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Case Reports
. 2017 Jun;11(6):OD18-OD20.
doi: 10.7860/JCDR/2017/27645.10094. Epub 2017 Jun 1.

Large Isolated Major Aortopulmonary Collateral Artery Causing Severe Pulmonary Hypertension in an Infant: A Rare and Challenging Diagnosis

Brajesh Kumar Kunwar  1 , Snigdha Paddalwar  2 , Mahesh Ghogare  3
Affiliations
Case Reports

Large Isolated Major Aortopulmonary Collateral Artery Causing Severe Pulmonary Hypertension in an Infant: A Rare and Challenging Diagnosis

Brajesh Kumar Kunwar et al. J Clin Diagn Res. 2017 Jun.

Abstract

Major Aortopulmonary Collateral Artery (MAPCA) as an isolated congenital anomaly, without evidence of any structural heart disease, is a very rare observation. Previously published reports indicate that symptomatic infants with large isolated MAPCA usually present with congestive heart failure or recurrent respiratory tract infections. To the best of our knowledge, the present case of an infant with large isolated MAPCA is a unique case with a diagnostic dilemma due to presentation with severe pulmonary hypertension as a predominant sign. The infant was managed successfully by percutaneous obliteration with amplatzer vascular plugs, along with perioperative and postoperative sildenafil.

Keywords: Amplatzer vascular plug; Computed tomography angiogram; Sildenafil.

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Figures

[Table/Fig-1]:
[Table/Fig-1]:
Echocardiography showing severe tricuspid regurgitation (TR) and dilated right atrium (RA) and right ventricle (RV).
[Table/Fig-2]:
[Table/Fig-2]:
Echocardiography showing dilated confluent pulmonary artery with normal branching pattern - left pulmonary artery (LPA), main pulmonary artery (MPA) and right pulmonary artery (RPA).
[Table/Fig-3]:
[Table/Fig-3]:
CT angiogram showing normal branching left pulmonary artery (LPA), main pulmonary artery (MPA) and right pulmonary artery (RPA).
[Table/Fig-4]:
[Table/Fig-4]:
CT angiogram showing a large major aortopulmonary collateral artery (MAPCA), arising from the descending thoracic aorta and supplying blood to upper and middle lobe of right lung.
[Table/Fig-5]:
[Table/Fig-5]:
Aortogram image in cathlab showing large isolated major aortopulmonary collateral artery (MAPCA) arising from descending thoracic aorta.
[Table/Fig-6]:
[Table/Fig-6]:
Successful delivery of (A) Proximal vascular plug of 8 mm size and (B) Distal vascular plug of 6 mm size.
[Table/Fig-7]:
[Table/Fig-7]:
Vascular plugs in-situ (A) Proximal vascular plug (post-release), (B) Distal vascular plug (post-release).
[Table/Fig-8]:
[Table/Fig-8]:
Follow-up echocardiography showing significant reduction in right atrium and right ventricle sizes and subsystemic pulmonary artery pressures.
See this image and copyright information in PMC

References

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