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A Rare Case of Pulmonary Atresia with Ventricular Septal Defect with a Right Sided Aortic Arch and a Calcified Pulmonary AVM Presenting in an Adult without Cyanosis.

Kulkarni DV, Hegde RG, Balani A, Joshi AR - Case Rep Radiol (2014)

Bottom Line: Pulmonary atresia with ventricular septal defect (PA-VSD) with pulmonary arterial supply arising from the aorta representing large MAPCAs associated with a right sided aortic arch is an uncommon anomaly.Most of the patients succumb to severe respiratory compromise or congestive cardiac failure very early.We report the clinical details and imaging findings of a case of PA-VSD with a right sided aortic arch and a calcified pulmonary arteriovenous malformation (AVM) in a 21-year-old postpartum female with no previous episodes of cyanosis who was diagnosed as having a cardiac anomaly on echocardiography when she presented with breathlessness during the 8th month of the pregnancy.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiology, Lokmanya Tilak Municipal Medical College and Lokmanya Tilak Municipal General Hospital, Sion, Mumbai 400022, India.

ABSTRACT
Pulmonary atresia with ventricular septal defect (PA-VSD) with pulmonary arterial supply arising from the aorta representing large MAPCAs associated with a right sided aortic arch is an uncommon anomaly. Most of the patients succumb to severe respiratory compromise or congestive cardiac failure very early. We report the clinical details and imaging findings of a case of PA-VSD with a right sided aortic arch and a calcified pulmonary arteriovenous malformation (AVM) in a 21-year-old postpartum female with no previous episodes of cyanosis who was diagnosed as having a cardiac anomaly on echocardiography when she presented with breathlessness during the 8th month of the pregnancy.

No MeSH data available.


Related in: MedlinePlus

Transthoracic echocardiography: 5-chamber view shows a large malaligned nonrestrictive subaortic VSD (thin arrow).
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fig2: Transthoracic echocardiography: 5-chamber view shows a large malaligned nonrestrictive subaortic VSD (thin arrow).

Mentions: Transthoracic 2D echo revealed a single large malaligned nonrestrictive ventricular septal defect (VSD) with right-to-left (R-L) shunt, >50% overriding of aorta and pulmonary atresia (Figures 2 and 3). Suprasternal echocardiography revealed a MAPCA arising from the aorta.


A Rare Case of Pulmonary Atresia with Ventricular Septal Defect with a Right Sided Aortic Arch and a Calcified Pulmonary AVM Presenting in an Adult without Cyanosis.

Kulkarni DV, Hegde RG, Balani A, Joshi AR - Case Rep Radiol (2014)

Transthoracic echocardiography: 5-chamber view shows a large malaligned nonrestrictive subaortic VSD (thin arrow).
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4279784&req=5

fig2: Transthoracic echocardiography: 5-chamber view shows a large malaligned nonrestrictive subaortic VSD (thin arrow).
Mentions: Transthoracic 2D echo revealed a single large malaligned nonrestrictive ventricular septal defect (VSD) with right-to-left (R-L) shunt, >50% overriding of aorta and pulmonary atresia (Figures 2 and 3). Suprasternal echocardiography revealed a MAPCA arising from the aorta.

Bottom Line: Pulmonary atresia with ventricular septal defect (PA-VSD) with pulmonary arterial supply arising from the aorta representing large MAPCAs associated with a right sided aortic arch is an uncommon anomaly.Most of the patients succumb to severe respiratory compromise or congestive cardiac failure very early.We report the clinical details and imaging findings of a case of PA-VSD with a right sided aortic arch and a calcified pulmonary arteriovenous malformation (AVM) in a 21-year-old postpartum female with no previous episodes of cyanosis who was diagnosed as having a cardiac anomaly on echocardiography when she presented with breathlessness during the 8th month of the pregnancy.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiology, Lokmanya Tilak Municipal Medical College and Lokmanya Tilak Municipal General Hospital, Sion, Mumbai 400022, India.

ABSTRACT
Pulmonary atresia with ventricular septal defect (PA-VSD) with pulmonary arterial supply arising from the aorta representing large MAPCAs associated with a right sided aortic arch is an uncommon anomaly. Most of the patients succumb to severe respiratory compromise or congestive cardiac failure very early. We report the clinical details and imaging findings of a case of PA-VSD with a right sided aortic arch and a calcified pulmonary arteriovenous malformation (AVM) in a 21-year-old postpartum female with no previous episodes of cyanosis who was diagnosed as having a cardiac anomaly on echocardiography when she presented with breathlessness during the 8th month of the pregnancy.

No MeSH data available.


Related in: MedlinePlus