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Table 7 Genetic tests and suggested work-up of probands and relatives with primary electrical diseases LQTS BrS CPVT Idiopathic VF ERS Genetic test Class Ia Class I Class Ia Class IIb Class IIb Proband Initial clinical test Cornerstone for diagnosis ECG Exercise test ECG and high precordial lead ECG Sodium channel blockers provocative testc Exercise test See Section 5.2.3, scenario 3 ECG Other tests/processes Exclude acquired LQTS Exclude phenocopyb Exclude phenocopyb/SHD Holter Echocardiography Follow-up 1–3 years dependent on level of risk Relatives Clinical screening ECG Exercise test (when feasible) From birth ECG and high precordial lead ECGs: start at 10 years Sodium channel blockers provocative testc: start .16 years unless clinically indicated180,181 ECG Exercise test From birth ECG and high precordial lead ECGs Exercise test Echocardiogram182 ECG Echocardiogram Follow-up Positive phenotype and/or Class IV/V variant 1–3 years dependent on level of risk Negative phenotype and no Class IV/V variant Discharge BrS, Brugada syndrome; CPVT, catecholaminergic polymorphic ventricular tachycardia; ECG, electrocardiogram; ERS, early repolarization syndrome; LQTS, long QT syndrome; VF, ventricular fibrillation. aIncluding neonatal genetic testing. bA phenocopy has characteristics of a genetic disease but is produced environmentally. cNot in case of a documented type 1 Brugada pattern. Ergonovine Same as acetylcholine. Intracoronary stepwise injection: RCA (20–60 mg) LCA (20–60 mg) over a period of 2–5 min. Same as acetylcholine Left main stenosis .50%, 3-vessel disease, 2-vessel disease with total occlusion, NYHA III/V HF, renal failure. Temporary wire should be placed for back-up pacing. Risk of cardiogenic shock. Same as acetylcholine. Cath lab. 143 Adenosine Exclude latent pre-excitation. 6, 12, 18 mg boluses up to maximum dose 24 mg or until AV block or pre-excitation occurs. Identification of accessory pathway. Asthma, sinus node disease, allergy to adenosine. Side effects: Bronchospasm, bradycardia, asystole, AF, seizure. Antagonist: theophylline. 5 min. Same as ajmaline. 144 AF, atrial fibrillation; AV, atrioventricular; BrS, Brugada syndrome; CA, cardiac arrest; CPVT, catecholaminergic polymorphic ventricular tachycardia; ECG, electrocardiogram; HF, heart failure; LCA, left coronary artery; NYHA, New York Heart Association; PVCs, premature ventricular complexes; PVT, polymorphic ventricular tachycardia; RCA, right coronary artery; SADS, sudden arrhythmic death syndrome; SHD, structural heart disease; VF, ventricular fibrillation; VT, ventricular tachycardia. Downloaded from academic.oup.com/eurheartj/article/43/40/3997/6675633 by guest on 07 October 2026