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 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 5  |  Issue : 1  |  Page : 85-87

A case of arrhythmogenic right ventricular dysplasia/cardiomyopathy with pneumothorax and degloving injury of the right upper limb


Department of Anaesthesiology, Government Medical College, Thiruvananthapuram, Kerala, India

Date of Submission08-Jan-2020
Date of Decision18-Jan-2020
Date of Acceptance24-Feb-2020
Date of Web Publication23-Apr-2020

Correspondence Address:
Dr. Varun Suresh
Department of Anaesthesiology, Government Medical College, Thiruvananthapuram, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/bjhs.bjhs_66_19

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  Abstract 


Arrhythmogenic right ventricular dysplasia / cardiomyopathy (ARVD / C) is a rare genetic cardiomyopathy characterized by fibro-fatty replacement primarily of the right ventricular myocytes,and can present as any of 'concealed' disease, 'overt arrhythmia' followed by sudden cardiac death, isolated right heart failure or biventricular failure: mimicking dilated cardiomyopathy. We present the successful management of a 65 year old male patient with ARVD/C presenting to us with trauma and Cardiogenic shock for limb saving emergency surgery.

Keywords: Arrhythmogenic right ventricular dysplasia, dilated cardiomyopathy, plakophilin


How to cite this article:
Suresh V. A case of arrhythmogenic right ventricular dysplasia/cardiomyopathy with pneumothorax and degloving injury of the right upper limb. BLDE Univ J Health Sci 2020;5:85-7

How to cite this URL:
Suresh V. A case of arrhythmogenic right ventricular dysplasia/cardiomyopathy with pneumothorax and degloving injury of the right upper limb. BLDE Univ J Health Sci [serial online] 2020 [cited 2020 Aug 15];5:85-7. Available from: http://www.bldeujournalhs.in/text.asp?2020/5/1/85/283087



Arrhythmogenic right ventricular (RV) dysplasia/cardiomyopathy (ARVD/C) is characterized by the fibrofatty replacement of RV myocytes. Cardiac electrical instability, ventricular tachycardia (VT), and biventricular failure are common in this autosomal dominant inherited condition,[1] with a male predominant prevalence rate of 1 in 5000.[2],[3] Epicardial degenerative fibro-fatty changes spread to the endocardium through the myocardial tissue here. Although the disease process in ARVD/C begins in the RV outflow tract, later stages of the disease involve cardiac apex, subtricuspid area, left ventricle (LV), and interventricular septum.[4],[5],[6] Polymorphic phenotypic expression of any of plakophilin-2, desmoglein-2, desmoplakin, desmocollin-2, and/or junctional plakoglobin, which are cardiac desmosomal proteins, has been attributed to ARVD/C.[7]

Beta-blockers, antiarrhythmic drugs, catheter ablation, implantable cardioverter/defibrillator, and heart transplantation are among the various therapeutic options in this cardiomyopathy. Intraoperative management in ARVD/C centers on measures to decrease both preload and afterload with diuretics and vasodilators, respectively, and stimulating the hypodynamic heart with inotropic agents. The management of a patient with ARVD/C undergoing noncardiac surgery is always a challenge to the anesthesiologist as ARVD/C is most commonly complicated by progressive congestive heart failure, malignant arrhythmias, and sudden cardiac death. We describe the management of a 65-year-old-male patient with ARVD/C who had a humerus bone fracture along with the degloving injury of right upper limb and blunt trauma chest with flail segment.


  Case Report Top


A 65-year-old male patient presented to the emergency department with an alleged history of roadside accident. Examination showed him to be conscious with 15/15 Glasgow Coma Scale score, heart rate (HR) of 100/min, and blood pressure (BP) of 90/60 mm Hg. Head-to-foot examination found a lacerated wound with degloving over right upper limb with abnormal mobility and tenderness over the right hemithorax.

Roentgenography [Figure 1] revealed the fracture of right 2, 3, and 4 ribs with flail segment and underlying pneumothorax and segmental fracture of the right humerus. A history of heart disease with the New York Heart Association 2 status on medications, namely spironolactone, aspirin, ramipril, carvedilol, and digoxin for the past 13 years was elicited, and the diagnosis of ARVD/C was confirmed from the previous records. Family history revealed a history of sudden cardiac death among kins. Transthoracic echocardiography showed cardiomyopathy with severe LV systolic dysfunction, ejection fraction 25%, severe mitral regurgitation, severe tricuspid regurgitation, severe pulmonary artery hypertension, mild aortic regurgitation, and global hypokinesia.
Figure 1: Chest X-ray showing traumatic flail segment

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The patient required the debridement of the wound and external fixator application of humerus. Preoperative electrocardiogram [Figure 2] showed significant ventricular premature complexes. Surgery was planned under general anesthesia (GA) as regional anesthesia (RA) with interscalene brachial plexus blockade could not be done due to lacerations in the local area. No premedication was administered.
Figure 2: Preoperative electrocardiogram showing multiple ventricular premature contractions

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Invasive hemodynamic monitoring was started before GA induction. Anesthesia was induced with intravenous (IV) morphine 9 mg, fentanyl 100 μg, midazolam 3 mg, and etomidate 10 mg. Endotracheal intubation was achieved with 8.5-mm cuffed endotracheal tube after skeletal muscle paralysis with IV succinylcholine. Vecuronium IV was administered after intubation. Anesthesia was maintained with oxygen, nitrous oxide, and desflurane (MAC 0.9–1). Hemodynamics were maintained with IV infusion of dopamine 5–20 μg/kg/min and dobutamine 5–20 μg/kg/min titrated to a mean BP of 70–80 mm Hg. IV esmolol intermittent boluses were given to circumvent episodes of tachycardia sustained intraoperatively with HR maintained between 100 and 120 bpm. Blood loss intraoperatively approximated around 700–900 mL, for which two units of cross-matched packed red cells were transfused, maintaining hemoglobin of 10 g/dL intraoperatively. Crystalloid IV fluids were given titrating to the central venous pressure (CVP) of 8–12 mm Hg. Postoperative mechanical ventilation (MV) was planned, and the patient was shifted to the intensive care unit (ICU) hemodynamically stable and unreversed from neuromuscular blockade.

Ionotropic support was gradually weaned off over 72 h. Weaning from MV was attempted after the 4th postoperative day. With the patient off ionotropes, full preoperative medication with spironolactone, ramipril, carvedilol, digoxin, and aspirin was restarted. Due to MV weaning failure, an elective tracheostomy was done on the 7th postoperative day. The patient was weaned off from MV by the 14th postoperative day. Further, ICU stay was uneventful, and the patient was discharged on the 18th postoperative day.


  Discussion Top


ARVD/ C passes through the following four phases: (1) concealed, (2) overt arrhythmia, (3) isolated RV failure, and (4) biventricular failure. Patients can be discovered in any phase of the disease.[8],[9]

There are insufficient data in the literature to make specific recommendations on the use or avoidance of any particular anesthetic drug or technique in this condition. Thus, whether GA or RA is used depends on the procedure and the degree of cardiac dysfunction. Perhaps, it is better to avoid epinephrine as an adjunct to RA and perhaps also avoid large doses of bupivacaine to reduce the risks of potential cardiac toxicity. There is likely to be a lower threshold for considering invasive hemodynamic monitoring, transesophageal echocardiography, or CVP monitoring to help guide intraoperative decision-making. The pulmonary artery catheter (PAC) should probably be avoided in patients with ARVD/C as these patients are likely more susceptible to the complications of catheter placement (VT and ventricular perforation). The benefits of any information gained from PAC are probably outweighed by their risks.[10]

The goals that should be incorporated in the anesthetic management of ARVD/C include prudent use of antiarrhythmic drugs, monitor serum electrolytes, avoid myocardial depression, maintain normovolemia, avoid overdose of drugs during GA, avoid decrease in RV/LV afterload, and avoid sudden hypotension when RA is the choice.

RA may be an alternative to GA in selected patients with ARVD/C. Brachial plexus blockade was not attempted in this case due to local area lacerations. Cervical epidural anesthesia was not attempted in view of ongoing cardiogenic shock.

This case requires reporting as ARVD/C is a rare condition encountered during anesthesia; moreover, the cardiac pathology here was compounded with flail chest. Multidisciplinary management between anesthetists, cardiologists, surgeons, and intensivists lead to successful perioperative management and optimal outcome in our case.

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

Departmental.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Corrado D, Basso C, Thiene G, McKenna WJ, Davies MJ, Fontaliran F, et al. Spectrum of clinicopathologic manifestations of arrhythmogenic right ventricular cardiomyopathy/dysplasia: A multicenter study. J Am Coll Cardiol 1997;30:1512-20.  Back to cited text no. 1
    
2.
Peters S, Trümmel M, Meyners W. Prevalence of right ventricular dysplasia-cardiomyopathy in a non-referral hospital. Int J Cardiol 2004;97:499-501.  Back to cited text no. 2
    
3.
Kies P, Bootsma M, Bax J, Schalij M, van der Wall E. Arrhythmogenic right ventricular dysplasia/cardiomyopathy: Screening, diagnosis, and treatment. Heart Rhythm 2006;3:225-34.  Back to cited text no. 3
    
4.
Thiene G, Basso C. Arrhythmogenic right ventricular cardiomyopathy: An update. Cardiovasc Pathol 2001;10:109-17.  Back to cited text no. 4
    
5.
Corrado D, Basso C, Schiavon M, Thiene G. Screening for hypertrophic cardiomyopathy in young athletes. N Engl J Med 1998;339:364-9.  Back to cited text no. 5
    
6.
Tabib A, Loire R, Chalabreysse L, Meyronnet D, Miras A, Malicier D, et al. Circumstances of death and gross and microscopic observations in a series of 200 cases of sudden death associated with arrhythmogenic right ventricular cardiomyopathy and/or dysplasia. Circulation 2003;108:3000-5.  Back to cited text no. 6
    
7.
Norgett EE, Hatsell SJ, Carvajal-Huerta L, Cabezas JC, Common J, Purkis PE, et al. Recessive mutation in desmoplakin disrupts desmoplakin-intermediate filament interactions and causes dilated cardiomyopathy, woolly hair and keratoderma. Hum Mol Genet 2000;9:2761-6.  Back to cited text no. 7
    
8.
Sen-Chowdhry S, Syrris P, Ward D, Asimaki A, Sevdalis E, McKenna WJ. Clinical and genetic characterization of families with arrhythmogenic right ventricular dysplasia/cardiomyopathy provides novel insights into patterns of disease expression. Circulation 2007;115:1710-20.  Back to cited text no. 8
    
9.
Wei J, Tang J, Xia L, Chen X, Wang DW. A case of arrhythmogenic right ventricular cardiomyopathy without arrhythmias. Diagn Pathol 2012;7:67.  Back to cited text no. 9
    
10.
Alexoudis AK, Spyridonidou AG, Vogiatzaki TD, Iatrou CA. Anaesthetic implications of arrhythmogenic right ventricular dysplasia/cardiomyopathy. Anaesthesia 2009;64:73-8.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2]



 

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