Congenital hepatic arteriovenous malformation: an unusual cause of neonatal persistent pulmonary hypertension.

Congenital hepatic arteriovenous malformations are rare anomalies, which typically present in infancy with congestive heart failure, anemia, and hepatomegaly. Morbidity and mortality is high if the condition is not recognized and treated promptly. Hepatic arteriovenous malformation associated with persistent pulmonary hypertension of the newborn has been reported in two cases in the literature. We report a neonate who was referred for management of persistent pulmonary hypertension and was subsequently diagnosed with a large hepatic arteriovenous malformation. He underwent coil embolization following which pulmonary hypertension resolved.

Two-dimensional echocardiographic valve measurements in healthy children: gender-specific differences.

The goal of this study was to create nomograms of echocardiographic two-dimensional valve dimensions based on a large group of children without heart disease. Children aged 0-18 years underwent standard echocardiographic evaluation. Referring diagnoses were chest pain, heart murmur, or syncope. Only patients with a structurally normal heart and normal systolic and diastolic function were included. All four valves were measured at their maximal dimensions. A total of 748 children (314 girls and 434 boys) met the inclusion criteria. Mean values and standard deviations were calculated, and z value nomograms based on body surface area were developed. Surprisingly, the boys had larger valve dimensions at all ages. These valve dimension differences were statistically significant for three of four valves even after adjustment for the differences in body sizes. The difference may be due to higher circulating blood volume in boys compared to that in girls. Because the differences are subtle, they reach statistical significance only when evaluated in a large group of subjects. Presented normal value data will be helpful in following cardiology patients and evaluating intervention strategy in patients with valve hypoplasia.

Dobutamine stress echocardiography in the evaluation of young patients with Kawasaki disease.

There has been no consistent approach to the follow-up of Kawasaki disease patients for remote coronary perfusion abnormalities. Dobutamine stress echocardiography (DSE) has become a standard method for evaluation of perfusion abnormalities in adults with coronary artery disease. In addition, DSE has been used with success in some pediatric patients. The purposes of this study were to evaluate safety and accuracy of DSE in the follow-up of patients with Kawasaki disease, to evaluate whether DSE adds any additional value to the resting echocardiogram, and to determine the association of DSE results with American Heart Association (AHA) risk level categories. DSE was performed 1 month to 13 years after acute Kawasaki disease in 47 patients (range, 3.8-22.6 years; 33 males and 16 females). Patients were stratified according to AHA risk level categories (I-V). Ischemia was defined as a new or worsening regional wall motion abnormality or >1 mm ST segment depression on the electrocardiogram during DSE. In 45/47 patients, DSE was completed successfully (i.e., achievement of target heart rate or development of ischemia). No patients in risk levels lower than V (i.e., patients without coronary artery stenoses) had positive DSE, whereas 2/4 (50%) in the risk level V category had positive DSE, both of whom had coronary occlusion >50% confirmed by angiography. Of the 2 AHA risk level V patients with negative DSE, 1 had extensive collateralization and the other had coronary obstruction <50%. DSE is a safe and feasible method for the evaluation of children with Kawasaki disease. DSE provides a confirmatory benefit and may be a useful screening alternative to cardiac catheterization during follow-up. Patients in AHA risk levels I-IV are unlikely to have dobutamine-induced coronary perfusion abnormalities. Patients in the risk level V category may or may not have positive DSE depending on the degree of both coronary obstruction and collateralization.