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 journalArticle

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 languageEnglish
JournalHeart and Lung
DOIs
Publication statusAccepted/In press - 2018 Jan 1

Fingerprint

Atrial Appendage
Hypertrophic Cardiomyopathy
Embolism
Siblings
Heart Ventricles
Heart Failure
Thrombectomy
Inborn Genetic Diseases
Heart-Assist Devices
Atrial Fibrillation
Thrombosis
Therapeutics

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 journalArticle

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