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: Minimally invasive approaches for combined aortic valve and ascending aortic surgery remain challenging. We report a case of utilizing a transaxillary approach (TAX) in combination with the RAM[®] system. Methods: A 65-year-old male presented with exertional angina and palpitations. He reported a one-year history of dizziness. Diagnostics revealed severe aortic regurgitation due to a calcified bicuspid aortic valve and an ascending aortic aneurysm measuring 57 × 54 mm. Left ventricular ejection fraction was reduced to 42%. Coronary artery disease was excluded. Surgery was performed via a right transaxillary mini-thoracotomy (3rd intercostal space) using endoscopic visualization. Cardiopulmonary bypass was established through femoral cannulation. After aortic cross-clamping and cardioplegic arrest, the bicuspid valve was excised and replaced with a bioprosthesis. A supracoronary ascending aortic replacement was performed using a Dacron graft. The RAM[®] system was used for annular suturing and proximal anastomosis, with automated fastener fixation. Results: The patient was extubated on postoperative day 1 and transferred to intermediate care on day 2. Postoperative recovery was uneventful, with no neurological deficits, bleeding, or other complications. Discharge occurred on postoperative day 9 in stable condition. Conclusions: This case highlights the feasibility and safety of a minimally invasive transaxillary approach for combined aortic valve and ascending aortic replacement using the RAM[®] system. This technique may expand the surgical armamentarium for complex aortic pathology while avoiding sternotomy. Further studies are required to evaluate reproducibility, long-term outcomes, and broader applicability.

Post-infarction left ventricular aneurysm is an uncommon but severe mechanical complication of transmural myocardial infarction, particularly in patients with delayed presentation or incomplete myocardial salvage. It may lead to profound distortion of left ventricular geometry, adverse remodelling, intraventricular thrombosis, mitral regurgitation, pulmonary hypertension, and advanced heart failure. We report the case of a 65-year-old male patient referred two months after a late-presenting anterior ST-segment elevation myocardial infarction caused by proximal occlusion of the left anterior descending coronary artery. At admission, the patient presented with severe decompensated heart failure, low-output status, multiorgan dysfunction, and a left ventricular ejection fraction of 12%. Transthoracic echocardiography and cardiac magnetic resonance imaging demonstrated a giant apical left ventricular aneurysm involving approximately 75% of the ventricular cavity, partial intraluminal thrombosis, extensive transmural scarring in the left anterior descending territory, and imaging features suggestive of a chronic contained free-wall rupture/pseudoaneurysmal component. Following multidisciplinary evaluation, the patient underwent surgical ventricular reconstruction using an endoventricular circular restoration technique guided by an intraventricular balloon sizer, combined with left internal thoracic artery bypass grafting to the left anterior descending artery. The early postoperative course required temporary inotropic, vasopressor, inhaled nitric oxide, and intra-aortic balloon pump support, followed by progressive haemodynamic recovery. The patient was discharged on postoperative day seven with functional improvement to NYHA class II. At six-month follow-up, he remained clinically stable without overt signs of heart failure, and echocardiography showed preserved ventricular geometry and improvement of left ventricular ejection fraction to 45%. This case highlights the potential role of carefully planned, balloon-guided surgical ventricular reconstruction in selected patients with giant post-infarction left ventricular aneurysms and end-stage heart failure when residual viable myocardium is present.

Background: It is known that mitral valve surgery produces an early reduction in hemodynamic overload after the procedure. However, the reduction in left ventricular (LV) ejection fraction (EF) may persist over the long term. Speckle-tracking echocardiography (STE) provides more sensitive indices of cardiac performance in MR. Myocardial work (MW) evaluates LV performance by considering the effect of LV afterload. The aim of this study was to describe the acute and late postoperative changes in cardiac function and hemodynamics using STE and MW indices in patients with primary MR and to find echocardiographic predictors of postoperative symptom improvement. Methods: Consecutive patients with severe MR and preserved LV EF undergoing mitral valve repair or replacement were prospectively enrolled. Exclusion criteria were atrial fibrillation, previous cardiac surgery, and concomitant aortic or coronary surgery. Patients underwent clinical, biohumoral and echocardiographic evaluation before surgery and within one week after surgery during the hospitalization period. Then, 32 of them underwent a new echocardiographic exam, completed by MW, at long-term follow-up. Results: Overall, 42 patients were enrolled (mean age 65 ± 12 years, 50% male, mean LV EF 59 ± 4%). After intervention, 48% of patients showed a reduction in LV EF (n = 20), while most patients showed reductions in STE and MW parameters across all chambers. Particularly, the global work index (GWI) was reduced in 79% of patients (n = 33). After a mean long-term follow-up of 26 ± 5 months, all STE parameters significantly improved in >70% of patients (n = 22), while LV EF maintained its initial reduction. A baseline value of LV GLS > -17.7% identified patients with persistence of symptoms at long-term follow-up (AUC 0.68, p = 0.02). Conclusions: After an initial decline, LV function detected by GLS improved over the long term after mitral valve surgery, paralleling a reduction in symptoms. STE indices might provide additional value for the long-term follow-up of patients undergoing mitral valve repair/replacement.

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.

Valve-sparing aortic root replacement (VSRR) requires coronary button reimplantation, and anomalous coronary anatomy may increase the risk of unexpected coronary malperfusion. A 38-year-old man with Marfan syndrome underwent VSRR for aortic root aneurysm and severe aortic regurgitation. The right coronary artery arose anomalously from the left sinus of Valsalva. Despite apparently satisfactory reconstruction, severe myocardial ischemia developed after coronary reimplantation during weaning from cardiopulmonary bypass. Intraoperative reassessment revealed no obvious kinking or obstruction of the right coronary artery, and bougie passage confirmed luminal patency. Because the mechanism of malperfusion remained unclear, bailout coronary artery bypass grafting (CABG) was performed. Hemodynamics stabilized promptly, and postoperative imaging confirmed graft patency. Geometric alteration of a reimplanted coronary artery may result in dynamic malperfusion even when reconstruction appears technically satisfactory. When persistent ischemia occurs without an identifiable anatomical cause, early bailout CABG may provide a practical treatment option.