Anesthesia and SADS Conditions

LONG QT syndrome (LQTS) is a malfunction of cardiac ion channels resulting from mutations involving genes encoding critical ion channels of the heart (congenital LQTS) or caused by metabolic abnormalities or drugs (acquired LQTS). In both instances the perturbed ion channels impair ventricular repolarization. Patients with LQTS often manifest prolongation of the QT interval and abnormal T wave morphology on 12-lead electrocardiogram secondary to delayed cellular repolarization and heterogeneity in dispersion of repolarization, which, when amplified by sympathetic activity, can lead to early after-depolarization, further dispersion of repolarization, and the formation of reentry circuits. These events can present as syncope, seizures, or sudden cardiac death secondary to a characteristic polymorphic ventricular tachycardia known as torsades de pointes (TdP).2,3 Symptomatic events may be triggered by physical activity and emotional stress.

Several medications, including anesthetic agents, may also interfere with cardiac repolarization, prolong the QT interval, and, sometimes, cause drug-induced TdP and sudden death.

Source: Kies et al., "Anesthesia for Patients with Congenital Long QT Syndrome", Anesthesiology 2005; 102:204-10

SADS Patients and families should take these materials (below) with them before undergoing a surgical procedure using anesthesia.

Resources for Anesthesiologists:

• Article: Nathan et al. "Case Scenario: Anesthesia-related Cardiac Arrest in a Child with Timothy Syndrome". Anesthesiology 2012; 117:1117-26

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• Article (excerpt above): Kies et al. "Anasthesia for Patients with Congenital Long QT Syndrome" Anesthesiology 2005; 102:204-10  

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• Important information about Timothy Syndrome

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