Hypoplastic Left Heart Syndrome

Recognize: - Cyanosis with minimal oxygen response - Tachypnea and increased work of breathing - Weak pulses and cool extremities - Poor feeding and lethargy - Low urine output - Metabolic acidosis Interpret: - Mixing between the circulations may be inadequate - Circulation may be ductal-dependent and failing as the ductus closes - Systemic blood flow may be insufficient - Pulmonary overcirculation may be stealing systemic output - Cardiac output is falling - The problem is structural rather than isolated pulmonary disease Act: - Notify the physician/NP immediately; call the neonatal resuscitation or rapid response team immediately for shock, apnea or a falling level of responsiveness - Support airway and breathing - Maintain reliable IV access - Prepare alprostadil (prostaglandin E1) if ordered - Prepare for echocardiography - Reduce infant stress by clustering care and limiting agitation - Anticipate transfer and cardiac intervention Monitor: - Oxygen saturation, preductal (right hand) and postductal (either foot) - Cardiac rhythm - Respiratory effort - Pulses and capillary refill - Blood pressure and temperature - Urine output - Lactate and acid-base status - Response...