Results 91 to 100 of about 2,024 (185)

Barth syndrome

open access: yesOrphanet Journal of Rare Diseases, 2013
First described in 1983, Barth syndrome (BTHS) is widely regarded as a rare X-linked genetic disease characterised by cardiomyopathy (CM), skeletal myopathy, growth delay, neutropenia and increased urinary excretion of 3-methylglutaconic acid (3-MGCA ...
Clarke Sarah LN   +16 more
doaj   +1 more source

Re-evaluation of hypoplastic left heart syndrome from a developmental and morphological perspective

open access: yesOrphanet Journal of Rare Diseases, 2017
Background Hypoplastic left heart syndrome (HLHS) covers a spectrum of rare congenital anomalies characterised by a non-apex forming left ventricle and stenosis/atresia of the mitral and aortic valves.
A. Crucean   +8 more
doaj   +1 more source

Maternal and Fetal Factors Associated With Mortality and Morbidity in a Multi–Racial/Ethnic Registry of Anti-SSA/Ro–Associated Cardiac Neonatal Lupus [PDF]

open access: yes, 2011
Background— Cardiac manifestations of neonatal lupus include conduction disease and, rarely, an isolated cardiomyopathy. This study was initiated to determine the mortality and morbidity of cardiac neonatal lupus and associated ...
Deborah Friedman   +15 more
core   +1 more source

Untreated ALCAPA diagnosed in gestational ultrasonography

open access: yesRadiology Case Reports
ALCAPA is a rare congenital anomaly that presents with left ventricular (LV) dysfunction and mitral valve regurgitation. The mortality rate is roughly 90% if the intracoronary collateral isn't significantly augmented.
Lam Truong Hoai, MD   +5 more
doaj   +1 more source

Clinical and Pathoanatomical Characteristics of Hypoplastic Left Heart Syndrome a Clinical Case

open access: yesJuvenis Scientia
Hypoplastic left heart syndrome (HLHS) is a common congenital heart defect, accounting for 4 to 16% of cases, and is one of the most prognostically unfavorable, involving single-­ventricle hemodynamics.
Chepelev, Alexandr S.   +2 more
doaj   +1 more source

Abnormal Flow Conditions Promote Endocardial Fibroelastosis Via Endothelial-to-Mesenchymal Transition, Which Is Responsive to Losartan Treatment. [PDF]

open access: yesJACC Basic Transl Sci, 2021
Oh NA   +8 more
europepmc   +1 more source

Mitral valve orifice area predicts outcome after biventricular repair in patients with hypoplastic left ventricles

open access: yesJournal of Cardiovascular Magnetic Resonance
: Background: Identification of risk factors for biventricular (BiV) repair in children with hypoplastic left ventricles (HLV) has been challenging.
David Liddle   +9 more
doaj   +1 more source

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