Patients  who  were  born  with  single  ventricle  and  had  undergone  surgical  correction  using  bidirectional  Glenn  shunt  and  Fontan  surgery, are  prone  to  develop  abnormal  systemic  venous  collaterals  due  to  high  pressure  circulation  which  can  lead  to  hypoxia  and  cyanosis  in  these  patients.^(1,2)

Recently most of such abnormal pathologies are treated by minimal interventional procedures (percutaneous catheterizations).

Case Report

Fourteen years old male with situs inversus, dextocardia, single ventricle, Patent Ductus Arteriosus  (PDA), transposition of great vessels (TGV), bilateral superior vena cavae (SVC) without  Innominate  vein,  pulmonary  stenosis  and  common  atrium.  during  first  year  of  life  the patient complained  of  cyanosis,  hypoxia  and  effort  intolerance.

At  age  of  2  years  he  underwent  bilateral  Bidirectional  Glenn  shunt,  on  follow  up  during  the following  2  years  the  patient  was  doing  well  and  the  Glenn  shunt  was  functioning  well.

Two  years  later  the  patient  underwent  completion  of  TCPC  (Total  cavopulmonary  connection) with  Right  Atrium  to left  pulmonary  artery  (LPA)  shunting.  The  patient  then  was  discharged,  On  follow  up  5  years  later, TCPC  was  found  functioning  well  but  he  has  aortic  valve  (AV)  valve  regurgitation  grade  I, despite  this  the  kid  had  grown  up  normally  and  didn’t  complain  of  hypoxia  and  investigations  didn’t  show  desaturation.

After  8  years  from  last  operation, the  patient   started  complaining  of  cyanosis  and  hypoxia (O₂  saturation around 80-8 % at rest and 70% on exercise), cardiac catheterization showed multiple hepato-venous collaterals  (Fig. 1, 2).

Fig.1: Catheterization angiogram,  shown inferior  vena  cava   and  collateral  in  the  left  sided  liver  tissue.

Fig. 2: Cathaterizaion  angiogram, shown  the  progression  of  the  die  from  the  intrahepatic collateral  to  confluent  one  above  the  diaphragm  to  the  left  atrium.

Patient underwent trial of Transcatheter embolisation to close these collaterals but the trial failed, so the patient had surgery.

Surgical procedure was done on beating heart off pump, with lower median sternotomy incision (5cm) (Fig. 3), opened by layers and adhesions were released, surgeon start dissection between the diaphragm and the heart until he could identified the collateral which was large in size (2cm), then the collateral was ligated (Fig. 4) and during next few seconds the oxygen saturation was rising from 80% to 96% (Fig. 5) and (Fig. 6).   The duration of the procedure was 87 minutes (Fig. 7).

Patient was discharged on the 4^th day post operation with no cyanosis and oxygen saturation 96%.

Fig. 3: Lower small  median  sternotomy  incision

Fig. 4: Collateral surrounded by the band before ligation

Fig. 5: Saturation 80% before ligation the collateral

Fig. 6: Saturation 96% after ligation the collateral

Fig. 7: End of operation

Discussion

This case is considered rare for several reasons, first of all the type of collateral which developed after bidirectional glenn shunt and total cavopulmonary connection is rare comparing to other types.  According to previous reports the common collaterals are abnormal superior vena caval connection and brachiocephalic vein followed by the left phrenic vein,⁽³⁾ usually these collaterals happen due to  increase in central venous pressure⁽⁴⁾ and such collaterals lead to systemic hypoxia but usually these  collaterals develop in early stages after the first surgery, but with our patient the late manifestation is  considered a rare occurence too.

The second reason is the method of treatment, as we mentioned above Transcatheter embolisation is  the method of choice but in some cases when it fails or there is contraindication surgery will be the  solution, the usual surgical procedure is done with on pump,⁽⁵⁾  but here we used small incision and  ligated the collateral with beating heart.

Finally, the result is satisfactory and follow up is recommended to identify any new collaterals or recanalization.

References

1. Marwah A, Khatri S.  Unusual systemic venous collateral channels to left atrium causing desaturation after fontan operation closed percutaneously.  Ann Pediatr Cardiol 2013; 6(2): 191-193.

2.Stümper O, Wright JG.  Late systemic desaturation after total cavopulmonary shunt operations.   Br Heart J 1995 Sep; 74(3): 282-286.

3.Gatzoulis MA, Shinebourne EA. Increasing cyanosis early after cavopulmonary connection caused by abnormal systemic venous channels.  Br Heart J 1995 Feb; 73(2): 182-186.

4.Usta E, Schneidera W.  Late desaturation due to collateral veins 10 years after total cavopulmonary shunt in left  atrial isomerism: surgical closure.  Published by European Association for Cardio-Thoracic Surgery 2007

5.Borches D, Brochet G.  Severe cyanosis after total cavopulmonary connection, corrected by surgical ligation of the suprahepatic veins.  Rev Esp Cardiol 2004; 57(4): 367-369.