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 ().
Our patient is a 92-year-old male with severe aortic stenosis whose past medical history includes umbilical and inguinal hernia, chronic diastolic heart failure, right bundle branch block, coronary artery disease, and obesity. He became significantly symptomatic in the last few months prior to his admission to the hospital, where he complained of worsened dyspnea on exertion and recurrent syncopal attacks. Physical examination did not show any significant findings except for a 5/6 systolic ejection murmur in the aortic area radiating to both carotids. Diagnostic cardiac catheterization showed no evidence of coronary artery disease. Carotid Doppler showed no significant stenosis. Electrocardiogram showed sinus rhythm with a right bundle branch block. Transesophageal echocardiography showed critical aortic valve stenosis, mean pressure gradient of approximately 72 mmHg, valve area of 0.46 cm2, and left ventricular ejection fraction of 60%. A 29 mm Edwards SAPIEN XT valve was implanted via transfemoral access. Confirmation of position was then done by aortography. The transesophageal echocardiogram confirmed the adequate position of the valve with minimal aortic insufficiency. There was no pericardial effusion with normal ventricular systolic function. Postdeployment aortogram showed paravalvular leak and patent coronary arteries.\nPostdeployment hemodynamics showed zero mmHg mean gradient pressure between the left ventricle and the aorta with left ventricular end diastolic pressure (LVEDP) ~ 10 mmHg.\nPost-TAVR day 1 routine transthoracic echocardiography (TTE) showed well-seated and normally functioning bioprosthetic aortic valve with a small posterior paravalvular leak. It also showed a small-sized continuous (systolic and diastolic phases) shunt, which communicated with the aortic root into the right ventricle (aorto-RV shunt) (Figures and ).\nSince the patient was asymptomatic following the procedure, he was managed conservatively with close follow-up. His repeat TTE a month after the procedure revealed no significant change from his post-TAVR day 1 TTE (). Therefore, we decided to continue with medical management and close follow-up, since the patient was asymptomatic.\nFollow-up with TTE a year later showed persistence of the aorto-RV fistula and significant improvement in the velocity across the shunt (), which may be due to a decrease in the fistula size rather than an increase in RV pressure as RV systolic pressure (RVSP) remained within normal limits on follow-up TTE.