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|Year : 2022 | Volume
| Issue : 1 | Page : 41-43
Cardiac sympathetic denervation for catecholaminergic polymorphic ventricular tachycardia
R Bansal, A Mahajan, S Vichare, Y Lokhandwala
Holy Family Hospital, Mumbai, Maharashtra, India
|Date of Submission||01-Aug-2020|
|Date of Decision||01-Dec-2020|
|Date of Acceptance||10-Dec-2020|
|Date of Web Publication||26-May-2021|
Holy Family Hospital, Mumbai, Maharashtra
Source of Support: None, Conflict of Interest: None
Catecholaminergic polymorphic ventricular tachycardia (CPVT), a rare inheritable fatal arrhythmogenic disorder, is difficult to diagnose and is a challenge to manage. A 21-years-old man presented with recurrent exertional syncope and complex multifocal ventricular ectopy. CPVT was diagnosed based on the clinical criteria, despite the absence of some classical findings. The patient underwent cardiac sympathetic denervation (CSD) after lifestyle modification and pharmacological management were ineffective. CSD proved to be effective. The patient did not have any exertional symptoms or recurrence of syncope at follow-up period of 1 year. The present case report adds to the growing evidence in favour of CSD for CPVT.
Keywords: Cardiac sympathectomy, sudden death, syncope
|How to cite this article:|
Bansal R, Mahajan A, Vichare S, Lokhandwala Y. Cardiac sympathetic denervation for catecholaminergic polymorphic ventricular tachycardia. J Postgrad Med 2022;68:41-3
|How to cite this URL:|
Bansal R, Mahajan A, Vichare S, Lokhandwala Y. Cardiac sympathetic denervation for catecholaminergic polymorphic ventricular tachycardia. J Postgrad Med [serial online] 2022 [cited 2022 Aug 12];68:41-3. Available from: https://www.jpgmonline.com/text.asp?2022/68/1/41/316964
| :: Introduction|| |
Catecholaminergic polymorphic ventricular tachycardia is a rare inheritable arrhythmogenic disorder, characterized by adrenergic induced polymorphic or bidirectional VT in the absence of structural heart disease. The presentation is usually in young patients with exertional palpitations or syncope, but occasionally sudden cardiac death may also be the first manifestation. Once a diagnosis is made, treatment options are limited, with activity restriction and pharmacological sympathetic blockade being the initial therapy. Beta blockers however do not have a reliable response required for this deadly disease. The other modality of an implantable cardioverter defibrillator (ICD) helps prevent some sudden cardiac deaths but may also be associated with frequent inappropriate shocks as also induce CPVT storms. Thus, ICD implantation should be considered only after good control of arrhythmia in this set of patients. A case of CPVT is being described who underwent CSD and responded well, confirming the usefulness of this underutilised modality.
| :: Case Report|| |
A 21-years-old man presented with exertional palpitations and two episodes of exertional syncope. There was no family history of sudden death. Physical examination was unremarkable. The resting ECG revealed normal sinus rhythm, no manifest pre excitation and a normal QT interval, but notably had multifocal premature ventricular complexes (PVCs, [Figure 1]a). A 24-hour Holter analysis demonstrated a PVC burden of 28% with complex ectopy pattern consisting of polymorphic PVCs, bigeminy and trigeminy; there were 20 episodes of non-sustained VT. Echocardiographic and cardiac MRI examinations were normal. On account of a young age with polymorphic PVCs without structural heart disease, a diagnosis of CPVT was entertained.
|Figure 1: (a) Resting electrocardiogram (ECG) showing sinus rhythm with multifocal premature ventricular complexes; (b) ECG during stage 4 of stress testing by Bruce protocol, demonstrating increased ventricular ectopy and runs of non-sustained ventricular tachycardia; (c and d)thoracoscopic view of the sympathetic chain before (left panel) and after (right panel) dissection|
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A treadmill exercise test induced significant increase in complex ventricular ectopy with one episode of non-sustained VT [Figure 1]b. However, characteristic bidirectional VT was not seen. Genetic testing for the RyR2 and Calsequestrin genes was also negative. Yet, a diagnosis of CPVT was made based on the expert consensus diagnostic criteria [Table 1]. The patient was advised exercise restriction and long acting propranolol, 40 mg twice daily, with a further plan for dose escalation. However, the patient remained symptomatic despite the maximal tolerated dose of propranolol.
|Table 1: Expert consensus criteria for diagnosis of catecholaminergic polymorphic ventricular tachycardia|
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Following a detailed discussion between the choice of ICD and CSD as the next line of therapy, the patient underwent the latter after informed consent. The procedure was performed with videoscopy-assistance, under general anesthesia with single lung ventilation; the first 4 throacic sympathetic ganglia were excised bilaterally [Figure 1]c, [Figure 1]d. The procedure was well tolerated without any complications, following which the patient improved significantly with reduction in ventricular ectopy. At a follow up of one year, the patient was minimally symptomatic with improved exercise capacity and no recurrence of syncope. The beta blockers were continued and the patient is kept on medical follow up, with a plan for ICD implantation in future if there is recurrence of arrhythmic syncope.
| :: Discussion|| |
A high index of suspicion is necessary for the diagnosis of CPVT. Classical findings of exercise induced bidirectional VT help in clinching the diagnosis but have a suboptimal sensitivity. Genetic testing for arrhythmic substrates can also be negative in one third of the cases. Thus, the clinical scenario should be supplanted with extensive investigations for making a diagnosis. In doubtful cases, extended monitoring with an implantable loop recorder or an epinephrine challenge can be useful.
Conventionally, CPVT does not have a curative treatment and has been proposed to be managed with exercise restriction, beta blockers and ICD implantation to minimise the risk of sudden cardiac death. Exercise restriction and beta blockers do reduce the risk of sudden cardiac death, but do not eliminate it. The residual risk warrants an ICD implantation. However, the expensive ICD therapy is not without its share of problems, especially in CPVT. As high as 46% of patients with CPVT may experience inappropriate shocks with ICD. Anti-tachycardia pacing is also ineffective in CPVT due to its polymorphic nature and a propensity towards acceleration of VT and its degeneration into ventricular fibrillation. The episodes of non-sustained VT which would have terminated spontaneously without device response can be instead a reason for unnecessary shocks. Furthermore, the anxiety induced by fear of repetitive shocks could by itself be proarrhythmic in these patients. Lastly, the problems of ICDs related to lead failures and requirement of multiple procedures for battery depletion also remain an important consideration, especially in the younger population. Thus, although considered as one of the frontline therapies, the ICD remains a far from ideal solution for CPVT.
After being first described by Jonesco in 1916, CSD has evolved as a treatment for several arrhythmias. Nevertheless, CSD remains a lesser known and underutilized treatment option for ventricular tachyarrhythmias refractory to drug therapy. The main advantage of CSD is that it leads to significant reduction of sympathetic input to the heart and a much superior control of arrhythmias as compared to pharmacological sympathetic inhibition, without myocardial depression. In a recent study by de Ferari et al., CSD in CPVT has been shown to decrease major cardiac events in one-third of patients on optimal medical therapy and to decrease the need of shock therapy by as high as 93% in patients who underwent ICD implantation. CSD can be unilateral (left sided) or bilateral, with the bilateral option being more effective. In a study by Vaseghi et al., three out of six patients who developed arrhythmia recurrence after left CSD, responded well to right CSD. Minimally invasive thoracoscopic bilateral CSD is the current state of art technique and is highly successful. The procedure can be done using two lung ventilation or single lung ventilation. The patient is usually extubated the same day and discharged on the third day. Side effects could be unilateral hand dryness (84%), colour or temperature variance between sides of the face (76%), abnormal sweating (54%), postoperative shoulder blade pain (48%), eyelid drooping (48%), decreased pupil size (42%) and chest pain (37%). Minimal surgical risk with short hospital stay, usually manageable side effects and an excellent arrhythmia control substantiated with improved quality of life make CSD an attractive choice of therapy in CPVT patients. Thus, it is suggested that CSD should be considered in CPVT as the next step after optimal medical therapy and before ICD implantation. It should also be remembered that in a recent study, an ICD was not associated with improved survival in CPVT patients. There was both a high rate of appropriate and inappropriate ICD shocks along with other device-related complications.
| :: Conclusion|| |
This case highlights the importance of CPVT as a cause of recurrent exertional syncope in young patients with structurally normal hearts. The diagnosis requires a high index of suspicion, supplemented by exercise testing. Exercise restriction, beta blockers and avoidance of stress form the cornerstones of therapy. ICD implantation has been considered as next line of therapy but lately the evidence in favour of CSD is accumulating. This case demonstrates the efficacy of CSD in such a patient.
Declaration of patient consent
The authors certify that appropriate patient consent was obtained.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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