First Reported Case of MitraClip Placement Due to Mitral Valve Flail in the Setting of Cardiac Amyloidosis.

Abstract

Amyloidosis is a protein misfolding disorder characterized by aggregation and deposition of amyloid fibrils in tissues and organs disrupting architecture and function. Although this disorder often results in a restrictive cardiomyopathy with diffuse biventricular thickening, biatrial dilation, and conduction disease, clinically significant valvular heart disease is rarely described. The MitraClip percutaneous edge-to-edge mitral valve repair is an important and effective treatment for patients with severe and symptomatic mitral regurgitation (MR). In patients at high risk for cardiac surgery, the MitraClip has shown excellent safety and durable results with 83% of patients demonstrating ≤2+ MR at 1-year and 87% of patients with New York Heart Association class I to II symptoms.1 We describe herein a patient with primary (AL) amyloidosis who suffered a ruptured mitral chord resulting in severe MR that was successfully treated using the MitraClip (Abbott Vascular, Minneappolis, MN) system. To our knowledge, this is the first reported case of degenerative MR related to cardiac amyloidosis that has been treated with a MitraClip. The patient is a 69-year-old man with a history of hypertension and hyperlipidemia. He presented one and half years before with worsening exertional dyspnea. Transthoracic echocardiography demonstrated normal ventricular function, severe biventricular hypertrophy, and bileaflet mitral valve thickening with mild prolapse (Figure 1; Movie I in the Data Supplement). Cardiac magnetic resonance imaging demonstrated diffuse right and left ventricular thickening with delayed enhancement suggestive of cardiac amyloidosis, and subsequent endomyocardial and bone marrow biopsies confirmed the …