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Golden Valley, MN, USA) was loaded into the AGADS with careful deairing of the system and the whole system was withdrawn to the ascending aorta.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