Diagnosis and management of complex cardiovascular pathology in a patient with type III Loeys-Dietz Syndrome

Type III Loeys-Dietz Syndrome (LDS) is a rare and progressive connective tissue disorder with a significant impact on the cardiovascular system. We report a case of a 47-year-old male diagnosed with type III LDS, who presented with a complex array of cardiovascular pathologies, such as aneurysmal dilatation of the aortic root and ascending aorta, along with annular dilatation and regurgitation of the aortic, mitral, and tricuspid valves. Genetic testing played a crucial role in establishing the diagnosis. A multivalvular surgical correction improved the patient’s cardiac function and overall condition.

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Journal
Chirurgia
Published
2024-09-01
DOI
https://doi.org/10.23736/s0394-9508.23.05621-8
Primary Topic
Connective tissue disorders research
Type
article
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article

Diagnosis and management of complex cardiovascular pathology in a patient with type III Loeys-Dietz Syndrome

Nickolai A. Tchurikov, Sergei V. GARMANOV, Vladimir A. MIRONENKO, Andrei M. Belyaev
Chirurgia
Connective tissue disorders research
article

Diagnosis and management of complex cardiovascular pathology in a patient with type III Loeys-Dietz Syndrome

Nickolai A. Tchurikov, Sergei V. GARMANOV, Vladimir A. MIRONENKO, Andrei M. Belyaev
article en

Abstract

Type III Loeys-Dietz Syndrome (LDS) is a rare and progressive connective tissue disorder with a significant impact on the cardiovascular system. We report a case of a 47-year-old male diagnosed with type III LDS, who presented with a complex array of cardiovascular pathologies, such as aneurysmal dilatation of the aortic root and ascending aorta, along with annular dilatation and regurgitation of the aortic, mitral, and tricuspid valves. Genetic testing played a crucial role in establishing the diagnosis. A multivalvular surgical correction improved the patient’s cardiac function and overall condition.

ChirurgiaVol. 37(4)
Engelhardt Institute of Molecular Biology (RU), Bakulev Scientific Center for Cardiovascular Surgery Russian Academy of Medical Sciences (RU)
Good health and well-being
Openalex Percentile: Top 84%
Connective tissue disorders research
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Diagnosis and management of complex cardiovascular pathology in a patient with type III Loeys-Dietz Syndrome — Nickolai A. Tchurikov, Sergei V. GARMANOV, et al. · Chirurgia (2024) | TGRS Research Map | TGRS