HCM At times, the first symptom may be SCD The typical murmur or family history should be sought in all other patients presenting with these signs or symptoms to assure that HCM is not overlooked Cardiology should be consulted when HCM is suspected or new symptoms are discovered Anomalous Coronary Arteries can also lead to SCD The most common variation is origin of the anomalous left coronary artery from the pulmonary artery (ALCAPA) Syncope and sudden death may be the presenting symptoms Patients may be asymptomatic until exertion when flow to the coronaries is impaired leading to ischemia, arrhythmia, or sudden death Bland White Garland Syndrome refers to presentation of ALCAPA in an infant around 10 weeks of age When the PVR drops significantly around this age, the anomalous LCA is not perfused and ischemia causes the infant to cry with feeds, develop cardiac wheezing, and respiratory distress CHF and SCD may ensue Some patients are asymptomatic until later in childhood or adolescence These older patients present with chest pain, syncope, or SCD Diagnosing a child with ALCAPA requires a high index of suspicion Clinical assessment should include EKG and echocardiogram in a laboratory skilled in imaging coronary arteries Chest radiograph can be helpful Treat with supplemental oxygen, sedation, and analgesia for chest pain Patients with syncope concerning for cardiac etiology, should have consultation with a cardiologist Consult cardiology for definitive diagnosis About 0.1% of all patients with WPW (i.e., ventricular pre-excitation) will experience SCD The mechanism of death starts with atrial fibrillation, which leads to VF if the atrial fibrillation is conducted rapidly via the accessory pathway to the ventricle Syncope in a patient with WPW is an indication for admission to the hospital for electrophysiology study for risk assessment and/or catheter ablation Generally, the use of digoxin is avoided in these patients CPVT, long QT syndrome (LQTS), and Brugada syndrome are congenital ion channelopathies that render the heart rhythm unstable In pediatric patients with any channelopathy, the first symptom may be sudden death Syncope is abrupt in onset with negligible prodrome and typically occurs during or just after exercise or during acute auditory stimulation (alarm clock) ( Fig 86.11 ) The first presentation of SCD may be syncope or cardiac arrest The EM provider must be vigilant for clues which might suggest aborted SCD rather than benign syncope Early recognition of patients with cardiac syncope or aborted SCD may save the patient, and prevent morbidity and mortality in close relatives by commencing screening evaluations