Outcomes of Children With Congenital Single Ventricle Heart
Summary
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.
Description
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.
Study Design
Observational Model: Defined Population, Observational Model: Natural History, Time Perspective: Longitudinal, Time Perspective: Retrospective
Conditions
Congenital Disorders
Location
Children's Healthcare of Atlanta
Atlanta
Georgia
United States
30322
Status
Active, not recruiting
Source
Children's Healthcare of Atlanta
Results (where available)
Links
- 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.
Mobius Syndrome
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)
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