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 89-year-old male with a prior history of laryngeal cancer status after surgical resection and radiation therapy with residual dysphagia, coronary artery disease status after coronary artery bypass grafting in 1992, severe aortic stenosis status after balloon valvuloplasty in 2013, pulmonary fibrosis, COPD on 2-liter continuous home O2 therapy, and TIA presented in our clinic with several years of progressive dyspnea and angina with exertion. He was admitted in the hospital at least five times over the past one year with chest pain and dyspnea. Diagnostic cardiac catheterization was done showing three out of four grafts being patent (SVG-RPLV completely occluded). Last year, he underwent balloon aortic valvuloplasty with a 22 mm balloon for severe aortic stenosis and the patient reported partial relief of symptoms for approximately six months after the procedure but now the symptoms are progressively getting worse. Transthoracic echocardiogram (TTE) showed concentric left ventricular hypertrophy, left ventricular ejection fraction of 60%, right ventricle mildly dilated with preserved systolic function, pulmonary artery systolic pressure of 34 mmHg, heavily calcified aortic valve annulus, mean pressure gradient of 68 mmHg, velocity of 517 cm/sec, and aortic valve area of 0.62 cm2. Cardiothoracic surgery team evaluated him for aortic valve replacement but the surgeon felt that patient is at very high risk for open-heart surgery. Per our TAVR team, his aortic valve annulus was too big (anteroposterior diameter 2.6 cm and mediolateral diameter of 2.8 cm per transesophageal echocardiogram, ) for commercially available Core Valve (Medtronic, USA). Ultimately, our TAVR team used a 29 mm Edward Sapien valve via transfemoral access. The procedure was complicated by complete heart block requiring temporary pacemaker backup. A permanent pacemaker was placed after 24 hours. Repeat TTE showed moderately dilated right ventricle with moderately reduced systolic function, pulmonary artery systolic pressure of 40 mmHg, Sapien prosthesis in aortic position with mild perivalvular leak and mean gradient of 9 mmHg, moderate sized continuous (systolic and diastolic phases) shunt which communicates with the aortic root around the prosthesis into the RV (aorto-RV shunt), and pulmonary flow (Qp) to systemic flow (Qs) ratio of 1.39. The patient was managed conservatively with close follow-up. At 30-day follow-up, the patient remained moderately to severely dyspneic. Patient was offered percutaneous closure of the aorto-RV fistula using devices like septal occluder (Amplatzer device) but patient refused it. His repeat TTE showed worsening of aorto-RV fistula and it also showed new small membranous VSD (see Video 1 in the Supplementary Material available online at ), which was not visible on the first postprocedural TTE. His condition progressively got worse. He changed his code status to comfort measures only and passed away in a couple of days.