Endoscopic mitral and tricuspid valve repair in a patient with dextrocardia, hypoplastic right lung, and scimitar syndrome: a case report

The treatment of primary mitral and secondary tricuspid regurgitation (MR, TR) in the setting of lung agenesis poses major technical challenges, and data on the feasibility of endoscopic tricuspid valve repair in adult congenital anatomy remain limited.

We report the case of a 76-year-old male with severe primary MR due to Barlow’s disease, torrential secondary TR, biatrial dilatation, persistent atrial fibrillation, and post-capillary pulmonary hypertension. Anatomical anomalies included dextrocardia, hypoplastic right lung, and scimitar vein syndrome. The patient had a history of surgical correction of an aortic coarctation at the age of 32 with residual stenosis but no proof of residual gradients in invasive assessment. Transcatheter repair was deemed unsuitable due to the massive dextrocardia, extensive leaflet pathology, and annular dilatation. The patient underwent endoscopic mitral and tricuspid repair with dual arterial and venous cannulation to avoid malperfusion of the upper body during extracorporeal circulation. Mitral valve repair included a surgical edge-to-edge repair, along with an implantation of an annuloplasty ring; secondary TR was repaired by ring annuloplasty too. Postoperatively, the patient experienced prolonged weaning and renal support. He was ultimately transferred to rehabilitation for uneventful recovery. Discharge echocardiography revealed good surgical results for the mitral valve and a residual moderate-to severe TR.

The present case highlights that an endoscopic approach for the treatment of mitral and tricuspid valve pathologies can be used in selected patients with dextrocardia. Tricuspid annuloplasty may reduce but not abolish tricuspid regurgitation in a combination of primary, congenital, and secondary aetiologies.