Unilateral pulmonary artery atresia
Unilateral pulmonary artery atresia (UPAA) or unilateral absence of the pulmonary artery (UAPA) is a variant of pulmonary artery atresia.
The estimated prevalence is around 1 in 200,000 young adults. The reported frequency on the right side is slightly greater for some reason 10.
Can be variable and include:
- asymptomatic: adult patients with UPAA are often asymptomatic 8
- chest pain
- pleural effusion
- recurrent chest infections: lack of arterial blood flow to the affected lung may result in poor delivery of inflammatory cells to sites of inflammation and impaired ciliary function
- reduced exercise intolerance
- pulmonary hypertension
- high-altitude pulmonary oedema
It commonly occurs on the side opposite to the side of the aortic arch.
The distal intrapulmonary branches of the affected artery usually remain intact and can be supplied by collateral vessels from other vessels such as bronchial, intercostal, internal mammary, subdiaphragmatic, subclavian, or even coronary arteries.
It occurs in association with other cardiac anomalies in approximately 60% of cases. They include:
- septal defects
- coarctation of aorta
- right aortic arch
- truncus arteriosus
- tetralogy of Fallot
In around 40% of cases they occur in isolation, where it is then termed as:
Features can vary dependent on which side is affected.
May be seen as volume loss to the ipsilateral lung and overinflation +/- herniation across the midline of the contralateral lung 4.
Allows direct visualisation of the absence of the affected pulmonary artery. Volume loss to the affected lung is also shown. Extensive collaterals can be visualised. May allow recognition of associated cardiac anomalies.
Recognised complications include:
Treatment and prognosis
Treatment is often around management of complications in asymptomatic individuals. There is often no commonly accepted consensus on a particular treatment strategy.
History and etymology
It is thought to have been first described by O Frantzel in 1868 3.
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
- 1. Reading DW, Oza U. Unilateral absence of a pulmonary artery: a rare disorder with variable presentation. Proc (Bayl Univ Med Cent). 2012;25 (2): 115-8. Free text at pubmed - Pubmed citation
- Ghanbari H, Feldman D, David S et-al. Unilateral absence of a left pulmonary artery: successful therapeutic response to a combination of bosentan and warfarin. Circ Cardiovasc Imaging. 2009;2 (6): e46-8. doi:10.1161/CIRCIMAGING.108.825745 - Pubmed citation
- 3. Frantzel O. Angeborener Defect der Rechten Lungenarterie. Virchows Arch Pathol Anat. 1868; 43: 420
- 4. Werber J, Ramilo J, London R et-al. CHEST Journal. 1983;84 (6): . doi:10.1378/chest.84.6.729
- 5. Deutsch MA, Thieme SF, Hinterseer M et-al. Adult presentation of combined unilateral atresia of the right proximal pulmonary artery and left patent ductus arteriosus: case report and embryological considerations. Int. J. Cardiol. 2010;141 (1): e4-7. doi:10.1016/j.ijcard.2008.11.125 - Pubmed citation
- 6. Nakwan N. Congenital unilateral pulmonary atresia with coronary-to-pulmonary collateral artery originating from left circumflex coronary artery. Eur J Cardiothorac Surg. . doi:10.1093/ejcts/ezu223 - Pubmed citation
- 7. Koga H, Hidaka T, Miyako K et-al. Age-related clinical characteristics of isolated congenital unilateral absence of a pulmonary artery. Pediatr Cardiol. 2010;31 (8): 1186-90. doi:10.1007/s00246-010-9787-5 - Pubmed citation
- 8. Kruzliak P, Syamasundar RP, Novak M et-al. Unilateral absence of pulmonary artery: pathophysiology, symptoms, diagnosis and current treatment. Arch Cardiovasc Dis. 2013;106 (8-9): 448-54. doi:10.1016/j.acvd.2013.05.004 - Pubmed citation
- 9. Ten Harkel AD, Blom NA, Ottenkamp J. Isolated unilateral absence of a pulmonary artery: a case report and review of the literature. Chest. 2002;122 (4): 1471-7. Pubmed citation
- 10. Sankhla D, Hussein S, George J et-al. Absence of Left Pulmonary Artery: Case report. Sultan Qaboos Univ Med J. 2011;9 (2): 180-3. Free text at pubmed - Pubmed citation
- 11. Aypak C, Yıkılkan H, Uysal Z et-al. Unilateral absence of the pulmonary artery incidentally found in adulthood. Case Rep Med. 2012;2012: 942074. doi:10.1155/2012/942074 - Free text at pubmed - Pubmed citation