Advertisement

A tale of two sisters with hypertrophic cardiomyopathy and recurrent embolism: When is the optimal timing of the intervention for left atrial appendage?

Published:September 22, 2018DOI:https://doi.org/10.1016/j.hrtlng.2018.08.010

      Abstract

      Hypertrophic cardiomyopathy (HCM) is an extremely heterogeneous genetic disease that affects the left ventricle (LV) and has a varied clinical course and phenotypic expression. Here, we report a case of two sisters with HCM who developed a massive refractory left atrial appendage (LAA) thrombus and recurrent embolism. The older sister, who was at a high surgical risk due to progressive LV systolic dysfunction with an ejection fraction of 19%, underwent LAA plication in combination with implantation of an LV assist device after progression to treatment-refractory heart failure at the age of 49. The younger sister underwent surgical thrombectomy, LAA plication, and Maze surgery before deterioration of heart failure at the age of 47. She was free from embolism and atrial fibrillation for 2years after surgery. Individualized therapeutic approaches targeting the LAA at a relatively early stage are required in the subgroups of HCM patients with left atrial dysfunction.

      Keywords

      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribe:

      Subscribe to Heart & Lung: The Journal of Cardiopulmonary and Acute Care
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

        • Otsuka H.
        • Arimura T.
        • Abe T.
        • et al.
        Prevalence and distribution of sarcomeric gene mutations in Japanese patients with familial hypertrophic cardiomyopathy.
        Circ J. 2012; 76: 453-461
        • Maron B.J.
        Hypertrophic cardiomyopathy: a systematic review.
        JAMA. 2002; 287: 1308-1320
        • Haruki S.
        • Minami Y.
        • Hagiwara N.
        Stroke and embolic events in hypertrophic cardiomyopathy: risk stratification in patients without atrial fibrillation.
        Stroke. 2016; 47: 936-942
        • Goette A.
        • Kalman J.M.
        • Aguinaga L.
        • et al.
        EHRA/HRS/APHRS/SOLAECE expert consensus on atrial cardiomyopathies: definition, characterization, and clinical implication.
        Heart Rhythm. 2017; 14: e3-e40
        • Hoit B.D.
        Left atrial size and function: role in prognosis.
        J Am Coll Cardiol. 2014; 63: 493-505
        • Tucker N.R.
        • Ellinor P.T.
        Emerging directions in the genetics of atrial fibrillation.
        Circ Res. 2014; 114: 1469-1482