Cirugía congénitos

World J Pediatr Congenit Heart Surg. 2025 Sep 24:21501351251360691. doi: 10.1177/21501351251360691. Online ahead of print.

ABSTRACT

ObjectivesA minority of patients with hypoplastic left heart syndrome (HLHS) are at extremely high risk for staged palliation and can be bridged-to-heart transplantation with bilateral pulmonary artery bands, ductal stenting, and single ventricle-ventricular assist device insertion (HYBRID + sVAD). The purpose of this analysis is to assess our learning curve associated with our first ten patients with functionally univentricular ductal-dependent systemic circulation who were supported with primary HYBRID + sVAD as bridge-to-heart transplantation.MethodsPatients were temporally separated into two cohorts: the first five and second five. Demographic, perioperative, and outcome data were collected. Continuous variables are described as median [IQR](range). Categorical variables are described as N (%). P values were calculated using Fisher exact t test for categorical variables and unpaired t tests for continuous variables.ResultsTen patients underwent HYBRID + sVAD operations for HLHS (2017-2022). Patients in the initial cohort and the most recent cohort were similar in age and weight. Liver dysfunction and renal dysfunction were more common in the first five patients (2/5 = 40%) versus the next five patients (0/5 = 0%). Length of sVAD support was longer in the most recent five patients (98 days [64-138] vs 154 days [134-225], P = .08); however, no increase in sVAD-associated stroke or bleeding was seen in the most recent five patients. Despite very similar demographic and preoperative profiles, only two of the first five patients (2/5 = 40%) survived to heart transplantation, while all of the next 5 (5/5 = 100%) were successfully bridged-to-cardiac transplantation with HYBRID + sVAD and are alive today.ConclusionsOur experience with primary HYBRID + sVAD as bridge-to-heart transplantation in neonates with HLHS demonstrates an important learning curve associated with this operation and approach.

PMID:40990805 | DOI:10.1177/21501351251360691

Pediatrics. 2025 Oct 1;156(4):e2025071349. doi: 10.1542/peds.2025-071349.

ABSTRACT

We present a case of infectious endocarditis in the immediate post-transplant period in a 7-year-old child. On postoperative day 6, an elevation in inflammatory markers was detected, and the echocardiogram revealed a pedunculated mass on the mitral valve. The patient underwent surgical resection on postoperative day 14, followed by a 6-week course of antibiotics. The patient demonstrated complete resolution of endocarditis and, at 5-year follow-up, has excellent allograft function without recurrence of endocarditis.

PMID:40987464 | DOI:10.1542/peds.2025-071349

Eur J Prev Cardiol. 2025 Sep 23:zwaf603. doi: 10.1093/eurjpc/zwaf603. Online ahead of print.

NO ABSTRACT

PMID:40986814 | DOI:10.1093/eurjpc/zwaf603

Eur J Cardiothorac Surg. 2025 Sep 23:ezaf310. doi: 10.1093/ejcts/ezaf310. Online ahead of print.

ABSTRACT

OBJECTIVES: Currently available materials in congenital cardiac surgery (CCS) are far from optimal, as they do not facilitate growth, remodelling, or renewal, resulting in poorer long-term outcomes due to material-related limitations. Bacterial cellulose (BC), a biogenic polymer-based material produced by Acetobacter xylinum, has emerged as a promising alternative exhibiting excellent bio- and hemocompatibility. This study aimed to develop BC specifically for application in CCS by modifying culturing conditions to enhance its biomechanical resilience.

METHODS: BC was produced according to a standard protocol, and its biomechanical properties were evaluated using inflation pressure testing, thickness measurement, and uniaxial tensile testing. To improve these characteristics, two modifications, a change in the growth media composition and an extended incubation time, were implemented and subsequently evaluated in five further test series. The long-term durability of BC patches was assessed in a fatigue tester for 20 ± 0.5 million cycles, and potential structural damage was investigated using scanning electron microscopy.

RESULTS: Utilizing the two modifications, BC patches demonstrated a capability to reach and maintain pressures exceeding 1000 mmHg with a durability of 100% (n = 24), compared to the standard 20.8% (n = 24) at 500 mmHg. The maximum tensile strength was enhanced from 0.311 ± 0.057 megapascal (MPa) to 0.986 ± 0.397 MPa (p < 0.0001), with a thickness of 3.89 ± 0.95 mm (p < 0.0001). In the long-term durability testing patches endured durations equaling six months without failure while retaining structural integrity.

CONCLUSIONS: The potential of BC for use in CCS was demonstrated by enhancing its biomechanical properties through culturing modifications, warranting further investigation and development of the biomaterial.

PMID:40986395 | DOI:10.1093/ejcts/ezaf310

Cureus. 2025 Aug 19;17(8):e90534. doi: 10.7759/cureus.90534. eCollection 2025 Aug.

ABSTRACT

Anomalous aortic origin of the right coronary artery (AAORCA) is a rare congenital anomaly. Although most patients with this anomaly remain asymptomatic, it is well established that this anomaly may lead to angina, myocardial infarction (MI), or sudden cardiac death (SCD) in the absence of atherosclerotic disease. In this article, we report three cases in the Middle East that presented with syncope in two patients, as well as diaphoresis and ECG changes during a nasal septoplasty surgery in the third. All patients were diagnosed with AAORCA with interarterial course using coronary computed tomography angiography (CTA) after initial evaluation. Although medical management varied over the three cases, all patients tolerated medical management and were referred for surgical interventions. One patient underwent a surgical procedure, with positive outcome afterwards. It is important to recognize the atypical presentations of the malignant course of AAORCA and demonstrate the importance of medical and surgical management in this disease, specially in understudied areas such as the Middle East.

PMID:40984897 | PMC:PMC12450288 | DOI:10.7759/cureus.90534

SAGE Open Med Case Rep. 2025 Sep 20;13:2050313X251377213. doi: 10.1177/2050313X251377213. eCollection 2025.

ABSTRACT

Over the last decade, pectus excavatum deformities have been advocated for correction in cardiac surgery procedures due to their effectiveness and reliability. However, it is uncertain for adult congenital cardiac disease. This is the first article to describe a simultaneous repair for a 72-year-old female who had adult congenital heart disease and pectus excavatum. Preoperative cardiac catheterization found her systolic peak-to-peak gradient at the right ventricular outflow tract stenosis was 69 mmHg, caused by a severely hypertrophied muscle bundle in the right ventricle. She also had a severe pectus excavatum with a Haller Index of 5.09. We performed resection of hypertrophied right muscle bundle at the ostium infundibulum with patch augmentation of the right ventriculotomy and Nuss procedure with bilateral cryoablation. She achieved excellent outcomes in both cardiac repair and chest wall repair in the year after this simultaneous surgery.

PMID:40984887 | PMC:PMC12450259 | DOI:10.1177/2050313X251377213

Indian J Thorac Cardiovasc Surg. 2025 Oct;41(10):1493-1497. doi: 10.1007/s12055-025-01996-7. Epub 2025 Aug 5.

ABSTRACT

A 5-year-old boy with a precordial murmur was initially diagnosed with anomalous systemic arterial (ASA) supply to the left lung lower lobe combined with arterial-pulmonary venous fistula (APVF) by transthoracic echocardiography (TTE), then evaluated by computed tomography angiography (CTA) and digital subtraction angiography (DSA). He received interventional therapy and recovered well. Although ASA supply to the lung lobes combined with APVF is usually diagnosed by CTA, it may also be first diagnosed by TTE in a patient who has a significant heart murmur. Careful TTE examination can improve the diagnostic rate of this cardiovascular abnormality.

PMID:40984852 | PMC:PMC12450170 | DOI:10.1007/s12055-025-01996-7

J Magn Reson Imaging. 2025 Sep 22. doi: 10.1002/jmri.70131. Online ahead of print.

ABSTRACT

BACKGROUND: Pulmonary regurgitant fraction (RF) in patients with repaired tetralogy of Fallot (rTOF) is an important indicator for therapeutic intervention. However, the impact of pulmonary artery geometry and turbulent flow on measurement accuracy is unclear.

PURPOSE: To evaluate the reliability of 4D flow MRI-derived and 2D phase contrast (2D-PC) RF assessments, identify clinically useful measurement locations, and investigate factors contributing to variability.

STUDY TYPE: Retrospective.

POPULATION/SUBJECTS: Thirty-four rTOF patients (21 female; age 45.2 ± 14.4 years).

FIELD STRENGTH/SEQUENCE: 3.0 T; balanced steady-state free precession (bSSFP) cine, time-resolved 2D-PC segmented gradient echo (2D-PC), and time-resolved velocity-sensitized 3D gradient echo with echo planar imaging readout (4D flow) sequences.

ASSESSMENT: MRI was performed before and 3 months after transcatheter pulmonary valve replacement (TPVI). Indexed right ventricular end-diastolic volume (RVEDVi) was assessed in pre/post bSSFP cine data to evaluate RV remodeling. MPA backward and forward flow volumes (BFV, FFV) and RF were measured using 2D-PC and 4D flow. Six equidistant 4D flow planes from the right ventricular outflow tract to the distal MPA were analyzed, with one additional plane matched to the 2D-PC location. MPA and vortex areas were also assessed for each 4D flow plane.

STATISTICAL TESTS: Spearman's rho assessed correlations between 2D-PC and 4D flow parameters, and area under the receiver operating characteristic curve (AUC) assessed the association between RF and the pre/post TVPI percentage change in RVEDVi.

SIGNIFICANCE: p < 0.05.

RESULTS: 2D-PC and 4D flow measurements at the matched MPA plane correlated significantly (RF: ρ = 0.65, FFV: ρ = 0.76, BFV: ρ = 0.87). RF varied by plane, and the plane 10 mm distal to the valve best associated with RVEDVi reduction after TPVI (AUC = 0.84). Changes in FFV between planes significantly correlated with dilatation and stenosis (ρ = 0.432, -0.618), whereas BFV correlated with dilatation alone (ρ = 0.581); however, vortex area enlargement showed no significant association with flow volume changes.

DATA CONCLUSION: 2D-PC and 4D flow measurements show high correlations, but 4D flow accuracy depends on plane location and geometry.

EVIDENCE LEVEL: 3.

TECHNICAL EFFICACY: Stage 2.

PMID:40984730 | DOI:10.1002/jmri.70131

Pediatr Surg Int. 2025 Sep 22;41(1):302. doi: 10.1007/s00383-025-06195-4.

ABSTRACT

PURPOSE: Pectus excavatum (PE) is the most common congenital chest wall deformity. While often perceived as a cosmetic issue, PE can impair cardiopulmonary function. Surgical correction has evolved from the Ravitch procedure to the minimally invasive Nuss technique, which offers reduced surgical risks and improved postoperative outcomes. However, diagnostic and therapeutic variability persists, highlighting the need for expert consensus.

METHODS: A 31-question web-based survey was distributed to CWIG members and PE specialists between November 2024 and January 2025. The survey explored five key domains: demographics, preoperative evaluation, surgical indications and timing, operative technique, and postoperative management. Responses from 100 international surgeons were analyzed.

RESULTS: The most common indications for surgery were severe deformity (88.9%), Haller index > 3.25 (78.8%), psychosocial distress (77.8%), and symptoms (77.8%). Technique selection was influenced by prior surgery (48%) and surgeon experience (45.9%). For complex cases, 90.8% preferred the double-bar technique. Most surgeons removed the bar within 2-3 years (86.6%). Postoperative pain was primarily managed with oral analgesics (64.6%) and IV pumps (47.5%). Early complications included pneumothorax (68.1%) and infections (62.8%); bar displacement (78%) was the most frequent late complication.

CONCLUSION: Despite widespread adoption of the Nuss procedure, significant variation remains in PE management. These findings emphasize the need for standardized, evidence-based guidelines to optimize patient outcomes.

PMID:40982104 | DOI:10.1007/s00383-025-06195-4

Reports (MDPI). 2025 Sep 19;8(3):185. doi: 10.3390/reports8030185.

ABSTRACT

Background and Clinical Significance: Atrial septal defects (ASDs), particularly the ostium secundum type, are congenital cardiac anomalies that can lead to serious complications if left untreated. Percutaneous closure using devices like the Amplatzer Septal Occluder (ASO) has become a widely accepted approach, although complications such as device embolization can occur. Case Presentation: We present a unique case of a 28-year-old woman who developed acute hemodynamic instability and arrhythmias following embolization of an Amplatzer device into the right ventricle during an ASD closure. Despite initial treatment with antiarrhythmic medication, the patient required urgent open-heart surgery for device retrieval and ASD closure. The surgery successfully involved pericardial patch closure of the ASD, device removal from the right ventricle, and the performance of the Kay procedure to address significant tricuspid regurgitation. Postoperative recovery was uneventful, with the patient stabilized and discharged in stable condition. Conclusions: This case highlights the critical need for rapid surgical intervention in cases of device embolization, and the importance of multidisciplinary coordination in managing such complex complications. The combination of ASD closure, device retrieval, and tricuspid valve repair led to a successful outcome, underscoring the importance of timely, decisive action in complex cardiovascular emergencies.

PMID:40981143 | PMC:PMC12452470 | DOI:10.3390/reports8030185

Thorac Cardiovasc Surg Rep. 2025 Sep 18;14(1):e29-e32. doi: 10.1055/a-2698-3658. eCollection 2025 Jan.

ABSTRACT

BACKGROUND: Ectopia cordis (EC) is an exceptionally rare congenital anomaly with a poor prognosis, particularly in resource-limited settings.

CASE DESCRIPTION: We present a newborn with complete thoracic EC who underwent surgical correction on day 5 of life. Although cardiac repositioning and soft tissue coverage were achieved, the infant died 72 hours postoperatively from septic shock, reflecting the significant challenges faced in such contexts.

CONCLUSION: This case underscores the urgent need for improved antenatal care and technical resources to enhance outcomes for complex congenital anomalies in sub-Saharan Africa.

PMID:40980777 | PMC:PMC12445977 | DOI:10.1055/a-2698-3658

Phys Ther Res. 2025;28(2):150-155. doi: 10.1298/ptr.25-E10341. Epub 2025 May 30.

ABSTRACT

OBJECTIVES: The need for early postoperative rehabilitation in patients with congenital heart disease (CHD) is increasing. However, rehabilitation settings in the pediatric intensive care unit (PICU) are usually determined subjectively by therapists. To address the lack of objective measurements, we sought to determine the effectiveness of near-infrared spectroscopy (NIRS) in evaluating skeletal muscles during rehabilitation of patients in the PICU. This case series aimed to clarify the characteristics of skeletal muscle oxygenation during exercise in 2 postoperative patients with CHD.

METHODS: The participants were two 6-month-female infant: one had undergone the Yasui operation (Case 1), and the other had undergone a bidirectional Glenn anastomosis (Case 2). Vital signs and tissue oxygen saturation (StO2) were measured during each exercise task, and the muscle oxygen extraction ratio (MOER), an index of the intramuscular oxygen extraction rate, was calculated. The results were compared between the two cases.

RESULTS: Case 1 showed no significant changes in vital signs, StO2, or MOER, whereas Case 2 had low oxygen saturation at rest and low StO2 during the exercise tasks. MOER increased during the exercise tasks.

CONCLUSIONS: The results showed that sitting did not impose a strong cardiopulmonary load on postoperative patients. However, in children with cyanotic cardiac disease, such as in Case 2, skeletal muscle oxygenation should be considered, and NIRS monitoring may be useful for safely performing rehabilitation.

PMID:40980345 | PMC:PMC12445354 | DOI:10.1298/ptr.25-E10341

Front Pediatr. 2025 Sep 4;13:1622729. doi: 10.3389/fped.2025.1622729. eCollection 2025.

ABSTRACT

Kawasaki disease (KD), or mucocutaneous lymph node syndrome, is a rare systemic inflammatory condition predominantly affecting children under 5 years of age. Complications such as giant coronary artery aneurysms, although rare due to advancements in treatment, remain life-threatening. Coronary artery bypass grafting (CABG) has been a well-established treatment for severe coronary lesions caused by KD. In rare cases of ischemic cardiomyopathy in pediatric patients, heart transplantation may be the only option. We report a case of a 15-year-old male with a history of KD diagnosed at 9 months of age, complicated by giant coronary aneurysms of the left anterior descending and right coronary arteries, who underwent a successful double CABG using the left internal mammary artery) and a saphenous vein graft.

PMID:40980138 | PMC:PMC12443707 | DOI:10.3389/fped.2025.1622729

Cureus. 2025 Aug 19;17(8):e90463. doi: 10.7759/cureus.90463. eCollection 2025 Aug.

ABSTRACT

BACKGROUND: Esophageal atresia (EA) is a rare congenital anomaly frequently associated with congenital heart disease (CHD). This study aimed to evaluate the incidence and characteristics of cardiac anomalies in EA patients treated at a tertiary center in Saudi Arabia.

METHODS: A retrospective review was conducted at National Guard Health Affairs-Riyadh. Medical records of 87 patients diagnosed with EA between 2000 and 2023 were analyzed for EA type, syndromic associations, cardiac anomalies, and the need for cardiac interventions. Statistical analysis was performed using IBM SPSS Statistics for Windows, Version 26 (Released 2019; IBM Corp., Armonk, New York).

RESULTS: Of the 87 patients, 75.9% had cardiac anomalies, which decreased to 42.5% when excluding patent ductus arteriosus (PDA) and patent foramen ovale (PFO). The most common EA type was Type C (80.5%). Syndromic diagnoses were identified in 17.3% of patients, with trisomy 21 being the most common. Cardiac anomalies were significantly associated with female gender (p = 0.013) and syndromic status (p = 0.007). Ventricular septal defect (VSD) and coarctation of the aorta were significantly more frequent in syndromic patients. Nine patients required cardiac intervention during the same admission. The need for cardiac intervention was significantly associated with syndromic status, suggesting that these patients may have more severe cardiac anomalies.

CONCLUSION: Cardiac anomalies excluding PDA and PFO occurred in 42.5% of EA patients. ASD and VSD were the most common findings. Syndromic and female patients had higher rates of cardiac anomalies and cardiac interventions. These results emphasize the need for thorough preoperative cardiac evaluation and multidisciplinary management in EA patients.

PMID:40978987 | PMC:PMC12444801 | DOI:10.7759/cureus.90463

J Cardiovasc Med (Hagerstown). 2025 Sep 1;26(9):477-486. doi: 10.2459/JCM.0000000000001757. Epub 2025 Aug 20.

ABSTRACT

AIMS: Bicuspid aortic valve (BAV) is a common congenital heart disease. However, pivotal randomized trials of transcatheter aortic valve replacement (TAVR) have excluded this population. There remains a lack of consensus on the optimal choice between balloon-expandible valves (BEVs) and self-expanding valves (SEVs) in this setting. This study aimed to compare the efficacy and safety of BEVs vs. SEVs in patients with BAV stenosis.

METHODS: A systematic search using four databases, including PubMed, Embase, Web of Science, and Cochrane CENTRAL, was conducted from inception to 26 November, 2024. Studies comparing the outcome of BEVs and SEVs in patients with BAV stenosis were included.

RESULTS: Nineteen studies comprising 3794 participants were included in this meta-analysis. Procedural mortality did not differ significantly between BEVs and SEVs [odds ratio (OR), 1.06; 95% confidence interval (95% CI) 0.42-2.69, P = 0.91]. Similarly, no differences were observed in all-cause mortality at 1 year or 3 years. BEVs were associated with a lower risk of permanent pacemaker implantation (OR, 0.60; 95% CI 0.48-0.76, P < 0.01) and moderate to severe paravalvular leakage (OR, 0.44; 95% CI 0.23-0.85, P = 0.01) compared with SEVs. However, BEVs were associated with a higher risk of annular rupture (OR, 2.80; 95% CI 1.05-7.49, P = 0.04).

CONCLUSION: BEVs and SEVs demonstrate similar survival outcomes from the procedural period up to 3 years of follow-up. However, the risk profiles for specific complications differ between the valve types. Valve selection for TAVR in patients with BAV stenosis should be considered based on individual anatomical characteristics and the associated risk of specific complications.

PMID:40977467 | DOI:10.2459/JCM.0000000000001757

Cardiol Young. 2025 Sep 22:1-5. doi: 10.1017/S104795112510958X. Online ahead of print.

ABSTRACT

OBJECTIVE: Contemporary management of aortic coarctation in adults is primarily by percutaneous intervention; however, this is not always possible. Intrapericardial ascending-to-descending aortic grafting is an alternative approach in adults with complex aortic coarctation. It can be performed off-pump and allows for a simultaneous treatment of concomitant cardiac lesions. Our aim was to examine the indications and the clinical long-term outcomes of intrapericardial ascending-to-descending aortic grafting in adults with aortic coarctation.

METHODS: We retrospectively reviewed demographic, clinical, surgical, and follow-up data of patients who underwent intrapericardial ascending-to-descending aortic grafting at a single tertiary centre between September 1994 and November 2016.

RESULTS: Ten patients were identified. Indications were primary intervention (n = 4), re-coarctation (n = 5), and iatrogenic coarctation of aorta after stent grafting (n = 1). There were no peri-operative deaths. During a mean follow-up of 13.6 ± 9.4 years, one patient died 11 years after surgery, unrelated to intrapericardial ascending-to-descending aortic grafting. All grafts remained patent with no anastomotic pseudoaneurysms at latest follow-up.The mean systolic blood pressure decreased from 154 ± 24 mmHg at baseline to 134 ± 20 mmHg at latest follow-up (p = 0.05), with the mean number of prescribed antihypertensive medications decreasing from 2.8 ± 1.5 preoperatively to 1.6 ± 0.5 (p = 0.11).

CONCLUSION: Intrapericardial ascending-to-descending aortic grafting is a safe and effective approach for adults with complex coarctation of aorta or re-coarctation not suitable for a percutaneous or conventional surgical approach. Improved blood pressure control and reduced anti-hypertensive use were observed long-term.

PMID:40977163 | DOI:10.1017/S104795112510958X

Cardiol Young. 2025 Sep 22:1-3. doi: 10.1017/S1047951125109566. Online ahead of print.

ABSTRACT

Coronary anomalies are a rare but potentially fatal congenital defect. Among high-risk anatomic features, totally anomalous origin of the coronary arteries from the pulmonary trunk is extremely rare, with only 57 cases reported in the literature as to our knowledge. We report a case of such a congenital anomaly with a successful surgical repair and good cardiac function recovery. We emphasise the need for awareness, since this anatomic presentation precludes collateral development and has, therefore, a higher mortality rate.

PMID:40977144 | DOI:10.1017/S1047951125109566

J Cardiovasc Magn Reson. 2025 Sep 19:101962. doi: 10.1016/j.jocmr.2025.101962. Online ahead of print.

ABSTRACT

BACKGROUND: Impaired right atrial (RA) function is strongly predictive of adverse outcomes in adults with pulmonary arterial hypertension (PAH) but remains incompletely understood in pediatric PAH. In this pediatric multicenter retrospective cohort study using cardiac magnetic resonance imaging (CMR), we analyzed RA size and phasic function and its associations with PAH severity.

METHODS: PAH and control pediatric patients from two centers who underwent CMR from 2010-2023 were identified. RA volumes were measured throughout the cardiac cycle using the single-plane, area-length method on a standard 4-chamber cine sequence. Total, conduit phase, and active phase stroke volume (SVi; indexed to BSA) and ejection fraction (EF) were calculated. A novel marker, the A/C ratio, was calculated as active/conduit SVi. RA size and phasic function measurements were correlated with clinical, hemodynamic, and non-RA CMR metrics of PAH severity and were associated with adverse events (Potts shunt, lung transplant listing/surgery, and/or death) using univariate and bivariate Cox proportional hazards regression analyses. Intra- and interrater reliability was analyzed using intra-class correlation coefficients (ICC).

RESULTS: Compared to controls (n=36), children with PAH (n=72) had higher RA volumes, lower conduit phasic function, and higher active phasic function. In PAH patients, minimum RA volume, active SVi, and A/C ratio directly correlated with NT-proBNP and right ventricular (RV) size, filling pressures, and afterload, while they were inversely correlated with exercise capacity and RVEF. RA conduit EF (cEF) correlations were reversed. During median follow-up of 3.2 years [IQR 1.0, 5.9], RA cEF and A/C ratio remained independent predictors of adverse events after adjustment for common metrics of PAH severity on bivariate analysis, including RVEF (RA cEF aHR 0.91 [95% CI: 0.83-0.99]; A/C ratio aHR 1.58 [95% CI: 1.09-2.29]) and indexed pulmonary vascular resistance (RA cEF aHR 0.83 [95% CI: 0.74-0.93]; A/C ratio aHR 1.79 [95% CI: 1.34-2.41]). RA volume measurements had excellent reliability (ICC >0.97).

CONCLUSION: Correlating with disease severity, impaired RA physiology in pediatric PAH is characterized by RA dilation, reduced conduit phasic function and compensatory augmentation of active phasic function. Assessment of RA size and phasic function is feasible and highly reproducible using standard CMR sequences.

PMID:40976452 | DOI:10.1016/j.jocmr.2025.101962

JACC Case Rep. 2025 Sep 17;30(28):105413. doi: 10.1016/j.jaccas.2025.105413.

ABSTRACT

Baffle stenosis and thrombus formation are recognized complications of atrial switch operation (ASO), with no concurrent cases documented. A 23-year-old man with dextro-transposition of the great arteries who had previously undergone Mustard ASO presented with progressive dyspnea, peripheral edema, and abdominal distension. Cardiac computed tomography (CT) showed severe bicaval baffle stenosis and 2 superior vena cava baffle thrombi, one occluding the stenosed segment. Mechanical thrombus fragmentation, systemic thrombolysis, and sequential stent implantation in both baffles achieved complete recovery. Follow-up cardiac CT confirmed patency of the stent and the absence of thrombi. The patient was discharged symptom free and on a daily regimen of acenocoumarin, with close follow-up. This case report emphasizes the value of cardiac CT in assessing baffle-related complications and guiding precise interventional strategies. In patients with a history of ASO and baffle complications, percutaneous stenting provides an effective alternative to surgical intervention.

PMID:40973326 | DOI:10.1016/j.jaccas.2025.105413

Biochim Biophys Acta Mol Basis Dis. 2025 Sep 17;1872(1):168052. doi: 10.1016/j.bbadis.2025.168052. Online ahead of print.

ABSTRACT

Bicuspid aortic valve (BAV) is a congenital malformation that predisposes individuals to thoracic aortic aneurysm (TAA), with endothelial dysfunction playing a pivotal role in its pathogenesis. Endothelial cell senescence is a hallmark of endothelial dysfunction, yet direct evidence linking endothelial senescence to BAV-TAA has not been established. In this study, we generated induced pluripotent stem cells (iPSCs) from both BAV-TAA patients and healthy controls, subsequently differentiating them into endothelial cells (iECs). Our findings revealed that BAV-TAA-iECs exhibited senescence phenotype, including impaired proliferation, diminished migratory capacity, upregulated senescence markers (p53, p21, p16), and a pronounced senescence-associated secretory phenotype (SASP). Transcriptomic analysis through RNA sequencing indicated aberrant activation of the FOXO signaling pathway in BAV-TAA-iECs which might contribute to BAV-TAA-iEC senescence. Inhibition of FOXO1 signaling using AS1842856 effectively reversed the senescence phenotype, restored endothelial nitric oxide synthase (eNOS) expression, attenuated SASP cytokine levels, and mitigated inflammation through the p65 and p38 signaling pathways. These findings suggest that endothelial cell senescence plays a critical role in the pathogenesis of BAV-TAA, and targeting FOXO1 signaling may represent a promising therapeutic strategy for BAV-associated aortic diseases.

PMID:40972881 | DOI:10.1016/j.bbadis.2025.168052