Left Ventricular Non-compaction in Holt-Oram Syndrome

Holt Oram Syndrome is an autosomal dominant condition with complete penetrance and which involves upper limb skeletal and cardiac abnormalities. The latter can be structural defects or involve the conduction system. This report details the occurrence of left ventricular non-compaction in multiple fa...

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Bibliographic Details
Published in:Heart, lung & circulation lung & circulation, 2016-06, Vol.25 (6), p.626-630
Main Authors: Kapadia, Renuka, BMed, Choudhary, Preeti, BSc Med, MBBS, FRACP, Collins, Nicholas, BMed, FRACP, Celermajer, David, MBBS, FRACP, PhD, Puranik, Rajesh, MBBS, FRACP, PhD
Format: Article
Language:English
Subjects:
Abnormalities, Multiple - physiopathology
Adult
Cardiomyopathies - physiopathology
Cardiovascular
Heart Defects, Congenital - physiopathology
Heart Septal Defects, Atrial - physiopathology
Heart Ventricles - physiopathology
Holt-Oram Syndrome
Humans
Left ventricular non-compaction
Lower Extremity Deformities, Congenital - physiopathology
Male
Upper Extremity Deformities, Congenital - physiopathology
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Summary:Holt Oram Syndrome is an autosomal dominant condition with complete penetrance and which involves upper limb skeletal and cardiac abnormalities. The latter can be structural defects or involve the conduction system. This report details the occurrence of left ventricular non-compaction in multiple family members with Holt Oram Syndrome. It is recommended that patients with the Holt Oram Syndrome be considered for comprehensive cardiac evaluation to exclude non-compaction cardiomyopathy as this may have significant prognostic implications.
ISSN:1443-9506
1444-2892
DOI:10.1016/j.hlc.2015.12.098