CLINICAL PEARLS AND PITFALLS Recognition of the most common presentation patterns for patients with CHD will guide appropriate therapy CHD should be considered in any neonate presenting with acute decompensation Evaluate femoral pulses in all newborns and infants to detect coarctation of the aorta Palliation or incomplete repair of a congenital heart defect in an infant is a red flag for a patient who may not tolerate other stressors Pediatric patients with cardiac disease often present nonspecific, gastrointestinal, or respiratory symptoms Endotracheal intubation and mechanical ventilation can significantly decrease cardiac demands Current Evidence Congenital heart malformations are the most common birth defects, affecting nearly 1% or approximately 40,000 live births/yr in the United States Nearly half of the deaths from CHD occur in the first year of life Approximately 15% of patients with CHD also have chromosomal abnormalities and 29% with CHD have other major noncardiac malformations This is a complex patient population who often seek medical care in the emergency department (ED) All forms of CHD occur along a spectrum For example, tetralogy of Fallot (TOF) may range from complete pulmonary atresia to very mild pulmonary stenosis, or absent pulmonary valve (no stenosis) Therefore, a newborn with TOF can present with fulminant cyanosis and shock or mild cyanosis or even congestive heart failure (CHF) It is not necessary to know the exact anatomical defect but rather to recognize the cardiac physiology based on vital signs, age, and symptoms Anatomical details will be defined by echocardiogram Full information is not necessary for recognition of common patterns of presentation and stabilization in the ED Clinical Considerations Clinical Recognition Many times when an infant or child presents to the ED, the diagnosis is not evident immediately and management is initiated without complete certainty of the underlying pathology This is especially true with CHD However, a