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.
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.