The first successful double switch operation for congenitally corrected transposition of the great arteries in Slovenia

Congenitally corrected transposition of the great arteries is a rare congenital heart defect characterized by atrioventricular and ventriculoarterial discordance and can be potentially associated with several other concomitant anomalies, such as ventricular septal defect or congenital complete atrioventricular block. Different surgical options are used for treating the condition. Anatomic repair yields the best long-term outcomes; however, it is technically the most challenging. We present a case of a 3-year old female patient who was electively admitted for complete repair after pulmonary artery banding and pacemaker implantation soon after birth. Subsequently, an anatomic repair (double switch) was performed with an uneventful postoperative course and excellent mid-term outcomes.

844 A Case of Pulmonary Artery Banding for Future Anatomical Repair in Congenitally Corrected Transposition of the Great Arteries (ccTGA)

This is a case of a patient prenatally diagnosed with ccTGA. Immediately after birth, postnatal transthoracic echocardiography diagnosed ccTGA with concomitant Ebstein’s anomaly of the tricuspid valve, without a ventricular septal defect (VSD). Initially, this dysplastic tricuspid valve had mild tricuspid regurgitation. However, by 12 months follow up, the patient was becoming symptomatic, and their tricuspid regurgitation had become severe. Due to the patients worsening symptoms and regurgitation, clinical intervention was pursued. The patient had Cardiovascular Magnetic Resonance performed to assess for myocardial fibrosis and to measure the size of each ventricle. After imaging and MDT discussion, the patient was deemed suitable for a double switch operation, a form of anatomical repair. However, since this patient did not have pulmonary stenosis (PS), they required a pulmonary artery (PA) band before anatomical repair. This procedure has dual benefits: to retrain the morphological left ventricle (mLV) to cope with systemic pressures, and to induce a septal shift, which can reduce the severity tricuspid regurgitation. At 18 months of age, this patient had their PA band placed. The patient immediately had a reduction in the severity of their tricuspid regurgitation. In the future, this patient will have cardiac catheterisation performed to measure mLV pressures and assess when the patient is ready for anatomical repair. The morphology of this patient is rare, as most patients with ccTGA have an associated VSD. This case describes a potential management pathway for a ccTGA patient without VSD and an Ebstenoid tricuspid valve. It also demonstrates the benefits of PA banding in a ccTGA patient with this morphology. This is a unique contribution, due to the rarity of this specific morphology and the limited evidence and guidance for its successful management.