An 86-year-old man with an history of degenerative aortic stenosis (, Video S) and treated by TAVI (Edwards Sapiens 3, 29 mm) 3 years ago, coronary artery disease (pre-TAVI coronary angiography with a 50–70% bifurcation lesion of the anterior descending involving the first diagonal branch), a permanent bicameral pacemaker implanted 6 months ago because sick sinus syndrome and arterial hypertension. In the month following the TAVI procedure, an Ao-RV fistula was noticed during a routine transthoracic echocardiography with a restrictive, non-significant shunt (, Videos S and S). No specific treatment was required and a standard pharmacological treatment with conventional clinical and echocardiography follow-up was programmed.\nDuring the last year, the patient had worsening heart failure symptoms (progressive oedema of the lower limbs, New York Heart Association dyspnoea class III, and acute pulmonary oedema episodes) which prompted multiple hospitalizations. A new severe mitral insufficiency was diagnosed at echocardiography follow-up by his cardiologist. After case review by our hospital heart team, a surgical mitral valve replacement was decided despite the complexity of the case and high perioperative risk (EUROSCORE II 28.18%, Clinical frailty scale 4/7 ‘Vulnerable’) due to the lack of other therapeutic options offering a complete treatment. He was admitted for elective surgery and patient consent was obtained before the procedure for the academic use of his clinical data. At admission, the patient had a regular cardiac rhythm a 70 b.p.m., with a pansystolic 3/6 mitral regurgitation murmur, basal bilateral pulmonary rales, a discrete lower limb oedema, and a blood pressure of 125/56 mmHg.\nThe preoperative transthoracic echocardiography confirmed a degenerative and calcified mitral valve with a posterior jet of severe regurgitation of mixed aetiology mainly due to tethering of P2 secondary to left ventricular remodelling (Carpentier classification IIIb; , Video S), a dilated left ventricle (telediastolic volume index of 97 mL/m2) with an ejection fraction of 46%, anterior wall hypokinesia (, Video S), a dilated left atrium (55 mL/m2), and a systolic pulmonary artery pressure estimated at 45 mmHg. The TAVI valve had a mean gradient of 6 mmHg and a surface of 2.2 cm2. An image consistent with an Ao-RV fistula, with restrictive continuous flow (Vmax 4.2 m/s) was noticed between the anterior aspect of the aortic valvular annulus, immediately distal to the valvular plan of the aortic prosthesis, and the right ventricular side of the interventricular septum, adjacent to crista supraventricularis with an anterior directed jet, this was coincident with annular calcifications in the base of the right coronary leaflet of the aortic valve, prior to TAVI (, , Videos S and S). A preoperative coronary angiography confirmed a severe stenosis (70–90%) of the proximal left anterior descending coronary artery (LAD) prompting the indication for coronary bypass. The mitral valve anatomy was considered not suitable for percutaneous edge to edge mitral valve repair because of severe annular calcification. The echocardiographic findings were confirmed by the intraoperative transoesophageal echocardiography (, Videos S and S) and direct surgical exploration of the diseased valve.\nThe patient underwent cardiac surgery under standard cardiopulmonary bypass (total time 156 min; aortic cross-clamp 138 min) with explantation of the TAVI (to better expose the mitral valve for replacement and the Ao-RV fistula for closure), surgical replacement of the mitral valve with a porcine stented bioprosthetic valve (St Jude Epic, size 31), coronary bypass (left internal thoracic artery to LAD), direct closure of the Ao-RV fistula (), and total aortic root replacement using a porcine stentless bioprosthetic valved aortic root (Medtronic Freestyle, size 27) because of anatomic alteration of the aortic root after TAVI explantation.\nThe patient had a standard post-operative recuperation and was transferred to a cardiac rehabilitation facility 2 weeks after surgery. Post-operative echocardiography confirmed the absence of Ao-RV and the normal function of both implanted valves (, Video S). The patient was followed in our outpatient clinic and is still doing well 2 months after the procedure.
An 88-year-old woman, with a medical history significant for coronary artery disease, hypertension, hyperlipidemia, and known severe aortic stenosis, presented to the Structural Heart Clinic with worsening fatigue and dizziness without syncope. She had exertional dyspnea with the New York Heart Association (NYHA) Class III-IV congestive heart failure symptoms and no angina. Transthoracic echocardiogram (TTE) showed severe aortic stenosis with mean gradient of 62 mmHg and an ejection fraction (EF) of 20%. Cardiac catheterization revealed patent coronary angiogram and an aortic valve area of 0.27 cm 2.\nShe was deemed to be high risk for open heart surgery (Society of Thoracic Surgeons score 26.782%). Due to the lifestyle-limiting symptoms and severe aortic stenosis, a balloon aortic valvuloplasty was initially performed with significant improvement in symptoms (NYHA II). Computed tomography and angiography showed mildly dilated aortic arch with significant atherosclerosis but no aneurysm of the aortic root or sinus of Valsalva.\nSix months later, due to the recurrence of symptoms, she underwent successful TAVR with 26 mm SAPIEN XT bioprosthetic valve (Edwards Lifesciences, Irvine, CA, USA) using standard technique without postdeployment dilation. Proper anatomic position, trivial paravalvular leak, and normal valve function were confirmed with transesophageal echocardiogram and completion aortogram. The patient required immediate surgical repair of the utilized iliac artery. Immediately postprocedure, the patient remained largely asymptomatic with stable cardiac function (EF 55%–60%) on echocardiogram.\nThe patient had an uneventful recovery and was discharged to home on the 3rd postoperative day. TTE at 4 days and again 2 weeks after the procedure showed good positioning and functioning of the bioprosthetic valve. A new small fistula connecting the right sinus of Valsalva to the RV [Figures , and Videos , ] was noted.\nA decision was made to closely monitor the fistula and intervene if patient were to develop symptoms.\nShe was hospitalized 3 months later with worsening of heart failure symptoms. Electrocardiogram and troponins were normal. Repeat echocardiogram showed enlargement of the fistula with a velocity of >4 m/s, a Qp:Qs of 2:1, an EF reduction from 55%–60% to 30%–35%, and moderate dilation of RV. In view of the extreme risks to undergo repeat cardiac surgical procedure, including percutaneous and open closure options, the patient decided to continue with medical management for heart failure. After another admission with similar symptoms few months later, she was discharged to a skilled nursing facility with hospice care and died due to progressive heart failure. Total duration between onset of fistula and death was 9 months.