Hypertrophic cardiomyopathy (HCM) is a genetic disorder in which left ventricular outflow tract obstruction critically affects symptoms and prognosis. Traditionally, left ventricular outflow tract obstruction was primarily attributed to septal hypertrophy with systolic anterior motion of the mitral valve. However, recent evidence highlights significant contributions from the mitral valve and papillary muscle anomalies, as well as an apical-basal muscle bundle observed in HCM patients. Accurate morphological assessment is essential when considering septal reduction therapy. While transesophageal echocardiography and cardiac magnetic resonance are recommended for assessing the anomalous structures, four-dimensional computed tomography offers superior spatial resolution and multiplanar reconstruction capabilities. These features enable the evaluation of details of the morphological anomalies, such as the apical-basal muscle band, papillary muscle anomalies, subaortic stenosis, and right ventricular outflow tract obstruction. Based on the detailed assessment of these morphological features, four-dimensional computed tomography has been utilized for planning of surgical correction in a comprehensive HCM center. This approach facilitates the intervention strategies and may improve outcomes in septal reduction therapy for obstructive HCM.

Background/Objectives: Robotically assisted mitral valve (MV) surgery is the least invasive surgical approach to the MV. The aim of the present study is to report our experience with robotically assisted MV repair, trying to define how experience could impact on postoperative results. Methods: This is a retrospective study on 144 patients who underwent robotic MV repair from November 2011 to March 2023. Patients were divided in two groups: Group 1, including 39 patients (November 2011-January 2013) operated using the Da Vinci Si system, and Group 2, including 105 patients operated (February 2020-March 2023) using the new Da Vinci Xi system. Results: Mean age was 58 ± 10 years. Increased use of external aortic clamp was observed in Group 2. A significant reduction of surgical times was observed: cardiopulmonary bypass time was 155 ± 44 min in Group 1 and 121 ± 36 min in Group 2 (p = 0.002), whereas cross-clamp time was 112 ± 25 min in Group 1 and 68 ± 39 min in Group 2 (p < 0.001). In-hospital mortality was 0.7%, and 10-year survival was 96 ± 2%. Freedom from reoperation was 100%. A higher percentage of complex and most complex MV repairs were performed in Group 2 (36% in Group 1 vs. 52% in Group 2, p = 0.001). Conclusions: Robotic-assisted MV repair is associated with excellent results. Experience is a key element to overcome the limitations of this technology. Finally, the robotic platform could improve results in difficult MV repair.

BACKGROUND: This case highlights several complications of a late and rare presentation of culture-negative Streptococcus pyogenes endocarditis of a previously repaired mitral valve with an annuloplasty ring including recurrent cardioembolic strokes, which was initially missed on transthoracic echocardiography.
METHODS: A 66-year-old Caucasian female with prior mitral valve prolapse status post mitral valve annuloplasty and left atrial appendage occlusion, followed by two strokes, presented with supraventricular tachycardia that resolved spontaneously. During an inpatient admission, she developed symptoms of another stroke, and imaging studies were suggestive of recurrent cardioembolic phenomenon. Additional workup revealed two small intra-atrial masses adherent to the mitral annuloplasty ring missed on prior evaluation for recurrent stroke. She underwent surgical repair in the setting of a chronic culture-negative infectious endocarditis with Streptococcus pyogenes and recovered well with no further cardioembolic phenomenon.
CONCLUSIONS: This case serves to highlight the importance of having a higher index of suspicion in any cardiac prosthesis patient for endocarditis when presenting with symptoms such as recurrent stroke, arrhythmias, and abnormal cardiac lab work. It also demonstrates the need for appropriate imaging with transthoracic echocardiography followed by transesophageal echocardiography and reviews surgical indications to diagnose and treat culture-negative endocarditis.

UNASSIGNED: Mitral valve (MV) disease including myxomatous degeneration is the most common form of valvular heart disease with an age-dependent frequency. Genetic evidence indicates that mutations of the transcription factor FOXC1 are associated with MV defects, including MV regurgitation. In this study, we sought to determine whether murine Foxc1 and its closely related factor, Foxc2, are required in valvular endothelial cells (VECs) for the maintenance of MV leaflets, including VEC junctions and the stratified trilaminar ECM (extracellular matrix).
UNASSIGNED: Adult mice carrying tamoxifen-inducible, vascular endothelial cell (EC), and lymphatic EC-specific, compound Foxc1;Foxc2 mutations (ie, EC-Foxc-DKO and lymphatic EC-Foxc-DKO mice, respectively) were used to study the function of Foxc1 and Foxc2 in the maintenance of MVs. The EC and lymphatic EC mutations of Foxc1/c2 were induced at 7 to 8 weeks of age by tamoxifen treatment, and abnormalities in the MVs of these mutant mice were assessed via whole-mount immunostaining, immunohistochemistry/RNAscope, Movat pentachrome/Masson Trichrome staining, and Evans blue injection.
UNASSIGNED: EC deletions of Foxc1 and Foxc2 in mice resulted in abnormally extended and thicker MVs by causing defects in the regulation of ECM organization with increased proteoglycan and decreased collagen. Notably, reticular adherens junctions were found in VECs of control MV leaflets, and these reticular structures were severely disrupted in EC-Foxc-DKO mice. PROX1 (prospero homeobox protein 1), a key regulator in a subset of VECs on the fibrosa side of MVs, was downregulated in EC-Foxc1/c2 mutant VECs. Furthermore, we determined the precise location of lymphatic vessels in murine MVs, and these lymphatic vessels were aberrantly expanded and dysfunctional in EC-Foxc1/c2 mutant MVs. Lymphatic EC deletion of Foxc1/c2 also resulted in similar structural/ECM abnormalities as seen in EC-Foxc1/c2 mutant MVs.
UNASSIGNED: Our results indicate that Foxc1 and Foxc2 are required for maintaining the integrity of the MV, including VEC junctions, ECM organization, and lymphatic vessel formation/function to prevent myxomatous MV degeneration.

BACKGROUND: Atrial functional mitral regurgitation (AFMR) is a newly discovered condition associated with longstanding atrial fibrillation. This retrospective study aimed to analyze the outcomes of the maze procedure and mitral regurgitation (MR) surgery in AFMR and atrial fibrillation in comparison with those in degenerative MR (DMR).
METHODS: Patients who underwent mitral valve repair/replacement with a maze procedure at a hospital (July 2012-August 2021) were included. We excluded patients aged below 18 years undergoing concomitant coronary artery bypass grafting or atrial septal defect repair and those with MR etiology other than ARMR or DMR.
RESULTS: We included 35 patients with AFMR and 50 patients with DMR. Patient characteristics and postoperative outcomes were not significantly different between the two groups. Long-term outcomes revealed no significant differences in the ratio of cardiac mortality, stroke, or hospital readmission. However, after the maze procedure, the sinus rhythm restoration rate was significantly lower (62% vs. 28.5%, p < 0.001), a junctional rhythm state (p < 0.001) and permanent pacemaker insertion for sick sinus syndrome (SSS) (p = 0.03) were significantly more common in AFMR than DMR. On postoperative transthoracic echocardiography (TTE), the pulmonary artery systolic pressure was significantly less decreased in the AFMR group than in the DMR group compared with that on preoperative TTE (p = 0.04).
CONCLUSIONS: AFMR showed excellent mitral valve surgery outcomes, similar to DMR, but had a significantly higher risk of pacemaker insertion for SSS after the maze procedure.

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BACKGROUND: Mitral annular calcification (MAC) is a progressive degenerative process associated with comorbidities and increased mortality. A staging system that considers extramitral cardiac damage in MAC may help improve patient selection for mitral valve interventions.
OBJECTIVE: This study sought to develop a transthoracic echocardiogram (TTE)-based cardiac staging system in patients with MAC and significant mitral valve dysfunction and assess its prognostic utility.
METHODS: We retrospectively evaluated all adults who underwent TTE over 1 year at Mayo Clinic with MAC and significant mitral valve dysfunction defined as mitral stenosis and/or at least moderate mitral regurgitation. Patients were categorized into 5 stages according to extramitral cardiac damage by TTE. All-cause mortality and heart failure hospitalization were assessed.
RESULTS: For the 953 included patients, the mean age was 76.2 ± 10.7 years, and 54.0% were women. Twenty-eight (2.9%) patients were classified in stages 0 to 1, 499 (52.4%) in stage 2, 115 (12.1%) in stage 3, and 311 (32.6%) in stage 4. At the 3.8-year follow-up, mortality was significantly higher in patients in stages 2 to 4 compared to stages 0 to 1 and increased with each stage. Survival differences were maintained after adjustment for age, diabetes mellitus, and glomerular filtration rate. The rate of heart failure hospitalization was significantly higher in stages 3 and 4 compared to stages 0 to 1. Similar results were observed in subgroup analysis in patients with moderate or severe MAC, predominant mitral stenosis, or predominant mitral regurgitation.
CONCLUSIONS: Using the proposed extramitral cardiac damage staging system in patients with MAC and significant mitral valve dysfunction, more advanced stages are associated with higher mortality.