A CHALLENGING CASE OF SEVERE MITRAL REGURGITATION TREATED WITH MITRAL VALVE REPLACEMENT IN PATIENT WITH REPAIRED TETRALOGY OF FALLOT

Abstract Background Acquired diseases in the adult congenital heart disease (ACHD) population are rarely encountered and their management is challenging due to limited data. Improved survival rates in this subgroup will likely lead to more cases in the future. History A 72–year–old male patient with repaired Tetralogy of Fallot (ToF) was admitted with mild dyspnea (NYHA class II). He had undergone palliative surgery at age 6 with a systemic–pulmonary shunt, followed by complete repair at 26 years old. His medical history also included arterial hypertension and atrial flutter treated with ablation. Trans–thoracic echocardiography (TTE) revealed severe mitral regurgitation due to a flail posterior leaflet from chordal rupture, moderate–to–severe tricuspid regurgitation, mild aortic regurgitation, severe left ventricular hypertrophy, EF 60%, mild right ventricular dilation and PAPs of 48 mmHg. ECG showed sinus bradycardia, LAFB, and RBBB. Trans–esophageal echocardiography (TEE) confirmed severe mitral regurgitation (EROA 0.4 cm²) with flail of posterior leaflet. Characteristics of the valve included MV area › 4 cm², posterior leaflet length › 10 mm, flail gap ‹ 10 mm and mean gradient 1 mmHg. Despite favorable parameters for mitral transcatheter edge–to–edge repair (TEER), the Heart Team decided to proceed with a minimally invasive surgical approach, considering the optimal conditions and the pre–operative angioCT findings. The surgical procedure was performed via a right axillary mini–thoracotomy with cardiopulmonary bypass and intra–aortic balloon clamping (Intraclude – Edwards) with cardioplegic arrest. After an initial attempt to repair the valve, a biological prosthesis (CE Magna Ease n° 31) was implanted since the water test was unsatisfactory due to extensive fibro–elastic deficiency of the valve. The post–operative course was uneventful. TTE showed good bioprosthesis function and moderate tricuspid regurgitation with PAPs of 35 mmHg. The patient was discharged home on the seventh post–operative day, without need forrehabilitation. Discussion Managing acquired degenerative diseases in ACHD patients is difficult due to the absence of standardized risk scores and limited data. The evaluation of the best choice for each patient is based on clinical–instrumental history and the experience of the Centers. Despite the emerging role of TEER, a minimally invasive approach with mitral valve replacement permitted optimal results and fast recovery for this patient.