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 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.