International journal of cardiology. Congenital heart disease最新文献_第8页

Excellent medium to long term outcomes after cardiac surgery for moderate and complex congenital heart disease, regardless of geographic location 不论地理位置如何，中度和复杂先天性心脏病心脏手术后中长期预后良好

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-03-25 DOI: 10.1016/j.ijcchd.2025.100579

Larissa Lloyd , Calum Nicholson , Geoff Strange , Rachael Cordina , David S. Celermajer , Michael M.H. Cheung

Objective

To compare the outcomes for repaired tetralogy of Fallot and Fontan patients who must travel from regional Victoria and interstate, in order to receive specialist congenital heart disease (CHD) surgery and ongoing care, with those of local patients.

Methods

This retrospective study included 332 patients who underwent tetralogy of Fallot (ToF) repair and 159 patients who underwent a Fontan procedure at Royal Children's Hospital (RCH) Melbourne between 2003 and 2017. Data was obtained from the National CHD Registry, linked with National Death Index data, and follow-up data from the Australian and New Zealand Fontan Registry.

Results

Equivalent outcomes were observed between location groups in both cohorts for all of the main outcomes of interest. Repaired ToF subjects were aged 0.76 years (IQR 0.52–3.33) at operation and 10.2 years (IQR 5.46–14.9) at last follow-up, whilst Fontan subjects were aged 4.94 (IQR 4.27–5.66) years at operation and 14.2 years (IQR 11.3–16.4) at last follow-up. Mortality rates were extremely low and did not significantly differ between geographic groups, with 10-year survival in the repaired ToF cohort 98.0 % in the City group, 98.1 % in the Regional group, and 98.8 % in the Interstate group; and 97.8 %, 92.3 %, and 97.5 % in the Fontan cohort, respectively.

Conclusions

In the Australian setting and with adequate planning and local follow-up options, patients travelling from regional areas or interstate for their CHD operations have similar outcomes, out to 21 years, compared to patients living locally.

目的比较法洛四联症和方丹四联症患者与当地患者的预后，这些患者必须从维多利亚地区和州际公路出发，才能接受专科先天性心脏病（CHD）手术和持续护理。方法本回顾性研究包括2003年至2017年在墨尔本皇家儿童医院（RCH）接受法洛四联症（ToF）修复的332例患者和接受Fontan手术的159例患者。数据来自国家冠心病登记处，与国家死亡指数数据相关联，并来自澳大利亚和新西兰Fontan登记处的随访数据。结果在两个队列的位置组之间，所有感兴趣的主要结果都是相同的。修复ToF患者手术时年龄为0.76岁（IQR 0.52-3.33），末次随访时年龄为10.2岁（IQR 5.46-14.9），而Fontan患者手术时年龄为4.94岁（IQR 4.27-5.66），末次随访时年龄为14.2岁（IQR 11.3-16.4）。死亡率极低，地理组间无显著差异，城市组修复ToF组的10年生存率为98.0%，地区组为98.1%，州际组为98.8%；Fontan组分别为97.8%、92.3%和97.5%。结论：在澳大利亚，通过适当的计划和当地随访选择，从地区或州际旅行的冠心病患者与居住在当地的患者相比，在21年内的预后相似。

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引用次数: 0

Temporal change in the age at time of death in adults with congenital heart disease 成人先天性心脏病患者死亡时年龄的时间变化

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-03-13 DOI: 10.1016/j.ijcchd.2025.100578

Zeyad Kholeif, Omar Abozied, Ahmed T. Abdelhalim, Sara ElZalabany, Amr Moustafa, Ali Ali, Alexander C. Egbe

Background

Among patients with congenital heart disease (CHD), life expectancy vary by CHD diagnosis, age of the population, and study era, making it difficult to make comparisons between studies. In this study, we aimed to address the knowledge gap regarding temporal change in age at the time of death over time across all CHD diagnoses using a large cohort of adults with CHD with longitudinal follow-up.

Methods

We studied adults with CHD that received care at Mayo Clinic from January 1, 2003 to December 31, 2023.

Results

We identified 9144 adults with CHD that received care within the study period. Of 9144 patients, 1046 (11 %) died, yielding incidence of death of 8.7 per 1000 patient-years. The incidence of death differed by CHD severity groups (7.1 versus 10.3 versus 19.3 per 1000 patient-years, p < 0.001, for simple versus moderate versus complex CHD, respectively). Similarly, the age at the time of death differed by CHD severity groups (66 ± 17 versus 57 ± 16 versus 43 ± 19 years, p < 0.001, for simple versus moderate versus complex CHD, respectively). The age at the time of death was significantly higher in the late era compared to the early era (58 ± 18 versus 50 ± 17 years p < 0.001), and this difference was consistent across CHD severity groups.

Conclusions

There was a temporal increase in the age at the time of death in adults with CHD, and this was consistent across CHD severity groups. The older age at the time of death suggests improved longevity in this population.

在先天性心脏病（CHD）患者中，预期寿命因冠心病诊断、人群年龄和研究时代而异，这使得研究之间的比较变得困难。在这项研究中，我们旨在通过纵向随访的大量冠心病成人队列，解决关于所有冠心病诊断中死亡时年龄随时间变化的知识差距。方法：我们研究了2003年1月1日至2023年12月31日在梅奥诊所接受治疗的成人冠心病患者。结果9144名成年冠心病患者在研究期间接受了护理。在9144例患者中，1046例（11%）死亡，死亡率为每1000例患者年8.7例。冠心病严重程度组的死亡发生率不同(7.1、10.3和19.3 / 1000患者-年，p <；0.001，分别为简单、中度和复杂冠心病)。同样，不同冠心病严重程度组的死亡年龄也不同(66±17岁vs 57±16岁vs 43±19岁，p <；0.001，分别为简单、中度和复杂冠心病)。死亡时的年龄晚期明显高于早期（58±18岁vs 50±17岁）p <；0.001)，这一差异在冠心病严重程度组中是一致的。结论：成年冠心病患者死亡时的年龄有时间上的增加，这在冠心病严重程度组中是一致的。死亡时的年龄越大，表明这一人群的寿命越长。

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引用次数: 0

The impact of tricuspid regurgitation on timing pulmonary valve replacement in ACHD patients after tetralogy of Fallot repair 三尖瓣反流对法洛四联修复后ACHD患者肺瓣膜置换术时机的影响

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-03-13 DOI: 10.1016/j.ijcchd.2025.100577

Gianluca Brancaccio , Veronica Bordonaro , Matteo Trezzi , Marcello Chinali , Delia Fugallo , Carolina D'Anna , Veronica Lisignoli , Marin Verrengia , Fabio Miraldi , Gianfranco Butera , Roberta Iacobelli , Benedetta Leonardi , Claudia Montanaro , Aurelio Secinaro , Lorenzo Galletti

Background

The emerging role of tricuspid regurgitation in the long-term outcome of the general population, poses the need for a review of its impact on patients with TOF and the timing for surgical PVR.

Methods

This is a retrospective, single Centre study. Clinical, imaging and surgical data of adults with repaired TOF with a transannular patch repair were collected from our database.

Results

149 patients (61 % male, mean age of 26.0 years) were selected. At a follow-up time of 25.5 years (95 % CI 24.5,26.4) 92 patients required isolated PVR at a mean time from TOF repair of 16.6 years (95 % CI 15.5,17.7) and at a mean age of 17.9 years (95 % CI 16.8,19.0). In the whole cohort, significant TR was associated with a two-fold increase likelihood of requiring PVR (log-rank p = 0-0.0023, HR 2.02; 1.09-4-47). Moderate to severe TR was more frequent in those who underwent VSD closure through a right atriotomy (17 versus 0, p = 0.0002) and affected time to PVR (22.10 yrs versus 18.07 yrs). Despite significant RV volume overload, only 16 % of the entire cohort had TR more than moderate, and the degree of TR and tricuspid valve annulus diameter correlated better with atrial rather than ventricular volumes and areas (r = 0.346; p = 0.0068).

Conclusions

TR severity of adults with transannula pacth repair of TOF correlats with surgical type of VSD closure and confers a two-fold increase in the likelihood of requiring PVR during follow-up. The close correlation of TV with atrial rather than ventricular volumes in this cohort suggests a link with the non-functional nature of TR.

背景三尖瓣反流在普通人群长期预后中的作用日益凸显，因此有必要审查其对 TOF 患者的影响以及进行 PVR 手术的时机。从我们的数据库中收集了经环状补片修复的成人 TOF 患者的临床、影像学和手术数据。结果选出了 149 名患者（61% 为男性，平均年龄 26.0 岁）。随访时间为 25.5 年（95 % CI 24.5,26.4），其中 92 例患者需要进行孤立 PVR，距离 TOF 修复的平均时间为 16.6 年（95 % CI 15.5,17.7），平均年龄为 17.9 岁（95 % CI 16.8,19.0）。在整个队列中，明显的 TR 与需要进行 PVR 的可能性增加两倍有关（log-rank p = 0-0.0023，HR 2.02；1.09-4-47）。在通过右心房切开术关闭 VSD 的患者中，中度至重度 TR 的发生率更高（17 对 0，P = 0.0002），并影响到 PVR 的时间（22.10 年对 18.07 年）。尽管 RV 容积超负荷严重，但整个队列中只有 16% 的患者 TR 超过中度，TR 程度和三尖瓣环直径与心房容积和面积的相关性比与心室容积和面积的相关性更好（r = 0.346；p = 0.0068）。在该队列中，TV与心房容积而非心室容积密切相关，这表明TR与非功能性有关。

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引用次数: 0

Atrial tachycardia in patients with repaired tetralogy of Fallot; its characteristics and catheter ablation outcome 修复法洛四联症患者心房性心动过速的研究其特点及导管消融效果

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-03-01 DOI: 10.1016/j.ijcchd.2024.100558

Qasim J. Naeemah , Miyako Igarashi , Noor K. Albakaa , Yuichi Hanaki , Noboru Ichihara , Chihiro Ota , Akira Kimata , Kojiro Ogawa , Naoto Kawamatsu , Tomoko Machino , Yuki Komatsu , Hiro Yamasaki , Akihiko Nogami , Masaki Ieda , Tomoko Ishizu

Background

Patients with repaired tetralogy of Fallot (TOF) now live longer. However, dysrhythmia becomes prevalent in adults with repaired TOF, especially atrial tachyarrhythmia.

Objective

To identify the characteristics of patients who develop atrial tachycardia (AT) and the mechanism of the clinical AT and the induced one.

Method

Seventy-seven patients with repaired TOF were enrolled. The patients were divided into two groups (AT and non-AT). Clinical and electrophysiologic data were studied.

Results

The mean age was 34 years. Twenty-three patients had AT (30 %). In AT group, the left ventricular ejection fraction was lower (58 ± 6 vs 62 ± 5; P = 0.011), the right and left atrial area (cm²) was larger (29 ± 13 vs 15 ± 5; P < 0.001, and 19 ± 3 vs 16 ± 4; P < 0.001, respectively), and the right ventricular S′ wave (cm/s) was smaller (8 ± 2 vs 10 ± 3; P = 0.029).

Patients with AT underwent catheter ablation, and 32 AT were ablated. The mechanism of AT was intra-atrial reentrant tachycardia in 14 AT (44 %), cavotricuspid isthmus-dependent in 12 AT (37 %), and focal activity in the remaining 6 AT (19 %). An important finding was that after the first AT was ablated, another AT was induced in 7 patients. The mechanism was focal in about half of them, in contrast to the first ablated AT, where the focal mechanism was the least common. After a median follow-up of 37 months, four patients had AT recurrence.

Conclusion

The patients with AT had biventricular dysfunction and bi-atrial dilatation. Aggressive induction and ablation of the induced AT may reduce the future AT recurrence.

背景：修复法洛四联症（TOF）的患者现在寿命更长。然而，心律失常在修复TOF的成年人中变得普遍，尤其是心房性心动过速。目的探讨心房心动过速（AT）患者的特点及临床和诱发性心房心动过速的发生机制。方法入选77例修复性TOF患者。患者分为AT组和非AT组。研究临床和电生理数据。结果患者平均年龄34岁。23例患者有AT（30%）。AT组左室射血分数较低（58±6 vs 62±5）；P = 0.011)，左右心房面积（cm2）较大(29±13 vs 15±5；P & lt;0.001, 19±3 vs 16±4；P & lt;右心室S波（cm/ S）较小(8±2 vs 10±3；p = 0.029)。AT患者行导管消融，32例AT消融。AT的发生机制为14例（44%）心房再入性心动过速，12例（37%）心房再入性心动过速，其余6例（19%）心房再入性心动过速。一个重要的发现是，在第一个AT消融后，7例患者又诱导了另一个AT。与第一次消融AT相比，其中大约一半的机制是焦点性的，焦点机制是最不常见的。中位随访37个月后，4例AT复发。结论AT患者存在双室功能障碍和双房扩张。积极诱导和消融诱发的AT可能减少未来AT的复发。

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引用次数: 0

Does stroke volume limit exercise capacity in TGA patients after the arterial switch operation? 动脉转换手术后，搏出量是否会限制 TGA 患者的运动能力？

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-02-15 DOI: 10.1016/j.ijcchd.2025.100576

Renée S. Joosen , Michiel Voskuil , Wieke G. de Pater , Sebastiaan W.H. van Wijk , Dominika Suchá , Abraham van Wijk , Heleen B.van der Zwaan , Gregor J. Krings , Tim Takken , Johannes M.P.J. Breur

Background

Patients with transposition of the great arteries (TGA) experience reduced exercise capacity after the arterial switch operation (ASO), possibly due to limited stroke volume. This study evaluates the role of stroke volume in reduced exercise capacity in these patients.

Methods

A retrospective analysis was conducted on TGA patients who underwent a transthoracic echocardiogram (TTE), cardiac magnetic resonance (CMR) and cardiopulmonary exercise test (CPET) within one year between September 2009 and February 2024 at the University Medical Center Utrecht. Excluding those with submaximal CPET results, the remaining patients were divided into <18 and ≥ 18 years old groups. Reduced exercise capacity was defined as a peak oxygen uptake (VO²peak) with a Z-score < -2. Left and right ventricular (LV and RV) data including volumes, function, strain and RV outflow tract obstructions were collected from TTE and CMR.

Results

A total of 126 patients (72 % male, mean age 19 ± 8 years) were included. Left ventricular function, RV volumes, function and strain were relatively preserved on CMR. Reduced VO²peak was seen in 55 % of patients ≥18 years, significantly more than those <18 years (23 %, p < 0.001). Reduced VO²peak was independently associated with time since ASO, body mass index, peak heat rate (HRpeak), and O²pulse. VO²peak showed weak to moderate correlations with time after ASO (R = −0.295,p < 0.001), body mass index (R = −0.468,p < 0.001) and HRpeak (R = 0.270,p = 0.002) and a strong correlation with O²pulse (R = 0.621,p < 0.001).

Conclusion

Exercise capacity in TGA patients after ASO might be limited by an impaired ability to increase stroke volume.

背景：大动脉转位（TGA）患者在动脉转换手术（ASO）后运动能力降低，可能是由于卒中容量有限。本研究评估卒中容量在这些患者运动能力降低中的作用。方法回顾性分析2009年9月至2024年2月在乌得勒支大学医学中心接受经胸超声心动图（TTE）、心脏磁共振（CMR）和心肺运动试验（CPET）检查的TGA患者。除CPET结果亚极大者外，其余患者分为18岁组和≥18岁组。运动能力降低定义为峰值摄氧量（vo2峰值），z分数为<；2。TTE和CMR收集左、右心室（LV和RV）数据，包括容积、功能、应变和RV流出道阻塞。结果共纳入126例患者，其中男性72%，平均年龄19±8岁。CMR上左室功能、左室容积、功能及应变均相对保存。在≥18岁的患者中，55%的患者出现vo2峰值降低，显著高于≥18岁的患者(23%,p <；0.001)。降低的vo2峰值与ASO后的时间、体重指数、峰值热率（HRpeak）和o2脉冲独立相关。vo2峰值与ASO后时间呈弱至中度相关性(R = - 0.295,p <；0.001)，体重指数(R = - 0.468,p <；0.001)和HRpeak (R = 0.270,p = 0.002)，与O2pulse (R = 0.621,p <；0.001)。结论ASO术后TGA患者的运动能力可能因增加卒中容量的能力受损而受到限制。

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引用次数: 0

Thrombus formation after the Norwood procedure: Incidence, risk factors, and its impact on late outcomes 诺伍德手术后血栓形成：发生率、危险因素及其对晚期预后的影响

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-02-15 DOI: 10.1016/j.ijcchd.2025.100575

Alessandra Poppe , Muneaki Matsubara , Jonas Palm , Thibault Schaeffer , Takuya Osawa , Carolin Niedermaier , Paul Philipp Heinisch , Nicole Piber , Bettina Ruf , Alfred Hager , Peter Ewert , Jürgen Hörer , Masamichi Ono

Objective

Thrombus formation is a feared complication after congenital heart surgery. We aimed to clarify the clinical characteristics associated with thrombus formation after the Norwood procedure.

Methods

All neonates who underwent the Norwood procedure between 2001 and 2022 were reviewed. The incidence and location of thrombus were evaluated. Risk factors for thrombus formation and its impact on survival were analyzed.

Results

Among 360 patients who were included, thrombus formation was detected in 42 patients (11.7 %) during the postoperative in-hospital period, with a median of 12 (range: 5–30) postoperative days. The most common site of thrombus was the superior vena cava in 9 (2.5 %) patients, followed by the right atrium in 8 (2.2 %). Patients who received a right ventricle to pulmonary artery conduit had a higher incidence of thrombus than those who received a modified Blalock-Taussig-Thomas shunt (16.4 vs. 7.7 %, p = 0.011). Patients with thrombus formation had a longer stay in the intensive care unit (ICU), than those without (median 21 vs. 13 days, p = 0.018). Survival after ICU discharge was lower in patients with thrombus than those without (57, 54, and 54 % vs 73, 71, and 70 % at 2, 4, and 6 years, respectively; p = 0.032). Restrictive atrial septal defect was identified as an independent risk for thrombus (odds ratio: 2.61; p = 0.005).

Conclusions

Thrombus formation was observed in 12 % of the patients during the hospital stay after the Norwood procedure and was associated with prolonged recovery and high mortality. A restrictive atrial septal defect was identified as a risk factor for thrombus formation.

目的：血栓形成是先天性心脏手术后令人担忧的并发症。我们的目的是阐明诺伍德手术后与血栓形成相关的临床特征。方法回顾性分析2001 ~ 2022年接受诺伍德手术的新生儿。评估血栓的发生率和位置。分析血栓形成的危险因素及其对生存的影响。结果纳入的360例患者中，42例（11.7%）患者在术后住院期间检测到血栓形成，中位数为12（范围：5-30）天。血栓最常见的部位为上腔静脉（2.5%），其次为右心房（2.2%）。接受右心室至肺动脉导管的患者血栓发生率高于接受改良Blalock-Taussig-Thomas分流术的患者（16.4% vs. 7.7%, p = 0.011）。血栓形成的患者在重症监护病房（ICU）的住院时间比没有血栓形成的患者更长（中位21天vs. 13天，p = 0.018）。在ICU出院后，血栓患者的生存率低于无血栓患者（分别为57、54和54% vs 73、71和70%，分别为2年、4年和6年）；p = 0.032)。限制性房间隔缺损被认为是血栓形成的独立危险因素(优势比：2.61；p = 0.005)。结论12%的患者在诺伍德手术后住院期间观察到血栓形成，并伴有较长时间的恢复和高死亡率。限制性房间隔缺损被认为是血栓形成的危险因素。

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引用次数: 0

The root of the matter: Linking oral health to chronic diseases prevention 问题的根源：将口腔健康与慢性病预防联系起来

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-02-12 DOI: 10.1016/j.ijcchd.2025.100574

Francesca D'Aiuto , Jeanie Suvan , Nisachon Siripaiboonpong , Michael A. Gatzoulis , Francesco D'Aiuto

Oral health is increasingly recognized as a vital component of general health, influencing various systemic systems. Periodontal diseases, particularly periodontitis, a chronic inflammatory condition affecting the gums and supporting tissues of teeth, have far-reaching implications beyond the oral cavity. Treating periodontitis not only benefits oral health but also plays a crucial role in reducing the burden of these chronic conditions, improving patient outcomes and lowering healthcare costs. Regular screenings for oral health issues, especially in patients with conditions such as cardiovascular disease or diabetes, should become standard practice in medical settings. Additionally, oral health professionals must be empowered to identify early signs of systemic diseases, creating a bidirectional flow of referrals between dentists and physicians. Ultimately, prioritizing oral health not only enhances individual well-being but also serves the greater public health good.

口腔健康越来越被认为是整体健康的重要组成部分，影响着各个系统。牙周病，特别是牙周炎，一种影响牙龈和牙齿支撑组织的慢性炎症，对口腔以外的疾病有着深远的影响。治疗牙周炎不仅有利于口腔健康，而且在减轻这些慢性疾病的负担、改善患者预后和降低医疗保健成本方面起着至关重要的作用。定期对口腔健康问题进行筛查，特别是对患有心血管疾病或糖尿病等疾病的患者，应成为医疗机构的标准做法。此外，口腔卫生专业人员必须有权识别全身性疾病的早期迹象，在牙医和医生之间建立双向转诊。最终，优先考虑口腔健康不仅可以提高个人福祉，还可以为更大的公共卫生服务。

引用次数: 0

My journey with pulmonary hypertension: From diagnosis to thriving 我的肺动脉高压之旅：从诊断到康复

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-01-31 DOI: 10.1016/j.ijcchd.2025.100572

Hall Skaara

引用次数: 0

Pulmonary arterial hypertension in Latin America. The age and comorbidity paradox 肺动脉高压在拉丁美洲。年龄和共病悖论

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-01-31 DOI: 10.1016/j.ijcchd.2025.100573

Tomas Pulido , Sofia de la Cruz-Perez , Daniela Valencia , Rafael Conde , Adrian Lescano , Nayeli Zayas

Pulmonary arterial hypertension (PAH) has been classically described as a disease in young adults, predominantly females with no comorbidities. However, in recent registries, the epidemiology has changed to older patients with comorbidities such as obesity, diabetes, systemic hypertension, and coronary heart disease. Nevertheless, there is not enough inclusion of these patients in clinical trials. In contrast, in Latin America, registries have shown that PAH patients are younger and have fewer comorbidities, which raises the question of whether Latin American patients present a different phenotype or if we are lagging behind developed countries and whether we will experience a change in epidemiology in the next couple of years. We analyzed these trends in this review.

肺动脉高压（PAH）一直被经典地描述为一种年轻人的疾病，主要是女性，没有合并症。然而，在最近的登记中，流行病学已经改变为有合并症的老年患者，如肥胖、糖尿病、全身性高血压和冠心病。然而，在临床试验中没有足够的纳入这些患者。相比之下，在拉丁美洲，登记显示PAH患者更年轻，合并症更少，这就提出了一个问题：拉丁美洲患者是否表现出不同的表型，或者我们是否落后于发达国家，以及我们是否会在未来几年内经历流行病学的变化。我们在这篇综述中分析了这些趋势。

引用次数: 0

New therapies in pulmonary arterial hypertension: Recent insights 肺动脉高压的新疗法：最近的见解

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-01-30 DOI: 10.1016/j.ijcchd.2025.100571

Giulia Guglielmi , Konstantinos Dimopoulos , S. John Wort

Pulmonary Arterial Hypertension (PAH) is a complex and progressive disease characterized by elevated pulmonary vascular resistance and right heart failure. Current therapies primarily focus on pulmonary vasodilation; however, novel approaches that target the underlying pathophysiological mechanisms—such as TGF-β signalling, epigenetic alterations, growth factors, inflammation, and extracellular matrix remodelling—are promising alternatives for improving treatment outcomes. This is a review of recent advances in the development of innovative therapeutic strategies for PAH.

The first section of this paper explores approaches targeting TGF-β signalling, both acting directly on receptors through drugs like Sotatercept and exogenous BMP9, and indirectly, inhibiting the degradation of key receptors, such as BMPR2. Subsequent sections describe treatments that target epigenetic regulators, e.g. poly (ADP-ribose) polymerase-1 (PARP-1) inhibitors and direct BRD4 antagonists, tyrosine kinase inhibitors (Seralutinib), and therapies aimed at inflammation, such as IL-6 inhibitors, CD-20 inhibitors, and monoclonal antibodies that prevent macrophage migration. Finally, strategies that target the serotonin pathway, and other metabolic and hormonal pathways are described.

This review includes both preclinical and clinical trial data that support efficacy, safety and the future potential of such therapies. Collectively, these therapeutic approaches can be valuable in treating PAH by targeting multiple aspects of its pathogenesis, potentially resulting in improved clinical outcomes for patients affected by this debilitating, life-limiting condition.

肺动脉高压（PAH）是一种复杂的进行性疾病，以肺血管阻力升高和右心衰为特征。目前的治疗主要集中在肺血管扩张；然而，针对潜在病理生理机制（如TGF-β信号传导、表观遗传改变、生长因子、炎症和细胞外基质重塑）的新方法有望改善治疗效果。本文综述了多环芳烃创新治疗策略的最新进展。本文第一部分探讨了针对TGF-β信号传导的方法，既可以通过sotaterept和外源性BMP9等药物直接作用于受体，也可以间接作用于抑制关键受体（如BMPR2）的降解。随后的章节描述了针对表观遗传调节因子的治疗，例如聚（adp -核糖）聚合酶-1 （PARP-1）抑制剂和直接BRD4拮抗剂，酪氨酸激酶抑制剂（Seralutinib），以及针对炎症的治疗，例如IL-6抑制剂，CD-20抑制剂和阻止巨噬细胞迁移的单克隆抗体。最后，描述了针对血清素途径以及其他代谢和激素途径的策略。本综述包括临床前和临床试验数据，这些数据支持此类疗法的有效性、安全性和未来潜力。总的来说，这些治疗方法可以通过针对其发病机制的多个方面来治疗多环芳烃，有可能改善这种使人衰弱、限制生命的疾病患者的临床结果。

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引用次数: 0