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 2 years 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.
| Original language | English |
|---|---|
| Journal | Heart and Lung |
| DOIs | |
| Publication status | Accepted/In press - 2018 Jan 1 |
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Keywords
- Echocardiography
- Hypertrophic cardiomyopathy
- Thrombus
ASJC Scopus subject areas
- Pulmonary and Respiratory Medicine
- Critical Care and Intensive Care Medicine
- Cardiology and Cardiovascular Medicine
Cite this
A tale of two sisters with hypertrophic cardiomyopathy and recurrent embolism : When is the optimal timing of the intervention for left atrial appendage? / Kimura, Mai; Kohno, Takashi; Makino, Shinji; Okuda, Shigeo; Nawata, Kan; Yanagisawa, Ryo; Kojima, Hidenori; Nishiyama, Takahiko; Aizawa, Yoshiyasu; Yuasa, Shinsuke; Murata, Mitsushige; Maekawa, Yuichiro; Okamoto, Kazuma; Shimizu, Hideyuki; Fukuda, Keiichi.
In: Heart and Lung, 01.01.2018.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - A tale of two sisters with hypertrophic cardiomyopathy and recurrent embolism
T2 - When is the optimal timing of the intervention for left atrial appendage?
AU - Kimura, Mai
AU - Kohno, Takashi
AU - Makino, Shinji
AU - Okuda, Shigeo
AU - Nawata, Kan
AU - Yanagisawa, Ryo
AU - Kojima, Hidenori
AU - Nishiyama, Takahiko
AU - Aizawa, Yoshiyasu
AU - Yuasa, Shinsuke
AU - Murata, Mitsushige
AU - Maekawa, Yuichiro
AU - Okamoto, Kazuma
AU - Shimizu, Hideyuki
AU - Fukuda, Keiichi
PY - 2018/1/1
Y1 - 2018/1/1
N2 - 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 2 years 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.
AB - 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 2 years 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.
KW - Echocardiography
KW - Hypertrophic cardiomyopathy
KW - Thrombus
UR - http://www.scopus.com/inward/record.url?scp=85053820533&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85053820533&partnerID=8YFLogxK
U2 - 10.1016/j.hrtlng.2018.08.010
DO - 10.1016/j.hrtlng.2018.08.010
M3 - Article
JO - Heart and Lung: Journal of Acute and Critical Care
JF - Heart and Lung: Journal of Acute and Critical Care
SN - 0147-9563
ER -