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.

A 76-year-old female was admitted with complaints of dyspnea on exertion and lower leg edema. She had undergone an aortic valve replacement thirty-nine years before and a redo aortic valve replacement and mitral valve replacement twenty-eight years before. She also had hemolytic anemia with jaundice. Echocardiography showed severe paravalvular leakage in the aortic and mitral valves, and a blood flow in the aortic annulus that flows from the aortic side into the left atrium. We diagnosed heart failure and hemolytic anemia due to paravalvular leakage and decided to perform a double-valve replacement for the third time. On operation, after removing the aortic valve through aortotomy, aorto- mitral fibrous continuity was extensively calcified and perforated, and its strength was not enough to sew the prosthetic valve to it. Therefore, we decided to perform the Commando procedure. Aortotomy was extended between the noncoronary aortic sinus and the left coronary aortic sinus until it reached the dome of the left atrium. After the prosthetic mitral valve was excised, annuloplasty of the posterior mitral annulus was performed using a bovine pericardial patch, and the new prosthesis mitral valve was implanted. The anterior part of the annulus corresponding to the aorto-mitral fibrous continuity was reconstructed by sewing the base of a two-tongued triangular bovine pericardial patch to the sewing cuff of the mitral prosthesis. After closing the left atrial ceiling with the posterior patch, the aortic prosthesis was secured to the aortic annulus and the pericardial patch. The anterior patch was used to close the right side of the aortotomy. The postoperative course was uneventful, and postoperative echocardiography revealed no paravalvular leakage.

Primary aldosteronism (PA) is the most common cause of secondary hypertension, and is associated with an increased incidence of cardiovascular events. PA itself is clinically classified into the following two types: unilateral PA, mostly composed of aldosteroneproducing adenoma (APA); and bilateral hyperaldosteronism, consisting of multiple aldosterone-producing micronodules (APMs) and aldosterone-producing diffuse hyperplasia. Histopathologically, those disorders above are all composed of compact and clear cells. The cellular morphology in the above-mentioned aldosterone-producing disorders has been recently reported to be closely correlated with patterns of somatic mutations of ion channels including KCNJ5, CACNA1D, ATP1A1, ATP2B3, and others. In addition, in non-pathological adrenal glands, APMs are frequently detected regardless of the status of the renin-angiotensin-aldosterone system (RAAS). Aldosterone-producing nodules have been also proposed as non-neoplastic nodules that can be identified by hematoxylin and eosin staining. These non-neoplastic CYP11B2-positive nodules could represent possible precursors of APAs possibly due to the presence of somatic mutations. On the other hand, aging itself also plays a pivotal role in the development of aldosterone-producing lesions. For instance, the number of APMs was also reported to increase with aging. Therefore, recent studies indicated the novel classification of PA into normotensive PA (RAAS-independent APM) and clinically overt PA.