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Unilateral Absence of the Left Pulmonary Artery With an Associated Vascular Anomaly in Adulthood.

Flores M, Letter H, Derrick E, Koury I - Cureus (2016)

Bottom Line: Left-sided pulmonary artery agenesis is a rare malformation that commonly requires childhood intervention secondary to associated congenital cardiovascular anomalies.We present an uncommon case of left-sided agenesis with an associated right-sided aortic arch and significant hypoplasia of the ipsilateral lung.Additionally, there is radiographic evidence of emphysema and pulmonary artery hypertension.

View Article: PubMed Central - HTML - PubMed

Affiliation: Diagnostic Radiology, Florida Hospital-Orlando.

ABSTRACT
Left-sided pulmonary artery agenesis is a rare malformation that commonly requires childhood intervention secondary to associated congenital cardiovascular anomalies. We present an uncommon case of left-sided agenesis with an associated right-sided aortic arch and significant hypoplasia of the ipsilateral lung. Additionally, there is radiographic evidence of emphysema and pulmonary artery hypertension. Pulmonary artery agenesis is not a common entity, but should be considered in adult patients presenting with recurrent pneumonias and radiographic evidence suggestive of pulmonary hypoplasia. A prompt diagnosis is beneficial for affected individuals who may be candidates for a revascularization procedure or embolization of collaterals. Earlier diagnosis also allows for proper management and follow-up care, considering pulmonary artery hypertension is a severe complication of pulmonary artery agenesis.

No MeSH data available.


Related in: MedlinePlus

CT chest, scout image.There is leftward shift of the cardiomediastinal silhouette (red arrow) with hyperinflation of the right lung; no evidence of left lobe collapse. The left hemithorax is asymmetrically smaller when compared to the right (black arrow). There is central left-sided bronchiectasis with opacification of the left lower lung field. Incidental note is made of a right-sided aortic arch (blue arrow). 
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FIG1: CT chest, scout image.There is leftward shift of the cardiomediastinal silhouette (red arrow) with hyperinflation of the right lung; no evidence of left lobe collapse. The left hemithorax is asymmetrically smaller when compared to the right (black arrow). There is central left-sided bronchiectasis with opacification of the left lower lung field. Incidental note is made of a right-sided aortic arch (blue arrow). 

Mentions: Subsequent contrast-enhanced computed tomography (CT) of the chest (Figures 1-5, Video 1) at our institution revealed left-sided pulmonary artery agenesis with an associated right-sided aortic arch and significant hypoplasia of the ipsilateral lung. Hyperinflation and expansion of the remaining right lung with evidence of emphysema and pulmonary artery hypertension were also observed. 


Unilateral Absence of the Left Pulmonary Artery With an Associated Vascular Anomaly in Adulthood.

Flores M, Letter H, Derrick E, Koury I - Cureus (2016)

CT chest, scout image.There is leftward shift of the cardiomediastinal silhouette (red arrow) with hyperinflation of the right lung; no evidence of left lobe collapse. The left hemithorax is asymmetrically smaller when compared to the right (black arrow). There is central left-sided bronchiectasis with opacification of the left lower lung field. Incidental note is made of a right-sided aortic arch (blue arrow). 
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4829407&req=5

FIG1: CT chest, scout image.There is leftward shift of the cardiomediastinal silhouette (red arrow) with hyperinflation of the right lung; no evidence of left lobe collapse. The left hemithorax is asymmetrically smaller when compared to the right (black arrow). There is central left-sided bronchiectasis with opacification of the left lower lung field. Incidental note is made of a right-sided aortic arch (blue arrow). 
Mentions: Subsequent contrast-enhanced computed tomography (CT) of the chest (Figures 1-5, Video 1) at our institution revealed left-sided pulmonary artery agenesis with an associated right-sided aortic arch and significant hypoplasia of the ipsilateral lung. Hyperinflation and expansion of the remaining right lung with evidence of emphysema and pulmonary artery hypertension were also observed. 

Bottom Line: Left-sided pulmonary artery agenesis is a rare malformation that commonly requires childhood intervention secondary to associated congenital cardiovascular anomalies.We present an uncommon case of left-sided agenesis with an associated right-sided aortic arch and significant hypoplasia of the ipsilateral lung.Additionally, there is radiographic evidence of emphysema and pulmonary artery hypertension.

View Article: PubMed Central - HTML - PubMed

Affiliation: Diagnostic Radiology, Florida Hospital-Orlando.

ABSTRACT
Left-sided pulmonary artery agenesis is a rare malformation that commonly requires childhood intervention secondary to associated congenital cardiovascular anomalies. We present an uncommon case of left-sided agenesis with an associated right-sided aortic arch and significant hypoplasia of the ipsilateral lung. Additionally, there is radiographic evidence of emphysema and pulmonary artery hypertension. Pulmonary artery agenesis is not a common entity, but should be considered in adult patients presenting with recurrent pneumonias and radiographic evidence suggestive of pulmonary hypoplasia. A prompt diagnosis is beneficial for affected individuals who may be candidates for a revascularization procedure or embolization of collaterals. Earlier diagnosis also allows for proper management and follow-up care, considering pulmonary artery hypertension is a severe complication of pulmonary artery agenesis.

No MeSH data available.


Related in: MedlinePlus