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 ().
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.