Catecholaminergic polymorphic ventricular
tachycardia is a rare malignant inherited arrhythmia
syndrome.[
1] It usually presents in childhood or young
adulthood with a history of physical or emotional
stress-induced syncope or cardiac arrest.[
1] Arrhythmia
is characterized by bidirectional or polymorphic
ventricular tachicardia in patients with a structurally
normal heart.[
2-
4]
Catecholaminergic polymorphic ventricular
tachycardia is associated with RYR2 and CASQ2
gene mutation. The inheritance pattern is autosomaldominant
and autosomal-recessive, respectively.[4,5]
These genes are related to calcium channels.[2] A family history of syncope or cardiac death is positive in
approximately 35% of patients with CPVT.[3]
Catecholaminergic polymorphic ventricular
tachycardia was first described in 1975 by Reid et
al.[3,4,6] Diagnosis can be challenging. As ECG usually
produces normal results in asymptomatic patients,
ECG is not specific.[2,4] The diagnosis is based on
ECG findings during exercise. During exercise,
typical ECG finding is bidirectional tachycardia.[1,4,7,8]
The medical treatment of CPVT is based on
beta-blockers.[1] Syncopal attacks can be controlled
by beta-adrenergic blockers. Nadolol is the preferred
agent thanks to its prolonged half-life. Therefore,
beta-blockers are effective for the acute phase and
they are the first-line of treatment.[1,3,7,9,10] Other
therapeutic options for patient with CPVT include
calcium channel blockers and/or flecainide. However,
an ICD should be planted in patients with recurrent
arrhythmias or cardiac arrest episodes.[1,3,4]
In this case, our patient presented due to exerciserelated
syncope with a prior history of cardiac arrest.
She was diagnosed as CPVT based on her medical
history and typical ECG findings. In the beginning,
she was treated with a single beta-blocker agent,
however, the patient presented again with cardiac arrest while she was on medical treatment. An automatic
ICD was implanted. The first ICD depleted in five
months. As a result, the second ICD was implanted;
however, it depleted one month later. The depletion
was caused by inappropriate multiple ICD shocks.
During a five-year follow-up, the patient remained
asymptomatic. At five-years, she had a repeated
syncopal episode as assessed by regular examinations.
Therefore, LCSD was recommended. An ICD was
recommended for CPVT patients who have cardiac
arrest or syncopal attack despite receiving maximum
dosage of beta-blocker therapy.[11,12] However, when
the symptoms persist despite beta blockade, calcium
channel blockers, ICD, and LCSD are effective
alternative treatments.[1,11,13]
Left cardiac sympathetic denervation is described
in 1971.[14,15] Surgically LCSD involves resection of
the lower half of the left stellate ganglion and the left
sided sympathetic chain. Left cardiac sympathetic
denervation has been used as an effective option for
patient with CPVT.[14,16] However, clinical experience
of LCSD in CPVT patients is limited. In addition,
there are few reports on the anesthetic procedure of
patients with CPVT. The goal of the treatment is to
manage the adrenergic stimulation, since adrenergic
stimulation may provoke arrhythmias.[13]
Operative management of these patients is often
challenging. There is a high risk of life-threatening
arrhythmia and sudden cardiac arrest. Anesthetic
management should be planned carefully.[13,17,18]
Careful planning and monitorization are critical to
ensure a safe operation. The operation room should
be appropriate for the induction. Hyper-hypotension,
bradycardia, tachycardia, hypothermia, hyperhypocapnia,
hypoxemia should be also controlled, as
these conditions can effect cardiac activity.[11,19]
Serum electrolytes should be measured. An external
defibrillator and blood pressure monitoring should
also be present in the operating room. Premedication is
necessary to avoid increased sympathetic activity.[19-21]
For premedication, midazolom is used in our
patient. Propofol was used in our patient for the
induction. Propofol has been used as induction and
maintenance agent in patients with CPVT to avoid
complications.[1] Rocuronium was administered to our
patient for muscle relaxation. Rocuronium can be safely
administered to this group of patients.[1] In this present
case, video-assisted thoracoscopic cardiac denervation,
a minimally invasive procedure, was made.
Bupivacaine and fentanyl were used for the pain
management and to maintain medical sympathetic
denervation. Then, a thoracal epidural catheter
was placed. For the management of postoperative
nausea and vomiting, ondansetron was initiated. No
significant complication and adverse effects were
noted during the operation.
In conclusion, anesthetic management of the
patient with CPVT requires careful monitoring of
cardiac parameter, understanding of risks, and good
management of postoperative pain control.
Declaration of conflicting interests
The authors declared no conflicts of interest with
respect to the authorship and/or publication of this
article.
Funding
The authors received no financial support for the
research and/or authorship of this article.
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