Limits...
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

(a) and (b) Coronal CT pulmonary angiography: MIP (maximum intensity projection) images reveal a right sided aortic arch with large arterial channels arising from the left lateral aspect of the descending thoracic aorta (thick arrows) representing MAPCAs supplying the pulmonary parenchyma. Also seen is a calcified lesion (thin arrow) in the lower lobe of the right lung with a vascular pedicle (arrow head).
© Copyright Policy - open-access
Related In: Results  -  Collection


getmorefigures.php?uid=PMC4279784&req=5

fig4: (a) and (b) Coronal CT pulmonary angiography: MIP (maximum intensity projection) images reveal a right sided aortic arch with large arterial channels arising from the left lateral aspect of the descending thoracic aorta (thick arrows) representing MAPCAs supplying the pulmonary parenchyma. Also seen is a calcified lesion (thin arrow) in the lower lobe of the right lung with a vascular pedicle (arrow head).

Mentions: CTPA revealed absent main pulmonary artery, right pulmonary artery, and left pulmonary artery with nonvisualisation of the right ventricular outflow tract indicating pulmonary atresia. Large arterial channels representing MAPCAs (2 on right and 1 on left) were seen arising from the aorta (one arterial channel arising from the descending thoracic aorta supplying blood to the RUL, one arterial channel seen arising from the arch of aorta supplying blood to the RML and RLL, and another large tortuous arterial channel arising from the descending thoracic aorta supplying the LUL and LLL) (Figures 4 and 6). Also seen was a large anterior 2.4-centimetre sized subaortic VSD, overriding of aorta, and right ventricular hypertrophy confirming the echocardiography findings (Figure 5). The absence of the native pulmonary vascular channels with MAPCAs supplying the lung directly indicates a type C PA-VSD.


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)

(a) and (b) Coronal CT pulmonary angiography: MIP (maximum intensity projection) images reveal a right sided aortic arch with large arterial channels arising from the left lateral aspect of the descending thoracic aorta (thick arrows) representing MAPCAs supplying the pulmonary parenchyma. Also seen is a calcified lesion (thin arrow) in the lower lobe of the right lung with a vascular pedicle (arrow head).
© Copyright Policy - open-access
Related In: Results  -  Collection

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

fig4: (a) and (b) Coronal CT pulmonary angiography: MIP (maximum intensity projection) images reveal a right sided aortic arch with large arterial channels arising from the left lateral aspect of the descending thoracic aorta (thick arrows) representing MAPCAs supplying the pulmonary parenchyma. Also seen is a calcified lesion (thin arrow) in the lower lobe of the right lung with a vascular pedicle (arrow head).
Mentions: CTPA revealed absent main pulmonary artery, right pulmonary artery, and left pulmonary artery with nonvisualisation of the right ventricular outflow tract indicating pulmonary atresia. Large arterial channels representing MAPCAs (2 on right and 1 on left) were seen arising from the aorta (one arterial channel arising from the descending thoracic aorta supplying blood to the RUL, one arterial channel seen arising from the arch of aorta supplying blood to the RML and RLL, and another large tortuous arterial channel arising from the descending thoracic aorta supplying the LUL and LLL) (Figures 4 and 6). Also seen was a large anterior 2.4-centimetre sized subaortic VSD, overriding of aorta, and right ventricular hypertrophy confirming the echocardiography findings (Figure 5). The absence of the native pulmonary vascular channels with MAPCAs supplying the lung directly indicates a type C PA-VSD.

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