Clinical introduction

A 39-year-old man was referred with a history of progressive shortness of breath, NYHA class II, for further evaluation. Clinical examination revealed prominent V-waves in the jugular venous pulse, a parasternal heave and a soft systolic murmur at the left sternal edge. Respiratory function tests were normal and d-dimer was negative. Echocardiography demonstrated severe RV dilation, severe tricuspid regurgitation and an apically displaced septal leaflet of the tricuspid valve typical of Ebstein's anomaly (see online supplementary appendix movie 1). An abnormality was noted attached to the atrial aspect of the tricuspid valve on transthoracic and transoesophageal imaging (Figure 1A, B as well as online …