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2013-04-01-09 Case of the week #348 © Chowdhury www.TheFetus.net 

Answer to the case of the week #348

July 04, 2013  - July 18, 2013 

Mayank Chowdhury, MD.

Mayflower Women's Hospital, Mehsana 384002, Pallav Imaging Institute, Ahmedabad, India.



Case report

This was a low risk pregnancy of a woman from a non-consanguineous couple. She was referred to our unit at 23rd week of gestation due to fetal cardiomegaly. The patient suffered from retinitis pigmentosa and her husband was partially blind. No other singularities were found in her anamnesis. 

During our ultrasound examination we found cardiomegaly with suspected abnormal connection leading from the left branch of the pulmonary artery to the left atrium. In our differential diagnostic process fistula of ductus arteriosus into the left atrium was also speculated. The most prominent finding was turbulent bidirectional flow going from the left side to the left atrium. Both outflow tracts of the heart were dilated with considerable left branch of the pulmonary artery.
 
The patient had delivered at term and the neonate manifested with low oxygen saturation. Cardiomegaly was present and postnatal examination also raised suspicion of a fistula leading to the left atrium. The final diagnosis of the left pulmonary artery to left atrium fistula (PALAF) (see drawing 1) was postulated during closed-heart surgery with placement of a cardiac implant closure device into the fistula. Postoperative course was normal; oxygen saturation returned to normal values immediately after the surgery and the child is doing well.

Editor's comment: Although fistulas between the right pulmonary artery and the left ventricle were described in the literature several times, we were able to find just one article describing the variant of the fistula between the left branch of the pulmonary artery and left atrium - Karnik et al., 1989 [1].

Drawing 1: Schematic drawing showing the anomaly presenting in the COW - fistula between the left pulmonary artery and left atrium.



Images 1, 2: 23rd week of gestation; the images show 4-chamber-view of the heart with cardiomegaly (RA - right atrium, RV - right ventricle, LV - left ventricle, LA - left atrium).

 
 
Images 3, 4: 23rd week of gestation; the images show color Doppler 4-chamber-view of the heart with bidirectional flow at the level of the left atrium - the red flow towards the left atrium represents the flow within the fistula between the left pulmonary artery (its portion is depicted as the blue part of the bidirectional flow) and the left atrium. (RA - right atrium, RV - right ventricle, LV - left ventricle, LA - left atrium).

 
 
Images 5, 6: 23rd week of gestation; the images show color Doppler 4-chamber-view of the heart with red flow towards the left atrium representing the fistula between the left pulmonary artery and the left atrium. (RA - right atrium, RV - right ventricle, LV - left ventricle, LA - left atrium).

 
 
Images 5, 6, 7, 8, and 9: 23rd week of gestation; the images show color Doppler 4-chamber-view of the heart with red flow towards the left atrium representing the fistula between the left pulmonary artery and the left atrium. (RA - right atrium, RV - right ventricle, LV - left ventricle, LA - left atrium).

 

 


 
Images 10, 11: 23rd week of gestation; the images show color Doppler 3-vessel-view of the heart with marked left pulmonary artery branch (PA - pulmonary artery, Ao - aorta, VCS - vena cava superior).

  

Image 12: 23rd week of gestation; the image shows 3-vessel-view of the heart with dilated pulmonary artery and aorta.


 
Videos 1, 2, 3, 4, and 523rd week of gestation; the video 1 shows color Doppler view of the heart with bidirectional flow at the level of the left atrium - the red flow towards the left atrium represents the flow within the fistula between the left pulmonary artery and the left atrium. The video 2 shows dilated outflow tracts of the heart with marked left branch of the pulmonary artery. The videos 3 and 4 show cardiomegaly and dilated outflow tracts of the heart. The video 5 shows color Doppler tomographic ultrasound imaging of the heart with bidirectional flow at the level of the left atrium representing the fistula between the left pulmonary artery and left atrium. 

 

 



References

1. Karnik AM, Nilsson U, Vijayaraghavan G, Hashmi J, Shuhaiber H. Direct communication between the left pulmonary artery and the left atrium. Chest. 1989 Oct;96(4):937-9.

2. Margaryan R, Arcieri L, Cantinotti M, Murzi B.: Surgical closure of big pulmonary artery-left atrial fistula. Interact Cardiovasc Thorac Surg. 2010 Jan;10(1):113-4. doi: 10.1510/icvts.2009.215350. Epub 2009 Oct 12


3. Camilleri L, Gabrillargues J, Lemaire JJ, Legault B, Brazzalotto I, Bailly P, Lusson JR, de Riberolles C.:  Congenital pulmonary arteriovenous fistula. Apropos of 2 cases. Arch Mal Coeur Vaiss. 1995 May;88(5):767-70.

4. Wojtalik M, Moll J, Szkutnik M, Frycz M, Religa Z, Zembala M, Grzybowski A, Kucewicz E, Goldstein L, Przybylski R, et al.  A case of direct right pulmonary artery-left atrium fistula. Kardiol Pol. 1992 Nov;37(11):317-9.

5. Lemoine G, Thibert M, Leca F, Rolland JC, Allegraud P, Temkine J.  Right pulmonary artery-left atrial fistula. Review of the literature apropos of a case. Arch Mal Coeur Vaiss. 1979 May;72(5):552-8.

 
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