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

Mai Kimura; Takashi Kohno; Shinji Makino; Shigeo Okuda; Kan Nawata; Ryo Yanagisawa; Hidenori Kojima; Takahiko Nishiyama; Yoshiyasu Aizawa; Shinsuke Yuasa; Mitsushige Murata; Yuichiro Maekawa; Kazuma Okamoto; Hideyuki Shimizu; Keiichi Fukuda

doi:10.1016/j.hrtlng.2018.08.010

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

Mai Kimura, Takashi Kohno, Shinji Makino, Shigeo Okuda, Kan Nawata, Ryo Yanagisawa, Hidenori Kojima, Takahiko Nishiyama, Yoshiyasu Aizawa, Shinsuke Yuasa, Mitsushige Murata, Yuichiro Maekawa, Kazuma Okamoto, Hideyuki Shimizu, Keiichi Fukuda

Research output: Contribution to journal › Article

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	https://doi.org/10.1016/j.hrtlng.2018.08.010
Publication status	Accepted/In press - 2018 Jan 1

Keywords

Echocardiography
Hypertrophic cardiomyopathy
Thrombus

ASJC Scopus subject areas

Pulmonary and Respiratory Medicine
Critical Care and Intensive Care Medicine
Cardiology and Cardiovascular Medicine

Access to Document

10.1016/j.hrtlng.2018.08.010

Fingerprint Dive into the research topics of 'A tale of two sisters with hypertrophic cardiomyopathy and recurrent embolism: When is the optimal timing of the intervention for left atrial appendage?'. Together they form a unique fingerprint.

Cite this

Kimura, M., Kohno, T., Makino, S., Okuda, S., Nawata, K., Yanagisawa, R., Kojima, H., Nishiyama, T., Aizawa, Y., Yuasa, S., Murata, M., Maekawa, Y., Okamoto, K., Shimizu, H., & Fukuda, K. (Accepted/In press). A tale of two sisters with hypertrophic cardiomyopathy and recurrent embolism: When is the optimal timing of the intervention for left atrial appendage? Heart and Lung. https://doi.org/10.1016/j.hrtlng.2018.08.010

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

Kimura, M, Kohno, T, Makino, S , Okuda, S, Nawata, K, Yanagisawa, R, Kojima, H, Nishiyama, T, Aizawa, Y, Yuasa, S, Murata, M, Maekawa, Y, Okamoto, K, Shimizu, H & Fukuda, K 2018, 'A tale of two sisters with hypertrophic cardiomyopathy and recurrent embolism: When is the optimal timing of the intervention for left atrial appendage?', Heart and Lung. https://doi.org/10.1016/j.hrtlng.2018.08.010

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. / A tale of two sisters with hypertrophic cardiomyopathy and recurrent embolism : When is the optimal timing of the intervention for left atrial appendage?. In: Heart and Lung. 2018.

@article{0279a847008a4edbbb1081270524fa2a,

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

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.",

keywords = "Echocardiography, Hypertrophic cardiomyopathy, Thrombus",

author = "Mai Kimura and Takashi Kohno and Shinji Makino and Shigeo Okuda and Kan Nawata and Ryo Yanagisawa and Hidenori Kojima and Takahiko Nishiyama and Yoshiyasu Aizawa and Shinsuke Yuasa and Mitsushige Murata and Yuichiro Maekawa and Kazuma Okamoto and Hideyuki Shimizu and Keiichi Fukuda",

year = "2018",

month = jan,

day = "1",

doi = "10.1016/j.hrtlng.2018.08.010",

language = "English",

journal = "Heart and Lung: Journal of Acute and Critical Care",

issn = "0147-9563",

publisher = "Mosby Inc.",

}

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

C2 - 30253876

AN - SCOPUS:85053820533

JO - Heart and Lung: Journal of Acute and Critical Care

JF - Heart and Lung: Journal of Acute and Critical Care

SN - 0147-9563

ER -