Newborns with a Congenital Heart Defect and Diastolic Steal Have an Altered Cerebral Arterial Doppler Profile.

OBJECTIVES: To compare trends in the anterior cerebral artery (ACA) Doppler markers of vascular flow for neonates with a congenital heart defect (CHD) with and without diastolic systemic steal during the first 7 days of life. METHODS: Prospective study recruiting newborns (≥35 weeks of gestation) with a CHD. Doppler ultrasound and echocardiography were performed daily from day 1 to 7. The cohort was divided into the presence/absence of holo-diastolic retrograde flow in the postductal aorta ("retrograde") on the last-available echocardiogram. Data extractors were masked to retrograde status. Mixed effect models (random slope/intercept) were constructed using RStudio. RESULTS: We enrolled 38 neonates with CHD. Retrograde aortic flow was present on the last echocardiogram in 23 (61%). Peak systolic velocity and mean velocity increased significantly over time, independent of retrograde status. However, having a "retrograde" flow status conferred a significant decrease over time of their ACA-end-diastolic velocity (ß = -5.75 cm/s, 95% CI -8.38 to -3.12, P < .001, when compared with the nonretrograde group), and a significant increase in the ACA resistive (ß = 0.16, 95% CI 0.10-0.22, P < .001) and pulsatility (ß = 0.49, 95% CI 0.28-0.69, P < .001) indexes. No subject presented retrograde diastolic flow in the ACA. CONCLUSIONS: In neonates with CHD in the first week of life, infants with echocardiographic signs of systemic diastolic steal within the pulmonary circulation have Doppler signs of cerebrovascular steal in the ACA.

Can cardiologists distinguish innocent from pathologic murmurs in neonates?

OBJECTIVES: To determine the sensitivity and specificity of the clinical assessment of murmurs in neonates, as performed by pediatric cardiologists, and to identify clinical features that predict the presence of congenital heart disease (CHD) in this population. STUDY DESIGN: Neonates (n = 201) referred for outpatient evaluation of a heart murmur were enrolled consecutively. After a clinical evaluation, the cardiologist documented whether the murmur was "likely innocent" or "likely pathologic." The cardiologist repeated his/her assessment after an electrocardiogram. Echocardiography served as the gold standard. RESULTS: The median age was 12 days (range, 2-31 days). CHD was present in 113 of 201 (56%). Clinical assessment alone identified patients with CHD with a sensitivity of 80.5% (95% CI, 73.2-87.8), specificity of 90.9% (95% CI, 84.9-96.9), positive predictive value of 91.9% (95% CI, 86.6-97.3), and negative predictive value of 78.4% (95% CI, 70.4-86.4). The addition of an electrocardiogram did not improve these test characteristics. Features that were predictive of CHD were murmur quality (P < .0001), location (P = .02), and timing (P = .04). No patients requiring catheter or surgical intervention were missed by clinical assessment. CONCLUSIONS: The prevalence of CHD in this referral population was high. Clinical assessment detected all complex CHD, although some simple lesions were missed. Murmur quality, location, and timing were predictive of CHD.

Cardiac pathology in sudden unexpected infant death.

OBJECTIVES: To determine the spectrum of cardiac pathology and circumstances of death in infants with sudden unexpected death and to define the impact of sudden cardiac deaths to overall sudden infant death. STUDY DESIGN: Retrospective analysis of all autopsies of infants with sudden death 7 days to 2 years of age between January 1987 and December 1999 in the province of Québec (Canada). RESULTS: Eighty-two cases of sudden death with cardiac pathology were found, representing 10% of the total number of sudden infant deaths. A structural malformation was present in the majority of cases (54%); however, cardiac pathology in anatomically normal hearts was also common (46%). Most (64%) anatomic malformations were detected before death compared with 13% of nonstructural heart disease. Although a major proportion of children were found dead during sleep, a significant number were described as being awake at time of death (32%). CONCLUSIONS: Heart disease is present in a significant percentage of autopsies of infants with sudden death. Structural heart malformations predominate, although nonstructural pathologic features of the heart are common and usually unrecognized before an autopsy is performed. Cardiac pathologic features are frequent when the child is witnessed to be awake at the time of sudden death.