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The aims of this study were (1) to describe the additive risk of performing cardiac surgery in neonates born ≤ 2.0 kg, after accounting for the baseline risks of low birth weight, and (2) to describe the additive risk of being born ≤ 2.0 kg in neonates undergoing cardiac surgery. We used a risk difference analysis in a retrospective cohort, 2006–2016. Neonates born ≤ 2.0 kg undergoing congenital heart surgery during initial postnatal admission were included. Data were standardized alternatingly for birth weight and cardiac surgical risk using national population data to estimate the number of deaths expected had they not required cardiac surgery or were they of normal weight. Of 105 neonates ≤ 2 kg, median birth weight was 1.6 kg (IQR 1.3–1.8 kg). Median gestational age was 33 weeks (IQR 31–35 weeks). Observed operative mortality was 14.3%; 0% for neonates ≤ 1.0 kg (CI 0–33.6%), 20.6% for neonates > 1.0–1.5 kg (CI 8.7–37.9%), and 12.9% for neonates > 1.5–2.0 kg (CI 5.7–23.9%). Among neonates ≤ 2.0 kg not undergoing cardiac surgery, expected mortality was 4.8% (CI 1.6–10.8); cardiac surgery increased the risk of mortality 9.5% (CI 1.7–17.4%). Conversely, the expected risk for normal birth weight neonates undergoing cardiac surgery was 5.7% (CI 2.1–12.0%); low birth weight increased the risk of mortality 8.6% (CI 0.5–16.6%). To continue making advancements in cardiac surgery, we must understand that the rate of mortality observed in normal weight infants is not a realistic target and that, despite advances, the risk attributable to the surgery remains higher among low birth weight patients.

BACKGROUND: Travel distance to surgical centers may be increased when coverage restrictions prevent children with congenital heart disease (CHD) from receiving care at out-of-state congenital heart surgery centers. We estimated the minimum travel distance to congenital heart surgery centers among publicly insured infants with time-sensitive CHD surgical needs, under two different scenarios: if they were and were not restricted to in-state centers. METHODS AND RESULTS: Using 2012 Medicaid Analytic extract data from 40 states, we identified 4,598 infants with CHD that require surgery in the first year of life. We calculated the minimum travel distance between patients’ homes and the nearest cardiac surgery center, assuming patients were and were not restricted to in-state centers. We used linear regression to identify demographic predictors of distance under both scenarios. When patients were not restricted to in-state centers, mean minimum travel distance was 43.7 miles, compared to 54.1 miles when they were restricted. For 5.9% of patients, the difference in travel distance under the two scenarios exceeded 50 miles. In six states, the difference in mean minimum travel distance exceeded 20 miles. Under both scenarios, distance was positively predicted by rural status, residence in middle-income zip codes, and white/non-Hispanic or American Indian/Alaskan Native race/ethnicity. CONCLUSIONS: For some publicly insured infants with severe CHD, facilitating the receipt of out-of-state care could mitigate access barriers. Existing efforts to regionalize care at fewer centers should be designed to avoid exacerbating access barriers among publicly insured CHD patients.

Cardiac catheterization is commonly performed in patients being supported by extracorporeal membrane oxygenation (ECMO). We aimed to evaluate the safety, benefit, and outcomes of catheterization in pediatric patients supported by ECMO. Retrospective review of cardiac catheterizations performed in patients ≤18 years of age while on ECMO at a large tertiary care center between January 2000 and May 2015. A total of 55 catheterizations were performed on 51 patients during 53 unique ECMO courses. Indications for ECMO include ventricular dysfunction (22), cardiac arrest (20), inability to wean from cardiopulmonary bypass (7), and persistent cyanosis (4). Catheterizations included purely diagnostic studies (11), atrial septostomies (34), stenting of vessels or surgical shunts (6), adjustment of a stent (1), coil embolization (1), and endomyocardial biopsy (1). Septostomy was elective in 58.8% of cases (20) and emergent in 41.2% (14). Forty-six catheterizations had either surgical or catheter intervention during the same or subsequent study (83.6%). High severity complications occurred in three patients (5.6%), including one death due to hemothorax after pulmonary artery stent placement. There were no complications during patient transport. In total, 38 out of 53 (71.7%) ECMO courses resulted in decannulation, 29 (54.7%) patients survived to discharge from the hospital, and 25 (47.2%) were alive at follow-up. Cardiac catheterization can be safely performed on patients supported by ECMO. Cardiac catheterization is a critical tool in the early recognition, diagnosis, and direct treatment of hemodynamic/anatomic abnormalities in patients supported by ECMO.

It is universally recognized that the prevention of sudden cardiac death (SCD) in youth is an important public health initiative. The best approach remains uncertain. Many European and Asian countries support the use of electrocardiograms (ECGs). In the United States, this is highly controversial. Many debate its cost-effectiveness. We designed a comprehensive economic model of two of the most prevalent causes of SCD identifiable by ECG, hypertrophic cardiomyopathy (HCM) and long QT syndrome (LQTS), to determine the drivers of uncertainty in the estimate of cost-effectiveness. We compared the cost-effectiveness of screening with history and physical examination (HP) plus ECG to the current United States standard, HP alone, for the detection and treatment of HCM and LQTS. We used a Markov model on a theoretical cohort of healthy 12-year-olds over a 70-year time horizon from a societal perspective, employing extensive univariable and probabilistic sensitivity analyses, to determine drivers of costs and effectiveness. The incremental cost-effectiveness of adding ECGs to HPs was $41,400/life-year saved. The model was highly sensitive to the effect of identification and treatment of previously undiagnosed individuals with HCM; however, it was insensitive to many variables commonly assumed to be significant, including the costs of ECGs, echocardiograms, and genetic testing, as well as the sensitivity and specificity of ECGs. No LQTS-related parameters were significant. This study suggests that the key to determining the cost-effectiveness of ECG screening in the United States lies in developing a better understanding of disease progression in the previously undiagnosed HCM population.

Sinusoids and coronary arterial fistulae are well described in fetuses and infants with single ventricles. Coronary arteriopathy is well described as a cause of myocardial infarction in adults and in children with familial hypercholesterolemias. To the best of our knowledge, pathologic alterations in coronary arteries (coronary arteriopathy) have only twice before been described as the cause of infarction in neonates. We present the case of a newborn with perinatal myocardial infarctions and death in the setting of extensive coronary arteriopathy and tricuspid atresia. The child had a pulseless arrest immediately after birth. Autopsy showed multiple areas of infarction ranging in age from acute to >10 days old.