International journal of cardiology. Congenital heart disease最新文献

Correlates of left ventricular hypertrophy in adults with coarctation of aorta and role in risk stratification 成人左室肥厚与主动脉缩窄的相关性及其在危险分层中的作用

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2026-03-01 Epub Date: 2025-11-22 DOI: 10.1016/j.ijcchd.2025.100642

Zeyad Kholeif, Ahmed E. Ali, Karim Ali, Marwan Ahmed, Ahmed Bahnasy, Meena Bai, Sara Aboelmaaty, Mohamed Ellabbad, Alexander C. Egbe

Background

Coarctation of aorta (COA) results in chronic left ventricular (LV) pressure overload and LV hypertrophy, which is associated with cardiovascular mortality. Therapies that decrease LV pressure overload result in regression of LV hypertrophy and improved survival. The goal of COA intervention is to relieve LV pressure overload, and the guidelines recommend the use of several COA severity indices as indications for intervention. These indices are Doppler mean gradient, systolic blood pressure (SBP), upper-to-lower-extremity SBP (ULE-SBP), aortic isthmus ratio, presence of collateral vessels, and exercise-induced hypertension (EIH). Although these indices are physiologically intuitive, it is unclear how many of these indices are required in order to recommend intervention, and the hierarchical importance of the different indices in patients that have some but not all the indices.

Methods

In this cross-sectional study, multivariate linear regression analysis was used to assess the correlation between LV mass index (LVMI) and indices of COA severity.

Results

Of the COA indices analyzed in 811 adult with repaired COA patients, aortic isthmus ratio had the strongest correlation with LVMI (β coefficients ±standard error −31.6 ± 13.8, p < 0.001), as compared to Doppler peak gradient (0.79 ± 0.22, p = 0.002) and SBP (0.53 ± 0.21, p = 0.03).

Conclusion

Aortic isthmus ratio had the strongest correlation with LVMI, which is a reflection of chronic LV pressure overload. An aortic isthmus ratio ≤0.7 was the optimal cut-off point to detect LV hypertrophy with good sensitivity and specificity. These results can potentially improve and simplify patient selection for COA intervention.

背景：主动脉缩窄（COA）导致慢性左室（LV）压力过载和左室肥厚，这与心血管疾病死亡率相关。减少左室压力过载的治疗可导致左室肥大的消退和生存率的提高。COA干预的目标是缓解左室压力过载，指南建议使用几个COA严重程度指标作为干预的指征。这些指标是多普勒平均梯度、收缩压（SBP）、上下肢收缩压（ULE-SBP）、主动脉峡比、侧支血管的存在和运动性高血压（EIH）。虽然这些指标在生理上是直观的，但目前尚不清楚需要多少这些指标才能推荐干预措施，以及不同指标在具有某些但不是全部指标的患者中的等级重要性。方法采用多变量线性回归分析，评价左室质量指数（LVMI）与COA严重程度指标的相关性。结果811例成人COA修复患者的COA指标中，主动脉峡部比值与LVMI （β系数±标准误差- 31.6±13.8,p < 0.001）、多普勒峰梯度（0.79±0.22,p = 0.002）和收缩压（0.53±0.21,p = 0.03）的相关性最强。结论主动脉峡比与LVMI相关性最强，是慢性左室压过负荷的反映。主动脉峡部比值≤0.7是检测左室肥厚的最佳分界点，具有良好的敏感性和特异性。这些结果可以潜在地改善和简化COA干预的患者选择。

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引用次数: 0

Evaluating the effect of renin-angiotension-aldosterone system inhibitors and beta blockers on the progression of bicuspid aortic valve aortopathy in adulthood: A retrospective cohort study 评估肾素-血管紧张-醛固酮系统抑制剂和受体阻滞剂对成年二尖瓣主动脉病变进展的影响：一项回顾性队列研究

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2026-03-01 Epub Date: 2026-01-12 DOI: 10.1016/j.ijcchd.2026.100653

Nili Schamroth Pravda , Elchanan Samuel , Tamir Bental , Ofir Brem , Ilan Richter , Keren Skalsky , Miri Schamroth Pravda , Yaron Shapira , Ran Kornowski , Guy Witberg

Purpose

Patients with a bicuspid aortic valve (BAV) often have an associated aortopathy and increased risk of aortic dissection. This study aimed to assess if the chronic use of renin-angiotensin-aldosterone system inhibitors (RAASi) or beta blockers (BB) may be associated with reducing the progression of aortic dilation in adult BAV patients.

Methods

A retrospective cohort study was performed including adult patients with BAV with serial echocardiography over ≥5 years. The cohort was subdivided according to chronic use of RAASi or BB. The primary outcome was a composite of progression of absolute ascending aortic diameter ≥4.5 cm or surgery for ascending aortic replacement.

Results

Included were 262 patients with mean age of 54.3 ± 19.5 years and 75.1 % male. The average follow-up time was 10.8 ± 0.8 years. The cumulative incidence of the primary endpoint was 14.6 % at 10-year follow up and 33.3 % at 15-year follow up. While those with RAASi (n = 39) had the primary endpoint numerically less that those without RAASi chronic therapy (12.8 % vs 24.8 %, p = 0.147), on multivariate analysis there was no significant decreased risk for the primary outcome. (HR = 0.89, 95 % CI [0.34–2.86], p = 0.97). Similarly, there was no significant decreased risk of the primary outcome amongst those with chronic beta blocker use following multivariate analysis (HR = 0.96, 95 % CI [0.37–2.51], p = 0.95).

Conclusions

Our findings suggest that the chronic use of RAASi or BB was not associated with a blunted progression of BAV aortopathy in the adult population. These results highlight the need for larger, randomised studies to validate these observations and further explore potential preventative strategies in this population.

目的：患有双尖瓣主动脉瓣（BAV）的患者通常伴有主动脉病变，主动脉夹层的风险增加。本研究旨在评估慢性使用肾素-血管紧张素-醛固酮系统抑制剂（RAASi）或β受体阻滞剂（BB）是否可能与减少成人BAV患者主动脉扩张的进展有关。方法采用回顾性队列研究方法，对≥5年的BAV成人患者进行连续超声心动图检查。该队列根据长期使用RAASi或BB进行细分。主要终点是绝对升主动脉直径≥4.5 cm进展或升主动脉置换术的综合结果。结果纳入262例患者，平均年龄54.3±19.5岁，男性占75.1%。平均随访时间10.8±0.8年。主要终点的累积发病率在10年随访时为14.6%，在15年随访时为33.3%。虽然RAASi患者（n = 39）的主要终点数值低于未接受RAASi慢性治疗的患者（12.8% vs 24.8%, p = 0.147），但在多因素分析中，主要结局的风险没有显著降低。（HR = 0.89, 95% CI [0.34-2.86], p = 0.97）。同样，在多变量分析中，慢性受体阻滞剂患者的主要结局风险没有显著降低（HR = 0.96, 95% CI [0.37-2.51], p = 0.95）。结论：我们的研究结果表明，在成人人群中，长期使用RAASi或BB与BAV主动脉病变的钝化进展无关。这些结果强调需要更大规模的随机研究来验证这些观察结果，并进一步探索这一人群的潜在预防策略。

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引用次数: 0

Last of the Eisenmengers: A Tribute 最后的艾森曼格：致敬

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2026-03-01 Epub Date: 2025-12-05 DOI: 10.1016/j.ijcchd.2025.100645

Craig Broberg

引用次数: 0

Right ventricular outflow tract stenting for obstructive lesions in adult congenital heart disease 成人先天性心脏病梗阻性病变右心室流出道支架置入术。

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2026-03-01 Epub Date: 2026-02-11 DOI: 10.1016/j.ijcchd.2026.100660

Miriam G. Zuniga Salcedo , Edgar García Cruz , José Antonio García Montes , Jorge Sanchez Nieto , Stephanie Angulo Cruzado , Edgar Francisco Carrizales Sepúlveda , Ramiro Flores Ramírez

Currently, the use of stents in the right ventricular outflow tract (RVOT) has been described as alternative for palliation in neonates with Tetralogy of Fallot (ToF) and critical infundibular stenosis, who are not candidates for total correction in a single stage. However, there is limited evidence of the benefit of this intervention in other clinical scenarios, and just a few reports in adults.

A retrospective study was conducted on 23 patients with significant RVOT obstruction who underwent RVOT stent placement and were not considered candidates for surgery. Patients 17 years old at the time of the procedure at our institution were included.

Median age at the time of procedure was 24 years (IQR 17-49). 34.8% had univentricular heart physiology. The most frequent diagnosis was ToF (43.5%). 56.5% had at least one previous systemic-pulmonary shunt. In 69.5% of the cases the clinical context that led to stent placement was due to an emergency, primarily thrombosis of a systemic-pulmonary shunt (43.5%). RVOT stent implantation was successful in 100% of the cases. There were 2 procedure-related deaths (8.7%). The median stent patency time was 18.7 months (IQR 0-112 months).

This is the first study that demonstrates the feasibility of RVOT stenting as a palliative method in adult patients with congenital heart disease in emergency settings. In addition to showing the possibility of its use in univentricular hearts, it demonstrates the long-term durability of RVOT stents as a palliative method, something never previously reported.

目前，右室流出道（RVOT）支架的使用已被描述为缓解法洛四联症（ToF）和严重漏斗狭窄的新生儿的替代方案，这些患者不适合单期完全矫正。然而，在其他临床情况下，这种干预的益处的证据有限，只有少数关于成人的报道。我们对23例RVOT梗阻患者进行了回顾性研究，这些患者接受了RVOT支架置入术，不考虑手术治疗。患者在我们机构接受手术时年龄为17岁。手术时的中位年龄为24岁（IQR 17-49）。34.8%有单室心脏生理。最常见的诊断是ToF（43.5%）。56.5%的患者既往至少有一次系统-肺分流。在69.5%的病例中，导致支架置入的临床情况是由于紧急情况，主要是系统-肺分流血栓形成（43.5%）。RVOT支架植入术100%成功。手术相关死亡2例（8.7%）。中位支架通畅时间18.7个月（IQR 0 ~ 112个月）。这是第一个证明RVOT支架置入术在急诊成人先天性心脏病患者中作为一种姑息治疗方法的可行性研究。除了显示其在单室心脏中使用的可能性外，它还证明了RVOT支架作为一种姑息方法的长期耐久性，这是以前从未报道过的。

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引用次数: 0

Two Italian fellows remember Richard Van Praagh 两个意大利人记得理查德·范普拉格

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2026-03-01 Epub Date: 2025-12-18 DOI: 10.1016/j.ijcchd.2025.100647

Bruno Marino, Angelo Restivo

Methods

two italian fellows remember its figure of scientist, teacher and friend.

Results

in 1964 Dr Van Praagh anticipated the new diagnostic approach to congenital heart disease and in the following years he published more than 300 articles clarifying what was not clear in the past. He was a fascinating pearson and teacher.

Conclusion

Richard Van Praagh was an example of pioneer, scientist and man and his contribution changed the knowledge of congenital heart disease.

方法两位意大利学者记住了它的科学家、老师和朋友的形象。1964年，Van Praagh博士预测了先天性心脏病的新诊断方法，在接下来的几年里，他发表了300多篇文章，澄清了过去不清楚的事情。他是一位令人着迷的学生和老师。结论richard Van Praagh是先驱者、科学家和人的典范，他的贡献改变了人们对先天性心脏病的认识。

引用次数: 0

The utility of 4D intracardiac echocardiography in transcatheter pulmonary valve replacement in adult congenital heart disease patients 4D心内超声心动图在成人先天性心脏病经导管肺瓣膜置换术中的应用

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2026-03-01 Epub Date: 2026-01-06 DOI: 10.1016/j.ijcchd.2026.100652

Eihab Ghantous , Hans Gao , Carlos Sisniega , Angela Li , Jamil Aboulhosn

Background

Intracardiac echocardiography (ICE) has become a critical imaging modality in adult congenital heart disease (ACHD) interventions, offering high-resolution intracardiac imaging without the need for intubation. In transcatheter pulmonary valve replacement (TCPVR), ICE may overcome limitations of traditional imaging, particularly in visualizing the right ventricular outflow tract (RVOT) and pulmonary valve.

Objectives

This study aimed to evaluate the feasibility, safety, and clinical utility of four-dimensional (4D) ICE in ACHD patients undergoing TCPVR.

Methods

Beginning in February 2023, we prospectively enrolled all patients undergoing TCPVR at a tertiary ACHD center. Patients underwent preprocedural imaging and clinical evaluation. 4D ICE was used pre- and post-valve implantation to assess cardiac anatomy and valve function. Patients without valve implantation or 4D ICE imaging were excluded.

Results

Of 55 referred patients, 45 underwent successful TCPVR with 4D ICE. The mean age was 42.6 ± 13.6 years, and 51 % were male. 4D ICE confirmed procedural indications, provided comprehensive anatomic and functional assessment, and detected post-implantation regurgitation in 35.6 % of cases, significantly more than angiography (11.1 %) or transthoracic echocardiography (4.4 %). Incidental but clinically relevant findings were identified in 33.3 % of patients, influencing management in 15.6 %. No ICE-related complications occurred.

Conclusions

4D ICE is a feasible, safe, and clinically valuable imaging tool during TCPVR in ACHD patients. It enhances procedural guidance, detects early valve-related complications, and identifies unexpected findings that affect management. Its integration into structural heart interventions may significantly improve outcomes, especially as technology continues to evolve.

心内超声心动图（ICE）已成为成人先天性心脏病（ACHD）干预的关键成像方式，无需插管即可提供高分辨率的心内成像。在经导管肺瓣膜置换术（TCPVR）中，ICE可以克服传统成像的局限性，特别是在显示右心室流出道（RVOT）和肺瓣膜方面。目的本研究旨在评估四维（4D） ICE在ACHD患者行TCPVR的可行性、安全性和临床应用。方法：从2023年2月开始，我们前瞻性地招募了所有在三级ACHD中心接受TCPVR的患者。患者接受术前影像学检查和临床评估。在瓣膜植入前后应用4D ICE评估心脏解剖和瓣膜功能。没有瓣膜植入或4D ICE成像的患者被排除在外。结果55例患者中，45例成功行TCPVR + 4D ICE。平均年龄42.6±13.6岁，男性占51%。4D ICE证实了手术指征，提供了全面的解剖和功能评估，35.6%的病例发现了植入后反流，明显高于血管造影（11.1%）或经胸超声心动图（4.4%）。在33.3%的患者中发现了偶然但与临床相关的发现，15.6%的患者影响了治疗。无ice相关并发症发生。结论4d ICE是一种可行、安全、有临床价值的成像工具。它加强了手术指导，发现早期与瓣膜相关的并发症，并识别影响管理的意外发现。将其整合到结构性心脏干预中可能会显著改善结果，特别是随着技术的不断发展。

{"title":"The utility of 4D intracardiac echocardiography in transcatheter pulmonary valve replacement in adult congenital heart disease patients","authors":"Eihab Ghantous , Hans Gao , Carlos Sisniega , Angela Li , Jamil Aboulhosn","doi":"10.1016/j.ijcchd.2026.100652","DOIUrl":"10.1016/j.ijcchd.2026.100652","url":null,"abstract":"<div><h3>Background</h3><div>Intracardiac echocardiography (ICE) has become a critical imaging modality in adult congenital heart disease (ACHD) interventions, offering high-resolution intracardiac imaging without the need for intubation. In transcatheter pulmonary valve replacement (TCPVR), ICE may overcome limitations of traditional imaging, particularly in visualizing the right ventricular outflow tract (RVOT) and pulmonary valve.</div></div><div><h3>Objectives</h3><div>This study aimed to evaluate the feasibility, safety, and clinical utility of four-dimensional (4D) ICE in ACHD patients undergoing TCPVR.</div></div><div><h3>Methods</h3><div>Beginning in February 2023, we prospectively enrolled all patients undergoing TCPVR at a tertiary ACHD center. Patients underwent preprocedural imaging and clinical evaluation. 4D ICE was used pre- and post-valve implantation to assess cardiac anatomy and valve function. Patients without valve implantation or 4D ICE imaging were excluded.</div></div><div><h3>Results</h3><div>Of 55 referred patients, 45 underwent successful TCPVR with 4D ICE. The mean age was 42.6 ± 13.6 years, and 51 % were male. 4D ICE confirmed procedural indications, provided comprehensive anatomic and functional assessment, and detected post-implantation regurgitation in 35.6 % of cases, significantly more than angiography (11.1 %) or transthoracic echocardiography (4.4 %). Incidental but clinically relevant findings were identified in 33.3 % of patients, influencing management in 15.6 %. No ICE-related complications occurred.</div></div><div><h3>Conclusions</h3><div>4D ICE is a feasible, safe, and clinically valuable imaging tool during TCPVR in ACHD patients. It enhances procedural guidance, detects early valve-related complications, and identifies unexpected findings that affect management. Its integration into structural heart interventions may significantly improve outcomes, especially as technology continues to evolve.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"23 ","pages":"Article 100652"},"PeriodicalIF":1.2,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145978174","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Point of care INR monitoring in cyanotic congenital heart disease-related compensatory erythrocytosis 紫绀型先天性心脏病相关代偿性红细胞增多症的监护点INR监测

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2026-03-01 Epub Date: 2026-01-19 DOI: 10.1016/j.ijcchd.2026.100655

Phidias Rueter , Freda Passam , David Celermajer , David Baker , Cristina Ma Biason , Danielle Osborn , Rachael Cordina

Introduction

People with cyanotic congenital heart disease (CHD) frequently require anticoagulation with warfarin. Point of care (POC) devices offer significant benefits such as patient-managed warfarin and rapid testing, however, there is limited experience with the use of POC in adult patients with CHD and high haematocrit.

Methods

This exploratory prospective cohort study recruited people between September 2021 and December 2023. Inclusion criteria were cyanotic CHD and oxygen saturations <93% and a Hct ≥0.51. Exclusion criteria were pregnancy, antiplatelet agents, recent thromboembolic event or major medical illness. A Coaguchek XS (Roche Diagnostics) was used.

Results

Five individuals were recruited to the warfarin group (Hct 0.54, 0.52–0.58, IQR 0.04) and five to the control group (Hct 0.55, 0.51–0.60, IQR 0.07). Difference in INR between methods was 0.1 (−0.3-0.2, IQR 0.2) in the warfarin and −0.1 (−0.3 – 0.1, IQR 0.2) in the cyanosis-control arms. Bland Altman analysis demonstrated no proportional bias. No eligible participants had Hct ≥0.60. One potential participant had Hct of 0.73 but POC testing was unrecordable. Correlation between POC and laboratory INR was r = 0.98, p < 0.01 (95 % CI 0.98–1.22) for the whole cohort, r = 1.0, p < 0.01 (95 % CI 0.75–1.50) in the warfarin group and r = 0.87, p = 0.06 (95 %CI -0.23-3.10) in the control group.

Conclusion

POC INR testing may be an accurate alternative for individuals with compensatory erythrocytosis and Hct up to 0.60.

紫绀型先天性心脏病（CHD）患者经常需要华法林抗凝。护理点（POC）设备提供了显著的好处，如患者管理华法林和快速检测，然而，在成年冠心病和高红细胞压积患者中使用POC的经验有限。方法本探索性前瞻性队列研究于2021年9月至2023年12月招募受试者。纳入标准为紫绀型冠心病，血氧饱和度≥93%，Hct≥0.51。排除标准为妊娠、抗血小板药物、近期血栓栓塞事件或重大内科疾病。使用Coaguchek XS （Roche Diagnostics）。结果华法林组5例（Hct = 0.54, 0.52 ~ 0.58, IQR = 0.04），对照组5例（Hct = 0.55, 0.51 ~ 0.60, IQR = 0.07）。两种方法的INR差异华法林组为0.1 (- 0.3-0.2,IQR 0.2)，青紫对照组为- 0.1 （- 0.3- 0.1,IQR 0.2）。Bland Altman分析显示无比例偏倚。没有符合条件的参与者Hct≥0.60。一名潜在参与者的Hct为0.73，但POC测试不可记录。整个队列中POC与实验室INR的相关性为r = 0.98, p < 0.01 (95% CI 0.98 - 1.22)，华法林组r = 1.0, p < 0.01 (95% CI 0.75-1.50)，对照组r = 0.87, p = 0.06 （95% CI -0.23-3.10）。结论poc INR检测对代偿性红细胞增多、Hct≥0.60的患者可作为准确的替代方法。

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引用次数: 0

Biventricular remodeling and function after Cone repair of Ebstein Anomaly: A review Ebstein异常椎体修复后双心室重构及功能的研究进展

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2026-03-01 Epub Date: 2025-12-24 DOI: 10.1016/j.ijcchd.2025.100649

Mimi X. Deng , Alison J. Howell , Osami Honjo , Mark K. Friedberg

The Cone reconstruction is the prevailing repair strategy for Ebstein anomaly (EA), providing near-anatomical restoration of the tricuspid valve (TV) and reliable reduction of tricuspid regurgitation (TR). Most of the risk of Cone repair is seen in the early postoperative period from dehiscence, with very low rates of mortality and reintervention thereafter. By correcting TV dysfunction and abnormal right ventricle (RV) geometry, ventricular reverse remodeling has been demonstrated through decreased right atrial and atrialized RV size, along with improved antegrade pulmonary flow and consequent left ventricular filling. Early postoperative RV dysfunction is common due to increased afterload upon addressing TR, with suggestion of delayed recovery. Cone repair augments left ventricular preload and preserves left ventricular function. The contrasting effects of ventricular plication and superior repositioning of TV during the Cone repair has led to discrepant reports in the trajectory of RV remodeling, which is further confounded by the lack of standardization in imaging measurements and timing of surveillance. Consistency in imaging protocol and lengthening follow-up will hopefully provide a more robust understanding about the evolution of the postoperative RV. Improvement in functional status and aerobic capacity can also be appreciated post-Cone repair, particularly in highly symptomatic patients. Innovation to promote remodeling of the Ebsteinoid heart include stem cell therapy at the time of surgical repair, which has shown promise in phase I clinical trial. Lastly, the goal of biventricular repair through Cone reconstruction can be facilitated by Starnes palliation to stabilize critical presentations, a paradigm shift that is increasingly adopted.

锥体重建是Ebstein畸形（EA）的主流修复策略，提供三尖瓣（TV）的接近解剖的修复和可靠的三尖瓣反流（TR）的减少。椎体修复的大部分风险发生在术后早期，因为椎体破裂，死亡率和此后的再干预率非常低。通过纠正TV功能障碍和右心室（RV）几何形状异常，右心房和心房化右心室尺寸减小，顺行肺血流改善，左心室充盈，证明了心室反向重构。术后早期右心室功能障碍是常见的，因为处理TR后负荷增加，提示恢复延迟。锥体修复增强左心室预负荷，保留左心室功能。在椎体修复过程中，心室收缩和电视复位的不同效果导致了RV重构轨迹的不同报道，这进一步被成像测量和监测时间缺乏标准化所混淆。成像方案的一致性和延长随访有望提供对术后右心室演变的更有力的了解。椎体修复后功能状态和有氧能力的改善也值得赞赏，特别是在症状严重的患者中。促进Ebsteinoid心脏重塑的创新包括手术修复时的干细胞治疗，这在I期临床试验中显示出希望。最后，通过椎体重建实现双心室修复的目标可以通过Starnes姑息治疗来稳定关键的表现，这是一种越来越被采用的范式转变。

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引用次数: 0

Hepatic cholate clearance as assessed by HepQuant-SHUNT is associated with clinical outcomes in individuals with Fontan circulation HepQuant-SHUNT评估的肝胆酸清除率与Fontan循环患者的临床结果相关

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2026-03-01 Epub Date: 2026-01-18 DOI: 10.1016/j.ijcchd.2026.100654

Yuli Y. Kim , Daniel Ganger , Alexis Z. Tomlinson , Isabella Z. Farkas , Jack Rychik , Joanne Imperial , Michael P. McRae , Greg T. Everson , Maarouf A. Hoteit

Background

Fontan-associated liver disease [FALD] is universal in individuals with Fontan circulation [FC]. The dual cholate clearance test is a noninvasive, flow-dependent measure of liver function. We aim to explore the association between cholate clearance and clinical outcomes in this population.

Methods

Prospective study of adults with FC ≥ 18 years from University of Pennsylvania [Penn] and Northwestern University [NU]. Hepatic clearance of oral d4-cholate and intravenous 13C-cholate were measured from peripheral venous blood and calculated as portal hepatic filtration rate [HFR], systemic HFR, and SHUNT% with elevated SHUNT% being >30 %. Primary outcome was transplant-free survival. Association between SHUNT% and primary outcome was assessed using Kaplan-Meier curves and log-rank test or Cox regression. Secondary outcome was composite of death, transplant, new onset heart failure, ascites, protein losing enteropathy, or hepatocellular carcinoma. Association between SHUNT% and composite outcome was analyzed by logistic regression.

Results

Fifty participants (35 Penn, 15 NU) were enrolled. Unadjusted 1-, 3-, and 5-year transplant-free survival was 96 %, 94 %, and 79 %, respectively. Individuals with elevated SHUNT% had significantly lower transplant-free survival at 5-years. The composite outcome was reached in 14/50 (29 %), including death (n = 4), combined heart-liver transplant (n = 4), or both (n = 2). Elevated SHUNT% was associated with 3.9-fold increase odds of composite outcome (95 % CI 1.02–15.3; p = 0.048).

Conclusions

An increase in shunting of portal blood into the systemic circulation as quantified by SHUNT% is associated with decreased transplant-free survival in individuals with FC and has excellent discrimination in identifying those at risk for mid-term adverse clinical events.

方丹相关性肝病[FALD]在方丹循环患者中是普遍存在的[FC]。双胆酸清除率试验是一种无创、血流依赖的肝功能测量方法。我们的目的是探索这一人群中胆固醇清除与临床结果之间的关系。方法对来自宾夕法尼亚大学[Penn]和西北大学[NU]的FC≥18岁成人进行前瞻性研究。通过外周静脉血测定口服4-胆酸盐和静脉注射13c -胆酸盐的肝脏清除率，计算门静脉肝滤过率[HFR]、全身HFR和SHUNT%，其中SHUNT%升高为30%。主要终点为无移植生存期。使用Kaplan-Meier曲线和log-rank检验或Cox回归评估SHUNT%与主要转归之间的关系。次要结局为死亡、移植、新发心力衰竭、腹水、蛋白质丢失性肠病或肝细胞癌。采用logistic回归分析SHUNT%与综合结局的关系。结果共入组50例（Penn 35例，NU 15例）。未调整的1年、3年和5年无移植生存率分别为96%、94%和79%。分流率升高的个体5年无移植生存率显著降低。达到复合结局的有14/50(29%)，包括死亡（n = 4）、心肝联合移植（n = 4）或两者兼有（n = 2）。SHUNT%升高与复合结局的几率增加3.9倍相关（95% CI 1.02-15.3; p = 0.048）。结论以SHUNT%为量化指标的门静脉血液进入体循环的增加与FC患者无移植生存期的降低有关，在识别中期不良临床事件风险方面具有很好的鉴别作用。

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引用次数: 0

Outcome of ventricular assist devices in adults with congenital heart disease – A single center case series 成人先天性心脏病患者心室辅助装置的疗效-单中心病例系列

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2026-03-01 Epub Date: 2025-12-20 DOI: 10.1016/j.ijcchd.2025.100648

Ahmed Younis , Zeyad M. Kholeif , William R. Miranda , Heidi M. Connolly , Mauricio T. Villavicencio , Joseph A. Dearani , Alexander C. Egbe

引用次数: 0