious Congenital Heart Disease in Very Low Birth Weight Infants

OBJECTIVE: To characterize serious congenital heart disease in very low birth weight (VLBW) infants (born at <1500 g or a gestational age of 22–29 weeks) in a large, international database. PATIENTS AND METHODS: We analyzed a database of 99 786 VLBW infants born or treated at 703 NICUs between calendar years 2006 and 2007. We defined serious congenital heart disease as 1 of 14 specific lesions or any other structural congenital heart disease that required surgical or medical treatment by initial hospital discharge or by the age of 1 year. We reviewed records for all infants with cardiac diagnoses and other genetic syndromes and associations to determine which had serious congenital heart disease. We excluded nonstructural disease as well as isolated and untreated atrial or ventricular septal defects. We determined the frequency of serious congenital heart disease, compared overall mortality rates of those with and without serious congenital heart disease, and determined the distribution of specific lesions and mortality for each diagnosis. RESULTS: Of 99 786 VLBW infants studied, 893 had serious congenital heart disease (8.9 per 1000). The most common lesions were tetralogy of Fallot (n = 166 [18.6% of those with serious congenital heart disease]), aortic coarctation (n = 103 [11.5%]), complete atrioventricular canal (n = 81 [9.1%]), pulmonary atresia (n = 73 [8.2%]), and double-outlet right ventricle (n = 68 [7.6%]). The mortality rate of those with serious congenital heart disease was 44%, compared with 12.7% in those without serious congenital heart disease (P < .0001). CONCLUSIONS: Serious congenital heart disease is probably more frequent in VLBW infants treated in NICUs than in the general live-born population, and the distribution reflects lesions associated with extracardiac malformations. VLBW infants with serious congenital heart disease have higher a mortality rate than those without, independent of other risk factors.

Pretransport and posttransport characteristics and outcomes of neonates who were admitted to a cardiac intensive care unit

OBJECTIVE. The objective for this study was to characterize the impact and the safety of transporting neonates with known or suspected cardiac abnormalities. METHODS. We reviewed retrospectively the charts and computerized records of 192 admissions to a cardiac ICU in 2002. Patients were included when they were <28 days of age at admission and were transported from adjacent obstetric facilities (local N = 70) or other inpatient medical facilities (transport N = 122). Demographic, clinical, pharmacologic, laboratory, and diagnostic information was obtained before transport (when available) and within 3 hours of arrival. Arrival status was considered optimal when measured metabolic and clinical parameters all were within range. Outcome variables included days on ventilator, days in ICU, days in hospital, and death. RESULTS. Of local admissions, 31 (44%) patients had 61 suboptimal arrival values, including pH <7.25 (n = 11), saturation <70% (n = 12), and temperature <36°C (n = 9). There were 69 undocumented values in 39 patients. Of transported patients, 55 (45%) had 86 suboptimal arrival values, including pH <7.25 (n = 8), saturation <70% (n = 14), and temperature <36°C (n = 13). There were 98 undocumented values in 53 patients. No in-transport deaths or catastrophic events occurred. Local admissions were more likely to have a prenatal diagnosis of heart disease and had more complex disease and higher mortality. Other outcome parameters were not significantly different between the 2 groups. Low admission arterial saturation, pH, and core temperature were not correlated with adverse outcome measures. CONCLUSIONS. Although we did not encounter major transport complications, opportunities exist to optimize arrival status and improve surveillance and documentation.

Serious Congenital Heart Disease and Necrotizing Enterocolitis in Very Low Birth Weight Neonates

BACKGROUND Infants with serious congenital heart disease (CHD) appear to be at increased risk for necrotizing enterocolitis (NEC). This study aimed to quantify the incidence and mortality of NEC among very low birth weight (VLBW) neonates with serious CHD, and identify specific CHD diagnoses at the highest risk for developing NEC. STUDY DESIGN Data were prospectively collected on 257,794 VLBW (401 to 1,500 g) neonates born from 2006 to 2011 and admitted to 674 Vermont Oxford Network US centers. Entries were coded for specific CHD diagnoses and reviewed for completeness and consistency. Survival was defined as alive in-hospital at 1 year or discharge. RESULTS Of eligible neonates, 1,931 had serious CHD. Of these, 253 (13%) developed NEC (vs 9% in infants without CHD, adjusted odds ratio [AOR] 1.80, p<0.0001). Mortality for neonates with CHD and no NEC was 34%, vs 55% for those with CHD and NEC (p<0.0001). Both groups of CHD patients had higher mortality than infants with NEC without CHD (28%, p<0.0001). Although NEC mortality overall decreases with higher birth weight, mortality for NEC and CHD together does not. CONCLUSIONS The incidence of NEC is significantly higher in VLBW neonates when CHD is present. The mortality of CHD and NEC together is substantially higher than that with each disease alone. Infants with atrioventricular canal appear to have higher risk for developing NEC than other CHD diagnoses. In addition to providing benchmark incidence and mortality data, these findings may have utility in the further study of the pathophysiology of NEC.

Identification of Critical Congenital Heart Disease in Vermont The Role of Universal Pulse Oximetry Screening in a Rural State

Objective. Pulse oximetry screening (POS) is becoming the standard of care in screening for critical congenital heart disease (CCHD). Our objective was to characterize the historical diagnostic pattern in a rural tertiary care children’s hospital and explore how universal POS might affect morbidity, mortality, and care delivery. Patients and Methods. We identified patients <6 months of age in the Vermont Children’s Hospital echocardiogram database with CCHD diagnosed between 2002 and 2011. Charts were reviewed to characterize timing of diagnosis, course, and outcome. The medical examiner was consulted to identify deaths due to undetected CCHD during the study period. Results. Of 60 329 live births, 73 (0.12 %) were diagnosed with CCHD. Of these, 31 (42%) were diagnosed prenatally, 34 (47%) were diagnosed by clinical examination in the nursery, 7 (9.6%) were diagnosed after nursery discharge, and 1 (1.4%) was born at home. The 8 patients not diagnosed by prenatal ultrasound or in the nursery were considered cases of undetected CCHD. Three had normal oxygen saturation (>95%) at diagnosis. Three presented with cardiovascular compromise. None died and all were well at the most recent follow-up. Review of autopsy reports from patients <6 months found no deaths from undetected CCHD during the study period. Conclusions. Over a 10-year period at our institution, universal POS could have identified 5 patients with undetected CCHD, possibly avoiding the need for resuscitation in 3. Examination of local diagnostic data may affect the cost/benefit considerations of universal POS initiatives in a setting of limited healthcare resources.

Fate of Moderate and Large Secundum Type Atrial Septal Defect Associated With Isolated Coarctation in Infants

A moderate to large secundum atrial septal defect associated with isolated coarctation in infancy is likely to close spontaneously. Nine of 12 defects, including 4 defects > or =8 mm in at least 1 dimension, had either closed or were believed to be hemodynamically insignificant at follow-up, supporting the strategy of a lateral thoracotomy approach to coarctation and deferral of atrial surgery.