Acute Management of Arrhythmias in Patients with Channelopathies



Fig. 8.1
A possible algorithm/pathway for diagnosis and treatment of arrhythmias in patients with channelopathies




Table 8.1
Conditions that can cause PVT/VF and potential therapies







































Clues

Test to consider

Diagnoses

Therapies

Long QT/T wave alternans

TdP

History of seizures

Specific triggers (loud noise)

ECG/monitor

Epinephrine challenge

Exercise stress test

Genetic testing

Congenital LQTS

Beta-blockers

Avoid QT-prolonging drugs

In LQT3: mexiletine/flecainide

PM/ICD

Stellatectomy

Incomplete RBBB with STE in leads V1–V2

Fever

ECG

Drug challenge

Genetic testing

BrS

Isoproterenol/quinidine

Antipyretic

Ablation

ICD

J-point elevation

ECG

Early repolarization

ICD

Short QT interval

ECG

SQTS

ICD

Quinidine or sotalol

Bidirectional VT exercise induced

Exercise stress test

Genetic testing

CPVT

Andersen-Tawil syndrome

Beta-blockers/flecainide/verapamil

ICD


Adapted from [15]: with permission




8.5 Indications for Hospitalization, Follow-Up, and Referral


Following the arrhythmic index event, channelopathy patient should be reevaluated for risk stratification and prevention of recurrences. Expert centers with a focus on inherited arrhythmias should be involved in complex cases [4].

Atrial arrhythmias in low-risk patients could be managed in out-of-hospital setting with referral to arrhythmia experts to set up indication for pharmacological or non-pharmacological strategy. Thromboembolism should be managed according to AF guidelines using CHA2DS2VASC score [20]. First line therapy consists in avoiding potentially pro-arrhythmic drugs and conditions: a complete list should be supplied to the patient and to the general practitioner.

CPVT and LQT patients should be advised to limit/avoid competitive sport, strenuous exercise, and exposure to stressful environments (which in LQT2 should include exposure to loud/abrupt noises, i.e., alarm bell); Brugada patients should avoid excessive alcohol intake and large meals and should be advised to a prompt treatment of fever [45].

Syncope and life-threatening arrhythmias require hospitalization.

Aborted sudden death and sustained ventricular arrhythmias require an ICD for secondary prevention [4, 15, 31, 45] with or without adjunctive therapy.

CPVT and LQTS patients should be treated with beta-blockers: nadolol and propranolol are the drugs of choice [1, 4, 33]; in patients with recurrent symptoms/arrhythmias already on beta-blockers, it should be considered flecainide for CPVT patients [33] and flecainide or ranolazine or mexiletine in LQT3 patients [4]; ICD and left cardiac denervation should be considered in patients refractory to pharmacological therapy [4, 33]. Repeated exercise stress test is used in CPVT patients to evaluate drug efficacy.

Brugada and SQTS patients symptomatic for syncope should be treated with ICD; quinidine therapy could be used as adjunctive therapy or in cases in which ICD is refused or contraindicated or in recurrent appropriate ICD intervention [22, 26].

Hydroquinidine has proven to play a role in AF recurrence prevention in Brugada patients [4, 21].

Refractory electrical storm could be evaluated for catheter ablation of triggers [15, 3941, 45].

All clinically diagnosed patients with LQTS and CPVT should undergo genetic evaluation if not previously performed, and it can be useful in Brugada (type1) patients and SQTS [42]. Routine genetic testing is not indicated for the survivor of an unexplained out-of-hospital cardiac arrest in the absence of a clinical index of suspicion for a specific cardiomyopathy or channelopathy [42] (Figs. 8.2 and 8.3)

A327403_1_En_8_Fig2_HTML.jpg


Fig. 8.2
Panel (a):12-lead ECG from a 9-year-old boy with ryanodine-positive CPVT shows a transition from triggered bidirectional ventricular tachycardia followed by brief polymorphic ventricular tachycardia to reentrant ventricular fibrillation. With permission from Elsevier Roses-Noguer F. et al. [43]: Copyright © 2014 Heart Rhythm Society. Panel (b): torsades de point. With permission from Van der Heide et al. [44]


A327403_1_En_8_Fig3_HTML.jpg


Fig. 8.3
Precordial leads ECG in patients with panel (a), long QT syndrome, heart rate 58 beats per minute, QTc 600 ms; panel (b), short QT syndrome, heart rate 52 beats per minute (bpm), QT 280 ms*; panel (c), Brugada syndrome, coved ST elevation in V1–V2. *With permission from Gaita F. et al. [7]


References



1.

Cerrone M, Priori SG. Genetics of sudden death: focus on inherited channelopathies. Eur Heart J. 2011;32:2109–18.PubMedCrossRef


2.

Patel C, Burke JF, Patel H, et al. Is there a significant transmural gradient in repolarization time in the intact heart? Cellular basis of the T wave: a century of controversy. Circ Arrhythm Electrophysiol. 2009;2(1):80–8.PubMedPubMedCentralCrossRef


3.

Hocini M, Pison L, Proclemer A, et al. Diagnosis and management of patients with inherited arrhythmias in Europe: results of the European Heart Rhythm Association Survey. Europace. 2014;16:600–3.PubMedCrossRef


4.

Priori SG, Wilde AA, Horie M, et al. HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm. 2013;10(12):1932–63.PubMedCrossRef

Oct 16, 2016 | Posted by in EMERGENCY MEDICINE | Comments Off on Acute Management of Arrhythmias in Patients with Channelopathies

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