Brexley's Story
Case Summary: Shone’s Complex
The Ward Family Heart Center at Children's Mercy provides comprehensive care for children with all types of heart conditions. Our team of 35 pediatric cardiologists and heart surgeons, together with specially trained nurses and staff members, work tirelessly to give your patient the best possible care.
Built from the heart, our dedicated team is committed to collaborating with you to alleviate the time and resource challenges associated with complex cardiology cases. From clinical and financial to social, psychological, and emotional support, together we can ensure top-tier patient care.
Read how our heart team at Children’s Mercy came together to help Brexley:
Patient Background:
Brexley, an 18-day-old infant, presented with acute respiratory arrest while in transit in Kiowa County, Kansas. Immediate resuscitation efforts were initiated by her parents, Gracie Gray and Kahl Huffman, followed by successful infant CPR performed by Master Trooper Evan Jacks of the Kansas Highway Patrol.
Initial Emergency Response:
Trooper Jacks, leveraging recent personal experience with infant CPR, managed to restore Brexley’s consciousness. The incident received national media coverage, highlighting the critical role of timely intervention.
Hospital Transfers and Diagnosis:
Post-resuscitation, Brexley was initially stabilized at a local hospital before being transferred to a medical center in Wichita. An echocardiogram revealed significant cardiac anomalies, necessitating further transfer to the Neonatal Intensive Care Unit (NICU) at Children’s Mercy.
Diagnosis:
Brexley was diagnosed with Shone’s Complex, characterized by multiple obstructive lesions in the left side of the heart.
Specific findings included:
Aortic valve stenosis
Hypoplastic aortic arch
Aortic coarctation
Pathophysiology: The patient’s initial asymptomatic presentation was attributed to a patent ductus arteriosus (PDA), which temporarily compensated for the obstructive lesions. The closure of the PDA precipitated the cardiac arrest due to inadequate systemic perfusion.
Interventions:
Balloon Aortic Valvuloplasty:
Performed by Dr. Ryan Romans to alleviate aortic valve stenosis.
Objective: Improve cardiac function to facilitate subsequent surgical interventions.
Aortic Arch Reconstruction:
Conducted by Dr. Edo Bedzra without cardiopulmonary bypass or hypothermia, minimizing procedural risks.
Outcome: Successful repair with no residual obstruction.
Postoperative Course: Brexley’s postoperative recovery included a hospital stay for weight gain and blood pressure stabilization. She was discharged in early June.
Follow-Up and Prognosis: Brexley continues to be monitored biweekly by her cardiologist. Long-term management will involve surveillance for potential re-interventions, common in patients with Shone’s Complex.
Family Adaptation: The patient’s family has demonstrated remarkable adaptability to their new medical reality, maintaining close communication with healthcare providers and support networks.
Conclusion: Brexley’s case underscores the importance of early detection, timely intervention, and comprehensive multidisciplinary care in managing complex congenital heart disease. The collaborative efforts of emergency responders and specialized cardiac teams were pivotal in her positive outcome.