Pulmonary artery atresia
Pulmonary artery atresia (or sometimes known as pulmonary atresia) is one of congenital cardiovascular anomaly in which there is complete disruption between the right ventricular outflow tract (RVOT) and the pulmonary trunk.
The estimated incidence is 1 in 10,000 births.
The term pulmonary atresia can cover a broad spectrum of abnormalities depending on the extent of the disruption.
It can be classified into 3 sub-types 1:
- pulmonary atresia with intact interventricular septum (PA-IVS)
- pulmonary atresia with VSD (PA-VSD)
- complex pulmonary atresia (pulmonary atresia with complex cardiac malformation)
- tetralogy of Fallot: PA-VSD is considered by some authors as a severe from 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 2
- heterotaxy syndrome: pulmonary atresia is present in two-fifths of right isomerism cases 9
- normal or mildly enlarged heart with poor / diminished pulmonary arterial vascular markings
- asymmetrical vascular suggest stenosis within pulmonary arterial tree
- there can be plethora due to horizontal arteries forming aorto-pulmonary collaterals
- mottled appearance as the lung periphery may suggest pleuro-pulmonary collateral formation
- severe cardiomegaly from massive right atrial dilatation 10
Echocardiography can characterise intracardiac anatomy. Has limited role in assessing pulmonary artery anatomy.
MDCT is better than echocardiography for pulmonary artery anatomy. It allows evaluation of the following 4:
- 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)
Allows direct visualisation of anomaly. Cine sequences may show a dilated non contracting right ventricle 5. 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 6.
- 1. Leonard H, Derrick G, O'Sullivan J et-al. Natural and unnatural history of pulmonary atresia. Heart. 2000;84 (5): 499-503. doi:10.1136/heart.84.5.499 - Free text at pubmed - Pubmed citation
- 2. Frank L, Dillman JR, Parish V et-al. Cardiovascular MR imaging of conotruncal anomalies. Radiographics. 2010;30 (4): 1069-94. doi:10.1148/rg.304095158 - Pubmed citation
- 3. Diethelm E, Soto B, Nath P et-al. The pulmonary vascularity in patients with pulmonary atresia and ventricular septal defect. RadioGraphics. 1985;5 (2): . doi:10.1148/radiographics.5.2.243
- 4. Rajeshkannan R, Moorthy S, Sreekumar KP et-al. Role of 64-MDCT in evaluation of pulmonary atresia with ventricular septal defect. AJR Am J Roentgenol. 2010;194 (1): 110-8. doi:10.2214/AJR.09.2802 - Pubmed citation
- 5. Kellenberger CJ, Yoo SJ, Büchel ER. Cardiovascular MR imaging in neonates and infants with congenital heart disease. Radiographics. 27 (1): 5-18. doi:10.1148/rg.271065027 - Pubmed citation
- 6. Tzifa A, Barker C, Tibby SM et-al. Prenatal diagnosis of pulmonary atresia: impact on clinical presentation and early outcome. Arch. Dis. Child. Fetal Neonatal Ed. 2007;92 (3): F199-203. doi:10.1136/adc.2006.093880 - Free text at pubmed - Pubmed citation
- 7. Daubeney PE, Sharland GK, Cook AC et-al. Pulmonary atresia with intact ventricular septum: impact of fetal echocardiography on incidence at birth and postnatal outcome. UK and Eire Collaborative Study of Pulmonary Atresia with Intact Ventricular Septum. Circulation. 1998;98 (6): 562-6. Circulation (link) - Pubmed citation
- 8. Krasemann T. Radiofrequency perforation: the optimum treatment for pulmonary atresia?. Heart. 2009;95 (18): 1473-4. doi:10.1136/hrt.2009.171579 - Pubmed citation
- 9. De Tommasi S, Daliento L, Ho SY et-al. Analysis of atrioventricular junction, ventricular mass, and ventriculoarterial junction in 43 specimens with atrial isomerism. Br Heart J. 1981;45 (3): 236-47. Free text at pubmed - Pubmed citation
- 10. Abbara S, Walker TG. Diagnostic imaging. AMIRSYS. ISBN:1416033408. Read it at Google Books - Find it at Amazon
Congenital heart disease
There is more than one way to present the variety of congenital heart diseases. Whichever way they are categorised, it is helpful to have a working understanding of normal and fetal circulation, as well as an understanding of the segmental approach to imaging in congenital heart disease.
congenital heart disease
- normal relationship between chambers and valves
- atrioventricular valves
- outflow tract
- great vessels
- venous inflow
- anomalous valves
- abnormal relationship of chambers and valves
- atrioventricular abnormality
- great vessel connection abnormality
- conotruncal cardiac anomalies
- pentalogy of Cantrell
- Shone syndrome
- congenital heart disease - chest x-ray approach
surgical repairs (mnemonic)
- arterial switch procedure
- Blalock-Taussig shunt
- double switch procedure
- Fontan procedure
- Glenn procedure
- Mustard repair
- Norwood procedure
- Pott shunt
- pulmonary artery banding
- Rastelli procedure
- Sano shunt
- Senning repair
- total repair of tetralogy of Fallot (TOF)
- unifocalisation procedure
- Waterston shunt