Introduction. Congenitally corrected transposition of the great arteries (ccTGA) is a rare cardiac malformation characterized by ‘double’ discordance of atrioventricular and ventriculo-arterial connections, resulting in a systemic tricuspid atrioventricular valve and a systemic right ventricle.
Clinical case. We describe a case report of 54 years-old male patient with ccTGA, diagnosed when he was 3 years-old. Patient performed regular follow-up. Since December 2019, the patient reported onset of effort dyspnoea (NYHA III), fatigue and exercise intolerance. Despite medication optimization, his symptoms persisted. He was referred at our Centre for specialist evaluation. Transthoracic echocardiography showed mild dilatation and dysfunction of right ventricle, massive systemic tricuspid valve regurgitation (EROA 42 mm2), due to multiple jets and systolic pulmonary artery pressure of 45 mmHg. As reported by most recent guidelines, indication was given for surgical replacement of the systemic tricuspid valve. However, the patient refused the cardiac surgery option. Transesophageal echocardiography (TEE) screening showed feasibility of percutaneous approach to tricuspid regurgitation. Being the jet central, the strategy was to place one device implant between the septal and the anterior leaflet (9-3 oriented), as central as possible. Following simultaneous leaflet capture and further optimization of leaflet, the result was excellent. Post-implant deployment TEE revealed only mild-to-moderate residual tricuspid regurgitation, with no tricuspid stenosis (mean gradient 2 mm Hg). The result was a percutaneous bicuspidization of a tricuspid systemic valve. The patient recovered quickly and was discharged to home 2 days after the procedure. Patient experienced significant improvement in functional status (NYHA I), exercise capacity and quality of life at 6 months follow-up.
Conclusions. In ccTGA patients, treatment of severe tricuspid regurgitation and improvement of the systemic ventricle’s systolic function is critical since progression of dysfunction of the RV and tricuspid regurgitation are considered key determinants for morbidity and mortality in these patients. Based on the results obtained in our case, percutaneous edge-to edge repair seems to be a reasonable alternative to the surgical approach and three-dimensional transesophageal echocardiography is imperative for the proper visualization of the leaflets outcome of the procedure and his durability.