Heart failure in a patient with Ebstein disease

 Ebstein's disease is a rare malformation of the tricuspid valve, accounting for approximately 1% of all cardiac malformations. It is an anomaly of the tricuspid valve where most often the 3 sheets are involved, to varying degrees, preventing the tricuspid valve from working properly. A 32 years old women was addressed to the hospital for palpitations, chronic dyspnea, edema of the lower limbs, and cyanosis. She has no history, nor known cardiovascular risk factors. The initial somatic examination showed signs of right heart failure with blood pressure at 132/75 mmHg, and heart rate at 130 beat per minute. At cardiac auscultation, we noted a systolic murmur on all the precordium, maximum at the tricuspid focus, without signs of left heart failure. The electrocardiogram revealed a common flutter. The echography showed an enormous dilation of the right cavities with atrialization of the ventricule by a low insertion of the tricuspid valve towards the right ventricule tip with offset between the mitral and tricuspid valve above 20mm. The doppler revealed severe tricuspid regurgitation with a pulmonary hypertension estimated at 86 mmHg. After optimal treatment by converting enzyme inhibitor, digitalics, and diuretics,the patient was referred for tricuspid valve replacement associated to an atrialized plication of the right ventricule. Ebstein's disease is a rare malformation of the tricuspid valve. The risk of sudden death remains high and unpredictable even in operated subjects with post-operative clinical improvement.