P1598 Ruptured Sinus of Valsalva: clinical and echocardiographic features at presentation and long-term results after surgical and percutaneous repair

Background Rupture of Sinus of Valsalva (rSOV) is a rare and potentially life-threatening condition often misdiagnosed. Percutaneous device closure has been replacing surgical repair as a treatment of choice. However, long-term outcome is poorly documented. Methods Echo database (2001-2019) was searched for patients >16y with rSOV. Clinical data were collected from ACHD database. Marfan Syndrome patients were excluded. Results Fifteen patients (42.2y, 10M) were diagnosed with rSOVbetween 1979 and 2019. HF symptoms at initial presentation were reported in 50%. All cases were diagnosed by TTE with finding of high velocity continuous flow from SOV to right heart. Two were initially misdiagnosed as VSD. Digital imaging recordings of 10/15 were available. The aortic root was dilated in 8/10. Four had asymmetrical SOV dilatation. LV and LA were dilated in 7/10 and 9/10. Significant aortic regurgitation was in 4. Two had RVOTO. RVSP was always raised. Echo findings are summarised in the Table. Mean FU after repair was 10,7y (0,9-39). Ten patients had surgical repair and 5 device closure. Seven had repair of concomitant lesions (4 VSD closure and 3 AVR) at the time of the procedure. One redo device closure was performed. During FU 1 died from Cardiomyopathy. At the latest FU significant improvement in functional class, LV and LA size and RVSP was found. Conclusion Patients with rSOV may present with acute HF. High velocity continuous flow from SOV to right heart on TTE is characteristic feature for diagnosis. Percutaneous closure is an attractive alternative to surgery in patients with isolated lesion. Significant haemodynamic improvement can be achieved with good long-term haemodynamic results Tot = 15 Initial findings Latest FU NYHA class 4 class I 2 class II 2 class III 4 class IV 3 unknown 11 class I 2 class II 1 class III 0 class IV* SOV (mm) 38.2 (33-44) STJ (mm) 36.2 (26-42 Asc Ao (mm) 30.3 (25-34) rupture site 8 RC sinus, 2 NC sinus Shunt to 8 RVOT, 1 RA, 1 RVOT + RA LVEDV (ml) 169.7 (114-330) 120 (67-230)* LVESV (ml) 66(42-130) 46 (28-80)* LAVi (ml/m2) 53.1 (30-129) 35 (24-53)* LV EF% 61.2 (57-65) 60.4 (50-70) RVSP (mmHg) 53.2 (37-130) 24.06 (15-32)** Pre VS post procedure p-value: * P Abstract P1598 Figure. PLAX in patient with ruptured RC sinus