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Golden Valley, MN, USA) was loaded into the AGADS with careful deairing of the system and the whole system was withdrawn to the ascending aorta.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.
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.