A 76-year-old woman, with a history of secundum atrial septal defect (ASD) patch closure 11 years earlier, presented with loss of appetite and dyspnea. She was treated for heart failure due to aortic regurgitation (AR), mitral regurgitation (MR), tricuspid regurgitation (TR), and atrial fibrillation (Af). Upon transfer to our department, she went into shock, leading to the introduction of V-A ECMO and IABP. Emergency surgeries, including aortic valve replacement (AVR), mitral valve replacement (MVR), tricuspid valve annuloplasty (TAP), and left atrial appendage closure, were performed. A second surgery for hemostasis was necessary, and V-A ECMO was removed on the second postoperative day. Transesophageal echocardiography revealed mitral bioprosthetic valve thrombosis. The patient was treated with heparin and warfarin, resulting in improved pressure gradients and removal of IABP by the seventh day. The sternum was closed on the seventeenth day, and she was transferred to the general ward on the thirty-ninth day. This case demonstrates the effective use of anticoagulant therapy for early valve thrombosis after mitral valve replacement under V-A ECMO.

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.

In aortic surgery involving shaggy aorta, surgical strategy to avoid embolism is crucial for each case. We applied the frozen elephant trunk technique to a patient with shaggy aorta. A 79-year-old man was admitted to our hospital for conservative treatment of acute Type B aortic dissection. Dissecting aneurysms of the aortic arch and descending aorta were shown to have rapidly dilated according to CT three weeks later. Preoperative contrast CT showed an ulcerated shaggy aorta from the aortic arch to the mid portion of the descending aorta. To utilize the benefit of the stent compared with the classical elephant trunk technique, we proposed that the frozen elephant trunk technique would be helpful in prevention of embolism. We therefore planned total arch replacement with the frozen elephant trunk technique and performed thoracic endovascular aortic repair. We employed the frozen elephant trunk technique in the first operation and balloon protection of the superior mesenteric artery and the renal artery in the second operation. The patient had an uneventful postoperative course without thromboembolism. The frozen elephant trunk technique may be helpful for patients with shaggy aorta to avoid thromboembolic events.