Document Detail


Isolated left ventricular hypertrabeculation/noncompaction in a Turner mosaic with male phenotype.
MedLine Citation:
PMID:  19317306     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
OBJECTIVES: Left ventricular hypertrabeculation (LVHT), also known as noncompaction, has been previously reported in a female patient with Turner syndrome (TS) with X0-karyotype, but has not been described in a male patient with a Turner mosaic.
CASE REPORT: In a 45-year-old man with short stature, facial dysmorphism, cryptorchism, hypospadia, but normal intellectual performance, TS was diagnosed upon cytogenetic evaluation and fluorescence in-situ hybridization revealing the karyotype mos45,X(28)/46,X,+mar(21)/47,X, + 2 mar(1). During an episode of heart failure at age 41 LVHT was detected in the posterolateral region on echocardiography also showing a slightly dilated left ventricle, severely reduced systolic function, and a moderate mitral and tricuspid insufficiency. On cardiac MRI LVHT was additionally seen in the lateral and anterior regions. Under adequate therapy, heart failure completely resolved but LVHT persisted.
CONCLUSIONS: LVHT may also occur in association with a mosaic TS with male phenotype. In such patients LVHT may not be accompanied by other congenital cardiac abnormalities but may be associated with severe cardiomyopathy resulting in rhythm abnormalities and heart failure.
Authors:
Hans Altenberger; Claudia Stöllberger; Josef Finsterer
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Acta cardiologica     Volume:  64     ISSN:  0001-5385     ISO Abbreviation:  Acta Cardiol     Publication Date:  2009 Feb 
Date Detail:
Created Date:  2009-03-25     Completed Date:  2009-06-05     Revised Date:  2013-05-28    
Medline Journal Info:
Nlm Unique ID:  0370570     Medline TA:  Acta Cardiol     Country:  Belgium    
Other Details:
Languages:  eng     Pagination:  99-103     Citation Subset:  IM    
Affiliation:
Universitätsklinik für Innere Medizin, Kardiologie, Internistiche Intensivmedizin, Paracelsus Medizinische Privatuniversität Salzburg, Austria.
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MeSH Terms
Descriptor/Qualifier:
Adrenergic alpha-Antagonists / therapeutic use
Angiotensin-Converting Enzyme Inhibitors / therapeutic use
Carbazoles / therapeutic use
Cardiomyopathy, Dilated / diagnosis*,  drug therapy,  genetics,  ultrasonography
Cardiotonic Agents / therapeutic use
Digoxin / therapeutic use
Diuretics / therapeutic use
Echocardiography
Furosemide / therapeutic use
Heart Ventricles / pathology*,  ultrasonography
Humans
Lisinopril / therapeutic use
Male
Middle Aged
Mineralocorticoid Receptor Antagonists / therapeutic use
Mosaicism*
Phenotype
Propanolamines / therapeutic use
Spironolactone / therapeutic use
Turner Syndrome / diagnosis*,  genetics
Ventricular Dysfunction, Left / diagnosis*,  drug therapy,  genetics,  ultrasonography
Chemical
Reg. No./Substance:
0/Adrenergic alpha-Antagonists; 0/Angiotensin-Converting Enzyme Inhibitors; 0/Carbazoles; 0/Cardiotonic Agents; 0/Diuretics; 0/Mineralocorticoid Receptor Antagonists; 0/Propanolamines; 0K47UL67F2/carvedilol; 20830-75-5/Digoxin; 52-01-7/Spironolactone; 54-31-9/Furosemide; 83915-83-7/Lisinopril

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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