Document Detail

Cyanotic congenital heart disease with decreased pulmonary blood flow in children.
MedLine Citation:
PMID:  10218082     Owner:  NLM     Status:  MEDLINE    
Of the "five T's" of cyanotic congenital heart disease--tetralogy of Fallot, TGA, TAPVC, truncus, and tricuspid valve abnormalities (tricuspid atresia, stenosis, and displacement)--the first and last are commonly associated with diminished PBF. The four features that comprise tetralogy of Fallot--right ventricular hypertrophy, VSD, overriding aorta, and subpulmonary stenosis--are all secondary to a single morphogenetic defect: failure of expansion of the subpulmonary conus. This also explains the variability in clinical presentation. When neonates need intervention, shunts are usually performed. Coronary arterial anatomy must be defined before repair, which is usually done after these infants are 3 months of age. Although children with repaired tetralogy of Fallot are not completely "normal," markedly increased longevity and improvement in quality of life can be achieved. When major associated defects are present, such as atrioventricular canal defect, diminutive pulmonary arteries or collateral vessels, or left heart lesions, the prognosis changes from excellent to merely good. Tetralogy of Fallot with absent pulmonary valve syndrome is physiologically different from other tetralogy of Fallot conditions and characterized primarily by airway obstruction from massive dilatation of the central and perihilar pulmonary arteries; repair with pulmonary artery reduction is necessary. Tricuspid valve abnormalities include atresia, hypoplasia (i.e., pulmonary atresia with intact ventricular septum), and displacement (i.e., Ebstein anomaly). The pathophysiology that dictates these children's clinical condition (and prognosis) relates to three factors: (1) status of the tricuspid valve, (2) presence and size of a VSD, and (3) TGA or normally related great arteries. Virtually all children with tricuspid valve abnormalities can be palliated; reparative options include repair using two-ventricle, one-ventricle, or 1-1/2 ventricle repair. Children with critical pulmonary stenosis generally have a normal tricuspid valve and right ventricle. Balloon dilation is usually the only therapy necessary.
J D Waldman; J A Wernly
Publication Detail:
Type:  Journal Article; Review    
Journal Detail:
Title:  Pediatric clinics of North America     Volume:  46     ISSN:  0031-3955     ISO Abbreviation:  Pediatr. Clin. North Am.     Publication Date:  1999 Apr 
Date Detail:
Created Date:  1999-05-06     Completed Date:  1999-05-06     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  0401126     Medline TA:  Pediatr Clin North Am     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  385-404     Citation Subset:  AIM; IM    
University of New Mexico Health Sciences Center, Children's Hospital Heart Center, Albuquerque, USA.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Cyanosis / etiology*
Decision Trees
Infant, Newborn
Pulmonary Circulation*
Pulmonary Veins / abnormalities*
Tetralogy of Fallot / complications*,  diagnosis,  physiopathology,  therapy
Transposition of Great Vessels / complications*,  diagnosis,  physiopathology,  therapy
Tricuspid Valve / abnormalities*
Truncus Arteriosus, Persistent / complications*,  diagnosis,  physiopathology,  therapy

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

Previous Document:  Left ventricular outflow obstruction.
Next Document:  Cyanotic congenital heart disease with increased pulmonary blood flow.