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Tricuspid valve duplication is an extremely rare condition and in most of the cases it is associated with other congenital cardiac malformations. Because of its rarity, the clinical presentation and the management are not defined yet. Clinical Case We report the case of an 18 y/o caucasian male, who was admitted to our Hospital in February 2018 for rapid atrial flutter not responsive to medical therapy (propanolol and digossin). He had a pre-natal diagnose of ventricular septum defect (VSD) and tricuspid straddling. At 1 year of age he underwent pulmonary artery bandage and one year later VSD closure was performed. Blood test showed sub-clinic hypothyroidism, probably related to previous amiodaron therapy. A transthoracic echocardiogram was obtained. The right atrium (RA) was severely dilated and the atrial septum dislocated towards left ventricle (LV); two right atrioventricular valves (tricuspid valves) were detected: the ‘true’ tricuspid opening was inside the right ventricle, and an ‘accessory‘ opening was located inside the LV and severely regurgitant into the RA; the mitral valve was morphologically and functionally normal; both ventricles were dilated with preserved systolic function; systolic pulmonary artery pressure was not detectable. A Cardiac Magnetic Resonance clearly delineated the anomaly. Atrial flutter radio frequency transcatheter ablation was succesfully performed before corrective surgery. The regurgitant accessory tricuspid orifice was closed with an heterologous pericardial patch and a right reduction atrioplasty was also done. The post-operative course was uneventful and only a mild paraseptal tricuspid jet with LV to RA shunt was present at post op echocardiography. After one year follow-up the patient remained asymptomatic, without arrhythmia recurrence. Conclusion DOTV is an extremely rare condition that could be responsible of severe tricuspid regurgitation. At the moment, there are not sufficient data to establish the correct timing for surgical intervention. In our case, the presence of severe tricuspid regurgitation, right atrium dilatation, biventricular overload and atrial flutter guided the clinical management and suggested surgical correction. Abstract P189 Figure.

P189 Duplication of the tricuspid valve (DOTV): case report of a rare congenital anomaly