2026年 · 第06卷第01期

Objective: To explore the risk factors for bird-beak configuration occurrence and its outcomes after thoracic endovascular aortic repair (TEVAR) for Stanford type B aortic dissection (TBAD). Methods: A total of 701 patients with TBAD diagnosed by thoracic aortic computed tomography angiography and treated using TEVAR from January 2010 to December 2022 were retrospectively enrolled. According to the occurrence of a bird-beak configuration in the proximal end of the stent-graft, the patients were divided into a bird-beak group (n = 291) and a non-bird-beak group (n = 410). The baseline and perioperative data, the incidence of clinical adverse events during follow-up were compared between the 2 groups. Multivariate logistic regression was performed to analyze the risk factors and prognosis associated with the formation of the bird-beak configuration. A sub-analysis was further undertaken within the bird-beak group based on the presence (n = 84) or absence (n = 207) of an immediate intraoperative endoleak. The Kaplan-Meier method was used to analyze the incidence of events in the bird-beak subgroup. Results: Baseline analysis showed that compared with the non-bird-beak group, the bird-beak group comprised significantly more men, and was associated with a higher frequency of calcium channel blocker use, but a lower proportion of stroke history (all P < 0.05). The perioperative data demonstrated that the bird-beak group received longer stents, had a higher percentage of proximal landings in aortic Zone 2, and exhibited a greater proportion of immediate intraoperative endoleaks (all P < 0.05). The distal stent diameter and the proportion of proximal bare stents were both significantly smaller in the bird-beak group than in the non-bird-beak group (P < 0.05). Multivariate logistic regression suggested that proximal landing in aortic Zone 2 (odds ratio (OR) = 1.839, 95% confidence interval (CI): 1.133-2.985; P = 0.014) and stent length (OR = 1.013, 95% CI: 1.002-1.025; P = 0.021) were independent risk factors for the formation of the bird-beak configuration, whereas the use of proximal bare stents (OR = 0.019, 95% CI: 0.010-0.039; P < 0.001) was an independent protective factor. Short- and long-term follow-up revealed no significant differences in the incidence of aortic-related or overall clinical adverse events between the 2 groups (all P > 0.05). In the subgroup analysis, the incidence of distal stent-graft-induced new entry, new endoleak, aortic-related adverse events, and overall clinical adverse events was higher in the immediate intraoperative endoleak group than in the non-immediate intraoperative endoleak group (all P < 0.05). Kaplan-Meier analysis further demonstrated that the immediate intraoperative endoleak group displayed a significantly higher incidence (Plog-rank = 0.005) of aorta-related adverse events and a greater overall incidence of clinical adverse events than the non-immediate intraoperative endoleak group (Plog-rank = 0.031). Conclusion: During TEVAR for TBAD, proximal landing in aortic Zone 2 and stent length are independent risk factors for bird-beak configuration formation, whereas proximal bare stent use serves as a protective factor. While the bird-beak configuration is associated with the occurrence of immediate intraoperative endoleak during TEVAR, it does not exert a significant adverse effect on the prognosis of patients with TBAD. However, the bird-beak configuration combined with an immediate intraoperative endoleak is associated with worse outcomes.

Objective: This study aimed to investigate the etiology, clinical consequences, and cardiovascular burden of pulmonary hypertension (PH) in patients with chronic kidney disease (CKD), particularly in severe CKD and end-stage renal disease. In addition, potential diagnostic markers were identified. Methods: In this single-center cross-sectional study, CKD patients (stage 3b–5) from the First Affiliated Hospital of Xi’an Jiaotong University were randomly matched 1:1 based on age, sex, and CKD stage between January 2020 and December 2022. Clinical and laboratory data—including blood cell counting, blood lipids, liver and kidney function, blood coagulation, N-terminal pro B-type natriuretic peptide, and troponin T—were collected from the Unified Digital Medical Record system. Additionally, transthoracic echocardiography and electrocardiogram results were collected upon patient admission. PH severity was classified as mild, moderate, or severe based on echocardiographic findings, and subgroup analyses were performed according to this classification. Results: A total of 440 CKD patients were enrolled with 220 patients diagnosed with PH (PH group) and 220 without PH (non-PH group). PH patients exhibited significantly lower red blood cell counts (2.76 (2.39, 3.20) × 1012/L vs. 3.14 (2.67, 3.73)×1012/L, P < 0.001), hemoglobin levels (82 (71, 93) g/L vs. 95 (79, 109) g/L, P < 0.001), and higher D-dimer (1.31(0.74, 2.75) mg/L vs. 0.83 (0.50, 1.47) mg/L, P < 0.001). Cardiac biomarkers, including N-terminal pro B-type natriuretic peptide (23,705 (6,809, 35,000) ng/L vs. 2,644 (665, 10,818) ng/L, P < 0.001) and troponin T (0.070 (0.032, 0.134) μg/L vs. 0.031 (0.013, 0.066) μg/L, P < 0.001), were markedly elevated in the PH group. Echocardiographic findings revealed significant right ventricular enlargement (29 (26, 32) mm vs. 27 (25,29) mm, P < 0.001) and a higher prevalence of pericardial effusion (62.7% vs. 25.0%, P < 0.001) in PH patients. Cardiac conduction abnormalities were more prevalent in PH patients, with significantly higher rates of atrial fibrillation, supraventricular premature beats, and right bundle branch block (all P < 0.05). There are 109 patients with mild PH, 83 with moderate PH, and 28 with severe PH. Left ventricular remodeling and functional impairment were observed in moderate and severe PH patients. Conclusion: Progressive PH in CKD patients is associated with significant cardiovascular abnormalities. Routine echocardiographic surveillance and biomarker monitoring are crucial for early risk stratification and management. Further research is needed to explore targeted therapies to improve outcomes in this high-risk population.

Objective: Diagnosing cardiac sarcoidosis (CS), giant cell myocarditis (GCM), and arrhythmogenic right ventricular cardiomyopathy (ARVC) is difficult because all 3 cardiomyopathies can involve both the right and left ventricles, leading to arrhythmias and heart failure. Notably, the extent and pattern of late gadolinium enhancement (LGE) on cardiac magnetic resonance (CMR) for each condition remain poorly characterized which further complicates their differentiation. This study aimed to evaluate the diagnostic efficacy of CMR parameters to improve the accuracy of their differential diagnosis. Methods: Twelve patients with CS, 8 with GCM, and 30 with ARVC in Fuwai Hospital who underwent CMR between July 2011 and May 2024 were retrospectively analyzed in this study. Cardiac structure, function, and scar quantification were performed on a post-processing software. LGE in the left ventricle was visually classified by layer: subepicardial, intramural, transmural, and subendocardial. Left ventricle segmentation followed the American Heart Association 17-segment model. Results: Patients with CS had the greatest left ventricular mass compared to those with GCM or ARVC (111.2 (84.7, 155.8)g vs. 77.3 (63.6, 103.5)g vs. 64.4 (54.3, 81.4)g, P = 0.002). Meanwhile, those with ARVC had the largest right ventricular dimension and outflow tract (P < 0.001), along with the most extensive right ventricular global longitudinal strain impairment compared with the CS or GCM group (-6.1% ± 4.9% vs. -14.8% ± 7.3% vs. -11.9% ± 4.9%, P < 0.001). CS patients presented with more LGE in the insertion area and less LGE in the right ventricular free wall than GCM and ARVC (all P < 0.001). The left ventricular layers showing the greatest vulnerability varied by condition: subepicardial for CS, subendocardial for GCM, and transmural for ARVC (all P < 0.02). Patients with CS and GCM presented with more extensive LGE than those with ARVC in the septal and anterior wall segments at the basal (segments 1–3) and mid-cavity (segments 7 and 8) levels of the left ventricle (all P < 0.05). However, at the apical level, patients with CS had less fibrotic involvement in segment 14 than patients with GCM or ARVC (P = 0.039). Conclusion: The 3 cardiomyopathies differed in the pattern and extent of lesion involvement. Insertion point enhancement favors a diagnosis of CS. More extensive right ventricular transmural LGE should prompt consideration of GCM or ARVC. At the basal and mid-cavity levels, the extent of LGE in anteroseptal segments was significantly reduced in patients with ARVC relative to that in patients with CS or GCM.

Endocytic adaptor proteins are essential regulators of intracellular trafficking, coupling receptor internalization with downstream signaling. Recent discoveries have deepened our understanding of how endocytic adaptor proteins contribute to both physiological and pathological processes in the vascular system. This review summarizes the emerging roles of key adaptor proteins—such as epsins, disabled-2, and adaptor protein 2—across multiple vascular cell types, including endothelial cells, vascular smooth muscle cells, and macrophages. These adaptors have diverse but essential functions, ranging from regulating angiogenic signaling and maintaining endothelial barrier integrity to modulating inflammatory responses, vascular smooth muscle cells phenotypic switching, lipid uptake, and structural remodeling of blood vessels. We also highlight advances in therapeutic strategies, such as nanomedicine, viral expression vectors, DNA nanostructures, and small molecule inhibitors that target endocytic adaptors and their function. Understanding the diverse and cell-specific roles of endocytic adaptor proteins is important for gaining insights into vascular disease mechanisms and uncovering novel targets for therapeutic interventions.

The prevalence of advanced heart failure (HF) is increasing with the aging population, with a high mortality rate despite guideline-directed medical therapy. Mechanical circulatory support and heart transplantation (HTx) are definitive treatments for patients with advanced HF. Temporary mechanical circulatory support devices, such as intra-aortic balloon pumps and extracorporeal membrane oxygenation, can be used to stabilize acute cases, while third-generation left ventricular assist devices, such as the HeartMate 3, provide durable support with reduced complications. However, HTx remains the gold standard, offering a median survival exceeding 15 years, although donor shortages limit its availability. Innovations in donor utilization and xenotransplantation aim to address this gap. Furthermore, timely referral, risk stratification, and multidisciplinary care are crucial to optimize outcomes. Continued advancements in mechanical circulatory support and HTx promise improved survival and quality of life for patients with advanced HF. This review highlights the evolving roles of mechanical circulatory support and HTx in the management of advanced HF, emphasizing timely intervention, individualized treatment, and ongoing innovation to improve patient outcomes.

Heart failure (HF) remains a leading cause of morbidity and mortality despite advances in guideline-directed medical therapy. Established pharmacological pillars—including angiotensin receptor-neprilysin inhibitors, beta blockers, aldosterone antagonists, and sodium-glucose cotransporter-2 inhibitors—have substantially improved outcomes in HF with reduced ejection fraction. However, significant therapeutic gaps persist, particularly in HF with preserved ejection fraction and HF with mid-range ejection fraction, with 30%–40% of patients with HF with reduced ejection fraction remaining symptomatic despite optimal therapy. This comprehensive review summarizes recent evidence on emerging HF therapeutics, examining 7 distinct therapeutic domains: myosin/sarcomere activators; mitochondrial-metabolic modulators; phosphodiesterase-9 inhibitors; anti-inflammatory/immunomodulatory agents; gene/RNA-based therapeutics; anti-fibrotic matrix modulators; and natriuretic receptor agonists. For each domain, a systematic analysis was performed of the mechanistic rationale, preclinical validation, clinical trial evidence, developmental stage, and translational barriers. Integration of these emerging therapies into precision medicine frameworks targeting specific pathophysiological phenotypes offers potential to further reduce morbidity and mortality associated with HF. This review emphasizes both scientific promise and practical barriers to clinical translation, providing a critical perspective on drug development trajectories and realistic prospects for clinical implementation in this rapidly evolving therapeutic landscape.

Subaortic stenosis is a form of left ventricular outflow tract obstruction, accounting for approximately 14% of cases. It occurs as an acquired lesion after repair of congenital heart disease and rarely following mitral valve repair or replacement. A 40-year-old female presented with dyspnea on minimal exertion, palpitations, and chronic hemolytic anemia. Her medical history included cardiac surgery at the age of 10 for closure of an atrial septal defect and mitral valve repair. Then, at the age of 28, she underwent another cardiac surgery to resect a subaortic stenosis, which recurred within one year. This recurrence led to a redo surgery involving mitral valve replacement with a 25-mm St. Jude prosthesis. Subsequent investigations demonstrated membranous subaortic stenosis with severe aortic valve insufficiency and partial de-insertion of the mitral valve with a small atrial septal defect. Management aimed to eliminate all systemic and structural factors contributing to increased flow in the left ventricular outflow tract, which are known to play an important role in the recurrence of subaortic stenosis. For this purpose, the patient underwent the fourth cardiac surgery involving aortic valve replacement, membrane resection, repair of the leak around the mitral valve, and atrial septal defect closure. She recovered within 2 months after surgery and resumed her job. During a 6-year follow-up period, she remained in good health with no evidence of cardiac dysfunction. Despite the considerable risks associated with repeated surgical interventions for recurrent subaortic stenosis, favorable long-term outcomes may outweigh these risks.

A 76-year-old female with rheumatic heart disease presented with progressive dyspnea 12 years after dual surgical bioprosthetic valve replacement (mitral: 25 mm; aortic: 21 mm). Diagnostic imaging revealed severe stenosis of both prostheses, with a mitral valve area of 0.8 cm2 and an aortic valve area of 1.06 cm2, complicated by a risk of left ventricular outflow tract obstruction (neo-left ventricular outflow tract obstruction area: 110 mm2). Given the high surgical risk, simultaneous transfemoral transcatheter aortic and mitral valve-in-valve implantation was performed using Edwards Sapien 3 valves. The Laceration of the Anterior Mitral leaflet to Prevent left ventricular Outflow Obstruction (LAMPOON) technique was employed to prophylactically divide the anterior bioprosthetic leaflet, preventing left ventricular outflow tract obstruction. Post-procedural recovery was uncomplicated, and echocardiography demonstrated restored valve function; the mitral mean gradient was 3 mmHg and the aortic mean gradient was 10 mmHg. This case demonstrates the feasibility of dual valve-in-valve therapy for patients with complex bioprosthetic degeneration.