| Isolated left ventricular hypertrabeculation/noncompaction in a Turner mosaic with male phenotype. | |
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MedLine Citation:
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PMID: 19317306 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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Hans Altenberger; Claudia Stöllberger; Josef Finsterer |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Acta cardiologica Volume: 64 ISSN: 0001-5385 ISO Abbreviation: Acta Cardiol Publication Date: 2009 Feb |
Date Detail:
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Created Date: 2009-03-25 Completed Date: 2009-06-05 Revised Date: 2009-06-11 |
Medline Journal Info:
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Nlm Unique ID: 0370570 Medline TA: Acta Cardiol Country: Belgium |
Other Details:
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Languages: eng Pagination: 99-103 Citation Subset: IM |
Affiliation:
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Universitätsklinik für Innere Medizin, Kardiologie, Internistiche Intensivmedizin, Paracelsus Medizinische Privatuniversität Salzburg, Austria. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adrenergic alpha-Antagonists
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therapeutic use Aldosterone 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 Mosaicism* Phenotype Propanolamines / therapeutic use Spironolactone / therapeutic use Turner Syndrome / diagnosis*, genetics Ventricular Dysfunction, Left / diagnosis*, drug therapy, genetics, ultrasonography |
| Chemical | |
Reg. No./Substance:
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0/Adrenergic alpha-Antagonists; 0/Aldosterone Antagonists; 0/Angiotensin-Converting Enzyme Inhibitors; 0/Carbazoles; 0/Cardiotonic Agents; 0/Diuretics; 0/Propanolamines; 20830-75-5/Digoxin; 52-01-7/Spironolactone; 54-31-9/Furosemide; 72956-09-3/carvedilol; 83915-83-7/Lisinopril |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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