209 a common case of aortic stenosis in a unique quadricuspid anatomy: management in real life

Abstract A 85-year-old lady with severe dyspnea presented in emergency dept. of our institution in Reggio Emilia last July. Typical signs and symptoms of heart failure were associated with high voltage at ECG and echocardiographic findings show a reduced EF (40%) with trans-aortic mean gradient of 42 mmHg, aortic valvular area of 0.8 cm2 and moderate aortic regurgitation. At CT scan analysis, a dismorphic quadricuspid aortic valve (QAV) was detected. This is a rare congenital heart disease generally unrelated to other cardiac abnormalities and usually diagnosed incidentally. Due to QAV stenosis rarity, many considerations in Trans Aortic Valve Implantation (TAVI) in QAV are unclear. Some individuals with QAV develop aortic regurgitation and rarely other cardiovascular complications such as aortic aneurysm. Because of its infrequency, it is difficult to characterize these patients and standardize their management. Tailored therapy is more than ever necessary. Our patient also had bicameral pacemaker for grade II Mobitz 2 atrioventricular blockage. At coronarography angiography mild atheromasia was detected; AngioCT reconstructions in figure 1 panel A and B show the presence of QAV. Severe wall atheromasia along the entire course of the aorta was also detected. A few days later the patient underwent the TAVI procedure. A coronary guidewire was positioned in right coronary artery in order to protect its ostium that was slightly lower and, as known from literature, QAV has a longer leaflet height and a shallower cusp depth. After implantation of a Balloon Expandable Valve, a chimney stenting was also performed. An excellent final result was achieved with a safe deployment and a 90/10 final position (last aortography frame in Figure 2, where both coronary arteries are well visible). Post-procedural echo follow-up showed a well functioning aortic prosthesis (with mean gradient of 9 mmHg). The case presented is particularly interesting mainly for three reasons: Extreme rarity of this condition that can be challenging especially in the elderly people with calcific valve affected by stenosis and regurgitation;Paucity of procedural data in literature on QAV;It is a real life management example with subsequent successful implantation of the BEV and chimney stenting in QAV;