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 accessOne-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 CareAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Prevalence and distribution of sarcomeric gene mutations in Japanese patients with familial hypertrophic cardiomyopathy.Circ J. 2012; 76: 453-461
- Hypertrophic cardiomyopathy: a systematic review.JAMA. 2002; 287: 1308-1320
- Stroke and embolic events in hypertrophic cardiomyopathy: risk stratification in patients without atrial fibrillation.Stroke. 2016; 47: 936-942
- EHRA/HRS/APHRS/SOLAECE expert consensus on atrial cardiomyopathies: definition, characterization, and clinical implication.Heart Rhythm. 2017; 14: e3-e40
- Left atrial size and function: role in prognosis.J Am Coll Cardiol. 2014; 63: 493-505
- Emerging directions in the genetics of atrial fibrillation.Circ Res. 2014; 114: 1469-1482
Article info
Publication history
Published online: September 22, 2018
Accepted:
August 22,
2018
Received in revised form:
August 15,
2018
Received:
February 24,
2018
Identification
Copyright
© 2018 Elsevier Inc. All rights reserved.
