Systemic arterial aneurysm complicated by thrombosis in an infant with resistant Kawasaki disease.

Kawasaki disease (KD) is a systemic vasculitis commonly associated with coronary artery aneurysms. Small-sized and medium-sized systemic arterial aneurysms have also been described, particularly in infants and patients with resistant KD. This case illustrates the presentation of a systemic arterial aneurysm complicated by arterial thrombosis and successful interventional management in a young infant.

Transthoracic Echocardiographic Assessment of Coronary Flow in the Diagnosis of Right Ventricular-Dependent Coronary Circulation in Pulmonary Atresia with Intact Ventricular Septum.

Right ventricular-dependent coronary circulation (RVDCC) is associated with pulmonary atresia with intact ventricular septum and is defined by two or more epicardial coronary arteries with atresia or severe stenosis resulting in the dependency of coronary supply by retrograde flow. The hypothesis of this study is that coronary Doppler flow patterns on echocardiography can be used to distinguish patients with RVDCC. Between 2007 and 2016, we reviewed 16 patients with pulmonary atresia or critical pulmonary stenosis. Patients were divided into two groups, those with RVDCC (determined by angiography or pathology evaluation) and those without. Echocardiographic evaluation of the coronary arteries included 2-dimensional measurements and pulse wave Doppler flow pattern in 3 epicardial coronary arteries. Velocity-time integral (VTI) and maximal velocity (Vmax) were measured and compared between the two groups. Three coronary flow patterns were demonstrated: (1) all antegrade flow, (2) antegrade to retrograde VTI flow ratio > 1, and (3) antegrade to retrograde VTI flow ratio ≤ 1. Of the 7 patients with RVDCC, 6 (86%) had evidence of flow pattern 3 in ≥ 2 of the 3 coronary arteries in contrast to 0 (0%) of the non-RVDCC patients (p = 0.001). Higher retrograde Vmax was associated with RVDCC (p < 0.001) and coronary artery dilatation with Z-score ≥ + 3 was also associated with RVDCC (p = 0.02). Echocardiographic evaluation of the coronaries can be useful in identifying RVDCC. More retrograde flow in at least two coronary arteries is strongly suggestive of RVDCC. Dilatation of the coronary arteries is also supportive evidence.

Complete Atrioventricular Septal Defect with Truncus Arteriosus and Interrupted Aortic Arch in the Fetus.

Complete atrioventricular septal defect is generally diagnosed on the four-chamber view of the fetal heart. This defect can be accompanied by additional outflow tract lesions, including truncus arteriosus. Although truncus arteriosus in isolation can be associated with interrupted aortic arch, we describe a fetal case with the rare association of all three diagnoses: complete atrioventricular septal defect with truncus arteriosus and interrupted aortic arch. This case points to the importance of evaluating the complete fetal heart even after one congenital cardiac abnormality is noted. The complexity of heart disease has implications for prenatal counseling and postnatal management.

Growth and weight gain of prepubertal children after cardiac transplantation.

This study evaluated changes in growth parameters after pediatric heart transplantation and identified factors associated with the changes after pediatric heart transplantation (OHT). We retrospectively evaluated the somatic growth of 46 children <11 yr of age who underwent OHT for changes in weight, height, and BMI. The patient age range was 3.5 months to 10.7 yr. Gain in Z score for weight and BMI was significant at six months post-OHT (mean weight Z score changed from -1.1 to -0.1 and mean BMI Z score changed from -0.1 to 1.3; p < 0.001). After six months post-OHT, there was no further significant change in weight or BMI Z score. Height Z score did not show significant change from pre-OHT at six months, one yr, or two yr post-OHT. Eight patients (17%) became overweight during the two-yr follow-up period as evidenced by a BMI Z score > 2. Multivariate analysis showed length of steroid treatment as a predictor for negative height Z score change, and age at transplant as a predictor for positive height Z score change. Post-OHT, weight significantly increases without proportional increases in height, resulting in a significant proportion of these children becoming obese. Length of steroid therapy is negatively related to the "catch-up" linear growth following OHT.

Echocardiographic predictors of outcome in fetuses with pulmonary atresia with intact ventricular septum.

OBJECTIVE: We sought to identify in utero predictors of postnatal outcomes in fetal patients with pulmonary atresia with intact ventricular septum (PAIVS) or critical pulmonary stenosis. BACKGROUND: Although PAIVS or critical pulmonary stenosis can be diagnosed in utero by echocardiography, our ability to predict outcomes is limited. METHODS: Fetal echocardiograms from 28 patients with PAIVS/critical pulmonary stenosis were retrospectively reviewed. Tricuspid valve (TV) annulus, right and left ventricular internal dimensions, and degree of tricuspid regurgitation were recorded. To establish normal fetal values, echocardiograms from healthy patients were analyzed in an identical fashion. RESULTS: Both a fetal TV z score of -4 or less beyond 23 weeks of gestation and a fetal TV annulus of 5 mm or less beyond 30 weeks of gestation were predictive of poor postnatal outcomes. In addition, right:left ventricular length or width less than 0.5 and/or the absence of tricuspid regurgitation were predictive of poor outcome. CONCLUSIONS: TV annulus size, right:left ventricular ratios, and presence of tricuspid regurgitation on fetal echocardiograms may aid in guiding prenatal counseling regarding postnatal outcome in PAIVS.