2025 Volume 54 Issue 6 Pages 276-279
Loeys-Dietz syndrome (LDS) is a rare genetic disorder characterized by systemic connective tissue abnormalities. Among its subtypes, LDS type 3 is associated with SMAD3 gene mutations and often presents with vascular and skeletal abnormalities. Narrow chest is a relative contraindication for minimally invasive cardiac surgery (MICS), yet this approach can be advantageous in connective tissue disorders where repeated surgeries may be anticipated. A 63-year-old woman with a previously unreported SMAD3 variant was diagnosed with LDS type 3. She presented with severe mitral regurgitation due to A2-3 prolapse. Her skeletal features included a narrow chest (anteroposterior diameter: 5 cm), scoliosis, and pectus excavatum. Totally endoscopic 3D mitral valve plasty was performed via a right minithoracotomy using a 2-port, 1-window approach. Mitral repair was successfully completed using artificial chordae and ring annuloplasty. Adequate exposure was achieved despite the narrow chest by retracting the pericardium and displacing the aorta using gauze packing. The mitral valve was clearly visualized using the 3D endoscopic camera, allowing safe repair of the A2-3 prolapse with four artificial chordae and a 29-mm Tailor ring. The patient was extubated 3.5 hours postoperatively and had an uneventful recovery except for transient atrial fibrillation. She was discharged on postoperative day 13 in sinus rhythm. Totally endoscopic MICS-MVP is feasible and beneficial even in patients with challenging thoracic anatomy due to connective tissue disease. It enables chest wall preservation and minimizes surgical trauma, which is particularly advantageous for LDS patients with lifelong surgical risk.
