pulmonary artery atresia
Absent left
main pulmonary artery with patent ductus arteriosus. a) Coronal, b) Sagittal MIP and c) rotated 3D VRT images. The white arrow refers to a dilated patent ductus aretriosus. Note the absence of the left main pulmonary artery and right sided aortic arch.
Pulmonary
artery atresia • Complex congenital heart disease: complete AV canal, L-TGA, pulmonary atresia with multiple MAPCAs & total anomalous pulmonary venous drainage (TAPVD) - Ganzer Fall bei Radiopaedia
Newborn with
cyanosis. CXR AP shows cardiomegaly and relatively decreased pulmonary vascularity.The diagnosis was pulmonary atresia.
Right
Pulmonary Artery Atresia with Right Lung Hypoplasia. Volume rendering reconstruction of the thoracic vessels, no right pulmonary artery or segmentar and subsegmentary vessels are seen on the right hemithorax
Right
Pulmonary Artery Atresia with Right Lung Hypoplasia. At the level of the main pulmonary artery and left pulmonary artery, no right pulmonary artery is identifiable
Right
Pulmonary Artery Atresia with Right Lung Hypoplasia. At the level of the carina, the difference in lung volume and deviation of the mediastinum to the right areclear as well as is the hyperinflation of the left lung
Right
Pulmonary Artery Atresia with Right Lung Hypoplasia. Postero anterior view of chest radiograph shows loss of right lung volume and mediastinal shift towards the right, with hyperinflation of the left lung
Pulmonary
artery atresia • Pulmonary artery atresia with major aortopulmonary collateral arteries - Ganzer Fall bei Radiopaedia
Pulmonary
artery atresia • Heterotaxy syndrome (dextrocardia, atrioventricular discordance, pulmonary atresia) - Ganzer Fall bei Radiopaedia
Pulmonary
artery atresia • Heterotaxy syndrome (dextrocardia, atrioventricular discordance, pulmonary atresia) - Ganzer Fall bei Radiopaedia
Pulmonary
artery atresia • Pulmonary atresia with ventricular septal defect and situs inversus totalis - Ganzer Fall bei Radiopaedia
Cyanotic
newborn. CXR AP shows decreased pulmonary vascularity and mild cardiomegaly.The diagnosis was pulmonary atresia with intact ventricular septum.
Pulmonary
artery atresia • Pulmonary atresia - Ganzer Fall bei Radiopaedia
Pulmonary
artery atresia • Pulmonary atresia with intact interventricular septum - Ganzer Fall bei Radiopaedia
Pulmonary
artery atresia • Pulmonary atresia - Ganzer Fall bei Radiopaedia
Absent left
main pulmonary artery with patent ductus arteriosus. CT pulmonary angiography coronal reformatted image showing hypoplastic left lung with abnormally elevated left diaphragmatic copula. Dorsal scoliotic deformity with convexity to the left side noted.
Absent left
main pulmonary artery with patent ductus arteriosus. CT pulmonary angiography, axial MIP image revealed oligemic left lung with absent left pulmonary arterial supply. This is associated with increased right pulmonary vasculature.
Absent left
main pulmonary artery with patent ductus arteriosus. a) Coronal, b) Sagittal MIP and c) rotated 3D VRT images. The white arrow refers to a dilated patent ductus aretriosus. Note the absence of the left main pulmonary artery and right sided aortic arch.
Absent left
main pulmonary artery with patent ductus arteriosus. a) Coronal, b) Sagittal MIP and c) rotated 3D VRT images. The white arrow refers to a dilated patent ductus aretriosus. Note the absence of the left main pulmonary artery and right sided aortic arch.
Absent left
main pulmonary artery with patent ductus arteriosus. CT pulmonary angiography, axial MIP image revealed oligemic left lung which is supplied with dilated intercostal arteries (yellow arrow).
Absent left
main pulmonary artery with patent ductus arteriosus. CT pulmonary angiography, axial MIP image revealed dilatation of the pulmonary trunk and right main pulmonary artery. Note absence of arterial flow to the left lung
Pictorial
review of the pulmonary vasculature: from arteries to veins. Pulmonary artery atresia. a Axial CT image demonstrating abrupt termination of the main pulmonary artery (white arrow) in a patient with tetralogy of Fallot with a small hypoplastic distal pulmonary artery (white arrowhead). b Bronchial artery dilatation off the descending aorta occurs to feed the pulmonary vasculature (white arrows) with the hypoplastic distal pulmonary artery again demonstrated (white arrowhead)
Pulmonary artery atresia (or sometimes known as pulmonary atresia) is a congenital cardiovascular anomaly in which there is complete disruption between the right ventricular outflow tract (RVOT) and the pulmonary trunk.
Epidemiology
The estimated incidence is 1 in 10,000 births.
Pathology
The term pulmonary atresia can cover a broad spectrum of abnormalities depending on the extent of the disruption.
Classification
It can be classified into three types :
- pulmonary atresia with intact interventricular septum (PA-IVS)
- pulmonary atresia with VSD (PA-VSD)
- complex pulmonary atresia (pulmonary atresia with complex cardiac malformation)
Variants
Associations
- tetralogy of Fallot: PA-VSD is considered by some authors as a severe form of tetralogy of Fallot
- truncus arteriosus (Collett and Edwards type IV) or pseudotruncus: recognized to be a form of PA-VSD rather than truncus arteriosus
- heterotaxy syndrome: pulmonary atresia is present in two-fifths of right isomerism cases
Radiographic features
Plain radiograph
PA-VSD
- normal or mildly enlarged heart with poor or diminished pulmonary arterial vascular markings
- asymmetrical vascular suggests stenosis within pulmonary arterial tree
- there can be plethora due to horizontal arteries forming aortopulmonary collaterals
- mottled appearance as the lung periphery may suggest pleuro-pulmonary collateral formation
PA-IVS
- severe cardiomegaly from massive right atrial dilatation
Echocardiography
Echocardiography can characterize intracardiac anatomy. Has a limited role in assessing pulmonary artery anatomy.
MDCT
MDCT is better than echocardiography for pulmonary artery anatomy. It allows evaluation of the following:
- length of pulmonary atresia
- presence of pulmonary artery confluence
- seagull appearance of the confluent pulmonary arteries and abbreviated pulmonary trunk
- size of main, right, and left pulmonary arteries at the origin and at the hilum
- presence of branch pulmonary artery stenosis
- sources of pulmonary blood flow to each lung, including the number of bronchopulmonary segments supplied by native pulmonary arteries and the distribution of major aortopulmonary collateral arteries (MAPCAs)
Cardiac MRI
Allows direct visualization of the anomaly. Cine sequences may show a dilated non contracting right ventricle . MR angiography allows detection of aortopulmonary collaterals and patent ductus arteriosus (if present).
Treatment and prognosis
- management varies depending on the presence of a VSD.
- one surgical technique includes the unifocalisation procedure which can be performed in cases with complete pulmonary artery atresia with major aortopulmonary collateral arteries (MAPCAs)
- prostaglandin E1 is used to keep the ductus open
Siehe auch:
und weiter:
- Persistierender Ductus arteriosus
- Herzfehler
- Lungenhypoplasie
- Varianten der Herzanatomie
- abnormal ductus venosus waveforms
- Isolated unilateral absence of pulmonary artery
- congenital pulmonary arterial stenosis
- pulmonary atresia with ventricular septal defect and situs inversus totalis
- Varianten Pulmonalarterie
- unilaterale Pulmonalarterienatresie
Assoziationen und Differentialdiagnosen zu Pulmonalatresie:
congenital
cardiovascular anomaly