Title:
Diffuse Arterial Spasm in a Young Patient With Turner Syndrome During Transcatheter Aortic Valve-in-Valve Replacement Sanchitha Guruchandrasekar, MBBS; Pedro Cox-Alomar, MD; Sylvia Oleck, MD; Steffan Sernich, MD; Kiran Mallula, MD
Introduction:
Surgical aortic valve replacement (SAVR) has traditionally been the standard of care for young patients with low to intermediate risk aortic stenosis. Recent trials have shown transcatheter aortic valve replacement (TAVR) to be non-inferior to SAVR. There are very limited case reports of diffuse arterial spasm associated with cardiac catheterization.
Clinical Case:
We present a 24-year-old female with Turner syndrome, bicuspid aortic valve, aortic stenosis and coarctation of aorta, who underwent balloon aortic valvuloplasty and coarctation repair during infancy. This was followed by surgical aortic root replacement with bioprosthetic valve and homograft at 4 years and repeat aortic root replacement with 23 mm Magna valve and 26 mm Hemashield conduit with septal myectomy at 11 years of age, due to severe aortic insufficiency and aortic root dilation. She presented in heart failure (NYHA III) secondary to severe stenosis and mild insufficiency of the valve. There was mild left ventricular systolic dysfunction. She also had a prior history of gastrointestinal bleeding from arteriovenous malformations in the jejunum that were resected. SAVR was not considered due to two previous sternotomies and the possible need for more surgeries considering her young age. The safety of long-term Warfarin treatment was also in question due to possible presence of arteriovenous malformations that may not have been completely resected. Hence, valve-in-valve (ViV) TAVR was considered the most appropriate treatment strategy. The first trans-femoral attempt was aborted due to severe diffuse vasospasm of the systemic arterial tree of most large vessels bilaterally despite adequate caliber on preprocedural CTA. The etiology of vasospasm was not clear. She was then rescheduled for ViV TAVR with a plan to attempt it via a trans-carotid approach. However, reassessment of the femoral vessels demonstrated adequate caliber that resulted in successful placement of 23 mm Edwards Sapien S3 valve via a trans-femoral approach. Post deployment, there was complete resolution of the stenosis with no sign of paravalvar leak. Echo revealed a well-positioned Sapien 3 valve with trivial insufficiency. She had no post-procedure complications.
Discussion:
This report highlights the utility of ViV TAVR in a young adult with congenital heart disease (ACHD) with multiple redo sternotomies. In the current era of non-inferiority of TAVR to SAVR procedure in low and intermediate-risk patients as demonstrated by recent trials, this treatment modality offers lesser invasive option for adults with congenital heart disease. Also, there is scant data on diffuse systemic arterial vasospasm in cardiac catheterization. In our case, there was no demonstrated etiology, but it was hypothesized to be related to exaggerated catecholamine sensitivity of the systemic arterial tree at the time of sheath insertion. It also stresses the fact for operators to be aware of this condition in order to avoid the morbidity that may be associated with other vascular approaches (transapical/transcarotid) compared to a typical transfemoral approach in these ACHD patients.