Dimarakis Ioannis, Tennyson Charlene, Karatasakis Aris, Macnab Anita, Dobson Laura E, Kadir Isaac, Feddy Lee, Callan Paul
Division of Cardiothoracic Surgery, Department of Surgery, University of Washington Medical Center, 1959 NE Pacific Street, Seattle, WA 98195, USA.
Department of Cardiothoracic Surgery, Manchester University Hospital NHS Foundation Trust, Wythenshawe Hospital, Southmoor Road, Manchester M23 9LT, UK.
Eur Heart J Case Rep. 2024 Dec 13;8(12):ytae649. doi: 10.1093/ehjcr/ytae649. eCollection 2024 Dec.
Bicuspid aortic valve (BAV) is the most common congenital heart defect (reported incidence of 0.5%-2%) and is commonly associated with proximal aortic dilation. Patients with severe aortic stenosis (AS) of BAV have been shown to have worse pre-operative left ventricular (LV) function as well as a higher incidence of post-operative heart failure hospitalization when compared with analogous patients with tri-leaflet aortic valve disease. While surgical aortic valve replacement (SAVR) may be favoured over transcatheter aortic valve implantation (TAVI) due to anatomical factors or concomitant aortopathy and coronary artery disease, surgical candidacy is often limited by prohibitive operative risk.
We report on three cases of severe AS in BAV with concomitant aortopathy and severe left ventricular dysfunction in whom we proceeded with SAVR with planned venoarterial extracorporeal membrane oxygenation (VA-ECMO) support and inotrope-assisted wean. All patients had severe LV dysfunction (ejection fraction < 25%) at baseline with gradual substantial improvement or normalization after successful SAVR.
These cases demonstrate the utility of planned VA-ECMO with SAVR and aortic root replacement as an integral component of the operative strategy for high surgical risk patients with severe BAV AS not amenable to TAVI. Appropriate pre-operative planning and consent for VA-ECMO as well as a multi-disciplinary approach involving anaesthesia, intensive care, and heart failure cardiology are the key to offering this option as an alternative to palliative medical therapy to a selected group of patients.
二叶式主动脉瓣（BAV）是最常见的先天性心脏缺陷（报告发病率为0.5%-2%），通常与升主动脉扩张相关。与三叶式主动脉瓣疾病的类似患者相比，BAV导致严重主动脉瓣狭窄（AS）的患者术前左心室（LV）功能较差，术后心力衰竭住院率较高。虽然由于解剖因素或合并主动脉病变和冠状动脉疾病，外科主动脉瓣置换术（SAVR）可能比经导管主动脉瓣植入术（TAVI）更受青睐，但手术候选资格往往受到高手术风险的限制。
我们报告了3例BAV合并严重主动脉病变和严重左心室功能障碍的严重AS患者，我们对其进行了SAVR，并计划采用静脉-动脉体外膜肺氧合（VA-ECMO）支持和血管活性药物辅助脱机。所有患者基线时均有严重左心室功能障碍（射血分数<25%），成功进行SAVR后逐渐有显著改善或恢复正常。
这些病例表明，对于不适合TAVI的严重BAV AS高手术风险患者，计划采用VA-ECMO联合SAVR和主动脉根部置换作为手术策略的一个组成部分是有用的。适当的术前VA-ECMO规划和同意，以及涉及麻醉、重症监护和心力衰竭心脏病学的多学科方法，是为选定患者群体提供这种替代姑息性药物治疗的关键。
