Cor Vasa 2019, 61(3):e314-e317 | DOI: 10.1016/j.crvasa.2018.07.004

Ebstein's anomaly with significant dysplasia of the tricuspid valve presenting at 73 years of age. A case report

Lumír Koc^a,b, Jiří Ondrášek^c, Tomáš Zatočil^a, Anna Nečasová^a, Jindřich Špinar^a,b

^a Department of Internal Medicine - Cardiology, University Hospital, Brno

^b Faculty of Medicine, Masaryk University, Brno

³ Center of Cardiovascular and Transplant Surgery, Brno

Ebstein's anomaly (EA) is a rare congenital heart defect, characterized by an anomalous tricuspid valve displaced towards the apex of the right ventricle (RV); it is usually accompanied by regurgitation. While its severe forms with an extremely dilated functional RV and oppression of the left heart chambers result in the development of severe heart failure at a young age and, in some cases, even prenatally, patients with mild EA may live until old age with the condition unrecognized. We present the case of a patient with a milder form of EA and a contribution of dysplasia, with the disease undiagnosed until his eighties when he developed heart failure accompanied by significant tricuspid regurgitation. Tricuspid valve replacement with a bioprosthesis and atrial septal defect occlusion were followed by significant improvement of RV function and remission of the patient's problems.

Keywords: Ebstein´s anomaly, Tricuspid regurgitation, Tricuspid valve dysplasia, Tricuspid valve replacement

Accepted: August 11, 2018; Published: June 21, 2019  Show citation