Left ventricular noncompaction

Gaisenok, O. V.; Kurnosov, P A; Бернс, С. А.

doi:10.17116/patol201880447

Cited by 3 publications

(2 citation statements)

References 49 publications

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“…LVNC is characterized by prominent left ventricular trabeculae, thin compacted layer, and deep intertrabecular recesses and is best visualized by CMR. 3 , 9 , 10 The prevalence of LVNC is reported as 0.05% in adults. 11 It can be associated with cardiomyopathies or congenital heart disease, and neuromuscular disorders.…”

Section: Discussionmentioning

confidence: 99%

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A Case Report of Thromboembolic Stroke in a Patient with Holt-Oram Syndrome

Abdelazeem¹,

Khan²,

Baral³

et al. 2022

Journal of Community Hospital Internal Medicine Perspectives

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Ischemic stroke associated with rare clinical syndromes represents less than 5% of etiologic factors. From those syndromes are Holt-Oram syndrome and Left ventricular non-compaction syndrome. We report a case of a 66 years old male with genetically confirmed Holt-Oram syndrome due to TBX5 mutation who presented with cryptogenic stroke most likely due to cardioembolic etiology. The patient has a history of moderate nonischemic cardiomyopathy due to an atypical pattern of left ventricular non-compaction confirmed by Cardiac Magnetic Resonance Imaging. The patient was treated appropriately with thrombolytic therapy and catheter-directed mechanical thrombectomy with minimal residual stroke symptoms. Holt-Oram syndrome is a genetic condition with variable clinical phenotypes, including cardiac manifestations. Left ventricular non-compaction syndrome is rare congenital cardiomyopathy defined as prominent left ventricular trabeculae, deep intertrabecular recesses, and a thin compacted layer. And only a few cases were reported with both conditions. Therefore, patients with the Holt-Oram Syndrome should get a comprehensive cardiac evaluation to exclude non-compaction cardiomyopathy, which may have significant prognostic implications.

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Section: Discussionmentioning

confidence: 99%

“…Cardiac transplantation can be considered in patients with heart failure refractory to medical management. 9 , 10 …”

Section: Discussionmentioning

confidence: 99%