Ebstein's disease. Study of 5 personal cases
Cariou, M.; Tatibouet, L.; Meudic, P.
La Semaine des Hopitaux Organe Fonde Par l'Association d'Enseignement Medical des Hopitaux de Paris 53(40): 2257-2265
1977
ISSN/ISBN: 0037-1777 PMID: 204042 Document Number: 113962
Ebstein's disease is a frequently cyanotic form of congenital heart disease. The lesions consist of low implantation in the right ventricle of the tricuspid valves, often associated with an atrial septal defect. Our five cases (two men and 3 women) had a variable clinical presentation depending on the degree of the variable clinical presentation depending on the degree of the lesions. A fairly exceptional fact should be emphasised, which is that 3 of our patients brought to term or almost to term 3 pregnancies and gave birth to live children. Ebstein's disease should be suspected when a large heart with a rugby football shape is discovered on X ray and E.C.G., together with right atrial hypertrophy, frequent elongation of the P-R interval, low voltage and complete right bundle branch block. The diagnosis can be confirmed by angiocardiography and catheterisation. Angiocardiography shows a large right atrium and a distal right ventricle in the form of a small chamber with thin walls. Catheterisation is essential and detects the atrial septal defect in 80% of cases and a pressure gradiant intermediate between atrial and ventricular pressures, revealing ventricular morphology. Surgical treatment will be either palliative (cavo-pulmonary anastomosis) or curative: Hunter's operation (Lillehei-Hardy) implantation of the tricuspid on the atrio-ventricular ring. The prognosis is then worse.