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Orthodontics

422 - Hypoplastic Left Heart Syndrome complicated by Perioperative Hypotension: A Case Study

Saturday, May 23, 2026
3:15 PM - 3:30 PM PST
Has Audio

    Presenting Author(s)

  • Meredith McEachern, DMD

    Pediatric Dental Resident
    University of Michigan, Ann Arbor, MI
    University of Michigan
    Allen Park, Michigan, United States

    • Research Mentor(s)

  • LS

    Larry Salzmann, DDS

    Clinical Associate Professor of Dentistry
    University of Michigan, Ann Arbor, MI
    Ann Arbor, Michigan, United States

    • Program Director(s)

  • JB

    James R. Boynton, DDS, MS

    Program Director
    University of Michigan Dental School
    Ann Arbor, Michigan, United States

Hypoplastic Left Heart Syndrome (HLHS) is a congenital heart defect characterized by the underdevelopment of the left-sided structures of the heart, including the mitral valve, left ventricle, aortic valve, ascending aorta, and aortic arch. Making up about 7-9% of all congenital heart disease, males have been demonstrated to have a greater predilection to develop this condition over females (1.5:1). More importantly, HLHS is responsible for 23% of cardiac deaths during the first week of life, making this condition the leading cause of neonatal mortality.

Given that these children suffer from either an absent or insufficiently-sized left ventricle, this defect leads to a significant deficit in oxygenated blood being pumped into the systemic circulation. Their smaller-sized aorta also places them at high risk for developing aortic valve atresia with atresia/severe stenosis of the mitral valve, leading to overall poor systemic blood perfusion. Atrial septal defects are also common in these children as they serve as nature’s way of compensating for the backlog of oxygenated blood. Due to the mixing of oxygenated and deoxygenated blood, systemic blood saturation is typically diminished to 75-85% within these children.

A multidisciplinary team approach is essential for the successful management of HLHS. The current management strategy of children afflicted with HLHS include: a lifetime follow-up with a cardiologist, a multi-targetted cardiac medication regimen, and surgical intervention when indicated.

As these children bear such a significant cardiac burden, their medical procedures are typically completed under general anesthesia as a means at promoting the safe delivery of their care. However, careful consideration is warranted as these children pose an increased risk for developing complications like cardiogenic shock, cyanosis and arrhythmias during surgery. Therefore, whether for dental or medical treatment under general anesthesia, these children require cross-functional team coordination at minimizing such complications from occurring.

This case report documents the perioperative management of severe hypotension in a child with HLHS requiring dental treatment under general anesthesia.