To access 4,300 diagnoses written by the world's leading experts in radiology, please log in or subscribe.Log inSubscribe
0
4
1
1
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
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
Related Anatomy
Loading...
Related Differential Diagnoses
Loading...
References
Tables
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
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
STATdx includes over 200,000 searchable images, including x-ray, CT, MR and ultrasound images. To access all images, please log in or subscribe.