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Published on: February 11, 2022
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.
Abstract:
Left ventricular non-compaction is a myocardial disorder characterized by excessive trabeculations and deep recesses that communicate with the ventricular cavity, which is thought to result from a failure of the trabecular regression that occurs during normal embryonic development. It carries a high mortality from heart failure or sudden cardiac death. A 15-year-old female patient was referred to our institution for moderate symptoms of heart failure. Echocardiography and MRI showed a bicuspid aortic valve with severe regurgitation, subaortic VSD, dilated left ventricle and left ventricular non-compaction with a moderately decreased ejection fraction, as well as isthmic coarctation and transverse arch hypoplasia. We elected to perform transaortic VSD closure and aortic valve replacement using a mechanical prosthetic valve on an arrested heart, and to address aortic coarctation and transverse arch hypoplasia using an extra-anatomic ascending-to-descending aorta bypass. Aortic cross-clamping was limited to 41 minutes. The postoperative recovery was rapid and the girl was discharged in NYHA class I with an estimated LVEF of 39%. Although management must be individualized, extra-anatomic bypass is a good single-stage approach for patients with complex coarctation and concomitant cardiovascular or myocardial disorders, reducing ischemic time and offering a better chance of successful weaning from cardiopulmonary bypass.
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