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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 11  |  Issue : 4  |  Page : 227-229

Persistent left superior vena cava and ipsilateral intraventricular hemorrhage in a preterm infant


1 Department of Pediatrics, School of Medicine, Louisiana State University Health Sciences Center, Shreveport, LA 71103, USA
2 Division of Neonatology, Louisiana State University Health Sciences Center, Shreveport, LA 71103, USA
3 Division of Cardiology, Louisiana State University Health Sciences Center, Shreveport, LA 71103, USA

Date of Submission14-Jul-2022
Date of Decision23-Jul-2022
Date of Acceptance23-Jul-2022
Date of Web Publication04-Oct-2022

Correspondence Address:
Shabih Manzar
Louisiana State University Health Sciences Center, 1501 Kings Highway, Shreveport, LA 71103
USA
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jcn.jcn_78_22

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  Abstract 


We describe a preterm infant with a peripherally inserted central catheter (PICC) placement. The PICC line was suspected to be in the persistent left superior vena cava (PLSVC), confirmed by echocardiogram. PLSVC is a rare anomaly. On routine screening, the infant was noted to have an ipsilateral intraventricular hemorrhage (IVH) postulated to be secondary to the altered retrograde blood flow through the PLSVC. No such association has been reported previously. The case presentation is followed by evidence supporting the postulated mechanism. Although the association between PLSVC and IVH could be coincidental, clinicians should be aware of the blood flow-related problem associated with PLSVC in preterm infants.

Keywords: Intraventricular hemorrhage, peripherally inserted central catheter, persistent left superior vena cava, preterm infant


How to cite this article:
Karunwi A, Manzar S, Jones R. Persistent left superior vena cava and ipsilateral intraventricular hemorrhage in a preterm infant. J Clin Neonatol 2022;11:227-9

How to cite this URL:
Karunwi A, Manzar S, Jones R. Persistent left superior vena cava and ipsilateral intraventricular hemorrhage in a preterm infant. J Clin Neonatol [serial online] 2022 [cited 2022 Dec 9];11:227-9. Available from: https://www.jcnonweb.com/text.asp?2022/11/4/227/357822




  Introduction Top


Persistent left superior vena cava (PLSVC) is a rare congenital anomaly. PLSVC has been associated with stroke in adults. We describe a neonate with PLSVC associated with ipsilateral intraventricular hemorrhage (IVH) postulated due to the altered SVC blood flow.


  Case Report Top


The infant was delivered through cesarean section at 23 1/7 weeks of gestation. The birth weight was 540 g. She was placed on a mechanical ventilator and started on total parental nutrition through an umbilical venous catheter. On day 11, a peripherally inserted central catheter (PICC) was placed on the left upper limb. The PICC line had taken an abnormal route suggesting persistent left superior vena cava (PLSVC) [Figure 1]a and [Figure 1]b. An echocardiogram was obtained that showed dilated CS with the tip of PICC in situ, confirming PLSVC [Figure 2]. The infant had an incidental finding of ipsilateral grade 2 intraventricular hemorrhage (IVH) [Figure 3]. The infant was followed up closely. She did not develop any significant hydrocephalus and was discharged home with follow-up in the pediatric neurosurgery and developmental clinic.
Figure 1: (a) Chest X-ray (anterior-posterior view) showing peripherally inserted central catheter (open white arrow) in the PLSVC. (b) Chest X-ray (cross-table lateral view) showing peripherally inserted central catheter (open white arrow) in the persistent left superior vena cava. PLSVC: Persistent left superior vena cava

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Figure 2: Echocardiogram in parasternal long-axis (PLAX) view in systole showing DCS with the catheter (white arrow) inside the coronary sinus. Mv: Mitral valve, DCS: Dilated coronary sinus, PLAX: Parasternal long axis

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Figure 3: Head ultrasound shows a 7.7-mm bleed in the left ventricle (open white arrow)

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  Discussion Top


PLSVC is a rare congenital anomaly. Chao et al.[1] assessed 19,488 neonates and found 56 to have PLSVC with an incidence of 0.29%. PLSVC is an embryological developmental defect that occurs roughly at 8 weeks of antenatal life [Figure 4].[2] In 92% of the cases, PLSVC drains into the right atrium (RA) through the coronary sinus (CS) and does not present with clinical manifestations, but its role in extremely premature infants has not been evaluated.[3]
Figure 4: Drawing showing the developmental stages of primitive venous system and embryology of normal SVC (right side) and persistent LSVC (left side), adapted from Azizova et al.[2] SVC: Superior vena cava, LSVC: Left superior vena cava, IVC: Inferior vena cava, AzV: Azygos vein, RSVC: Right superior vena cava, PLSVC: Persistent left superior vena cava

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PLSVC has been associated with stroke.[4] The possible mechanism for IVH noted in our case could be due to the critically altered superior vena cava flow.[5] The rate of occurrence of unilateral bleed in premature babies is reported as 48.8%.[6] Among many factors associated with IVH, elevated venous pressure is one of the contributing factors. The left-sided subependymal germinal matrix drains into the left internal cerebral vein, which normally drains into the SVC through the left brachiocephalic trunk [Figure 5].[7] However, with PLSVC, the blood from the left-sided subependymal germinal matrix flows directly into the PLSVC, which in our case was noted to be draining into the RA through the CS. In adults, during atrial systole, the retrograde blood flow through CS and PLSVC may not produce any symptoms. Still, a minimal fluctuation in the blood flow dynamics in extremely premature infants could be detrimental. The back pressure and flow could aggravate venous pressure and accentuate IVH. Recently, such an association of reversed internal cerebral vein flow and IVH has been reported.[8] An increase in venous flow from the retrograde blood coming from the CS into the PLSVC has been shown.[9] [Figure 5] shows the direction of retrograde blood flow.
Figure 5: Drawing showing the retrograde flow through the coronary sinus into PLSVC (adapted from Gerlis et al.[9]) and blood flow pattern in normal and left-sided superior vena cava. PLSVC: Persistent left superior vena cava

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As stated earlier, PLSVC usually does not present with clinical manifestations and could be an incidental finding. Recently, a case report showed PLSVC in a 79-year-old man not diagnosed earlier.[10] However, if noted in preterm infants, a close follow-up is advised. Although the association between PLSVC and IVH observed in our case could be coincidental, clinicians should be aware of the blood flow-related problem associated with PLSVC in preterm infants.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Chao YJ, Chen MR, Hung WL, Yeh SJ, Lin SM. Impact of persistent left superior vena cava detection in a normal neonatal population through echocardiography. Acta Cardiol Sin 2019;35:600-4.  Back to cited text no. 1
    
2.
Azizova A, Onder O, Arslan S, Ardali S, Hazirolan T. Persistent left superior vena cava: Clinical importance and differential diagnoses. Insights Imaging 2020;11:110.  Back to cited text no. 2
    
3.
Subramanian N, Sridhar A. Systemic venous anomalies: Echocardiographic evaluation. J Indian Acad Echocardiogr Cardiovasc Imaging 2020;4:344-9.  Back to cited text no. 3
  [Full text]  
4.
Park CJ, Upadhya B, Pu M, Zhou L. Diagnosis of persistent left superior vena cava: Lessons learned from a recurrent stroke case. CASE (Phila) 2020;4:320-3.  Back to cited text no. 4
    
5.
Kluckow M, Evans N. Low superior vena cava flow and intraventricular haemorrhage in preterm infants. Arch Dis Child Fetal Neonatal Ed 2000;82:F188-94.  Back to cited text no. 5
    
6.
Stam J. Thrombosis of the cerebral veins and sinuses. N Engl J Med 2005;352:1791-8.  Back to cited text no. 6
    
7.
Merhar SL, Tabangin ME, Meinzen-Derr J, Schibler KR. Grade and laterality of intraventricular haemorrhage to predict 18-22 month neurodevelopmental outcomes in extremely low birthweight infants. Acta Paediatr 2012;101:414-8.  Back to cited text no. 7
    
8.
Tanaka K, Sakamoto R, Imamura H, Naramura T, Matsumoto S, Iwai M, et al. Reversal of blood flow in deep cerebral vein in preterm intraventricular hemorrhage: two case reports. BMC Pediatr 2020;20:517.  Back to cited text no. 8
    
9.
Gerlis LM, Gibbs JL, Williams GJ, Thomas GD. Coronary sinus orifice atresia and persistent left superior vena cava. A report of two cases, one associated with atypical coronary artery thrombosis. Br Heart J 1984;52:648-53.  Back to cited text no. 9
    
10.
Tang PT, Chan K. Isolated persistent left superior vena cava. N Engl J Med 2022;386:879.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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