International journal of cardiology. Congenital heart disease最新文献_第6页

Trends in mortality from pulmonary hypertension amongst population with congenital heart disease in the United States from 1999 to 2020: A CDC WONDER analysis 1999年至2020年美国先天性心脏病患者肺动脉高压死亡率趋势：CDC WONDER分析

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-09-01 Epub Date: 2025-06-21 DOI: 10.1016/j.ijcchd.2025.100602

Allahdad Khan , Tehreem Asghar , Kumail Mustafa Ali , Muhammad Saeed , Saniya Ishtiaq , Fatima Ashfaq , Jamil Nasrallah , Laiba Yumn , Usman Ali Akbar , Peter Collins , Raheel Ahmed

Background

Congenital heart disease (CHD) is an umbrella term describing a variety of structural cardiac malformations at birth. CHD affects approximately 1 % of live births, generating a large adult population with these abnormalities. Pulmonary hypertension (PH) in patients with adult CHD is heterogeneous, based on the type of defect and associated conditions, but is a known cause of adverse outcome.

Method

We retrieved death certificate data from the CDC-WONDER database using ICD codes (I27.0, I27.2, I27.8, and I27.9., and Q20-26). Crude mortality rates (CMRs) and age-adjusted mortality rates (AAMRs) per 100,000 persons were calculated. Temporal trends were examined using the annual percent change (APC) and average annual percent change (AAPC) determined by Joinpoint regression.

Result

From 1999 to 2020, an overall declining pattern was observed in the mortality rate. Men and women with congenital heart disease and pulmonary hypertension in the U.S. experienced a statistically significant decline in mortality rates. In terms of races, among White individuals, the decline was the most pronounced. All four U.S. census regions experienced statistically significant declines in mortality due to pulmonary hypertension among individuals with congenital heart disease. The rate of decline was steeper in rural areas compared to urban ones.

Conclusion

This study highlights that previously implemented targeted interventions significantly contributed to the reduction of mortality amongst patiemnts with congenital heart disease and pulmonary hypertension in the U.S. Still improvements are required in certain areas, including female gender, Hispanic or Latino, and the Northeast and West regions.

先天性心脏病（CHD）是描述出生时各种结构性心脏畸形的总称。冠心病影响约1%的活产婴儿，导致大量成年人患有这些异常。成人冠心病患者的肺动脉高压（PH）是异质性的，基于缺陷的类型和相关条件，但它是已知的不良结果的原因。方法使用ICD代码（I27.0、I27.2、I27.8和I27.9）从CDC-WONDER数据库中检索死亡证明数据。Q20-26)。计算了每10万人的粗死亡率（cmr）和年龄调整死亡率（AAMRs）。采用年变化百分数（APC）和平均年变化百分数（AAPC）进行时间趋势分析。结果1999 - 2020年，死亡率总体呈下降趋势。在美国，患有先天性心脏病和肺动脉高压的男性和女性的死亡率在统计上有显著下降。就种族而言，在白人中，这种下降最为明显。在美国所有四个人口普查地区，先天性心脏病患者因肺动脉高压导致的死亡率在统计上都有显著下降。与城市地区相比，农村地区的下降速度更快。结论：本研究强调，先前实施的有针对性的干预措施显著有助于降低美国先天性心脏病和肺动脉高压患者的死亡率，但在某些地区仍需改进，包括女性、西班牙裔或拉丁裔以及东北和西部地区。

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引用次数: 0

Clinical outcomes of children operated for congenital heart diseases in Rwanda: a 14-year retrospective analysis 卢旺达先天性心脏病患儿手术治疗的临床结果：14年回顾性分析

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-09-01 Epub Date: 2025-06-28 DOI: 10.1016/j.ijcchd.2025.100605

Providence Akingeneye , David J. Bradley , Joseph Mucumbitsi , Naphtal Nyilimanzi , Yves Mutabandama , Emmanuel K. Rusingiza

Background

Congenital heart disease surgery and catheterization for Rwandan children has historically been accomplished with the assistance of visiting external teams and referrals abroad. However, follow-up information on patient-specific clinical outcomes is lacking.

Methods

This is a retrospective cross-sectional study of clinical outcomes among Rwandan children under 15 years of age at the time of heart surgery operated between 2006 and 2020. Data from two Rwandan teaching hospitals were collected from paper and digital patient charts.

Results

Among the 406 children included in our study, 59.1 % were female; 30.5 % and 28.1 % of the children were underweight and stunted, respectively. Tetralogy of Fallot was the most common cyanotic CHD. Of procedures, VSD repair was the most performed operation and PDA closure represented the majority of therapeutic catheterizations. Postoperatively complications such as pneumonia occurred in 6 %, residual shunt in 17 % and residual mild or greater stenosis in 41 %. The overall mortality was 3.7 %, with sepsis as the leading cause of death. Intensive care unit stay longer than seven days, symptomatic heart failure before surgery, and being underweight were significantly associated with complications. Cardiopulmonary bypass over 60 min was associated with mortality among operated children.

Conclusion

Procedural treatment of CHD in Rwandan children was predominantly accomplished by visiting missions during the study period. Of patients with sufficient clinical follow up data, survival was 96 % and complications occurred in 35 %. Nutritional compromise is a correlate of poor outcome. A centralized cardiac registry might mitigate the observed loss of clinical follow up data in 14 % of children.

历史上，卢旺达儿童的先天性心脏病手术和导管术是在访问外部团队和国外转诊的帮助下完成的。然而，缺乏患者特异性临床结果的随访信息。方法：这是一项回顾性横断面研究，对2006年至2020年期间卢旺达15岁以下儿童进行心脏手术的临床结果进行研究。来自两家卢旺达教学医院的数据从纸质和数字患者图表中收集。结果纳入研究的406例患儿中，女性占59.1%；30.5%的儿童体重不足，28.1%的儿童发育不良。法洛四联症是最常见的紫绀型冠心病。在手术中，室间隔缺损修复是最常见的手术，PDA关闭是大多数治疗性导管。术后并发症如肺炎的发生率为6%，残留分流的发生率为17%，残留轻度或较大狭窄的发生率为41%。总死亡率为3.7%，败血症是主要死亡原因。重症监护病房住院时间超过7天、术前症状性心力衰竭和体重过轻与并发症显著相关。超过60分钟的体外循环与手术儿童的死亡率相关。结论在研究期间，卢旺达儿童冠心病的程序性治疗主要通过访问特派团完成。在临床随访资料充足的患者中，生存率为96%，并发症发生率为35%。营养不良与不良结果有关。集中的心脏登记可能会减轻14%儿童临床随访数据的缺失。

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引用次数: 0

Late diagnosis of severe aortic coarctation in a 65-year-old endurance athlete: Insights into exercise physiology and collateral compensation 65岁耐力运动员重度主动脉缩窄的晚期诊断：运动生理学和侧支代偿的见解

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-09-01 Epub Date: 2025-06-30 DOI: 10.1016/j.ijcchd.2025.100606

Arundhati Sharma , Anubhuti Sharma , Kalyan Chaliki , Marco Antonio Fernandes , Francois Marcotte , Satyajit Reddy

Background

Coarctation of the aorta (CoA) is a relatively rare congenital narrowing of the distal aortic arch to proximal descending thoracic aorta, usually diagnosed in infancy or early childhood with upper extremity hypertension as a typical finding. Late detection in asymptomatic, physically active older adults is rare and can be clinically significant.

Case presentation

We present the case of a 65-year-old lifelong endurance athlete found to have transient severe left ventricular (LV) systolic dysfunction after completing a marathon. Stress testing revealed a hypertensive response, prompting cardiac MRI, which detected a new severe juxta-ductal CoA with collateralization. The patient underwent successful endovascular stenting with improved blood pressure response and stabilization of LV function.

Conclusion

This case underscores the importance of thorough cardiovascular assessment in older athletes and highlights the impact of lifelong exercise in compensating for congenital defects like CoA.

背景主动脉缩窄（CoA）是一种相对罕见的先天性主动脉弓远端至近段胸降主动脉狭窄，通常在婴儿期或幼儿期诊断，典型表现为上肢高血压。在无症状、体力活动的老年人中，晚期发现是罕见的，可能具有临床意义。病例介绍：我们报告了一位65岁的终身耐力运动员，在完成马拉松后发现有短暂的严重左心室收缩功能障碍。压力测试显示高血压反应，促使心脏MRI检查发现新的严重导管旁CoA伴侧支。患者成功接受血管内支架植入术，血压反应改善，左室功能稳定。结论本病例强调了对老年运动员进行全面心血管评估的重要性，并强调了终身运动对补偿先天性缺陷（如CoA）的影响。

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引用次数: 0

Incidence and risk factors for the development of pulmonary arteriovenous malformations after stage 2 palliation 2期缓解后肺动静脉畸形发生的发生率和危险因素

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-09-01 Epub Date: 2025-07-18 DOI: 10.1016/j.ijcchd.2025.100611

Lea Behrend , Thibault Schaeffer , Muneaki Matsubara , Jonas Palm , Teresa Lemmen , Nicole Piber , Paul Philipp Heinisch , Stanimir Georgiev , Alfred Hager , Peter Ewert , Jürgen Hörer , Masamichi Ono

Objective

This study evaluated the current incidence of pulmonary arteriovenous malformations (PAVMs) following stage 2 palliation (S2P).

Methods

Patients who underwent S2P, either through a bidirectional cavopulmonary shunt (BCPS) or the Kawashima procedure (KP) between 1992 and 2022, were reviewed. The cumulative incidence of PAVMs was compared between BCPS and KP. Risk factors for the development of PAVMs were identified.

Results

Among 682 patients who underwent S2P, 661 (96.9 %) underwent BCPS and 21 (3.1 %) KP. Median age at S2P was 5.1 (interquartile ranges (IQR): 3.6–9.6) months. During the median interstage follow-up of 1.6 (IQR: 1.6–2.2) years, PAVMs developed in 11 (1.6 %) patients (1.1 % (n = 7) after BCPS and 19.0 % (n = 4) after KP). Cumulative incidence of PAVMs was higher in patients after KP than those after BCPS (p < 0.001). PAVMs were observed in the right lung in 9 patients and both lungs in two. One patient with biliary atresia died of progressive PAVMs and liver cirrhosis after KP, and the remaining 10 patients underwent Fontan completion with a median interval of 1.9 (IQR: 1.5–2.4) years. PAVMs improved in all patients (9 resolutions and 1 improved). Independent risk factors for the development of PAVMs were KP (hazard ratio (HR): 16.364, p < 0.001) in all patients, anomalous pulmonary venous connection (HR: 6.772, p = 0.023) in BCPS patients, and hypoplastic left heart syndrome (HR: 18.819, p = 0.018) in KP patients.

Conclusions

The incidence of PAVMs after S2P is very low after BCPS but still relevant after KP. Resolution or improvement of PAVMs is probable after Fontan completion.

目的评价2期姑息（S2P）后肺动静脉畸形（pavm）的发生率。方法回顾了1992年至2022年间通过双向腔隙肺分流术（BCPS）或Kawashima手术（KP）接受S2P的患者。比较BCPS组和KP组的累计pavm发生率。确定了发生pavm的危险因素。结果682例S2P患者中，661例（96.9%）行BCPS， 21例（3.1%）行KP。S2P时的中位年龄为5.1个月（四分位间距（IQR）： 3.6-9.6个月）。在1.6年（IQR: 1.6 - 2.2）的中位期间随访期间，11例（1.6%）患者出现了pavm，其中BCPS组为1.1% (n = 7)， KP组为19.0% （n = 4）。KP患者的累积pavm发生率高于BCPS患者（p < 0.001）。9例患者右肺出现pavm， 2例患者双肺出现pavm。1例胆道闭锁患者在KP后死于进行性pavm和肝硬化，其余10例患者完成Fontan治疗，中位间隔为1.9 （IQR: 1.5-2.4）年。所有患者的pavm均有所改善（9例缓解，1例改善）。所有患者发生pavm的独立危险因素为KP（风险比（HR）： 16.364, p < 0.001）， BCPS患者肺静脉连接异常（HR: 6.772, p = 0.023）， KP患者左心发育不全综合征（HR: 18.819, p = 0.018）。结论S2P术后pams发生率极低，而KP术后仍有相关性。在丰滩完工后，可能会解决或改善pavm。

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引用次数: 0

Clinical outcomes after implantation of bioprosthetic valves in adults with congenital heart disease 成人先天性心脏病生物瓣膜置入术的临床效果

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-09-01 Epub Date: 2025-06-03 DOI: 10.1016/j.ijcchd.2025.100598

Andrea R. Hsu , Snigdha Karnakoti , Ahmed T. Abdelhalim , Jason H. Anderson , C. Charles Jain , William R. Miranda , Elizabeth H. Stephens , Joseph A. Dearani , Hartzell V. Schaff , Heidi M. Connolly , Alexander C. Egbe

Background

Bioprosthetic valves are frequently implanted in adults with congenital heart disease (CHD), but comprehensive data on bioprosthetic valve longevity and outcomes across all valve positions are lacking. The purpose of this study was to determine the incidence and risk factors for bioprosthetic valve dysfunction (BVD) across all valve positions in adults with CHD.

Method

BVD was defined as Doppler mean gradient >40 mmHg and/or severe prosthetic/periprosthetic regurgitation for aortic or pulmonary bioprostheses, or Doppler mean gradient >10 mmHg and/or severe prosthetic/periprosthetic regurgitation for mitral or tricuspid bioprostheses.

Results

There were 1221 adults with CHD that underwent implantation of a bioprosthetic valve (age 39 ± 16 years, males 617 [51 %]). At the time of valve implantation, 755 (62 %), 325 (27 %), 176 (14 %), and 21 (1.7 %) received a bioprosthetic valve in the pulmonary, tricuspid, aortic, and mitral positions, respectively. The 10-year cumulative incidence of BVD was 28 %, 36 %, 43 %, and 68 % for pulmonary, tricuspid, aortic, and mitral bioprosthetic valves, respectively (p < 0.001). Using the pulmonary valve position as the reference group, valve position (tricuspid [HR 1.26, 95 % CI 1.09–1.58), aortic [HR 1.64, 95 % CI 1.22–1.97], mitral [HR 2.01, 95 % CI 0.83–4.05]) were associated with higher risk of BVD. Younger age was associated higher risk of BVD (HR 0.95, 95 % CI 0.86–0.98 per 5-year increment).

Conclusions

These data support the existing evidence that left-sided bioprosthetic valves demonstrate reduced durability in young patients and should be included in counseling and clinical decision-making.

成人先天性心脏病（CHD）患者经常植入生物瓣膜，但缺乏关于所有瓣膜位置的生物瓣膜寿命和预后的综合数据。本研究的目的是确定成人冠心病患者所有瓣膜位置生物瓣膜功能障碍（BVD）的发生率和危险因素。方法将dbvd定义为主动脉或肺生物假体的多普勒平均梯度为40 mmHg和/或严重假体/假体周围反流，二尖瓣或三尖瓣生物假体的多普勒平均梯度为10 mmHg和/或严重假体/假体周围反流。结果1221例成人冠心病患者行生物瓣膜植入术（年龄39±16岁，男性617例[51%]）。在瓣膜植入时，分别有755例（62%）、325例（27%）、176例（14%）和21例（1.7%）在肺动脉、三尖瓣、主动脉和二尖瓣位置植入了生物瓣膜。肺动脉瓣、三尖瓣、主动脉瓣和二尖瓣生物瓣膜10年累计BVD发生率分别为28%、36%、43%和68% (p <；0.001)。以肺动脉瓣位置为参照组，瓣膜位置（三尖瓣[HR 1.26, 95% CI 1.09-1.58]、主动脉瓣[HR 1.64, 95% CI 1.22-1.97]、二尖瓣[HR 2.01, 95% CI 0.83-4.05]）与BVD的高风险相关。年龄越小，BVD风险越高（每5年增加危险度0.95,95% CI 0.86-0.98）。结论这些数据支持了现有的证据，即左侧生物假体瓣膜在年轻患者中表现出耐久性降低，应纳入咨询和临床决策。

{"title":"Clinical outcomes after implantation of bioprosthetic valves in adults with congenital heart disease","authors":"Andrea R. Hsu , Snigdha Karnakoti , Ahmed T. Abdelhalim , Jason H. Anderson , C. Charles Jain , William R. Miranda , Elizabeth H. Stephens , Joseph A. Dearani , Hartzell V. Schaff , Heidi M. Connolly , Alexander C. Egbe","doi":"10.1016/j.ijcchd.2025.100598","DOIUrl":"10.1016/j.ijcchd.2025.100598","url":null,"abstract":"<div><h3>Background</h3><div>Bioprosthetic valves are frequently implanted in adults with congenital heart disease (CHD), but comprehensive data on bioprosthetic valve longevity and outcomes across all valve positions are lacking. The purpose of this study was to determine the incidence and risk factors for bioprosthetic valve dysfunction (BVD) across all valve positions in adults with CHD.</div></div><div><h3>Method</h3><div>BVD was defined as Doppler mean gradient >40 mmHg and/or severe prosthetic/periprosthetic regurgitation for aortic or pulmonary bioprostheses, or Doppler mean gradient >10 mmHg and/or severe prosthetic/periprosthetic regurgitation for mitral or tricuspid bioprostheses.</div></div><div><h3>Results</h3><div>There were 1221 adults with CHD that underwent implantation of a bioprosthetic valve (age 39 ± 16 years, males 617 [51 %]). At the time of valve implantation, 755 (62 %), 325 (27 %), 176 (14 %), and 21 (1.7 %) received a bioprosthetic valve in the pulmonary, tricuspid, aortic, and mitral positions, respectively. The 10-year cumulative incidence of BVD was 28 %, 36 %, 43 %, and 68 % for pulmonary, tricuspid, aortic, and mitral bioprosthetic valves, respectively (p < 0.001). Using the pulmonary valve position as the reference group, valve position (tricuspid [HR 1.26, 95 % CI 1.09–1.58), aortic [HR 1.64, 95 % CI 1.22–1.97], mitral [HR 2.01, 95 % CI 0.83–4.05]) were associated with higher risk of BVD. Younger age was associated higher risk of BVD (HR 0.95, 95 % CI 0.86–0.98 per 5-year increment).</div></div><div><h3>Conclusions</h3><div>These data support the existing evidence that left-sided bioprosthetic valves demonstrate reduced durability in young patients and should be included in counseling and clinical decision-making.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"21 ","pages":"Article 100598"},"PeriodicalIF":0.8,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144306423","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

Utilizing optical coherence tomography to explore pulmonary arteries and pulmonary vascular disease in Fontan patients 利用光学相干断层扫描探查方丹患者肺动脉和肺血管疾病

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-09-01 Epub Date: 2025-06-03 DOI: 10.1016/j.ijcchd.2025.100599

François Côté , Leonaes Brahel Tatchinda Kuete , Réda Ibrahim , Jean-Claude Tardif , Colombe Roy , François-Pierre Mongeon , Annie Dore , Blandine Mondésert , Paul Khairy , Marie-A. Chaix

Background

The Fontan procedure is the palliative intervention of choice for patients with single ventricle physiology. Pulmonary vascular disease (PVD) is a major contributor to Fontan circulatory failure. Our study utilized optical coherence tomography (OCT) imagery in pulmonary arteries to better characterize PVD in Fontan patients.

Methods

In a prospective pilot study, OCT images were acquired during cardiac catheterization in a distal left pulmonary artery (PA) branch (A10). The PA intima-media ratio and wall-vessel ratio (i.e., difference between the external elastic membrane area and the luminal area divided by the external elastic membrane area) were measured off-line.

Results

A total of 9 Fontan patients (5 women), median age of 23.0 (21.0,38.0) years, were included. All patients exhibited an intima-to-media ratio above 1.0. The ratio exceeded 2.8 in patients with high pulmonary vascular resistance. No difference is observed between patients for the wall-vessel ratio. No relation between time of completion of Fontan and intima-media ratio is identified.

Conclusion

OCT shows promise in evaluating PVD in patients with Fontan palliation. PA remodeling is characterized by intimal hyperplasia with medial regression. Further studies are required to determine the role of OCT in guiding treatment decisions and assessing therapeutic responses.

背景Fontan手术是单心室生理患者的姑息性干预选择。肺血管疾病（PVD）是方坦循环衰竭的主要原因。我们的研究利用光学相干断层扫描（OCT）成像的肺动脉更好地表征PVD在Fontan患者。方法在一项前瞻性先导研究中，在左肺动脉远端（PA）分支（A10）心导管插入术期间获得OCT图像。离线测量PA内膜-中膜比和血管壁比（即外弹性膜面积与管腔面积之差除以外弹性膜面积）。结果共纳入Fontan患者9例（女性5例），中位年龄23.0（21.0,38.0）岁。所有患者的内膜中膜比均大于1.0。在肺血管阻力高的患者中，该比值超过2.8。两组患者的血管壁比无差异。Fontan的完成时间与内膜-中膜比例没有关系。结论oct在评估Fontan姑息治疗患者PVD方面有较好的应用前景。PA重塑的特征是内膜增生伴内侧退行。需要进一步的研究来确定OCT在指导治疗决策和评估治疗反应中的作用。

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引用次数: 0

GORE® CARDIOFORM ASD occluder thrombus in a patient of rheumatoid arthritis occurred three days after deployment GORE®CARDIOFORM ASD闭塞血栓在类风湿关节炎患者部署后3天发生

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-09-01 Epub Date: 2025-06-16 DOI: 10.1016/j.ijcchd.2025.100600

Naomi Nakagawa, Yusuke Shigemitsu, Kengo Okamoto

A 67-year-old female patient with rheumatoid arthritis that was well suppressed by medications except for steroids had catheter closure of the secundum atrial septal defect (ASD). After the administration of aspirin and intravenous heparin, a 32-mm of GORE® CARDIOFORM ASD occluder was successfully deployed. On postprocedural day 3, transthoracic echocardiography showed mobile echogenic mass of 9 mm on the right side of the device. Intravenous heparin 10 IU/kg/hr was administrated continuously, and clopidogrel was added. On the following day, the mobile mass was not visible. Even though the occurrence rate of device thrombus is not high, device thrombosis can become a major complication of device closure of ASD because it may cause systemic and/or pulmonary embolism. Administration of dual antiplatelet therapy or other anticoagulant therapy should be considered if the patients have a higher risk of thrombosis.

67岁女性类风湿关节炎患者，除类固醇外药物抑制良好，导管关闭第二房间隔缺损（ASD）。在给予阿司匹林和静脉肝素后，一个32毫米的GORE®CARDIOFORM ASD闭塞器成功部署。术后第3天，经胸超声心动图显示装置右侧有9mm的可移动回声肿块。持续静脉滴注肝素10 IU/kg/hr，同时加用氯吡格雷。第二天，移动的团块就看不见了。尽管器械血栓的发生率不高，但器械血栓形成可成为ASD器械关闭的主要并发症，因为它可能导致全身和/或肺栓塞。如果患者有较高的血栓形成风险，应考虑双重抗血小板治疗或其他抗凝治疗。

引用次数: 0

Prognostic value of right ventricular-pulmonary arterial coupling in adults with repaired coarctation of aorta 成人主动脉缩窄修复后右心室-肺动脉耦合的预后价值

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-09-01 Epub Date: 2025-07-03 DOI: 10.1016/j.ijcchd.2025.100608

Alexander C. Egbe, William R. Miranda, Charles Jain, Heidi M. Connolly, Luke J. Burchill, Barry A. Borlaug

Background

Right ventricular (RV) systolic dysfunction and pulmonary hypertension is present in 20 % of adults with repaired coarctation of aorta (COA). However, the prognostic value of RV to pulmonary artery (RV-PA) coupling in this population is unknown. The purpose of this study was to assess the relationship between RV-PA coupling and clinical outcomes (heart failure hospitalization and all-cause mortality) in this population.

Methods

Retrospective cohort study of adults with repaired COA and normal RV systolic function defined as RV free wall strain ≥ -24 %. RV-PA coupling was assessed using tricuspid annular plane systolic excursion/RV systolic pressure (TAPSE/RVSP) ratio.

Results

Of 509 patients (median age 32 [20–45] years; men 290 [57 %]), the average TAPSE and RVSP were 22 ± 5 mm and 33 ± 9 mmHg, respectively, and TAPSE/RVSP ratio was 0.78 (0.56–0.96) mm/mmHg. Of 509 patients, 51 (10 %) died and 43 (8 %) were hospitalized for heart failure during a median follow-up of 8.5 (4.9–10.4) years. TAPSE/RVSP was associated with all-cause mortality (hazard ratio 0.82, 95 % confidence interval 0.78–0.86, per 0.1 mm/mmHg), and heart failure hospitalization (hazard ratio 0.86, 95 % confidence interval 0.79–0.93, per 0.1 mm/mmHg) after adjustment for anatomic lesions, cardiovascular interventions, comorbidities, and echocardiographic indices. The inclusion of TAPSE/RVSP ratio in the risk models improved prognostic power of the models to predict all-cause mortality (C-statistics difference 0.046, p < 0.001), and heart failure hospitalization (C-statistics difference 0.031, p = 0.007).

Conclusions

TAPSE/RVSP ratio was associated with outcomes in COA patients with normal RV systolic function, suggesting that abnormal RV-PA coupling may be present prior to the onset of overt RV systolic function in this population.

背景：20%的成人修复主动脉缩窄（COA）患者存在右心室（RV）收缩功能障碍和肺动脉高压。然而，RV与肺动脉（RV- pa）耦合在该人群中的预后价值尚不清楚。本研究的目的是评估该人群中RV-PA偶联与临床结果（心力衰竭住院和全因死亡率）之间的关系。方法回顾性队列研究成人COA修复，右心室收缩功能正常，右心室游离壁应变≥- 24%。采用三尖瓣环形平面收缩偏移/RV收缩压（TAPSE/RVSP）比评估RV- pa耦合。结果509例患者中位年龄32岁[20-45]；男性290例（57%），平均TAPSE和RVSP分别为22±5 mm和33±9 mmHg， TAPSE/RVSP比值为0.78 (0.56 ~ 0.96)mm/mmHg。509例患者中，51例（10%）死亡，43例（8%）因心力衰竭住院，中位随访时间为8.5年（4.9-10.4年）。在校正解剖病变、心血管干预、合共病和超声心动图指标后，TAPSE/RVSP与全因死亡率（风险比0.82,95%置信区间0.78-0.86，每0.1 mm/mmHg）和心力衰竭住院（风险比0.86,95%置信区间0.79-0.93，每0.1 mm/mmHg）相关。在风险模型中纳入TAPSE/RVSP比值提高了模型预测全因死亡率的预后能力(c -统计差异0.046,p <；心力衰竭住院（c -统计差异0.031,p = 0.007）。结论stapse /RVSP比值与右心室收缩功能正常的COA患者的预后相关，提示该人群在右心室明显收缩功能出现之前可能存在异常的RV- pa耦合。

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引用次数: 0

Pulmonary arterial hypertension treatment. A new era 肺动脉高压治疗。新时代

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-09-01 Epub Date: 2025-05-27 DOI: 10.1016/j.ijcchd.2025.100594

Sophia Anastasia Mouratoglou , Alexandra Arvanitaki , George Papadopoulos , Rogerio Souza , George Giannakoulas

Pulmonary arterial hypertension (PAH) is a progressive and life-threatening condition characterized by elevated pulmonary vascular resistance, ultimately leading to right heart failure and death. Advances in the understanding of PAH pathophysiology have driven the development of targeted therapies that improve functional capacity, quality of life and survival. The current treatment paradigm emphasizes early and aggressive therapy, with combination therapy now considered standard of care, offering superior outcomes compared to monotherapy. Herewith, we examine the underlying pathophysiological mechanisms of PAH and explore the modes of action and clinical efficacy of major therapeutic classes, providing a comprehensive overview of contemporary treatment strategies.

肺动脉高压（PAH）是一种进行性和危及生命的疾病，其特征是肺血管阻力升高，最终导致右心衰和死亡。对多环芳烃病理生理学的理解的进步推动了靶向治疗的发展，这些治疗可以改善功能能力、生活质量和生存率。目前的治疗模式强调早期和积极的治疗，与单一治疗相比，联合治疗现在被认为是标准治疗，提供了更好的结果。在此，我们研究了多环芳烃的潜在病理生理机制，并探讨了主要治疗类别的作用模式和临床疗效，提供了当代治疗策略的全面概述。

引用次数: 0

Mechanical valve replacement in adults with Fontan physiology is an effective palliation in selected patients. 机械瓣膜置换术对患有Fontan生理的成人患者是一种有效的缓解方法。

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-07-26 eCollection Date: 2025-09-01 DOI: 10.1016/j.ijcchd.2025.100612

Amr Moustafa, Ahmed T Abdelhalim, William R Miranda, Heidi M Connolly, Elizabeth H Stephens, Joseph A Dearani, Alexander C Egbe

Background: Valve regurgitation is associated with systemic ventricular dysfunction and mortality in patients with Fontan palliation. Valve repair and valve replacement with bioprostheses have limited durability, and outcomes after valve replacement with mechanical prostheses are poorly defined. The study objectives were to assess: (1) Operative mortality, valve-related complications (stroke, mechanical valve thrombosis, major bleeding complication, prosthetic valve endocarditis, and valve reoperation), and late mortality/transplant. (2) Postoperative changes in ventricular volumes and ejection fraction; (3) Postoperative changes in the clinical indices of disease severity.

Method: Retrospective cohort study of adults (age ≥18 years) with Fontan palliation who underwent isolated aortic or atrioventricular valve replacement at Mayo Clinic (2003-2023). Preoperative and postoperative evaluations (clinical evaluation, echocardiogram, biomarkers) were performed 6 months preoperatively and 6-24 months postoperatively, respectively.

Results: Overall, 17 patients underwent isolated atrioventricular (N = 9) or aortic valve (N = 8) replacement with mechanical prosthesis (age 27 ± 8 years; 9 [53 %] males). There was no operative mortality. There was postoperative improvement in functional capacity (NYHA class), heart failure indices (NT proBNP), and hepatorenal function (MELD-XI score). There was a postoperative decrease in systemic ventricular volumes, without change in ejection fraction. The 10-year cumulative incidence of valve-related complications and death/transplant was 15 % and 18 %, respectively.

Conclusions: Atrioventricular valve and aortic valve replacement with mechanical prostheses was associated with low (zero) operative mortality, low risk of valve-related complications, and clinical improvement. This suggests that valve replacement with mechanical prostheses may be an effective palliation in selected patients and may delay the need for transplant in this population.

背景：Fontan姑息治疗患者的瓣膜返流与全身性心室功能障碍和死亡率相关。生物假体瓣膜修复和瓣膜置换术的耐久性有限，机械假体瓣膜置换术后的结果也不明确。研究目的是评估：(1)手术死亡率、瓣膜相关并发症（中风、机械性瓣膜血栓形成、大出血并发症、人工瓣膜心内膜炎和瓣膜再手术）和晚期死亡率/移植。(2)术后心室容积和射血分数的变化；(3)术后疾病严重程度临床指标的变化。方法：回顾性队列研究2003-2023年在梅奥诊所（Mayo Clinic）接受Fontan姑息治疗的孤立主动脉瓣或房室瓣置换术的成人（年龄≥18岁）。术前6个月和术后6-24个月分别进行术前和术后评价（临床评价、超声心动图、生物标志物）。结果：总的来说，17例患者接受了孤立房室（N = 9）或主动脉瓣（N = 8）机械假体置换术（年龄27±8岁；9例[53%]男性）。无手术死亡率。术后功能容量（NYHA分级）、心力衰竭指数（NT proBNP）和肝肾功能（MELD-XI评分）均有改善。术后全身心室容积下降，射血分数无变化。瓣膜相关并发症和死亡/移植的10年累积发生率分别为15%和18%。结论：采用机械假体替代房室瓣和主动脉瓣与低（零）手术死亡率、低瓣膜相关并发症风险和临床改善相关。这表明，在某些患者中，机械假体瓣膜置换术可能是一种有效的缓解方法，并可能延迟移植的需要。

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