Adult Pulmonary Valve Replacement: A Simple and Reproducible Technique
Summary
Pulmonary valve replacement in the adult population is an uncommon operation. The majority of native valve pathology in adults involves the mitral, aortic, and occasionally tricuspid valves. On the other hand, right ventricular outflow tract and pulmonary valve disease is quite common in children, especially with tetralogy of fallot, truncus arteriosus, pulmonary atresia/ventricular septal defect, and double outlet right ventricle. Unfortunately, right ventricular pathology often develops in adulthood as a result of pulmonary insufficiency or pulmonary stenosis created by previous childhood operations. Without the valve size constraints present at prior operations, these patients can be well-served by the placement of adult-sized bioprosthetic valves. There are limited descriptions in the adult cardiac literature of the actual technique of pulmonary valve replacement. We present our current technique and the pitfalls encountered when performing pulmonary valve replacement in adults.
Description
This will be primarily a technique paper. The only clinical data points I am interested in are the presence of pulmonary stenosis, pulmonary insufficiency or perivalvular leak on the initial post-operative echocardiogram. I estimate reviewing 50 patients' charts dated March 22.2003 through and including March 22.2006 at Emory Hospital .
Primary Aim: Present an effective technique for pulmonary valve replacement
Secondary Aim: Present our excellent technical results of PVR in our adult congenital patients
Study Design
Observational Model: Defined Population, Observational Model: Natural History, Time Perspective: Longitudinal, Time Perspective: Retrospective
Conditions
Congenital Disorders
Location
Emory University
Atlanta
Georgia
United States
30306
Status
Terminated
Source
Emory University
Results (where available)
Links
- Source: http://clinicaltrials.gov/show/NCT00384163
- 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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