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Golden Valley, MN, USA) was loaded into the AGADS with careful deairing of the system and the whole system was withdrawn to the ascending aorta.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