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*Corresponding author: King Faisal Specialist Hospital and Research Centre, PO Box 3354, Riyadh, Kingdom of Saudi Arabia. E-mail: valeria.pergola@gmail.com PRESENTATION OF THE CASE A 21-year-old male known to have atrial septal defect (ASD), ventricular septal defect (VSD) and an Ebsteinoid tricuspid valve (TV) underwent ASD and VSD repair and TV replacement with a size 22 mm aortic homograft when he was four years old. The patient had been followed up in our institution, developing progressive TV stenosis and regurgitation. In 2013 he underwent a Bruce protocol exercise test and he was able to achieve 13.1 METS (10 min 55”). He had started complaining of shortness of breath over the past two years. The patient denied any history of loss of consciousness, syncope or chest pain. He presented recently claiming severe shortness of breath. Clubbing and cyanosis of the fingers was present. SpO2 on room air was 80-84%. ECG showed normal sinus rhythm and right bundle branch block (RBBB). On laboratory examination, red blood cells (7.3×1,012/L) and bilirubin (41.7 μmol/L) were elevated. Chest X-ray demonstrated a mildly enlarged heart size. Transthoracic echocardiography (TTE) showed normal left ventricular (LV) size and function, a moderately dilated right ventricle (RV) with moderately to severely impaired function; TV homograft presented grade III-IV regurgitation and TV area by pressure half time was 0.7 cm2. RV systolic pressure was normal. A persistent left superior vena cava (PLSVC) with unroofed coronary sinus (UCS) was demonstrated with saline echo contrast injection via the left antecubital vein, showing bubbles draining through the left atrium (LA) then passing to the LV before appearing in the right atrium (RA) and RV (Figure 1). CASE SUMMARY

eurointervention

How should I treat a left persistent superior vena cava with unroofed coronary sinus associated with severe stenosis and regurgitation of aortic homograft placed in the tricuspid position?