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CASE REPORT
Year : 2023  |  Volume : 17  |  Issue : 1  |  Page : 101-103

Anesthesia management of living donor liver transplantation in a patient with scimitar syndrome


Department of Anesthesia, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia

Correspondence Address:
Muhammad Shabbir
King Faisal Specialist Hospital and Research Centre, MBC-72, P O Box 3354, Riyadh 11211
Saudi Arabia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/sja.sja_553_22

Rights and Permissions
Date of Submission28-Jul-2022
Date of Decision03-Aug-2022
Date of Acceptance05-Aug-2022
Date of Web Publication02-Jan-2023
 

  Abstract 


Scimitar syndrome is a rare congenital anomaly with a hallmark of an abnormal drainage of pulmonary veins into inferior vena cava instead of the left atrium; this creates a curvilinear radiological pattern resembling a sword (scimitar) on a chest radiograph, thus attracting the name. This case report highlights the challenges during liver transplantation, and perioperative anesthetic management of a patient with an uncorrected Scimitar Syndrome.

Keywords: Aberrant pulmonary veins, liver transplantation, Scimitar


How to cite this article:
Shabbir M, Majeed A, Baig MA, Shajar MA, Iqbal T. Anesthesia management of living donor liver transplantation in a patient with scimitar syndrome. Saudi J Anaesth 2023;17:101-3

How to cite this URL:
Shabbir M, Majeed A, Baig MA, Shajar MA, Iqbal T. Anesthesia management of living donor liver transplantation in a patient with scimitar syndrome. Saudi J Anaesth [serial online] 2023 [cited 2023 Feb 3];17:101-3. Available from: https://www.saudija.org/text.asp?2023/17/1/101/364873




  Introduction Top


Scimitar syndrome, designated by a curvilinear pattern on chest X-ray, is a rare condition with abnormal pulmonary veins draining a part or all of the right lung into the inferior vena cava (IVC), possibly accompanied by the pulmonary artery supplying the effected part of the lung arising from systemic arterial supply, and right lung hypoplasia.[1] It may have a circulatory impact resembling a significant left to right shunt and pulmonary hypertension. We would like to report the anesthetic management of an adult with uncorrected congenital Scimitar syndrome, presented for elective living donor liver transplant which has not been reported before.


  Case Report Top


A 69-year-old, 50 kg female, known case of hypertension, type 2 diabetes, atrial fibrillation (AF), and interval progression of hepatocellular carcinoma with liver cirrhosis, was scheduled for living donor liver transplantation (LDLT). Pre-operative assessment revealed no cardio-respiratory symptoms, except an irregularly irregular pulse (68 – 70 beats per minute). She was receiving Metoprolol 25 mg and Rivaroxiban daily.

Chest X-ray showed an incidental scimitar sign along with a smaller right lung shadow, and a marked displacement of the mediastinum to the right side [Figure 1]. Computerised tomography scan of chest and abdomen confirmed hypoplastic right lung with venous drainage to the IVC; arterial supply of the upper part was demonstrated from the pulmonary artery but in the lower part arose abnormally from the abdominal aorta superior to the celiac trunk. Cardiomegaly with enlarged pulmonary vessels and bicaval dilatation were also noted [Figure 2].
Figure 1: Chest X-ray showing Scimitar sign

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Figure 2: Aberrant circulation seen on CT scan. (a) pulmonary vein. (b) draining into inferior vena cava. (c) pulmonary artery arising from aorta

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Transthoracic echo revealed; mildly elevated right ventricular pressure (30-35 mm of Hg), moderate regurgitation through mitral and tricuspid valves and persistent patent foramen ovale (PFO), the left ventricular ejection fraction (LVEF) of 50-55%. Myocardial perfusion scan showed a normal LVEF (67%), and a small inferior wall defect of mild severity and partial reversibility with normal thickening on gated images which was considered to be an attenuation artifact as the left heart catheterization confirmed patent coronary arteries. The right heart catheterization revealed right atrial, ventricular, and pulmonary artery pressures within normal physiological range; the shunt fraction (Qp/Qs) was derived using pulmonary and arterial oxygenation as 1.2 and regarded to be mild.

Multi-disciplinary review noted cardio-thoracic advice against corrective surgery prior to improved liver function; liver transplantation was therefore scheduled with the impression that such improvement would not be otherwise achievable.

Anesthetic management goals were set to include: optimizing heart function with normal systemic blood pressure for the age; goal directed fluid therapy to avoid volume overload and pulmonary congestion; avoidance of shunt reversal – intra-cardiac by maintaining a meticulous balance in the pulmonary and systemic vascular resistance, and extra-cardiac by repeated comparative evaluation of mixed venous and systemic arterial blood gas analysis; and not the least the prevention of potential air embolism in the presence of PFO by strict removal of air bubbles from the IV lines, tubing, and stopcocks.

Anesthesia was induced with fentanyl 50 mcg, propofol 70 mg, and atracurium 50 mg, and maintained with sevoflurane 0.5 to 1.0 MAC along with oxygen in air and continuous infusions of fentanyl (0.5-2.0 mcg/kg/h), and atracurium (0.5 mg/kg/h); standard ASA monitoring instituted; additionally radial and femoral arterial lines and a pulmonary artery catheter continuously evaluating pulmonary artery pressure and core body temperature were sited. Point of care testing provided through frequent blood gas analysis and rotational thromboelastography (ROTEM).

During neohepatic phase, the de-clamping of IVC and Portal Vein were done slowly, to avoid hemodynamic instability. This was tolerated well with ongoing epinephrine (0.05 to 0.08 mcg/kg/min) and norepinephrine (0.05 to 0.2 mcg/kg/min) infusions. Epinephrine was weaned off within next 20 min. Central venous pressure (CVP) remained high, between 15 and 20 cm of H2O despite administering diuretics, octreotide infusion was started at 1 mcg/kg/hour, which helped maintain CVP value below 13 cm of H2O. Estimated blood loss was around 1000 milliliters. She received about 900 milliliters of packed red cells, and 2000 milliliters of albumin 5%.

The patient was transferred to intensive care unit (ICU) sedated, her lungs were ventilated, and octreotide and norepinephrine infusions continued. Sedation was stopped and her trachea was extubated uneventfully the next morning. She was discharged from ICU on the 4th post-op day.


  Discussion Top


The rare appearance of a scimitar (Turkish sword) sign on a chest X-ray requires further evaluation including echocardiography verifying the scimitar vein and associated cardiac alterations[2]; three-dimensional tomography or cardiac magnetic resonance imaging are fundamental for visualizing the anomalous pulmonary vein and clarifying the patient's anatomy.[3] Cardiac catheterization and angiography can confirm the diagnosis, giving detailed information about the patient's anatomy and the severity of pulmonary hypertension.

Three groups are based on reducing severity of clinical picture: neonatal, children (1-10 years old), and adults.[4] The infants are symptomatic soon after birth developing severe pulmonary hypertension and cardiac failure with high mortality[3],[5]; in adults, it usually is an incidental finding with mild symptoms and a benign prognosis and is not associated with pulmonary hypertension.

Treatment starts with the management of the pulmonary hypertension; surgery including lobectomies and interruption of the anomalous systemic flow through embolization or direct ligation is recommended in patients with persistent heart failure, recurrent infectious diseases, or refractory pulmonary hypertension.[6],[7]

Perioperative control of this extra-cardiac shunt is particularly difficult. During orthotropic liver transplantation in an uncorrected scimitar anomaly the IVC clamp can impede the pulmonary drainage producing pulmonary edema and exaggerating pulmonary hypertension; unclamping it after connecting the liver graft may produce excessive flow in the IVC and congestion of the graft.

In our patient, the additional challenge was to avoid excessive drop in pulmonary vascular resistance in the sections of the lung with regular circulation to avoid reversal of right-to-left shunt through PFO, not the least due to the presence of AF with the potential to reduce atrial clearance.

Our case report demonstrates that by establishing and adhering to clear anesthetic goals made it possible to safely anaesthetize this patient. The presentation of the milder disease certainly favored the good outcome; whether similar outcomes could be expected in the pediatric variety of scimitar syndrome remains unknown.

Declaration of patient consent

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

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Snellen HA, van Ingen HC, Hoefsmit EC. Patterns of anomalous pulmonary venous drainage. Circulation 1968;38:45-63.  Back to cited text no. 1
    
2.
Schramel FMNH, Westermann CJJ, Knaepen PJ, van den Bosch JMM. The scimitar syndrome: Clinical spectrum and surgical treatment. Eur Respir J 1995;8:1966-201.  Back to cited text no. 2
    
3.
Karthekeyan RB, Yachendra, Kumar SM, Rao S, Vakamudi M, Komarakshi B, et al. Pneumonectomy in scimitar syndrome – Is it correct? Ind J Thorac Cardiovasc Surg 2008;24:176-9.  Back to cited text no. 3
    
4.
Vida VL, Guariento A. A sword threatening the heart: The Scimitar Syndrome. JTCVS Tech 2020;1:75-80.  Back to cited text no. 4
    
5.
Korkmaz AA, Yildiz CE, Onan B, Guden M, Cetin G, Babaoglu K. Scimitar syndrome: A complex form of anomalous pulmonary venous return. J Card Surg 2011;26:529-34.  Back to cited text no. 5
    
6.
Çiçek S, Arslan AH, Ugurlucan M, Yildiz Y, Ay S. Scimitar syndrome: The curved Turkish sabre. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2014;17:56-61.  Back to cited text no. 6
    
7.
Elsallabi O, Smer A, De Vrieze B, Sirineni G. Scimitar syndrome. J Gen Intern Med 2016;31:253-4.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2]



 

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