Complex aortic coarctation, regurgitant bicuspid aortic valve with VSD and ventricular non-compaction: a challenging

P O Myers1, C Tissot, M Cikirikcioglu

  • 1Division of Cardiovascular Surgery, Geneva University Hospitals and Medical School, Geneva, Switzerland. patrick.myers@hcuge.ch

Insights

Left ventricular non-compaction, a heart muscle disorder, requires complex surgical repair. An extra-anatomic bypass effectively treated a teen with coarctation and non-compaction, improving heart function.

Area of Science:

  • Cardiovascular Medicine
  • Pediatric Cardiology
  • Cardiac Surgery

Background:

  • Left ventricular non-compaction (LVNC) is a congenital myocardial disorder associated with high morbidity and mortality from heart failure and sudden cardiac death.
  • Complex congenital heart defects, including bicuspid aortic valve, ventricular septal defect (VSD), and aortic coarctation, often require multi-stage surgical interventions.

Observation:

  • A 15-year-old female presented with moderate heart failure symptoms.
  • Diagnostic imaging revealed severe aortic regurgitation, subaortic VSD, dilated left ventricle, LVNC with moderately reduced ejection fraction, isthmic coarctation, and transverse arch hypoplasia.

Findings:

  • A single-stage surgical approach was performed, including transaortic VSD closure and aortic valve replacement with a mechanical prosthesis.
  • Aortic coarctation and hypoplasia were addressed simultaneously using an extra-anatomic ascending-to-descending aorta bypass, limiting aortic cross-clamping time to 41 minutes.
  • The patient experienced rapid postoperative recovery, discharged with improved heart function (estimated LVEF 39%) and in NYHA class I.

Implications:

  • Extra-anatomic bypass is a viable single-stage strategy for complex coarctation combined with other cardiovascular or myocardial disorders.
  • This approach can reduce ischemic time, facilitate cardiopulmonary bypass weaning, and improve outcomes in pediatric patients with complex congenital heart disease.
  • Individualized management is crucial, but this technique offers a promising option for complex cases.

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