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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
TERMINOLOGY
Abbreviations
- Pulmonary atresia with ventricular septal defect (PA-VSD)
- Pulmonary atresia with intact ventricular septum (PA-IVS)
Definitions
- Absence of communication between right ventricle (RV) and main pulmonary artery (MPA)
- 2 distinct types based on interventricular septum
- PA-IVS (intact ventricular septum)
- Complete atresia of pulmonary valve
- RV hypoplasia (single ventricle physiology) or dilation
- Normal-sized PAs
- Lungs supplied by PDA and PFO
- RV to coronary connections in 50% at birth
- Tricuspid valve hyopplasia/dysplasia
- PA-VSD (ventricular septal defect)
- Ranges from imperforate pulmonary valve (extreme variant of TOF, seen in 10%) to hypoplastic/absent pulmonary arteries and branches (pseudotruncus, truncus arteriosus type 4)
- Major aortopulmonary collateral arteries (MAPCAs) supply 1 or both lungs
- 2 functional ventricles exist
- RV blood exits through overriding aorta and supplies systemic and pulmonary circuit
- PA-IVS (intact ventricular septum)
- Category: Cyanotic, cardiomegaly, decreased ± irregular pulmonary vascularity
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
- Wright LK et al: Long-term outcomes after intervention for pulmonary atresia with intact ventricular septum. Heart. ePub, 2019
- Presnell LB et al: An overview of pulmonary atresia and major aortopulmonary collateral arteries. World J Pediatr Congenit Heart Surg. 6(4):630-9, 2015
- Frank L et al: Cardiovascular MR imaging of conotruncal anomalies. Radiographics. 30(4):1069-94, 2010
- Rajeshkannan R et al: Role of 64-MDCT in evaluation of pulmonary atresia with ventricular septal defect. AJR Am J Roentgenol. 194(1):110-8, 2010
- François CJ et al: Unenhanced MR angiography of the thoracic aorta: initial clinical evaluation. AJR Am J Roentgenol. 190(4):902-6, 2008
- Gaca AM et al: Repair of congenital heart disease: a primer--Part 2. Radiology. 248(1):44-60, 2008
- Gaca AM et al: Repair of congenital heart disease: a primer-part 1. Radiology. 247(3):617-31, 2008
- Dorfman AL et al: Magnetic resonance imaging evaluation of congenital heart disease: conotruncal anomalies. J Cardiovasc Magn Reson. 8(4):645-59, 2006
- Greil GF et al: Imaging of aortopulmonary collateral arteries with high-resolution multidetector CT. Pediatr Radiol. 36(6):502-9, 2006
- Boechat MI et al: Cardiac MR imaging and MR angiography for assessment of complex tetralogy of Fallot and pulmonary atresia. Radiographics. 25(6):1535-46, 2005
- Roche KJ et al: Assessment of vasculature using combined MRI and MR angiography. AJR Am J Roentgenol. 182(4):861-6, 2004
- Baque J et al: Evaluation of pulmonary atresia with magnetic resonance imaging. Heart. 87(2):159, 2002
- Okada M et al: Modified Blalock-Taussig shunt patency for pulmonary atresia: assessment with electron beam CT. J Comput Assist Tomogr. 26(3):368-72, 2002
- Holmqvist C et al: Pre-operative evaluation with MR in tetralogy of fallot and pulmonary atresia with ventricular septal defect. Acta Radiol. 42(1):63-9, 2001
- Doyle TP et al: Tetralogy of Fallot and pulmonary atresia with ventricular septal defect. In: Moller JH et al: Pediatric Cardiovascular Medicine. 1st ed. Philadelphia: Churchill Livingstone. 391-408, 2000
- Freedom RM: Pulmonary atresia and intact ventricular septum. In Moller JH et al: Pediatric Cardiovascular Medicine. 1st ed. Philadelphia: Churchill Livingstone. 442-60, 2000
- Powell AJ et al: Accuracy of MRI evaluation of pulmonary blood supply in patients with complex pulmonary stenosis or atresia. Int J Card Imaging. 16(3):169-74, 2000
- Ichida F et al: Evaluation of pulmonary blood supply by multiplanar cine magnetic resonance imaging in patients with pulmonary atresia and severe pulmonary stenosis. Int J Card Imaging. 15(6):473-81, 1999
- Westra SJ et al: Cardiac electron-beam CT in children undergoing surgical repair for pulmonary atresia. Radiology. 213(2):502-12, 1999
- Choe YH et al: MR imaging of non-visualized pulmonary arteries at angiography in patients with congenital heart disease. J Korean Med Sci. 13(6):597-602, 1998
- Frank H et al: Magnetic resonance imaging of absent pulmonary valve syndrome. Pediatr Cardiol. 17(1):35-9, 1996
- Taneja K et al: Comparison of computed tomography and cineangiography in the demonstration of central pulmonary arteries in cyanotic congenital heart disease. Cardiovasc Intervent Radiol. 19(2):97-100, 1996
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Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
TERMINOLOGY
Abbreviations
- Pulmonary atresia with ventricular septal defect (PA-VSD)
- Pulmonary atresia with intact ventricular septum (PA-IVS)
Definitions
- Absence of communication between right ventricle (RV) and main pulmonary artery (MPA)
- 2 distinct types based on interventricular septum
- PA-IVS (intact ventricular septum)
- Complete atresia of pulmonary valve
- RV hypoplasia (single ventricle physiology) or dilation
- Normal-sized PAs
- Lungs supplied by PDA and PFO
- RV to coronary connections in 50% at birth
- Tricuspid valve hyopplasia/dysplasia
- PA-VSD (ventricular septal defect)
- Ranges from imperforate pulmonary valve (extreme variant of TOF, seen in 10%) to hypoplastic/absent pulmonary arteries and branches (pseudotruncus, truncus arteriosus type 4)
- Major aortopulmonary collateral arteries (MAPCAs) supply 1 or both lungs
- 2 functional ventricles exist
- RV blood exits through overriding aorta and supplies systemic and pulmonary circuit
- PA-IVS (intact ventricular septum)
- Category: Cyanotic, cardiomegaly, decreased ± irregular pulmonary vascularity
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
- Wright LK et al: Long-term outcomes after intervention for pulmonary atresia with intact ventricular septum. Heart. ePub, 2019
- Presnell LB et al: An overview of pulmonary atresia and major aortopulmonary collateral arteries. World J Pediatr Congenit Heart Surg. 6(4):630-9, 2015
- Frank L et al: Cardiovascular MR imaging of conotruncal anomalies. Radiographics. 30(4):1069-94, 2010
- Rajeshkannan R et al: Role of 64-MDCT in evaluation of pulmonary atresia with ventricular septal defect. AJR Am J Roentgenol. 194(1):110-8, 2010
- François CJ et al: Unenhanced MR angiography of the thoracic aorta: initial clinical evaluation. AJR Am J Roentgenol. 190(4):902-6, 2008
- Gaca AM et al: Repair of congenital heart disease: a primer--Part 2. Radiology. 248(1):44-60, 2008
- Gaca AM et al: Repair of congenital heart disease: a primer-part 1. Radiology. 247(3):617-31, 2008
- Dorfman AL et al: Magnetic resonance imaging evaluation of congenital heart disease: conotruncal anomalies. J Cardiovasc Magn Reson. 8(4):645-59, 2006
- Greil GF et al: Imaging of aortopulmonary collateral arteries with high-resolution multidetector CT. Pediatr Radiol. 36(6):502-9, 2006
- Boechat MI et al: Cardiac MR imaging and MR angiography for assessment of complex tetralogy of Fallot and pulmonary atresia. Radiographics. 25(6):1535-46, 2005
- Roche KJ et al: Assessment of vasculature using combined MRI and MR angiography. AJR Am J Roentgenol. 182(4):861-6, 2004
- Baque J et al: Evaluation of pulmonary atresia with magnetic resonance imaging. Heart. 87(2):159, 2002
- Okada M et al: Modified Blalock-Taussig shunt patency for pulmonary atresia: assessment with electron beam CT. J Comput Assist Tomogr. 26(3):368-72, 2002
- Holmqvist C et al: Pre-operative evaluation with MR in tetralogy of fallot and pulmonary atresia with ventricular septal defect. Acta Radiol. 42(1):63-9, 2001
- Doyle TP et al: Tetralogy of Fallot and pulmonary atresia with ventricular septal defect. In: Moller JH et al: Pediatric Cardiovascular Medicine. 1st ed. Philadelphia: Churchill Livingstone. 391-408, 2000
- Freedom RM: Pulmonary atresia and intact ventricular septum. In Moller JH et al: Pediatric Cardiovascular Medicine. 1st ed. Philadelphia: Churchill Livingstone. 442-60, 2000
- Powell AJ et al: Accuracy of MRI evaluation of pulmonary blood supply in patients with complex pulmonary stenosis or atresia. Int J Card Imaging. 16(3):169-74, 2000
- Ichida F et al: Evaluation of pulmonary blood supply by multiplanar cine magnetic resonance imaging in patients with pulmonary atresia and severe pulmonary stenosis. Int J Card Imaging. 15(6):473-81, 1999
- Westra SJ et al: Cardiac electron-beam CT in children undergoing surgical repair for pulmonary atresia. Radiology. 213(2):502-12, 1999
- Choe YH et al: MR imaging of non-visualized pulmonary arteries at angiography in patients with congenital heart disease. J Korean Med Sci. 13(6):597-602, 1998
- Frank H et al: Magnetic resonance imaging of absent pulmonary valve syndrome. Pediatr Cardiol. 17(1):35-9, 1996
- Taneja K et al: Comparison of computed tomography and cineangiography in the demonstration of central pulmonary arteries in cyanotic congenital heart disease. Cardiovasc Intervent Radiol. 19(2):97-100, 1996
