High risk and low incidence diseases: Acyanotic congenital heart disease

Critical acyanotic congenital heart lesions are uncommon but potentially life-threatening causes of neonatal shock and cardiovascular collapse.

This review highlights key aspects of acyanotic CHD, including presentation, diagnosis, and management in the emergency department (ED), based on current evidence.

Acyanotic CHD encompasses a number of conditions, including critical coarctation of the aorta (CoA), interrupted aortic arch (IAA), critical aortic stenosis (AS), and anomalous left coronary artery from the pulmonary artery (ALCAPA). These conditions should be considered in critically ill neonates presenting with shock and cardiovascular collapse, often without prominent cyanosis. Evaluation involves a thorough assessment of the patient's cardiovascular and respiratory status. Diagnostic tools include pulse oximetry, laboratory testing, electrocardiogram, chest radiograph, and ultrasonography. Management requires prompt recognition and hemodynamic stabilization, which may involve the administration of prostaglandin, vasoactive/inotropic agents, and airway management. Expert consultation and coordination of transfer to a tertiary or quaternary care center are crucial. Surgical repair serves as the definitive treatment.

A thorough understanding of acyanotic congenital heart disease is essential for emergency clinicians to effectively diagnose and manage these potentially life-threatening conditions.