Ebstein pulmonary hypoplasia

32 babies aged under three months underwent emergency echocardiography before further investigation and treatment. The diagnosis was made in 30 cases, a significant improvement on clinical examination alone. The echocardiographic analysis was concentrated on:--the interventricular septum,--septo-aortic continuity,--mitro-aortic continuity,--the position of the aorta,--the dimensions of the aorta, from which the size of the pulmonary artery could be deduced in cases of VSD with pulmonary obstruction. This analysis is completed by a study of the other cardiac structures, to give a rapid and reliable diagnosis...

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The reliability of prenatal ultrasound (US) for the detection of structural heart defects was investigated in a consecutive population of patients referred for prenatal US. Twenty-eight of 49 fetuses (57%) shown to have one or more heart defects with physical examination or autopsy were found to have congenital heart disease with prenatal US. There were 66 cardiac defects in 49 fetuses, 33 of which were entirely or partly identified (50%). Defects correctly diagnosed included hypoplasia of either ventricle, common atrioventricular canal, tetralogy of Fallot, transposition of the great vessels, and Ebstein anomaly...

The morphologic spectrum of malformations of the tricuspid valve in 14 fetal and 5 neonatal heart specimens is described. Echocardiography during fetal life had revealed the valvular malformations along with dilation of the right-sided chambers of the heart and atrioventricular (AV) valve incompetence. This functional impairment reflected either Ebstein's malformation (10 cases) or dysplasia of valve leaflets having a normal junctional attachment (9 cases). The right atrium and right ventricle were markedly enlarged, and heart weight was increased to 181% (median) and 179% (median) in the groups with Ebstein's malformation and valvular dysplasia, respectively...

The echocardiographic studies and clinical course of 27 fetuses (mean gestational age 26.9 weeks) diagnosed in utero with tricuspid valve disease and significant tricuspid regurgitation were reviewed. The diagnosis of Ebstein's anomaly was made in 17 of the fetuses, 7 had tricuspid valve dysplasia with poorly developed but normally attached leaflets and 2 had an unguarded tricuspid valve orifice with little or no identifiable tricuspid tissue. One fetus was excluded from data analysis because a more complex heart lesion was documented at autopsy...

A case of Ebstein's anomaly with pulmonary hypoplasia diagnosed in utero by color Doppler echocardiography in a 26 weeks fetus is presented. We emphasize the importance of ultrasound in the early detection of this congenital abnormality that allowed our medical team to offer correct assistance to a severe ill child at birth.

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...

The echocardiographic findings in a case of Uhl's anomaly, or congenital hypoplasia of the right ventricular myocardium, are reported. Diastolic opening of the pulmonary valve is described. Comparison is made with echocardiograms in Ebstein's disease of the tricuspid valve, and it is suggested that echocardiography can help in differentiating these two entities. In addition to the pulmonary valve finding, increased right ventricular dimension, delayed tricuspid closure and abnormality (prolapse) of the mitral valve were noted...

The special pathology of reconstructable or only prosthetically correctable congenital malformations of the mitral valve is described on the basis of the following examples taken from our own operative and autopsy material of the last 5 years: 1. Congenital isolated mitral stenosis in female twins (7 month old infant and 33 month old child). 2. Congenital isolated mitral insufficiency in a 7 1/2 year old boy. 3. Combined mixed mitral valve malformations with a parachute valve-like mitral valve anomaly, combined with hypoplasia of the ascending and descending aortas, in a 6 1/2 year old girl...

The morphological features of a series of 37 specimens of pulmonary atresia with intact ventricular septum were reviewed with particular emphasis on features which might influence the results of pulmonary valvotomy. The degree of right heart hypoplasia was quantified by measuring right and left heart dimensions and comparing them with 20 normal infant hearts. Right ventricular cavity size was usually smaller than normal but constituted a spectrum ranging from tiny to a dilated ventricle larger than normal. There was a positive correlation between triscuspid annular size and right ventricular size but no correlation between the size of the pulmonary artery and the right ventricle...

Prostaglandin-E (PGE) infusions have been used in an attempt to increase ductal patency in 11 infants aged one to 99 days with cyanotic heart disease. PGE1 was used in nine infants and PGE2 in two. Five patients had pulmonary atresia, four extreme pulmonary stenosis, one Ebstein's anomaly and one simple transposition of the great arteries. All but the oldest infant showed a satisfactory increase in oxygen saturation (average 36%) attributed to dilatation of the ductus. The failure in one infant may have been due largely to hypoplasia of the left pulmonary artery...