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先天性心脏病和心力衰竭成人的超重与肥胖:来自PATHFINDER-CHD注册研究的真实世界证据。

Overweight and Obesity in Adults with Congenital Heart Disease and Heart Failure: Real-World Evidence from the PATHFINDER-CHD Registry.

作者信息

Pittrow Robert D, Kaemmerer Harald, Freiberger Annika, Achenbach Stefan, Bischoff Gert, Dewald Oliver, Ewert Peter, Engel Anna, Freilinger Sebastian, Hörer Jürgen, Holdenrieder Stefan, Huntgeburth Michael, Kaemmerer-Suleiman Ann-Sophie, Pittrow Leonard B, Kaulitz Renate, Klawonn Frank, Mellert Fritz, Nagdyman Nicole, Neidenbach Rhoia C, Schmiedeberg Wolfgang, Pittrow Benjamin A, Ury Elsa, von Scheidt Fabian, Harig Frank, Suleiman Mathieu N

机构信息

Department of Cardiac Surgery, University Hospital Erlangen, Friedrich-Alexander-University Erlangen-Nürnberg, 91054 Erlangen, Germany.

International Center for Adults with Congenital Heart Disease, Department of Congenital Heart Disease and Paediatric Cardiology, German Heart Center Munich, Technical University Munich, 80636 Munich, Germany.

出版信息

J Clin Med. 2025 Jun 27;14(13):4561. doi: 10.3390/jcm14134561.

Abstract

The PATHFINDER-CHD Registry is a prospective, multicenter, non-interventional registry across tertiary care centers in Germany. The aim is to analyze real-world data on adults with congenital heart defects (ACHD) or hereditary connective tissue disorders who have manifest heart failure (HF), a history of HF, or are at significant risk of developing HF. This analysis investigates the prevalence and clinical impact of overweight and obesity in this unique population. As of 1st February, 2025, a total of 1490 ACHD had been enrolled. The mean age was 39.4 ± 12.4 years, and 47.9% were female. Patients were categorized according to Perloff's functional class and the Munich Heart Failure Classification for Congenital Heart Disease (MUC-HF-Class). The most common congenital heart disease (CHD) in this cohort was Tetralogy of Fallot, transposition of the great arteries, and congenital aortic valve disease. Marfan syndrome was the most common hereditary connective tissue disease. Of the patients, 46.1% were classified as overweight (32.8%) or obese (13.3%), while 4.8% were underweight. The highest prevalence of overweight (47.1%) was observed among patients who had undergone palliative surgery, whereas untreated patients showed the highest proportion of normal weight (57.2%). Cyanotic patients were predominantly of normal weight. Patients with univentricular circulation exhibited significantly lower rates of overweight and obesity (35%; = 0.001). Overweight and obesity were statistically significantly associated with arterial hypertension, diabetes mellitus, and sleep apnea (all < 0.001). High BMI was linked to increased use of HF-specific medications, including SGLT2 inhibitors ( = 0.040), diuretics ( = 0.014), and angiotensin receptor blockers ( = 0.005). The data highlight the clinical relevance of overweight and obesity in ACHD with HF, emphasizing the need for individualized prevention and treatment strategies. The registry serves as a critical foundation for the optimization of long-term care in this population.

摘要

“探索者 - 冠心病注册研究”是一项在德国三级医疗中心开展的前瞻性、多中心、非干预性注册研究。其目的是分析患有先天性心脏病(ACHD)或遗传性结缔组织疾病且已出现心力衰竭(HF)、有心力衰竭病史或有显著发生心力衰竭风险的成年人的真实世界数据。该分析调查了这一独特人群中超重和肥胖的患病率及其临床影响。截至2025年2月1日,共纳入了1490例ACHD患者。平均年龄为39.4±12.4岁,47.9%为女性。患者根据佩洛夫功能分级和慕尼黑先天性心脏病心力衰竭分类(MUC - HF - Class)进行分类。该队列中最常见的先天性心脏病(CHD)是法洛四联症、大动脉转位和先天性主动脉瓣疾病。马凡综合征是最常见的遗传性结缔组织疾病。在这些患者中,46.1%被归类为超重(32.8%)或肥胖(13.3%),而4.8%体重过轻。在接受姑息手术的患者中观察到超重的患病率最高(47.1%),而未经治疗的患者体重正常的比例最高(57.2%)。发绀型患者主要体重正常。单心室循环患者超重和肥胖的发生率显著较低(35%; = 0.001)。超重和肥胖与动脉高血压、糖尿病和睡眠呼吸暂停在统计学上显著相关(均 < 0.001)。高体重指数与增加使用心力衰竭特异性药物有关,包括钠 - 葡萄糖协同转运蛋白2抑制剂( = 0.040)、利尿剂( = 0.014)和血管紧张素受体阻滞剂( = 0.005)。这些数据突出了超重和肥胖在伴有心力衰竭的ACHD中的临床相关性,强调了个性化预防和治疗策略的必要性。该注册研究是优化这一人群长期护理的关键基础。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0666/12249734/9610f8c1c9f6/jcm-14-04561-g001.jpg

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