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 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 53  |  Issue : 5  |  Page : 191-194

Surgical treatment of Stanford type A dissection for a patient with situs inversus


1 Department of Cardiovascular Surgery, School of Medicine, Taipei Medical University; Division of Cardiovascular Surgery, Department of Surgery, Taipei Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, Taipei; Department of Ophthalmology, Chang Gung Memorial Hospital, Linkou, Taiwan
2 Division of Cardiovascular Surgery, Department of Surgery, Taipei Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, Taipei; Department of Cardiovascular Surgery, School of Medicine, Tzu Chi University, Hualien, Taiwan

Date of Submission08-Jan-2019
Date of Decision16-May-2019
Date of Acceptance13-May-2020
Date of Web Publication19-Oct-2020

Correspondence Address:
Kuei-Ton Tsai
Division of Cardiovascular Surgery, Department of Surgery, Taipei Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, No. 289, Jianguo Road, Xindian District, New Taipei City, 231
Taiwan
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/fjs.fjs_4_19

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  Abstract 


Situs inversus totalis represents a positional anomaly of fetal development, in which the heart is right sided with inverted atria, along with mirror-imaged malposition of other visceral organs. Situs inversus totalis is frequently associated with concurrent cardiac anomaly of the patient. Therefore, in the event of life-threatening conditions such as acute aortic dissection, effective surgical planning, and optimal management are crucial to survival. We report the rare case of a 66-year-old male with situs inversus totalis and type A aortic dissection who, sustaining abdominal visceral ischemia and paraplegia at the initial presentation, had then undergone successful aortic graft reconstruction. Besides, a brief review of literature is presented with regard to situs inversus and aortic dissection.

Keywords: Aortic dissection, cardiovascular surgery, situs inversus


How to cite this article:
Ling XC, Tsai KT. Surgical treatment of Stanford type A dissection for a patient with situs inversus. Formos J Surg 2020;53:191-4

How to cite this URL:
Ling XC, Tsai KT. Surgical treatment of Stanford type A dissection for a patient with situs inversus. Formos J Surg [serial online] 2020 [cited 2020 Nov 24];53:191-4. Available from: https://www.e-fjs.org/text.asp?2020/53/5/191/298502




  Introduction Top


Situs inversus totalis is a congenital positional anomaly, in which the heart is right-sided with inverted atria, along with mirror-imaged malposition of other viscera. The incidence of situs inversus totalis ranges from 1/10,000 to 1/20,000. Pathophysiology includes malrotation during the bending of the primitive cardiac tube in forming the heart during fetal development. Type A aortic dissection, complicated with situs inversus totalis, is exceedingly rare and difficult to manage. We describe the case of a 66-year-old male with acute type A aortic dissection and situs inversus totalis, who already had abdominal visceral ischemia and paraplegia due to extreme lower body malperfusion at the initial dissection presentation.


  Case Report Top


The institutional review board of Taipei Tzu Chi Hospital approved this study (IRB No. 07-CR-025). A 66-year-old male with uncontrolled hypertension experienced acute chest pain with radiation to back for hours before presenting at our emergency department. Upon arriving, he remained oriented, yet hypotensive. The accompanying symptoms included cold sweating, right arm numbness, and loss of bilateral low limbs muscle power with absent femoral pulses. Vital signs were as follows: body temperature 36.6°C, blood pressure 80/64 mmHg, and pulse rate 110/min. White blood cell count, prothrombin time, activated partial thromboplastin time, and serum hemoglobin level are within the normal ranges. Venous blood gas analysis indicated metabolic acidosis with respiratory acidosis status (pH: 7.159, pCO2: 62.5 mmHg, pO2: 21.2 mmHg, HCO3: 21.7 mmol/L, TCO2: 23.6 mmol/L, ABE: −10.8 mmol/L, BEecf: −7.0 mmol/L, SBC 17.7 mmol/L, O2 sat.: 20.8%) at the emergency department. Chest X-ray showed dextrocardia, right-sided aortic arch, and visceral situs inversus [Figure 1]. Computed tomographic (CT) angiography revealed situs inversus totalis with Stanford type A aortic dissection and presence of hemopericardium. The dissected aortic intima flap was extending into right common carotid, subclavian arteries with extreme compression of thoracic aortic true lumen, resulting in vague opacification of celiac and superior mesenteric arteries. There was no further contrast enhancement of the aorta below superior mesenteric artery level [Figure 2]a.
Figure 1: Whole body plain X-ray revealing dextrocardia, right-sided aortic arch, visceral situs inversus with left-sided liver

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Figure 2: (a) Reconstructed images before operation showing right-sided aortic arch, mirror-imaged sequence of supra-aortic branches, and extreme thoracic aortic true lumen compression with no further contrast enhancement of aorta below superior mesenteric artery level, suggesting lower body malperfusion (*The bulging picture inside the distal aortic arch appearing to be the undeleted right pulmonary artery during image-reformatting). (b) Reconstructed computed tomographic angiography before discharge showing proximal aortic graft replacement, residual type B aortic dissection without aneurismal dilatation, patent aortic arch branches, good enhancement of visceral arteries, and smooth continuity of bilateral iliofemoral arteries

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Cardiovascular surgeon was consulted, and emergent salvage operation was performed due to impending tamponade. Median sternotomy was performed, and pericardium was incised open first for tamponade relief. The three branches of the aortic arch were completely reversed in mirror image, which in the sequence was left brachiocephalic (innominate) artery, right common carotid artery, and right subclavian artery [Figure 3]. Besides, the apex of the heart was on the right side with the superior and inferior vena cavae located on the left side. Extracorporeal circulation was then established through bi-arterial cannulation of left axillary and femoral artery grafts (8 mm Impra Carboflo Vascular Graft; Bard Peripheral Vascular Ins., Tempe, AZ, USA) and venous cannulation of left-sided morphological right atrium. The systemic temperature was cooled down, and right-sided systemic ventricle was vented through left superior pulmonary vein. Under deep hypothermia, the systemic flow was stopped, and the brain was continuously perfused via left axillary artery with clamping of left innominate and right common carotid branches. The heart was protected by retrograde delivery of cold Histidine-Tryptophan-Ketoglutarate cardioplegic solution. The dissected ascending aorta was incised open and meticulously examined. We discovered a complex intima tear, which started just before left innominate artery takeoff and circled spirally down to the aortic arch between left innominate artery and right common carotid artery. The dissected supracoronary ascending aorta and proximal arch, including the whole primary intima tear, were excised and replaced with a straight segment of 28 mm collagen-impregnated aortic woven graft (Intergard; Intervascular, La Ciotat Cedex, France). The transected left innominate artery, extended by an interposition graft, was then anatomically reimplanted onto the reconstructed aortic graft. With the proximal end of the aortic graft clamped and declamp of arch braches, the systemic circulation was restarted through perfusion of the left axillary artery graft. The body temperature was rewarmed. The incompetent aortic valve was excised and replaced with a 25-mm stented bioprosthesis (Epic Supra Aortic Valve; St. Jude Medical, St. Paul, MN, USA), followed by the completion of proximal aortic anastomosis. The surgeon at the left side of the operating table performed most of the main procedures. The cardiopulmonary bypass data were as follows: deep hypothermic temperature 20°C, unilateral antegrade cerebral perfusion time (low body arrest time) 80 min, cardiac ischemic time 171 min, total bypass time 315 min.
Figure 3: Intraoperative photograph revealing that the three branches of the aortic arch were completely reversed in mirror image, which in sequence were left brachiocephalic (innominate) artery (i), right common carotid artery (II), and right subclavian artery

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The patient regained his consciousness soon after surgery, yet he had a stormy postoperative course mostly from lower body malperfusion involving multiple organs before operation. Postoperative events included re-sternotomy for the evacuation of retained mediastinal hematoma due to coagulopathy, hemodialysis for acute renal failure, prolonged fasting, parenteral nutrition and strong antibiotics for sequelae from abdominal visceral ischemia, paraplegia and long-term ventilator dependence due to high-level thoracic spinal cord infarction involving the function of respiratory muscles. Finally, he had bowel and renal function recovery in the subsequent weeks and was weaned off ventilator therapy 2 months later. He stayed in the hospital for another 2 months for respiratory and paraplegia rehabilitation. Spinal magnetic resonance imaging demonstrated T2 signal change over lower thoracic spinal cord consistent with cord ischemic infarct. Nerve conduction velocity confirmed no nerve response in the lower limbs. Computed tomographic angiography before discharge depicted status-postproximal aortic graft replacement, residual type B aortic dissection without aneurismal dilatation, patent aortic arch branches, good visualization of visceral arteries, and smooth continuity of bilateral iliofemoral arteries [Figure 2]b. Currently, he is well oriented and is being followed up at outpatient clinic with strict antihypertensive regimen, as well as actively participating in paraplegia rehabilitation program.


  Discussion Top


Dextrocardia is defined as a positional abnormality in which the apex of the heart is directed toward the right side of the thorax. The reported incidence for dextrocardia was 0.02%.[1] During the period of fetal development, the cardiac tube serves as a primitive heart, which then begins to evolve into normal configuration and starts to bend to form a loop. For situs inversus or dextrocardia, the heart apex usually starts out in the left hemithorax but travels to the right hemithorax. Dextrocardia or situs inversus totalis was frequently associated with concurrent cardiac anomaly.[2]

In terms of management, aortic dissection in situ s inversus totalis is extremely rare. There was one reported case by Magishi et al. whereby an explosion accident caused an acute aortic dissection of Stanford type A in a 57-year-old male with situs inversus totalis.[3] Niino et al. reported another case of a 33-year-old male with acute aortic dissection Stanford type A with uneventful postoperative course.[4] Regarding our patient, the surgical technique adopted might not be different from other Stanford type A aortic dissection repairs. However, the mirror-imaged anatomic position, along with shock and devastating systemic malperfusion prior to surgery, presented a formidable challenge to the perioperative management. Lawton et al. reported that the combination of severe acidosis (BE >−10) and abdominal malperfusion was almost uniformly fatal in the surgical management of Stanford type A aortic dissection.[5] Despite severe acidosis (pH <7.1 and BE >−10) and extensive lower body malperfusion involving spinal cord, abdominal viscera and low extremities, our patient survived all dismals except paraplegia sequela. The incidence of spinal cord infarction with aortic dissection, clearly more common with type B aortic dissection than with type A dissection, was reported to be 2%–3% of all patients.[6] Acute occlusion of the artery of Adamkiewicz, the major radicular arterial supply to the mid and lower thoracic cord levels, is postulated to be the cause of aortic dissection-induced paraplegia. Despite immediate surgery and restoration of distal aortic blood flow through the true lumen, less than half of these patients showed substantial motor recovery and the paraplegia might become permanent.[7]

We attribute the successful surgical outcome to the cooperation and communication between two experienced surgeons and the surgical team involved. During the operation for this situs inversus patient with type A aortic dissection, most of the major procedures were performed by the surgeon standing on the left side of operating table. By maintaining surgeon's competence on both sides of the patient without the need to switch positions, the surgical process was expedited and organ ischemic time was alleviated.


  Conclusion Top


Situs inversus totalis with dextrocardia and right-sided aortic arch may present unique challenge to cardiovascular surgeons, especially when it comes in an acute type A aortic dissection with lower body malperfusion. Although the rarity of these combined situations and its devastating status, a successful outcome could be achieved through seamless cooperation and communication within the healthcare team.

Declaration of patient consent

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

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Van PR, Weinberg PM, Smith SD. Malposition of the heart. In: Hugh A, editor. Moss & Adams' Heart Disease in Infants, Children, and Adolescents, Including the Fetus and Young Adult. Baltimore: Williams & Wilkins; 2012. p. 530-89.  Back to cited text no. 1
    
2.
Bédard E, Shore DF, Gatzoulis MA. Adult congenital heart disease: A 2008 overview. Br Med Bull 2008;85:151-80.  Back to cited text no. 2
    
3.
Magishi K, Izumi Y, Ishikawa N, Kimura F. Stanford type A acute aortic dissection caused by blunt trauma in a patient with situs inversus. Ann Thorac Surg 2006;81:2294-6.  Back to cited text no. 3
    
4.
Niino T, Shiono M, Inoue T, Hata M, Sezai A, Negishi N. A case of acute aortic dissection type A in a patient with situs inversus. Ann Thorac Surg 2003;75:1963-5.  Back to cited text no. 4
    
5.
Lawton JS, Moon MR, Liu J, Koerner DJ, Kulshrestha K, Damiano RJ Jr., et al. The profound impact of combined severe acidosis and malperfusion on operative mortality in the surgical treatment of type A aortic dissection. J Thorac Cardiovasc Surg 2018;155:897-904.  Back to cited text no. 5
    
6.
Hagan PG, Nienaber CA, Isselbacher EM, Bruckman D, Karavite DJ, Russman PL, et al. The International registry of acute aortic dissection (IRAD): New insight into an old disease. JAMA 2000;283:897-903.  Back to cited text no. 6
    
7.
Sandridge L, Kern JA. Acute descending aortic dissections: Management of visceral, spinal cord, and extremity malperfusion. Semin Thorac Cardiovasc Surg 2005;17:256-61.  Back to cited text no. 7
    


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  [Figure 1], [Figure 2], [Figure 3]



 

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