Tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries (TOF/PA/MAPCAs)
- Ritu Asija, MD
Ritu Asija, MD
- Clinical Assistant Professor, Department of Pediatrics
- Division of Pediatric Cardiology, Stanford University
- Stanton Perry, MD
Stanton Perry, MD
- Associate Professor of Pediatrics (Cardiology)
- Stanford University
- Section Editors
- David R Fulton, MD
David R Fulton, MD
- Section Editor — Pediatric Cardiology
- Associate Professor of Pediatrics
- Harvard Medical School
- Candice Silversides, MD, MS, FRCPC
Candice Silversides, MD, MS, FRCPC
- Section Editor — Congenital Heart Disease
- Associate Professor of Medicine
- University of Toronto
Tetralogy of Fallot with pulmonary valve atresia and major aortopulmonary collateral arteries (TOF/PA/MAPCAs) is the most extreme variant of TOF, in which complete atresia of the pulmonary valve replaces pulmonary stenosis.
The definition, anatomy, physiology, clinical presentation, management, and outcome of TOF/PA/MAPCAs will be reviewed here.
TOF/PA is relatively rare, with a reported incidence of 0.7 per 10,000 live births in the Baltimore-Washington Infant Study . While TOF is the most common cyanotic congenital heart lesion, TOF/PA/MAPCAs is considered to be the most extreme form of TOF and accounts for approximately one-fifth of all cases of TOF .
TOF/PA is a complex lesion that includes characteristic features of TOF (anterior malaligned ventricular septal defect [VSD] and overriding aorta) with PA. PA may be limited to the valve itself (membranous PA) or involve the subpulmonary infundibulum (muscular PA), and results in no antegrade flow from the right ventricle (RV) to the pulmonary artery. (See "Pulmonary atresia with intact ventricular septum (PA/IVS)", section on 'Pulmonary valve atresia'.)
The lack of antegrade pulmonary blood flow in utero leads to a range of morphologic findings in the pulmonary artery vasculature. If the ductus arteriosus (DA) is present, confluent true pulmonary arteries of variable size may develop. Without flow through the DA, MAPCAs, fetal vessels derived from the splanchnic vascular plexus, may persist after birth . These vessels connect the systemic and pulmonary arterial vasculature, thereby supplying pulmonary blood flow. MAPCAs are tortuous vessels that arise directly from the aorta or its branches. MAPCAs vary in number and origin, follow circuitous routes to reach central, lobar, and segmental pulmonary arteries, and have variable areas and locations of stenosis. Their arborization pattern is unpredictable and often incomplete, leaving some lung segments with excessive or insufficient flow, and they can become narrow over time [4,5]. As a result, a given segment of the lung may be supplied solely from the true pulmonary arteries, solely from the MAPCAs, or both. The morphology of the pulmonary vasculature and MAPCAs plays a critical role in determining management decisions. (See 'Surgical intervention' below.)
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- CLINICAL PRESENTATION
- Fetal presentation
- Postnatal presentation
- - Cardiac examination
- - Tests
- Pulse oximetry
- Chest radiography
- Laboratory testing
- FURTHER EVALUATION
- - Cardiac catheterization
- - MR and CT angiography
- DIFFERENTIAL DIAGNOSIS
- Initial medical treatment
- Surgical intervention
- - Our approach
- POSTOPERATIVE MANAGEMENT
- HEALTHCARE MAINTENANCE
- Endocarditis prophylaxis
- SUMMARY AND RECOMMENDATIONS