Anish R Katta, Shivni Patel, Miza S Hammoud, Amol Pande, Andrew Toth, Samuel M Hoenig, Yibei Zhu, Lin Chin, Rashed Mahboubi, Justin A Robinson, Tara Karamlou
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Echocardiographic data on systemic atrioventricular valvular regurgitation and systemic ventricular dysfunction were analysed. A time-varying coefficient model evaluated these factors' impact on health-related quality of life.</p><p><strong>Results: </strong>Anatomic repair had significantly higher PROMIS-10 Physical T-scores compared to physiologic repair (median 50.9 vs 41.6, <i>p</i> = 0.04). MacNew Social scores were significantly higher for medical management compared to physiologic repair (median 6.8 vs 6.0, <i>p</i> = 0.02). Echocardiographic analysis revealed that systemic ventricular dysfunction had a stronger immediate impact on health-related quality of life. Systemic atrioventricular valve regurgitation showed a delayed negative effect, significant at 6-10 years after echo, although this effect gradually decreased over time. Anatomic repair patients had better systemic ventricular function (84.6% normal), and less systemic atrioventricular valve regurgitation (69.2% had none) compared to medical management and physiologic repair groups.</p><p><strong>Conclusions: </strong>Anatomic repair improves health-related quality of life in congenitally corrected transposition of the great arteries patients, with physiologic repair showing some gains over time. Health-related quality of life data should provide important guidance regarding treatment decisions, especially in well-balanced congenitally corrected transposition of the great arteries patients.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1595-1601"},"PeriodicalIF":0.7000,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Health-related quality of life outcomes for congenitally corrected transposition of the great arteries patients across the therapeutic spectrum.\",\"authors\":\"Anish R Katta, Shivni Patel, Miza S Hammoud, Amol Pande, Andrew Toth, Samuel M Hoenig, Yibei Zhu, Lin Chin, Rashed Mahboubi, Justin A Robinson, Tara Karamlou\",\"doi\":\"10.1017/S104795112510142X\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Objective: </strong>Congenitally corrected transposition of the great arteries is a rare congenital cardiac condition with varied presentations, complicating treatment decisions. 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Echocardiographic analysis revealed that systemic ventricular dysfunction had a stronger immediate impact on health-related quality of life. Systemic atrioventricular valve regurgitation showed a delayed negative effect, significant at 6-10 years after echo, although this effect gradually decreased over time. Anatomic repair patients had better systemic ventricular function (84.6% normal), and less systemic atrioventricular valve regurgitation (69.2% had none) compared to medical management and physiologic repair groups.</p><p><strong>Conclusions: </strong>Anatomic repair improves health-related quality of life in congenitally corrected transposition of the great arteries patients, with physiologic repair showing some gains over time. 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引用次数: 0
摘要
目的：先天性纠正性大动脉转位是一种罕见的先天性心脏病，其表现多样，使治疗决策复杂化。本研究评估医疗管理、生理修复和解剖修复对健康相关生活质量的影响。方法：对克利夫兰诊所240例先天性大动脉转位患者中的50例进行横断面随访（1995-2020）。使用MacNew和promise -10问卷评估健康相关生活质量。分析全身房室瓣膜反流和全身心室功能不全的超声心动图资料。时变系数模型评估了这些因素对健康相关生活质量的影响。结果：与生理性修复相比，解剖修复的promise -10物理t评分明显更高（中位数50.9 vs 41.6, p = 0.04）。与生理修复相比，医疗管理的MacNew Social评分明显更高（中位数6.8 vs 6.0, p = 0.02）。超声心动图分析显示，全身性心室功能障碍对健康相关生活质量有更强的直接影响。系统性房室瓣膜返流表现出延迟的负面影响，在超声后6-10年显著，尽管这种影响随着时间的推移逐渐减弱。解剖修复组与生理修复组相比，全身心室功能（84.6%正常）更好，全身房室瓣膜返流（69.2%无）更少。结论：解剖修复改善了先天性大动脉转位患者的健康相关生活质量，随着时间的推移，生理性修复显示出一些改善。与健康相关的生活质量数据应该为治疗决策提供重要指导，特别是在平衡良好的先天性纠正大动脉转位患者中。
Health-related quality of life outcomes for congenitally corrected transposition of the great arteries patients across the therapeutic spectrum.
Objective: Congenitally corrected transposition of the great arteries is a rare congenital cardiac condition with varied presentations, complicating treatment decisions. This study evaluates the impact of medical management, physiologic repair, and anatomic repair on health-related quality of life.
Methods: A cross-sectional follow-up was conducted on 50 congenitally corrected transposition of the great arteries patients from a cohort of 240 at Cleveland Clinic (1995-2020). Health-related quality of life was assessed using MacNew and PROMIS-10 questionnaires. Echocardiographic data on systemic atrioventricular valvular regurgitation and systemic ventricular dysfunction were analysed. A time-varying coefficient model evaluated these factors' impact on health-related quality of life.
Results: Anatomic repair had significantly higher PROMIS-10 Physical T-scores compared to physiologic repair (median 50.9 vs 41.6, p = 0.04). MacNew Social scores were significantly higher for medical management compared to physiologic repair (median 6.8 vs 6.0, p = 0.02). Echocardiographic analysis revealed that systemic ventricular dysfunction had a stronger immediate impact on health-related quality of life. Systemic atrioventricular valve regurgitation showed a delayed negative effect, significant at 6-10 years after echo, although this effect gradually decreased over time. Anatomic repair patients had better systemic ventricular function (84.6% normal), and less systemic atrioventricular valve regurgitation (69.2% had none) compared to medical management and physiologic repair groups.
Conclusions: Anatomic repair improves health-related quality of life in congenitally corrected transposition of the great arteries patients, with physiologic repair showing some gains over time. Health-related quality of life data should provide important guidance regarding treatment decisions, especially in well-balanced congenitally corrected transposition of the great arteries patients.
期刊介绍：
Cardiology in the Young is devoted to cardiovascular issues affecting the young, and the older patient suffering the sequels of congenital heart disease, or other cardiac diseases acquired in childhood. The journal serves the interests of all professionals concerned with these topics. By design, the journal is international and multidisciplinary in its approach, and members of the editorial board take an active role in the its mission, helping to make it the essential journal in paediatric cardiology. All aspects of paediatric cardiology are covered within the journal. The content includes original articles, brief reports, editorials, reviews, and papers devoted to continuing professional development.
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