The 4-year-old girl underwent subaortic VSD repair surgery in our hospital on October 24, 2011 when she was 2 years old. Ten days after surgery, transthoracic echocardiography showed that the VSD was properly repaired but there was a fistula of right sinus of Valsalva (RSOV) to the right ventricle (RV). With the fistula’s diameter of 1.5 mm, color Doppler showed a continuous shunt from RSOV to RV without aortic regurgitation (AR), and physical examination showed a new grade 3/6 continuous murmur at the left sternal border. Since no rupture of sinus of Valsalva was found in the preoperative echocardiography ordetected during surgery, it was believed that this fistula was a postsurgical complication. Because the patient was asymptomatic and the fistula was small, her parents decided to observe her for progression over a period of time. During 21 months of observation, transthoracic echocardiography (once every 3 months) showed that the fistula’s diameter increased from 1.5 mm to 2.5 mm with a high-velocity (4.5 m/s) continuous Doppler flow (, , ). Meanwhile, the diastolic diameter of LV also increased from 32 mm to 39 mm. The patient was still asymptomatic and the continuous murmur at the left sternal border became grade 4/6.\nAlthough the initial treatment recommendation was surgery, the patient’s parents were more inclined to avoid reopening the heart and using cardiopulmonary bypass and eliminating radiation exposure during percutaneous closure. With experience in closed VSDs using minimally invasive transthoracic device closure guided by TEE, we explored the feasibility of adopting this technique to close the fistula. After the potential risks were explained in detail, the parents gave their informed consent for occlusion of the fistula of RSOV under TEE guidance.\nUnder general anesthesia, TEE confirmed the diagnosis of fistula of the RSOV to RV () with a fistula diameter of 2.5 mm. Antibiotic prophylaxis (Cefotiam, 40 mg/kg body weight) and full heparinization (1 mg/kg body weight) were administered. The free wall of RV was exposed via a 3-cm incision in the inferior sternum and was palpated to locate the area of puncture before a purse-string suture was placed at this location. Under the real-time guidance andmonitoring of TEE (Vivid 7 Dimension, GE, Horten, Norway), a trocar was introduced into RV within the purse-string, and then a guide-wire was passed through the trocar and managed into RSOV through the fistula, at which point the trocar was removed (). A 7-Fr delivery sheath was fed over the wire and inserted into RSOV through the fistula. With this done, the guide-wire was pulled out, and the previously prepared loading sheath, which contained a 4 mm ordinary symmetric VSD occluder (Shanghai Shape Memory Alloy Corporation, Shanghai, China), was connected to the delivery sheath. The occluder device was then advanced into RSOV through the sheath and thus deployed.\nAfter deployment, TEE showed that the occluder had a stable position with abolition of the shunt, and no AR occurred (). No other periprocedural complications were noted. The patient was asked to take 70 mg of aspirin orally daily for 6 months.\nThree months after intervention, the patient was in good condition with no cardiac murmurs on examination.\nTransthoracic echocardiography revealed satisfactory position of the occluder with no residual shunt, aortic regurgitation (), device embolization or infective endocarditis. The diastolic diameter of LV had decreased from 39 mm to 36 mm. Electrocardiography and chest X-ray also showed normal ().
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.