Left cardiac sympathetic denervation for the treatment of inherited arrhythmia syndromes: salvation for the desperate?
M. Voskuil
J. F. van der Heijden
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In the large majority of patients, ventricular arrhythmias are
caused by myocardial ischaemia and/or infarction because of
underlying coronary artery disease. Also, nonischaemic
cardiomyopathy, infiltrative disease (e.g. sarcoidosis,
amyloidosis) and infectious disease (e.g. viral myocarditis) are
reasonably frequent causes of ventricular arrhythmias. Obviously, in
these cases, treating the underlying disease is the primary goal.
However, ventricular arrhythmias also occur in patients with
primary inherited arrhythmia syndromes such as
catecholaminergic polymorphic ventricular tachycardia (CPVT) and
long-QT syndrome (LQTS). CPVT and LQTS are rare causes
of ventricular arrhythmias, and predominantly occur in young
patients, with apparently structurally normal hearts. The
ventricular arrhythmias occur mainly under conditions of
increased sympathetic activity (e.g. physical or emotional stress)
[1]. In these patients, besides removal of triggers, the
cornerstone of treatment of these diseases is -adrenoreceptor
blockade. Treatment with beta-blockers is currently a class I
indication in clinically diagnosed patients suffering from
ventricular arrhythmias [2]. Beta-blockade should be titrated up to an
effective level. If not sufficient, adding flecainide in the case
of CPVT appears to be a sensible approach [3]. Implantation
of an internal cardioverter defibrillator (ICD) can be
considered in the (rare) patient not adequately controlled with
medical interventions. However, the use of an ICD alone is also
not the panacea because of the catecholamine release,
triggered by the pain and fear generated by ICD shocks, which
may subsequently initiate arrhythmic storms and/or multiple
shocks and may even lead to death [4]. Therefore,
continuation of medical therapy in the maximal tolerable dose is
mandatory, also after ICD implantation.
Left cardiac sympathetic denervation (LCSD) may be
considered for patients with CPVT and LQTS who suffer from
recurrent ventricular tachycardias despite maximal
pharmacological therapy [5]. Historically, sympathectomy has been
used as a treatment modality for angina pectoris [6].
From the 1970s cardiac sympathetic denervation has
been described for the treatment of (treatment-resistant)
ventricular arrhythmias [7].
In the current issue of the Netherlands Heart Journal,
Olde Nordkamp and co-workers present their
experience of LCSD in treating therapy-resistant patients with
inherited arrhythmia syndromes. [8] They are a tertiary
referral centre for this patient cohort in the Netherlands,
being the only centre in this country performing this
surgical procedure. The baseline characteristics of the
described patients show the disease state, since all
patents were on beta-blockade and the majority of
patients suffering from CPVT were also taking flecainide.
Despite maximal medical therapy these patients were
still experiencing adverse events (arrhythmias, syncope).
The majority of patients underwent a video-assisted
thoracoscopic approach, some using the supraclavicular
a p p r o a c h a nd o n e p a t i e n t u n d e r w e n t LC S D v i a
thoracotomy.
The clinical results presented are reassuring; in
general, a nice 87 % of symptomatic patients experienced a
decreased cardiac event rate after LCSD. However, only
47 % of the patients did not experience any cardiac
events, while 53 % of the patients remained
symptomatic (although to a lesser extent) during a median
follow-up of 34 (IQR 1677) months. These numbers
are comparable with previous reports from other
(foreign) centres [9].
This reduction of ventricular arrhythmias after LCSD,
however, comes at a cost; the risk for serious peri-procedural
complications was 12 % (2 out of 17 patients) in the
current patient cohort. One patient had a permanent
postoperative Harlequin face and one (severely affected)
LQT (type 8) patient died postoperatively because of
multi-organ failure after several problems following
multiple arrhythmic storms. Furthermore, three patients
suffered from a post-procedural pneumothorax and one
patient experienced a transient Horners syndrome. It is,
however, to be expected that more experience with this
treatment modality will result in a reduction of the
(serious) adverse events in the future.
Therefore, the conclusion of this retrospective analysis
investigating the safety and efficacy of LCSD of patients with
LQTS and CPVT, not responding well to pharmacological
therapy, must be that it offers a valuable addition to the
medical therapy for patients with CPVT and LQTS, suffering
from repetitive ICD shocks and/or ventricular arrhythmias.
However, the other side of the coin must be taken into account
in all cases, since it is not a minor intervention without risks. If
risks and benefits are discussed and understood properly by
the (in general young) patient it offers a potential escape for
the often-traumatic events of otherwise untreatable ventricular
arrhythmias and ICD shoc (...truncated)