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.
A 22-year-old man was referred for evaluation of exertional fatigue. He was previously diagnosed with a ventricular septal defect (VSD). He was not on any medical therapy. On examination, there were no overt signs of congestive heart failure. A systolic precordial thrill was palpable and a harsh 5/6 continuous murmur was audible. The pulse was high volume with blood pressure of 135/65 mmHg. Electrocardiogram (ECG) demonstrated sinus rhythm and incomplete right bundle branch block.\nTransthoracic echocardiography (TTE) revealed normal situs and connections with marked left ventricular (LV) enlargement; LV end diastolic/end systolic dimensions (LVEDd/LVESd) were 62/33 mm. No hypertrophy and no regional wall motion anomalies were noted and there was hyperactive LV function with LV fractional shortening of 47%. A sizable fistula with a large flow was detected shunting from RCAS-RV. There was no VSD or aortic valve regurgitation (AR).\nThe patient was scheduled for cardiac catheterization under transesophageal echo (TEE) guidance. The procedure was performed under general anesthesia with endotracheal intubation. The TEE interrogation disclosed an 8-mm fistula confirming the TTE findings [Figures and ]. A hemodynamic evaluation was performed with a normal pulmonary artery (PA) pressure 30/4 (15) mmHg and a QP/QS ratio of 3:1. The right anterior oblique projection aortogram illustrated the RCAS-RV irregular fistula which measured 8 mm in the aortic orifice and 9 mm in the mid portion.\nAn arterio-venous loop was established by retrograde crossing of the fistula using a 0.025” Terumo guidewire (Terumo Medical Corporation, Somerset, NJ, USA) from the aorta to the RV followed by a multipurpose (MP) 5F catheter (Cordis, Miami, FL, USA). At this stage, a noodle wire (NW) (AGA Medical Corporation, Golden Valley, MN, USA) was introduced and was snared out from the pulmonary artery (PA) via the inferior vena cava (IVC) through the femoral vein using a 15-mm pfm snare (Produkte fur die Medizin AG. Cologne, Germany). An AGA 7F delivery sheath (AGADS) was introduced through the femoral vein to join the MP catheter in the IVC. The assembly of the MP with the AGADS was gently pushed over the NW to the level of the aortic arch. A 12 × 10 mm Amplatzer Duct Occluder (ADO) (AGA Medical Corporation, Golden Valley, MN, USA) was loaded into the AGADS with careful deairing of the system and the whole system was withdrawn to the ascending aorta. The ADO was partially deployed in the ascending aorta and the entire system was withdrawn to allow engagement of the 12-mm ADO disk with the aortic aspect of the RCAS-RV fistula. At that stage, the AGADS was further withdrawn to allow full deployment of the ADO. Following TEE evaluation of adequate ADO position and exclusion of AR, the ADO was released from the delivery system with TEE and aortogram confirming the final result [Figures and ]. A trivial color-flow Doppler residual shunt was noted on TEE [] and ascending aortography, immediately after ADO release, but it vanished on an outpatient evaluation 10 days following the procedure. No peri-procedural complications were noted. A very good LV function was maintained with LVEDd/LVESd 49/30 mm and marked reduction of LV size. Treatment with acetylsalicylic acid 100 mg daily was initiated after the procedure for 6 months.