PubMed · 647249
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1978-05-20. Ebstein's anomaly.. https://pubmed.ncbi.nlm.nih.gov/647249/
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A 3-month-old infant with corrected transposition of the great arteries and severe systemic atrioventricular valve regurgitation due to "Ebstein-like anomaly" is reported. Through a right thoracotomy and longitudinal left atrial incision, a 19-mm St. Jude Medical valve was implanted into the annulus without removing the native valve. He is doing well 7 months after operation.
The hearts of six neonates with Ebstein's anomaly of the tricuspid valve who died in the 1st month of life were compared with hearts of six age- and size-matched control neonates. All six hearts had morphologically severe disease with gross right atrial dilation and marked apical displacement of the tricuspid valve. All had a secundum atrial septal defect and four had additional cardiac lesions (pulmonary atresia in two, ventricular septal defect in two). There was significant thinning of the right ventricular free wall distal to the tricuspid valve (3 +/- 0.2 mm vs. control 4.2 +/- 0.2, p less than 0.01) and right ventricular fiber diameter was reduced (7.2 +/- 0.3 microns vs. control 11.4 +/- 0.6, p less than 0.001). The fibrous tissue content of both right and left ventricular free walls was increased (right, 29.3 +/- 2.6% vs. control 8.7 +/- 1.1, p less than 0.001; left, 23.2 +/- 1.5% vs. control 8.5 +/- 0.7%, p less than 0.001). Although the right ventricular abnormalities might be explained by hemodynamic stress in utero, abnormalities of the left ventricular free wall suggest that either genetic or nonhemodynamic environmental factors are involved in the morphogenesis of this condition. Increased right and left ventricular fibrosis may contribute to the poor early outcome in this group and may predispose to late complications, such as subnormal exercise performance, hemodynamic deterioration or late sudden death that may occur in patients with Ebstein's anomaly who survive the neonatal period.
OBJECTIVE: To assess the degree of agreement between the fetal echocardiographic and postmortem examination of hearts from fetuses with severe malformations of the tricuspid valve. DESIGN: A retrospective study to analyse echocardiographic recordings and make comparisons with postmortem findings. SETTING: Tertiary referral centre for fetal echocardiography. Institute for cardiac morphology. PATIENTS: 19 cases shown to have severe malformation of the tricuspid valve by fetal echocardiography that died in the prenatal or neonatal period. MAIN OUTCOME MEASURES: Correlations between morphology and measurements made at echocardiography and necropsy. RESULTS: The echocardiographic diagnosis was Ebstein's malformation in seven and tricuspid valvar dysplasia in 12 fetuses. These findings were confirmed in six and eight cases at necropsy. In one false positive diagnosis of Ebstein's malformation, necropsy showed dysplasia of the leaflets of the tricuspid valve without displacement. In four cases with the echocardiographic diagnosis of valvar dysplasia, necropsy showed displacement, the hallmark of Ebstein's malformation. Associated malformations that are known to worsen prognosis were predicted correctly by echocardiography. Taking the mean duration of four weeks between echocardiographic and postmortem investigations, both methods showed cardiomegaly causing lung hypoplasia, right atrial dilatation, and relative hypoplasia of the pulmonary trunk, morphometric factors that may be responsible for the poor outcome. Mostly good agreement existed between the echocardiographic and postmortem measurements if cases with an interval of more than eight weeks between the measurements were excluded. CONCLUSION: Fetal echocardiography was proved to be a reliable technique in differentiating the variants of tricuspid valvar disease, in diagnosing associated cardiac lesions, and in predicting quantitative factors that can define the subsequent outcome.