Fontan operation through a right lateral thoracotomy to treat Cantrell syndrome with severe ectopia cordis

Area of Science:

• Pediatric Cardiac Surgery
• Congenital Heart Disease
• Thoracic Surgery

Background:

• Cantrell syndrome with ectopia cordis presents significant surgical challenges, particularly median sternotomy.
• Tricuspid atresia complicates surgical management in these patients.
• Previous interventions included a left modified Blalock-Taussig shunt and ventriculo-peritoneal shunt closure.

Observation:

• Severe ectopia cordis and sternal defects were noted.
• A defect in the middle and inferior sternum was present.
• The patient had undergone prior surgical procedures.

Findings:

• A right lateral thoracotomy approach was utilized for extracardiac total cavopulmonary connection.
• A right modified Blalock-Taussig shunt was established.
• Coil embolization was performed on the existing left modified Blalock-Taussig shunt.

Implications:

• This surgical strategy may offer a feasible alternative for complex congenital heart defects like Cantrell syndrome.
• Thoracotomy provides an alternative access route when sternotomy is contraindicated.
• Further research is warranted to evaluate long-term outcomes of this approach.