Intrauterine hemodynamics of tricuspid valve dysplasia in a growth-restricted infant.

We present a case of fetal tricuspid valve dysplasia, which was diagnosed at 22 weeks of gestation during a routine obstetrical examination. Serial fetal echocardiographic evaluation revealed progressive right ventricular outflow tract obstruction and persistent cardiomegaly. A female infant weighing 1,916 g was delivered by elective cesarean section at 38 weeks of gestation. Longstanding compression of the fetal lungs secondary to the persistent cardiomegaly resulted in severe respiratory distress and cyanosis immediately after birth. Although, mechanical ventilation and continuous infusion of prostaglandin were instituted, the infant died of respiratory failure at 21 days of age. During the pregnancy, the fetus exhibited intrauterine growth restriction, but hydrops did not occur. In regard to the fetal hemodynamics in this cardiac anomaly, transatrial communication is essential for fetal survival. The diameter of the fossa ovalis, which is a marker of transtrial blood flow, was adequate in this case. However, marked enlargement of the right heart associated with regurgitation interfered with left ventricular filling and output, which resulted in restriction of the combined ventricular output and intrauterine fetal growth restriction.

Revision of diagnostic guidelines for Kawasaki disease (the 5th revised edition).

Diagnostic guidelines for Kawasaki Disease was revised to meet the present situation in 2002. This issue intends to explain new guidelines and their backgrounds. Major alterations are interpretation of cases with 4 or fewer febrile days shortened by early intravenous immunoglobulin treatment, and the clinical importance of atypical (incomplete, or suspected) cases.

Prenatal diagnosis of congenital heart disease: clinical experience and analysis.

Over a five-year period, we reviewed 19 fetuses who were prenatally diagnosed with congenital heart disease, including hemodynamically significant arrhythmias. Five of them had fetal tachyarrhythmias, and 14 had structural heart disease. The outcomes were: six intrauterine deaths, five neonatal deaths, and three infant surgeries. Six of the fetuses had chromosomal abnormalities, four had extracardiac anomalies, and two had hydrops fetalis. Of the 96 neonates with congenital heart disease found during the study period, the overall detection rate was 20%; 16% of the neonates with structural cardiac defects and 83% of the neonates with arrhythmias. Some of the complex cardiac defects with normal fetal four-chamber view were difficult to detect prenatally. During the course of the pregnancy, 37% of the fetuses with prenatally diagnosed congenital heart disease were found to have intrauterine growth retardation, and 26% were found to have an abnormal amniotic fluid volume. In view of our findings, a comprehensive screening system should be more frequently considered in order to improve both detection rate and perinatal management.