Prenatal Diagnosis and Outcome of Ebstein's Anomaly and Tricuspid Valve Dysplasia in Relation to Lung Hypoplasia

Abstract

Although not all newborns with Ebstein's anomaly present with severe cardiomegaly in utero, some of them cannot live after birth because of the lung hypoplasia. T o clarifi the relationship between the intrauterine cardiomegaly and the outcome of the patients with Ebstein$ anomaly or tricuspid valve dysplasia, we calculated the ratio of the area of the heart against the thorax (CTAR) in the transverse view of the thorax at the level of the cardiac four-chamber view in the fetal echocardiogram and compared it to the outcome. The study population consisted of four patients with Ebstein's anomaly and one patient with tricuspid valve dysplasia who were diagnosed i n utero. The ranges obtained from 53 normal fetal cases were 20% 5 8% (mean t 2 SD) at below 20 week's gestation, 25% 2 10% at the gestational age of 21 to 30 weeks, and 29% 2 6.4% at the gestational age of 31 to 40 weeks. The CTARs of these five cases measured 81.6%, 51 %, 55.2%, 47.5%, and 75.6%, respectively, and were abnormully higher than the normal value. Two fetuses died i n utero with severe hydrops fetalis. Two fetuses whose cardiothoracic ratios by chest X ray were 100% died at twelve hours of life. One patient died at 11 0 days. The cross-sectional area of the thorax was smaller than the normal range in 3 (cases 1, 4, and 5) out of these 5 cases. Thus, we conclude that fetal Ebstein's anomaly and tricuspid valve dysplasia associated with massive tricuspid regurgitation with a large CTAR ratio (higher than 50%) and small thoracic cross-sectional area has a very poor prognosis both prenatally and neonatally. (ECHOCARDIOGRAPHY, Volume 11, May 1994) prenatal diagnosis, lung hypoplasia, Ebsteids anomaly, tricuspid dysplasia, cardiothoracic area ratio (CTAR) Ebstein's anomaly is known to have a very wide spectrum of clinical features from a case with an almost normal life span to a case of intrauterine fetal death.'s2 Tricuspid valve dysplasia is a very rare congenital anomaly with very severe regurgitation of the tricuspid valve showing gelatinous nodules and short chordae tendineae of the tricuspid valve and no features of Ebstein's a n~r n a l y .~.~ We have experienced five cases with severe prenatal tricuspid regurgitation, four of whom had Ebstein's anomaly and one who had tricuspid valve dysplasia. We studied the accuracy of prenatal diagnosis of these cases to evaluate the usefulness of prenatal diagnosis of Ebstein's anomaly and tricuspid valve dysplasia. Then, the cardiothoracic area ratio (CTAR) was measured and compared to the normal value in order to assess the severity and outcome of the disease ( Table 1). Diagnostic AccuracyThe gestational period at diagnosis ranged from 23 weeks and 2 days (case 1) to 36 weeks showed massive tricuspid valve regurgitation

Has fetal echocardiography improved the prognosis of congenital heart disease? Comparison of patients with hypoplastic left heart syndrome with and without prenatal diagnosis

“…In recent years, as fetal echocardiography has been gaining in popularity, the number of cases of prenatal diagnosis of congenital heart disease has increased 1 –3 . It is generally accepted that the greater the cardiac anomaly, the easier the diagnosis is to make, as shown in the Metropolitan Atlanta Congenital Defects Program (MACDP) report 4 .…”

Successful neonatal balloon aortic valvoplasty following prenatal diagnosis of critical aortic stenosis

“…Dilation of the left ventricle and left atrium secondary to massive mitral regurgitation may also cause hypoplasia of the lung or ventilatory insufficiency by postnatal compression, as occurred in fetal cases with Ebstein's malformation or tricuspiddysplasia. 7 As Satomi Figure 2. Chest x-ray at birth (A) showed cardiomegaly (cardiothoracic ratio (CTR) ofO.…”

“…The cardiothoracic area ratio as judged echocardiographically was 0.45 ( Figure I). 7 There was a small amount of pericardial effusion. She was transferred to our hospital and had a cesarean section at 34 gestational weeks.…”

Screening for congenital heart disease in high-risk population: Indications for fetal echocardiography

“…Fetuses with congenital heart disease also have a significant risk of having a chromosomal abnormality 24 . Therefore, complete cardiac examination should be performed in all fetuses with anomalies detected by karyotyping and vice versa 2 .…”

“…Non-immune fetal hydrops abnormalnosti 24 . Zato je fetalna ehokardiografija neophodna kod svih fetusa sa detektovanim anomalijama posredstvom kariotipa i obrnuto 2 .…”