Sample Interventional Challenging Case: Transcatheter Closure of an Aorto-Right Ventricular Fistula Complicating Transcatheter Aortic Valve Replacement for Bicuspid Aortic Stenosis

Author: Kenta Nakamura, Sammy Elmariah, Jonathan Passeri, Meagan M. Wasfy, James Harkness, Jonathan G. Teoh, Nandini M. Meyersohn, Eliza Teo, Brian Ghoshhajra, Ignacio Inglessis, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA

Case Submission:

  • Patient Initials or Identifier Number: DD
  • Relevant History and Physical Exam: A 54-year-old man with O2-dependent chronic obstructive pulmonary disease, pulmonary hypertension, chronic kidney disease, ischemic cardiomyopathy, and bicuspid aortic valve presented with worsening exertional dyspnea. Physical examination was consistent with severe aortic stenosis.
  • Relevant Test Results Prior to Catheterization: The patient was found to have severe bicuspid aortic valve stenosis with peak/mean aortic valve gradients of 95/57 mmHg and left ventricular ejection fraction of 35%. Given his high operative risk, the heart valve team decided to proceed with transcatheter aortic valve replacement (TAVR).
  • Relevant Catheterization Findings and Hospital Course: Transfemoral TAVR was performed using a 29 mm Sapien XT valve (Edwards Lifesciences, Irvine, CA). Upon deployment, severe valvular regurgitation was noted due to a non-functioning valve leaflet. A second 29-mm Sapien XT valve was deployed with minimal residual regurgitation. Aortography and transesophageal echocardiography revealed a stable focal disruption of the right coronary cusp at the upper border of the valve stent. The patient remained hemodynamically stable until five days later when he developed decompensated right heart failure and multi-organ dysfunction. Cardiac computed tomography and transesophageal echocardiography revealed an aortic root pseudoaneurysm originating from the right sinus of Valsalva at the distal edge of the TAVR stent with fistulous communication to the right ventricle. Surgical evaluation concluded that the patient’s comorbid conditions and acute decompensation precluded surgical repair, so transcatheter closure was planned.
  • Interventional Management: The defect was crossed and a guiding sheath was advanced across the defect into the right ventricle. An 18-mm Amplatzer “Cribriform” Septal Occluder (St. Jude Medical, St. Paul, MN) was successfully deployed, reducing the left-to-right shunt to only trivial severity. The patient’s heart failure syndrome improved markedly and he was discharged nine days later.

Case Description: We report a case of a 54-year-old man with symptomatic severe bicuspid aortic stenosis (AS) deemed to be at extreme risk for surgical valve replacement. Transcatheter aortic valve replacement (TAVR) was complicated by focal disruption of the right coronary cusp that progressed over the subsequent 5 days to a pseudoaneurysm with fistulous communication to the right ventricle with resulting hemodynamic compromise. The defect was crossed and then closed using an 18-mm septal occluder with dramatic reduction in the severity of left-to-right shunt. The patient’s heart failure syndrome improved markedly and he was discharged nine days later. We suspect that the TAVR prosthesis was deployed asymmetrically due to valve calcification and bicuspid valve morphology, traumatizing the right sinus of Valsalva. This case demonstrates that iatrogenic aorto-right ventricular fistula is a rare complication of TAVR, that may be more likely to occur in bicuspid AS, and that transcatheter closure of these defects is a viable treatment option in cases with elevated surgical risk.

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