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CASE REPORT
Ahead of print publication  

Persistent left superior vena cava: A case series in Kano, Northwest Nigeria


1 Department of Paediatrics, Federal Medical Centre Nguru, Nguru, Nigeria
2 Department of Paediatrics, Cardiopulmonary Unit, Aminu Kano Teaching Hospital, Kano, Nigeria
3 Department of Paediatrics, Aminu Kano Teaching Hospital, Bayero University, Kano, Nigeria

Date of Submission24-Apr-2020
Date of Decision29-Aug-2020
Date of Acceptance21-Sep-2020

Correspondence Address:
Nuhu Abubakar Garba,
Department of Paediatrics, Federal Medical Centre Nguru, Nguru
Nigeria
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/mjdrdypu.mjdrdypu_207_20

  Abstract 


Persistent left superior vena cava (PLSV) is an abnormality of drainage of the embryological left cardinal vein, persisting as left superior vena cava and commonly emptying into the right atrium through the coronary sinus. Other drainage patterns include draining into the left pulmonary vein or into the left atrium through its roof and sometimes through the coronary sinus when the defect exists between the coronary sinus and the left atrium. The aim of this report is to indicate the existence of PLSV in this environment and as one of the rarest abnormal venous connections limitedly reported in Nigeria. PLSV is a systemic venous drainage abnormality. Although it may be asymptomatic, it can also present with cardiac failure and severe life-threatening arrhythmias. Dilated coronary sinus during transthoracic echocardiography should be a pointer to a suspected PLSV.

Keywords: Coronary sinus, persistent left cardinal vein, persistent left superior vena cava



How to cite this URL:
Garba NA, Ahmadu I, Abubakar MS, Asani MO, Aliyu I. Persistent left superior vena cava: A case series in Kano, Northwest Nigeria. Med J DY Patil Vidyapeeth [Epub ahead of print] [cited 2021 Dec 6]. Available from: https://www.mjdrdypv.org/preprintarticle.asp?id=319297




  Introduction Top


Persistent left superior vena cava (PLSV) is an abnormality of systemic venous drainage, resulting from failure of regression of the left cardinal vein.[1],[2] PLSV has a prevalence of about 0.5% in general population and may be up to 10% in children with congenital anomalies.[1],[2],[3],[4] Embryonically, the right and left anterior cardinal veins through the right duct of Cuvier's drain into the right atrium through the sinus venosus.[5] The right cardinal vein together with the right duct of Cuvier eventually forms the superior vena cava, while the left cardinal vein degenerates.[5],[6] The left cardinal vein may persist as a left superior vena cava which drains anomalously within the cardiac venous network.[5] A different anatomical drainage pattern of PLSV has been described. However, the drainage through the coronary sinus is most prevalent.[1],[7],[8]

The aim of this report is to indicate the existence of PLSV as one of the rarest abnormal venous connections limitedly reported in Nigeria.


  Case Reports Top


Case 1

A 2-month-old female infant, who was delivered via a noneventful spontaneous vaginal delivery with a birth weight of 2.8 kg, presented with the complaints of fever and difficulty in breathing. She was predominantly breastfed and immunized according to the Nigerian immunization schedule. The mother claimed that she was growing well as compared to the elder sibling. She was the second child of the family in nonconsanguineous settings. Examination findings revealed that the child was in respiratory distress; she had axillary temperature of 37.9°C and was not cyanosed. She was dyspneic with subcostal and intercostal recessions, and the respiratory rate was 54/min. Saturation at room air was 84%, but it rose to 95%–98% following management. She had crepitation on the right axillary and subscapular region. The pulse rate was 148/min and the apex beat was displaced inferolaterally (5th intercostal space lateral to the midclavicular line). Heart sounds were first, and second; which were normal with a pan systolic murmur which was loudest at the left lower sternal edge. She had a good oral hygiene with a normal palate. She had a tender hepatomegaly of 4 cm below the right costal margin along the midaxillary line. The provisional diagnosis of bronchopneumonia in heart failure with background congenital heart disease (suspected ventricular septal defect [VSD]) was performed. Bedside hemoglobin concentration was 9.3 g/dl with white blood cells (WBC) of 9.0 × 103, granulocyte of 3.9 × 103, and lymphocytes of 4.6 × 103. Electrolytes, urea, and creatine were essentially normal (Na 143 mmol/l, K 4.2 mmol/l, HCO3 20 mmol/l, Cl 106 mmol/l, urea 2.3, and creatinine 42 mmol/l). The chest radiography showed cardiomegaly [Figure 1] while echocardiography diagnosed PLSV draining into the right atrium with unroofing of coronary sinus [Figure 2] and mild dilatation of the left atrium and normal right superior vena cava draining into the right atrium. There were perimembranous VSD measuring 3.5 mm in diameter and secundum atrial septal defect measuring 5 mm.
Figure 1: Chest radiography indicating cardiomegaly

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Figure 2: Plax view demonstrating dilated coronary sinus

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Case 2

A 3-year-old girl presented with complaints of fever, cough, and recurrent difficulty in breathing. She was immunized for age and had normal developmental milestone. The mother also noticed she was not gaining weight, despite adequate feeding. Examination revealed small-for-age girl, who was in respiratory distress, febrile (axillary temperature 38.0°C), and not cyanosed. The pulse rate was 128 bpm, it was regular with full volume; the blood pressure was 90/60 mmHg, measured from the right arm, while in the sitting position. The heart sounds were 1st and 2nd; were normal with grade three pan systolic murmur. Respiratory rate was 63 bpm, she was dyspneic (subcostal and intercostal recessions), saturation at room air was between 84% and 92%, and she had bilateral crepitation in the lung fields. She had a full soft abdomen with a tender hepatomegaly of 6 cm below the right costal margin. A diagnosis of bronchopneumonia with congestive heart failure secondary to congenital heart disease (VSD) was performed. She was placed on IV furosemide, oral spironolactone, and ceftriaxone. The laboratory investigations revealed WBC of 5.9 × 103/UL, lymphocytes count of 2.8 × 103, granulocyte count 2.7 × 103/μL, and HgB 11.1 g/dl. Electrolyte, urea, and creatinine showed: Na 126 mmol/l, K 4.4 mmol/l, HCO3 20 mmol/l, Cl 90 mmol/l, urea 3.9 mmol/l, and creatinine 43 mmol/l. The chest radiography revealed cardiomegaly and echocardiography revealed multiple moderate-sized secundum atrial septa defects and bilateral superior vena cava. The left superior vena cava was draining into the unroofing coronary sinus [Figure 3].
Figure 3: Subcostal view showing dilated coronary sinus

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All patients are currently been followed up at the pediatric cardiology clinic while awaiting surgery.


  Discussion Top


PLSV is an uncommon systemic venous drainage abnormality in children and adults. It is a congenital anomaly arising from failure of obliteration of the left anterior cardinal veins, which may persist on the left side draining into the atrium through different venous channels. The drainage is usually through the coronary sinus but could also drain anomalously to the left atrium[9] or left pulmonary vein.

We reported a case series of persistent superior vena cava draining into the right atrium through the unroofing coronary sinus in the presence of normally drained right superior vena cava into the right atrium.

The first case was associated with ventricular septal and secundum atria septal defects in keeping with findings documented in the literature.[3] PLSV in isolation may be asymptomatic, but the presence of ventricular septal and atrial septal defects could lead to heart failure as was observed in the first case, which may also be complicated by bronchopneumonia. Similarly, the second case also had bronchopneumonia with multiple secundum atrial septal defects which predisposed the child to heart failure.

Coronary sinus dilatation is a common finding in PLSV;[10] however, it could also be dilated in total anomalous pulmonary venous return, tricuspid atresia, or severe left ventricular hypertrophy, but these were ruled out following echocardiographic examinations. Normal saline bubble with the injection of contrast into the cubital veins in conjunction with echocardiography could also be used to diagnose PLSV. The injected saline contrast will pass through the coronary sinus before draining into the right atrium. Other important image modalities such as cardiac magnetic resonance imaging or computed tomography angiography are also important in diagnosing PLSV where available which further strengthens the diagnosis. Life-threatening atrial fibrillation, heart failure, and cerebral abscess have also been reported in adults with PLSV. Surgical intervention, especially in children with other associated congenital heart disease, has been reported to improved survival. The surgical technique involves anastomosis of PLSV to the right superior vena cava, and other associated cardiac defects could also be corrected.[11],[12]

Limitation

Genetic analysis was not done in these cases; the facility for karyotyping/DNA analysis is scare in most resource-limited settings.


  Conclusion Top


PLSV is an uncommon systemic venous drainage abnormality. Although it may be asymptomatic, it can also present with cardiac failure, especially when associated with other cardiac defects and chest infection. Dilated coronary sinus during transthoracic echocardiography should be a pointer to a suspected PLSV.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Snider AR, Server GA. Abnormal Vascular Connections and Structure. In: Snider AR, editor. 2nd ed.. New York, Philadelphia: Mosby; 1974. p. 453-4.  Back to cited text no. 1
    
2.
Kurtoglu E, Cakin O, Akcays S. Persistent left superior vena cave draining into the coronary sinus: A case report. Cardiol Res 2011;2:249-55.  Back to cited text no. 2
    
3.
Eldin GS, Els-Segaier M, Galalal MO. High prevalence of persistent superior vena cava determined by echocardiography in patients with congenital heart disease in Saudi Arabia. Libyan J Med 2013;2:21679-3.  Back to cited text no. 3
    
4.
Irwin RB, Greaves SM, Schimit M. Left superior vena cave: Revisited. Eur J Cardiovasc Imaging 2012;13:284-91.  Back to cited text no. 4
    
5.
Galindo FA, Gwiteirre L, Escribano D. Clinical significant of persistent superior vena cava diagnosed in fatal life. Ultrasound Obset Gyneacol 2007;30:152-61.  Back to cited text no. 5
    
6.
Lee MS, Pande RL, Rao B, Landzberg MJ, Kwong RY. Cerebral abscess due to persistent left superior vena cava draining into the left atrium. Circulation 2011;124:2362-4.  Back to cited text no. 6
    
7.
Boyer R, Sidhu R, Ghandforoush A, Win T, Heidari A. Persistent left superior vena cava: Implications of surgical management. J Investig Med High Impact Case Rep 2019;7:1-3.  Back to cited text no. 7
    
8.
Povoski SP, Khabiri H. Persistent left superior vena cava: Review of the literature, clinical implications, and relevance of alterations in thoracic central venous anatomy as pertaining to the general principles of central venous access device placement and venography in cancer patients. World J Surg Oncol 2011;9:174-84.  Back to cited text no. 8
    
9.
Alghamdi MH, Elfaki W, Al-Habshan F, Aljarallah AS. Bilateral superior vena cava with right superior vena cava draining into left atrium. J Saudi Heart Assoc 2015;27:123-6.  Back to cited text no. 9
    
10.
Tripathi SP, Nabar AA, Kerkar PG, Telkar HB, Udare AS. Bilateral superior venae cavae with crisscross atrial drainage. Circulation 2015;132:e365-8.  Back to cited text no. 10
    
11.
Kowar A, NaitoY, Fuwara K. Operative techniques for persistent left superior vena cava draining into the left atrium. Ann J Thorac Surg 1996;62:1185-90.  Back to cited text no. 11
    
12.
Ozcan EE, Szeplaki G, Merkely B, Geller L. Isolation of persistent left superior vena cava during atrial fibrillation ablation. Indian Pacing Electrophysiol J 2015;15:130-2.  Back to cited text no. 12
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

 
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