A tale of two sisters with hypertrophic cardiomyopathy and recurrent embolism: When is the optimal timing of the intervention for left atrial appendage?
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...
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| Veröffentlicht in: | Heart & lung 2019-05, Vol.48 (3), p.198-200 |
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| creator | 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 |
| description | 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. |
| doi_str_mv | 10.1016/j.hrtlng.2018.08.010 |
| format | Article |
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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.</description><identifier>ISSN: 0147-9563</identifier><identifier>EISSN: 1527-3288</identifier><identifier>DOI: 10.1016/j.hrtlng.2018.08.010</identifier><identifier>PMID: 30253876</identifier><language>eng</language><publisher>United States: Elsevier Inc</publisher><subject>Atrial Appendage - diagnostic imaging ; Atrial Appendage - surgery ; Cardiomyopathy, Hypertrophic - complications ; Cardiomyopathy, Hypertrophic - diagnosis ; Cardiomyopathy, Hypertrophic - surgery ; Echocardiography ; Echocardiography, Transesophageal ; Female ; Heart Atria ; Heart Diseases - diagnosis ; Heart Diseases - etiology ; Heart Diseases - surgery ; Heart Failure - physiopathology ; Humans ; Hypertrophic cardiomyopathy ; Middle Aged ; Recurrence ; Thrombectomy - methods ; Thrombosis - diagnosis ; Thrombosis - etiology ; Thrombosis - surgery ; Thrombus ; Time Factors</subject><ispartof>Heart & lung, 2019-05, Vol.48 (3), p.198-200</ispartof><rights>2018 Elsevier Inc.</rights><rights>Copyright © 2018 Elsevier Inc. 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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.</abstract><cop>United States</cop><pub>Elsevier Inc</pub><pmid>30253876</pmid><doi>10.1016/j.hrtlng.2018.08.010</doi><tpages>3</tpages><orcidid>https://orcid.org/0000-0001-5593-7552</orcidid><orcidid>https://orcid.org/0000-0003-0377-2287</orcidid><orcidid>https://orcid.org/0000-0002-7874-9527</orcidid></addata></record> |
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| identifier | ISSN: 0147-9563 |
| ispartof | Heart & lung, 2019-05, Vol.48 (3), p.198-200 |
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| source | MEDLINE; ScienceDirect Journals (5 years ago - present) |
| subjects | Atrial Appendage - diagnostic imaging Atrial Appendage - surgery Cardiomyopathy, Hypertrophic - complications Cardiomyopathy, Hypertrophic - diagnosis Cardiomyopathy, Hypertrophic - surgery Echocardiography Echocardiography, Transesophageal Female Heart Atria Heart Diseases - diagnosis Heart Diseases - etiology Heart Diseases - surgery Heart Failure - physiopathology Humans Hypertrophic cardiomyopathy Middle Aged Recurrence Thrombectomy - methods Thrombosis - diagnosis Thrombosis - etiology Thrombosis - surgery Thrombus Time Factors |
| title | A tale of two sisters with hypertrophic cardiomyopathy and recurrent embolism: When is the optimal timing of the intervention for left atrial appendage? |
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