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

研究成果: Article査読

抄録

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.

本文言語English
ページ(範囲)198-200
ページ数3
ジャーナルHeart and Lung
48
3
DOI
出版ステータスPublished - 2019 5 1

ASJC Scopus subject areas

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

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