Transcatheter bicuspid aortic valve replacement in Turner syndrome: A unique experience of interventional cardiologist

A 69-year-old short-statured Turner syndrome (TS) patient with a history of poliomyelitis in childhood and moderate bicuspid aortic stenosis (BAS) reported worsening dyspnea and fatigue over six months. A repeat transthoracic echocardiogram revealed progression to severe aortic stenosis with dilated ascending aorta (AA). As part of the work-up for aortic valve replacement, the patient underwent cardiac catheterization, which revealed a severely calcified AV with an area of 0.5 sq. cm and a mean gradient of 37 mmHg. On coronary angiography, there was 70% stenosis of the proximal left anterior descending artery (LAD). Due to poor rehabilitation potential, she was deemed high-risk for surgical aortic valve replacement. A recommendation for transcatheter aortic valve replacement (TAVR) with stenting of the proximal LAD was made. Dilated AA was managed conservatively with serial noninvasive imaging. The patient underwent TAVR with Edwards-Sapien valve (23 mm S3) and stenting of proximal LAD. The procedure was successful without complications. To our knowledge, our patient is the first case of TAVR in BAS with aortopathy in TS. <Learning objective: Therapeutically, transcatheter aortic valve replacement is an off-label indication for bicuspid aortic stenosis. In real-life practice, many of these patients are poor surgical candidates and demand careful and judicious decision-making in the presence of diverse clinical scenarios. In such circumstances, a multidisciplinary approach with shared decision-making is required to recommend best possible therapeutic solution in the presence of limited data.>.