Sequence 4170, syndromes with risk of sudden death type, will populate when a history of syndromes of sudden death are captured. If the patient has a history of a syndrome with risk of sudden death, sequence 4170 seeks to capture the type. The first selection we will review is Brugada syndrome. This is a polymorphic ventricular tachycardia that occurs in the absence of structural heart disease. It is associated with the baseline ACG pattern during sinus rhythm showing a right bundle branch block with ST elevation in leads V1 through V3. They can also be characterized by documentation of ACG patterns associated with Brugada syndrome, some of which may be unmasked when provoked with drugs. The most common genetic mutations identified for this syndrome are in a sodium channel gene. Therefore, sodium channel blocking agents may exacerbate the ACG features and clinical presentation. This syndrome typically presents before the age of 50. CPVT is a highly malignant inheritable cardiac channelopathy in individuals without structural heart disease and QT prolongation on ECG. It is often thought of being a disease of childhood, setting itself in before the age of 21 with symptoms such as syncope or sudden cardiac arrest. However, there is an adult form that presents between the ages of 32 and 48. This syndrome is triggered by physical or emotional stress in patients who ECG is normal. Idiopathic or primary ventricular tachycardia or fibrillation occurs in patients without structural heart disease, metabolic abnormalities, or the prolonged QT syndrome. Prolonged QT syndrome describes a group of inherited channelopathies that confer risks of polymorphic ventricular tachycardia and sudden cardiac death. A clinical diagnosis is required and is based on clinical presentation and ECG. Genetic testing is generally advised as certain variants are responsible for this syndrome. This cannot be determined by the abstractor based on a long QT on ECG. Short QT syndrome refers to the ECG manifestation of accelerated cardiac repolarization. Acquired disease, which is the most common cause, results from electrolyte disturbances or drugs in addition to hypercalcemia, hyperkalemia, and acidosis. Short QT syndrome manifests with digoxin, androgen use, increased vapal tone, and after ventricular fibrillation. Short QT syndrome is a ring, sporadic, or autosomal dominant disease that manifests with atrial and ventricular arrhythmias, sudden cardiac death, and shortened QT. Cardiac arrest occurs as the presenting symptom in up to 40% of the cases. Mutations in potassium and calcium channels have been identified as disease causing.

Brugada syndrome

CPVT

Prolonged QT syndrome

Short QT syndrome

sudden cardiac death