Pulmonary Atresia

Pulmonary atresia is a type of congenital cardiovascular anomaly in which there is complete disruption between the right ventricular outflow tract (RVOT) and the central pulmonary arteries. The term 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 inter ventricular septum (PA-IVS)
* pulmonary atresia with VSD (PA-VSD)
* complex pulmonary atresia ( pulmonary atresia with complex cardiac malformation )

Radiographic features

CXR
PA - VSD 3:

* 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

MRI

Allows direct visualisation of anomaly. Cine sequences may show a dilated non contracting right ventricle 6. MR angiography allows detection of aortopulmonary collaterals and patent ductus arteriosus (if present)

Associations

tetralogy of Fallot : PA-VSD is considered by some authors as a severe from of tetralogy of Fallot

Treatment

  • management significantly varies depending on the presence of a VSD.
  • prostaglandin E1 is used to keep the ductus open 5.
Pulmonary%20atresia.jpg

Article Source : Dr Yuranga Weerakkody, Radiopaedia

References

* 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. Burrows PE, Freedom RM, Benson LN et-al. Coronary angiography of pulmonary atresia, hypoplastic right ventricle, and ventriculocoronary communications. AJR Am J Roentgenol. 1990;154 (4): 789-95. AJR Am J Roentgenol (abstract) - Pubmed citation
* 3. Elizabeth Diethelm et. al, The pulmonary vascularity in patients with pulmonary atresia and ventricular septal defect. RadioGraphics , Volume 5, Number 2 March 1985
* 4. Ramiah Rajeshkannan et.al, Role of 64-MDCT in Evaluation of Pulmonary Atresia With Ventricular Septal Defect, AJR 2010; 194:110-118
* 5. Aphrodite Tzifa et. al. Prenatal diagnosis of pulmonary atresia: impact on clinical presentation and early outcome, Arch Dis Child Fetal Neonatal Ed 2007;92:F199-F203 doi:10.1136/adc.2006.093880
* 6. Christian J. Kellenberger et.al, Cardiovascular MR Imaging in Neonates and Infants with Congenital Heart Disease : January 2007 RadioGraphics, 27, 5-18

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