Outcomes of Children With Congenital Single Ventricle Heart
Congenital heart disease affects 1 in 100 newborn babies each year and more than 2,000,000 Americans have a congenital heart defect. One common defect treated at Children's Healthcare of Atlanta at Egleston is single ventricle heart. Due to these overwhelming numbers, the use of diagnostic imaging technology to assess these defects and heart function is an important step in the evolving care of this patient group.
The definition of single ventricle can mean a ventricle that is hypoplastic, too small, or completely absent. Either the right or left ventricle may be affected. There may also be other cardiac anomalies present. Infants with a single ventricle develop distress after birth when the ductus arteriosus and foramen ovale close. This may happen within hours or days of delivery. The severity of symptoms is determined by the degree of defect. But, single ventricle patients will not survive without treatment. Patients with only one functioning ventricle can usually expect either a heart transplant or a series of palliative surgeries or sometimes both. Their care is very complex requiring a team of dedicated practitioners to manage drug therapy, medical support and surgery.
At Children's Healthcare of Atlanta at Egleston, it is standard of care for a patient with a single ventricle heart to undergo many non-invasive imaging studies and sometimes invasive studies such as heart catheterization. Results of the studies provide valuable information used for treatment decisions and evaluation of heart function. We propose to do a retrospective chart review of patient data including a review of their invasive and non-invasive studies.
Observational Model: Defined Population, Observational Model: Natural History, Time Perspective: Longitudinal, Time Perspective: Retrospective
Children's Healthcare of Atlanta
Active, not recruiting
Children's Healthcare of Atlanta
Results (where available)
- Source: http://clinicaltrials.gov/show/NCT00266968
- Information obtained from ClinicalTrials.gov on July 15, 2010
Medical and Biotech [MESH] Definitions
Myasthenic Syndromes, Congenital
A heterogeneous group of disorders characterized by a congenital defect in neuromuscular transmission at the NEUROMUSCULAR JUNCTION. This includes presynaptic, synaptic, and postsynaptic disorders (that are not of autoimmune origin). The majority of these diseases are caused by mutations of various subunits of the nicotinic acetylcholine receptor (RECEPTORS, NICOTINIC) on the postsynaptic surface of the junction. (From Arch Neurol 1999 Feb;56(2):163-7)
Lipodystrophy, Congenital Generalized
Congenital disorders, usually autosomal recessive, characterized by severe generalized lack of ADIPOSE TISSUE, extreme INSULIN RESISTANCE, and HYPERTRIGLYCERIDEMIA.
Congenital Disorders Of Glycosylation
A genetically heterogeneous group of heritable disorders resulting from defects in protein N-glycosylation.
Urea Cycle Disorders, Inborn
Rare congenital metabolism disorders of the urea cycle. The disorders are due to mutations that result in complete (neonatal onset) or partial (childhood or adult onset) inactivity of an enzyme, involved in the urea cycle. Neonatal onset results in clinical features that include irritability, vomiting, lethargy, seizures, NEONATAL HYPOTONIA; RESPIRATORY ALKALOSIS; HYPERAMMONEMIA; coma, and death. Survivors of the neonatal onset and childhood/adult onset disorders share common risks for ENCEPHALOPATHIES, METABOLIC, INBORN; and RESPIRATORY ALKALOSIS due to HYPERAMMONEMIA.
A syndrome of congenital facial paralysis, frequently associated with abducens palsy and other congenital abnormalities including lingual palsy, clubfeet, brachial disorders, cognitive deficits, and pectoral muscle defects. Pathologic findings are variable and include brain stem nuclear aplasia, facial nerve aplasia, and facial muscle aplasia, consistent with a multifactorial etiology. (Adams et al., Principles of Neurology, 6th ed, p1020)
Infants with congenital long QT syndrome (LQTS) and 2:1 atrioventricular block (AVB) have been recognized as a clinical subset of children with LQTS. The purpose of this study is to descr...
Review the outcome of treatment with endothelin receptor antagonists (bosentan or sitaxsetan) alone or in combination with Sildenafil (a PDE-5 inhibitor) in adult patients with pulmonary h...
It is now estimated that the number of adults with congenital heart disease in the U.S is over 800,000. Unfortunately, these patients, in some way, have become a lost population. They hav...
The aim of this study is the identification of familial congenital arrhythmogenic disorders and their clinical follow-up.
The primary objective of this study is to determine the clinical benefits of percutaneous intervention to improve pulmonary blood flow on oxygen saturations, symptoms, exercise tolerance a...
Congenital disorders of glycosylation (CDG) is a booming class of metabolic diseases. Its number has increased nearly fourfold (to 45) since 2003, the year of the Komrower lecture, entitled 'Congenita...
The mammalian hair follicle (HF) is an active skin appendage which operates hair cycles throughout life. Recent advances in molecular genetics have led to the identification of many genes expressed in...
Congenital loss of innervation to the extra-ocular muscles (EOMs) can have a profound effect on the target muscle. This has been well recognised in Duane's retraction syndrome. However, it has been le...
Inherited thrombocytopathies are much less frequent in comparison to acquired platelet function disorders. However, congenital disorders can lead to severe bleeding tendency and are often not diagnose...
AimWe thought of assessing the prevalence and predictors of feeding disorders in patients with congenital heart defects after neonatal cardiac surgery. METHODS: Retrospective study of 82 consecutive n...