|Year : 2022 | Volume
| Issue : 4 | Page : 227-229
Persistent left superior vena cava and ipsilateral intraventricular hemorrhage in a preterm infant
Adenike Karunwi1, Shabih Manzar2, Ryan Jones3
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 Submission||14-Jul-2022|
|Date of Decision||23-Jul-2022|
|Date of Acceptance||23-Jul-2022|
|Date of Web Publication||04-Oct-2022|
Louisiana State University Health Sciences Center, 1501 Kings Highway, Shreveport, LA 71103
Source of Support: None, Conflict of Interest: None
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|| |
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|| |
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|| |
PLSVC is a rare congenital anomaly. Chao et al. 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]. 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.
|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. 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. The possible mechanism for IVH noted in our case could be due to the critically altered superior vena cava flow. The rate of occurrence of unilateral bleed in premature babies is reported as 48.8%. 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]. 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. An increase in venous flow from the retrograde blood coming from the CS into the PLSVC has been shown. [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.) 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. 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.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]