Acute severe bioprosthetic aortic regurgitation complicated by cardiogenic shock represents a high-risk clinical scenario requiring urgent intervention. Conventional medical therapy is often insufficient, and options for mechanical circulatory support remain limited because severe aortic regurgitation is traditionally considered a relative contraindication to transvalvular support devices.

Background/Objectives: Centralisation of open abdominal aortic aneurysm (AAA) repair is widely advocated, but supporting evidence often conflates institutional volume with individual surgeon expertise. We report outcomes of open AAA repair at a small- to medium-volume centre and assess the contributions of surgeon experience, multidisciplinary perioperative care, and supervised surgical teaching. Methods: Retrospective analysis of all consecutive patients undergoing open AAA repair between January 2017 and December 2023. Endovascular procedures were excluded. The primary endpoint was 30-day all-cause mortality. Secondary endpoints included perioperative morbidity, acute kidney injury (AKI), length of stay, and overall survival. Results: A total of 195 patients underwent open AAA repair (162 elective; 33 urgent/emergency), with a mean of 27.9 cases annually. Mean age was 70.2 years (SD 8.1), and 85.6% were male. Thirty-day mortality was 1.5% overall (3/195) and 1.2% (2/162) for elective cases, with no significant difference between elective and urgent/emergency cases (1.2% vs. 3.0%; p = 0.430). No perioperative strokes occurred; myocardial infarction occurred in three patients (1.5%). Median operative time was 261 min (IQR 210-320), reflecting systematic supervised teaching in approximately 95% of cases. AKI occurred in 10.3%; clinically significant renal insufficiency at 30 days occurred in 5.1%, and no patient required permanent renal replacement therapy. The perioperative reintervention rate was 9.2%, and median hospital stay was 8 days (IQR 7-12). At a mean follow-up of 62.3 months (SD 25.1), overall mortality was 7.7% and aneurysm-related mortality 1.0%. Kaplan-Meier estimated overall survival was 98.5%, 95.8%, and 89.0% at 1, 3, and 7 years, respectively. Conclusions: Favourable outcomes can be achieved at small- to medium-volume centres through concentrated surgeon expertise, structured teaching, and a high-functioning multidisciplinary team. These findings support a more nuanced assessment of open AAA repair that considers surgeon experience, team performance, and demonstrated outcomes rather than institutional volume alone.

Complex aortic arch branching variants with multiple direct branches and variant vertebral artery origins are rare and typically incidental. We report a postmortem-confirmed case of a rare five-vessel aortic arch configuration identified in a 65-year-old man who underwent elective mitral and tricuspid valve repair for severe mitral regurgitation. The postoperative course was complicated by delayed neurological deterioration and fatal tonsillar herniation. Examination of the thoracic aorta revealed a five-branch aortic arch consisting of the right common carotid artery, left common carotid artery, left vertebral artery arising directly from the arch, left subclavian artery, and an aberrant right subclavian artery with a retroesophageal course. The right vertebral artery originated anomalously from the proximal right common carotid artery. This rare vascular configuration highlights the embryological complexity of multi-branch aortic arch variants and suggests potential implications for vertebrobasilar perfusion during cardiothoracic procedures. Recognition of such variants through careful preoperative imaging of the supra-aortic vessels may help reduce unforeseen perioperative risks.

GNPTAB-related disorders comprise the phenotypes mucolipidosis II (ML II) and mucolipidosis IIIα/β (ML IIIα/β), and phenotypes intermediate between ML II and ML IIIα/β. ML II is evident at birth and slowly progressive; death most often occurs in early childhood. Orthopedic abnormalities present at birth may include thoracic deformity, kyphosis, clubfeet, deformed long bones, and/or dislocation of the hip(s). Growth often ceases in the second year of life; contractures develop in all large joints. The skin is thickened, facial features are coarse, and gingiva are hypertrophic. All children have cardiac involvement, most commonly thickening and insufficiency of the mitral valve and, less frequently, the aortic valve. Progressive mucosal thickening narrows the airways, and gradual stiffening of the thoracic cage contributes to respiratory insufficiency, the most common cause of death. ML IIIα/β becomes evident at about age three years with slow growth rate and short stature; joint stiffness and pain initially in the shoulders, hips, and fingers; gradual mild coarsening of facial features; and normal to mildly impaired cognitive development. Pain from osteoporosis becomes more severe during adolescence. Cardiorespiratory complications (restrictive lung disease, thickening and insufficiency of the mitral and aortic valves, left and/or right ventricular hypertrophy) are common causes of death, typically in early to middle adulthood. Phenotypes intermediate between ML II and ML IIIα/β are characterized by physical growth in infancy that resembles that of ML II and neuromotor and speech development that resemble that of ML IIIα/β.

Coronary-subclavian steal syndrome (CSSS) is an uncommon but clinically important entity that may occur after coronary artery bypass grafting (CABG) with internal mammary artery conduits. Hemodynamically significant proximal left subclavian artery (LSA) stenosis can alter distal branch vessel flow and, in patients with prior left internal mammary artery (LIMA)-based CABG, may raise concern for coronary-subclavian steal physiology. Concurrent vertebral artery flow reversal may further predispose to cerebrovascular insufficiency. We report a case of a 75-year-old male with an extensive cardiovascular history, including prior CABG, transcatheter aortic valve replacement (TAVR), thoracic endovascular aortic repair (TEVAR), and a cardiac implantable electronic device, who presented with dizziness and was found to have severe proximal left subclavian artery stenosis. Computed tomography angiography demonstrated severe (~90%) proximal LSA stenosis, while duplex ultrasonography revealed alternating vertebral artery flow consistent with evolving steal physiology. Invasive hemodynamic assessment confirmed a significant trans-stenotic gradient (>25 mmHg). Given the patient's prior LIMA-based CABG, severe proximal LSA stenosis raised concern for possible coronary-subclavian steal physiology, although direct angiographic confirmation of retrograde LIMA flow was not available. The patient underwent a hybrid endovascular approach utilizing intravascular ultrasound (IVUS)-guided lesion assessment and optimization, drug-coated balloon (DCB) angioplasty, and precise deployment of a balloon-expandable covered stent (GORE VIABAHN VBX; Flagstaff, AZ: W. L. Gore & Associates). The procedure restored antegrade subclavian flow, normalized the trans-stenotic pressure gradient, and preserved the vertebral artery origin. This case demonstrates the technical feasibility of a multimodality endovascular approach for severe proximal LSA stenosis with evolving vertebrobasilar steal physiology in a high-risk post-CABG patient. Integration of IVUS guidance, DCB therapy, and covered stent technology may assist procedural optimization in carefully selected patients, although further studies are required to define the long-term role of this strategy in supra-aortic trunk disease.

Mitral regurgitation (MR) frequently coexists with severe aortic stenosis and may persist or worsen after surgical aortic valve replacement (SAVR) despite afterload reduction. Following SAVR, abrupt ventricular unloading can unmask hyperdynamic ventricular function, promote systolic anterior motion and left ventricular outflow tract (LVOT) obstruction, resulting in dynamic MR and hemodynamic instability. A thorough preoperative assessment of ventricular geometry and mitral valve anatomy is crucial to identify patients at increased risk of post-SAVR LVOT obstruction. We describe a case of severe dynamic MR and LVOT obstruction following SAVR, successfully managed through comprehensive echocardiographic evaluation and tailored hemodynamic optimization, ultimately avoiding unnecessary percutaneous mitral intervention.

Reintervention after truncus arteriosus repair is frequently driven by progressive neoaortic (truncal) valve regurgitation. In many patients, valve dysfunction is primarily associated with annular and root dilatation rather than intrinsic leaflet pathology. We present a 6-year-old child with prior neonatal repair of truncus arteriosus who developed severe truncal valve regurgitation in the setting of marked annular dilatation and quadricuspid truncal valve. The patient underwent truncal valve repair by tricuspidization, annular reduction and root stabilization. Replacement of the right pulmonary valve was performed. Postoperative echocardiography demonstrated excellent truncal valve function with only a trace of regurgitation. At 2-month follow-up, valve function remained stable. This case highlights the importance of annular reduction and valve-preserving strategies in truncal valve surgery.