Prevalence of coronary artery lesions on the initial echocardiogram in Kawasaki syndrome.

OBJECTIVES: To determine whether coronary artery lesions (ectasia and aneurysm) are commonly observed on the initial echocardiogram of patients with acute Kawasaki syndrome, whether coronary artery ectasia and/or aneurysms occur more frequently in patients with incomplete Kawasaki syndrome than in those patients with complete findings, and whether earlier diagnosis and treatment of Kawasaki syndrome are associated with less frequent occurrence of coronary artery ectasia and/or aneurysm. DESIGN: A retrospective medical record review. SETTING: A tertiary care pediatric hospital. PARTICIPANTS: One hundred patients treated for Kawasaki syndrome between July 1, 1998, and June 30, 2003, who were identified by a medical record search. MAIN OUTCOME MEASURE: Prevalence of coronary artery lesions (ectasia and aneurysm) on the initial and subsequent echocardiograms. RESULTS: Forty-four percent of patients had a coronary artery lesion (31% with ectasia, 13% with aneurysm) on the initial echocardiogram. Patients with incomplete Kawasaki syndrome were treated significantly later (median, 10 days) and had a significantly higher occurrence of coronary artery aneurysms over the course of their illness (37%) than those with complete Kawasaki syndrome, who were treated at a median of 7 days (P<.001) and had a 12% aneurysm occurrence (P = .009). Patients treated by day 7 of illness had a less frequent occurrence of aneurysm (6%) compared with those patients treated between days 8 and 10 of illness (27%) (P = .03). CONCLUSIONS: Coronary artery lesions are frequently detected on the initial echocardiogram of children with Kawasaki syndrome. If future studies show ectasia to have a relatively high degree of specificity for Kawasaki syndrome, the initial echocardiography may be a useful adjunctive diagnostic test.

Effectiveness of pulse oximetry screening for congenital heart disease in asymptomatic newborns.

OBJECTIVE: To determine the sensitivity, specificity, predictive value, and accuracy of a program of pulse oximetry screening of asymptomatic newborns for critical congenital cardiovascular malformation (CCVM). METHODS: Pulse oximetry was performed on asymptomatic newborns in the well-infant nurseries of 2 hospitals. Cardiac ultrasound was performed on infants with positive screens (saturation 24 hours). Data regarding true and false positives as well as negatives were collected and analyzed. RESULTS: Oximetry was performed on 11 281 asymptomatic newborns, and 3 cases of CCVM were detected (total anomalous pulmonary venous return x2, truncus arteriosus). During the study interval, there were 9 live births of infants with CCVM from a group of 15 fetuses with CCVM detected by fetal echocardiography. Six infants with CCVM were symptomatic before screening. There was 1 false-positive screen. Two infants with negative screens were readmitted (coarctation, hypoplastic left pulmonary artery with aorto-pulmonary collaterals). Other cardiac diagnoses in the database search were nonurgent, including cases of patent foramen ovale, peripheral pulmonic stenosis, and ventricular septal defect. The prevalence of critical CCVM among all live births was 1 in 564 and among the screened population was 1 in 2256 (sensitivity: 60%; specificity: 99.95%; positive predictive value: 75%; negative predictive value: 99.98%; accuracy: 99.97%). CONCLUSIONS: This screening test is simple, noninvasive, and inexpensive and can be administered in conjunction with state-mandated screening. The false-negative screen patients had lesions not amenable to detection by oximetry. The sensitivity, specificity, and predictive value in this population are satisfactory, indicating that screening should be applied to larger populations, particularly where lower rates of fetal detection result in increased CCVM prevalence in asymptomatic newborns.