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KEY FACTS
Terminology
Imaging
Clinical Issues
TERMINOLOGY
Abbreviations
- Pulmonary atresia (PAt)
Synonyms
- Sometimes referred to as truncus arteriosus type 4 or pseudotruncus (misnomer)
Definitions
- 2 distinct entities, differentiated by presence or absence of ventricular septal defect (VSD)
- PAt, VSD, multiple aortopulmonary collateral arteries (MAPCAs): Hypoplastic/absent pulmonary arteries (PAs); MAPCAs supply 1 or both lungs
- Type A: Normal-sized PAs with small aortopulmonary (AP) collaterals
- Type B: Mild to moderate PA hypoplasia with multiple AP collaterals
- Type C: Markedly hypoplastic PAs with pulmonary flow from numerous AP collaterals
- PAt, intact ventricular septum (VS): Normal-sized PAs supplied by patent ductus arteriosus (PDA), patent foramen ovale (PFO)
- Coronary sinusoids or fistulas are often present
- PAt, VSD, multiple aortopulmonary collateral arteries (MAPCAs): Hypoplastic/absent pulmonary arteries (PAs); MAPCAs supply 1 or both lungs
- Both are characterized by underdevelopment of right ventricular outflow tract (RVOT) & pulmonary valve
- PAt, VSD, MAPCAs: At extreme end of spectrum of RVOT-obstructive (Fallot-type) heart lesions with complex & highly variable PA anatomy
- Category: Cyanotic, cardiomegaly, ↓ &/or irregular pulmonary vasculature
- Hemodynamics: Extreme outflow obstruction of RV; (almost) entire cardiac output goes into dilated overriding ascending aorta
- 2 distinct entities, differentiated by presence or absence of ventricular septal defect (VSD)
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Echocardiographic Findings
Angiographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
Selected References
- Gindes L et al: Prenatal diagnosis of major aortopulmonary collateral arteries (MAPCA) in fetuses with pulmonary atresia with ventricular septal defect and agenesis of ductus arteriosus. J Matern Fetal Neonatal Med. 1-9, 2021
- Katz JD et al: A 15-year-old with tetralogy of Fallot with pulmonic atresia, multiple aorticopulmonary collateral arteries and bilateral peripheral pulmonary stenosis. Cureus. 12(10):e11151, 2020
- Sinha M et al: Imaging characteristics and associations in twisted atrioventricular connections on multidetector computed tomography angiography. J Card Surg. 35(11):2979-86, 2020
- Fishbein GA et al: Tricuspid and pulmonic valve pathology. Curr Cardiol Rep. 21(7):54, 2019
- Rato J et al: Ebstein's anomaly with 'reversible' functional pulmonary atresia. BMJ Case Rep. 12(12), 2019
- Enaba MM et al: Multidetector computed tomography (CT) in evaluation of congenital cyanotic heart diseases. Pol J Radiol. 82:645-59, 2017
- Jonas SN et al: Pulmonary valve anatomy and abnormalities: a pictorial essay of radiography, computed tomography (CT), and magnetic resonance imaging (MRI). J Thorac Imaging. 31(1):W4-12, 2016
- Byeon JH et al: Coronary artery collateral flow and its effect on myocardial perfusion in a patient with unilateral pulmonary artery atresia. Cardiol Young. 1-4, 2015
- Ryan JR et al: A novel approach to neonatal management of tetralogy of Fallot, with pulmonary atresia, and multiple aortopulmonary collaterals. JACC Cardiovasc Imaging. 8(1):103-4, 2015
- Rajiah P et al: CT and MRI of pulmonary valvular abnormalities. Clin Radiol. 69(6):630-8, 2014
- Morozov AA: [Surgical treatment of pulmonic atresia with defect of interventricular septum and collateral lung circulation.] Vestn Khir Im I I Grek. 172(5):115-9, 2013
- Séguéla PE et al: Critical stenosis of a right ventricle to coronary artery fistula seen at dual-source CT in a newborn with pulmonary atresia and intact ventricular septum. Pediatr Radiol. 41(8):1069-72, 2011
- Kawel N et al: Preoperative evaluation of pulmonary artery morphology and pulmonary circulation in neonates with pulmonary atresia--usefulness of MR angiography in clinical routine. J Cardiovasc Magn Reson. 12:52, 2010
- Formigari R et al: Prevalence of bilateral patent ductus arteriosus in patients with pulmonic valve atresia and asplenia syndrome. Am J Cardiol. 70(13):1219-20, 1992
- Hruda J et al: Diagnostic selective balloon occlusion technique in pulmonic valve atresia and ventricular septal defect. Am J Cardiol. 63(18):1408-9, 1989
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References
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Tables
KEY FACTS
Terminology
Imaging
Clinical Issues
TERMINOLOGY
Abbreviations
- Pulmonary atresia (PAt)
Synonyms
- Sometimes referred to as truncus arteriosus type 4 or pseudotruncus (misnomer)
Definitions
- 2 distinct entities, differentiated by presence or absence of ventricular septal defect (VSD)
- PAt, VSD, multiple aortopulmonary collateral arteries (MAPCAs): Hypoplastic/absent pulmonary arteries (PAs); MAPCAs supply 1 or both lungs
- Type A: Normal-sized PAs with small aortopulmonary (AP) collaterals
- Type B: Mild to moderate PA hypoplasia with multiple AP collaterals
- Type C: Markedly hypoplastic PAs with pulmonary flow from numerous AP collaterals
- PAt, intact ventricular septum (VS): Normal-sized PAs supplied by patent ductus arteriosus (PDA), patent foramen ovale (PFO)
- Coronary sinusoids or fistulas are often present
- PAt, VSD, multiple aortopulmonary collateral arteries (MAPCAs): Hypoplastic/absent pulmonary arteries (PAs); MAPCAs supply 1 or both lungs
- Both are characterized by underdevelopment of right ventricular outflow tract (RVOT) & pulmonary valve
- PAt, VSD, MAPCAs: At extreme end of spectrum of RVOT-obstructive (Fallot-type) heart lesions with complex & highly variable PA anatomy
- Category: Cyanotic, cardiomegaly, ↓ &/or irregular pulmonary vasculature
- Hemodynamics: Extreme outflow obstruction of RV; (almost) entire cardiac output goes into dilated overriding ascending aorta
- 2 distinct entities, differentiated by presence or absence of ventricular septal defect (VSD)
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Echocardiographic Findings
Angiographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
Selected References
- Gindes L et al: Prenatal diagnosis of major aortopulmonary collateral arteries (MAPCA) in fetuses with pulmonary atresia with ventricular septal defect and agenesis of ductus arteriosus. J Matern Fetal Neonatal Med. 1-9, 2021
- Katz JD et al: A 15-year-old with tetralogy of Fallot with pulmonic atresia, multiple aorticopulmonary collateral arteries and bilateral peripheral pulmonary stenosis. Cureus. 12(10):e11151, 2020
- Sinha M et al: Imaging characteristics and associations in twisted atrioventricular connections on multidetector computed tomography angiography. J Card Surg. 35(11):2979-86, 2020
- Fishbein GA et al: Tricuspid and pulmonic valve pathology. Curr Cardiol Rep. 21(7):54, 2019
- Rato J et al: Ebstein's anomaly with 'reversible' functional pulmonary atresia. BMJ Case Rep. 12(12), 2019
- Enaba MM et al: Multidetector computed tomography (CT) in evaluation of congenital cyanotic heart diseases. Pol J Radiol. 82:645-59, 2017
- Jonas SN et al: Pulmonary valve anatomy and abnormalities: a pictorial essay of radiography, computed tomography (CT), and magnetic resonance imaging (MRI). J Thorac Imaging. 31(1):W4-12, 2016
- Byeon JH et al: Coronary artery collateral flow and its effect on myocardial perfusion in a patient with unilateral pulmonary artery atresia. Cardiol Young. 1-4, 2015
- Ryan JR et al: A novel approach to neonatal management of tetralogy of Fallot, with pulmonary atresia, and multiple aortopulmonary collaterals. JACC Cardiovasc Imaging. 8(1):103-4, 2015
- Rajiah P et al: CT and MRI of pulmonary valvular abnormalities. Clin Radiol. 69(6):630-8, 2014
- Morozov AA: [Surgical treatment of pulmonic atresia with defect of interventricular septum and collateral lung circulation.] Vestn Khir Im I I Grek. 172(5):115-9, 2013
- Séguéla PE et al: Critical stenosis of a right ventricle to coronary artery fistula seen at dual-source CT in a newborn with pulmonary atresia and intact ventricular septum. Pediatr Radiol. 41(8):1069-72, 2011
- Kawel N et al: Preoperative evaluation of pulmonary artery morphology and pulmonary circulation in neonates with pulmonary atresia--usefulness of MR angiography in clinical routine. J Cardiovasc Magn Reson. 12:52, 2010
- Formigari R et al: Prevalence of bilateral patent ductus arteriosus in patients with pulmonic valve atresia and asplenia syndrome. Am J Cardiol. 70(13):1219-20, 1992
- Hruda J et al: Diagnostic selective balloon occlusion technique in pulmonic valve atresia and ventricular septal defect. Am J Cardiol. 63(18):1408-9, 1989
