Cocomello Lucia, Taylor Kurt, Caputo Massimo, Cornish Rosie P, Lawlor Deborah A
MRC Integrative Epidemiology Unit, Population Health Sciences, Bristol Medical School, University of Bristol, Oakfield House, Bristol, United Kingdom.
Bristol Heart Institute, Bristol, United Kingdom.
Front Cardiovasc Med. 2022 Jun 10;9:870474. doi: 10.3389/fcvm.2022.870474. eCollection 2022.
Advances in the management of congenital heart disease (CHD) patients have enabled improvement in long-term survival even for those with serious defects. Research priorities (for patients, families and clinicians) have shifted from a focus on how to improve survival to exploring long-term outcomes in patients with CHD. A comprehensive appraisal of available evidence could inform best practice to maximize health and well-being, and identify research gaps to direct further research toward patient and clinical need. We aimed to critically appraise all available published systematic reviews of health and well-being outcomes in adult patients with CHD.
We conducted an umbrella review, including any systematic reviews that assessed the association of having vs. not having CHD with any long-term health (physical or mental), social (e.g., education, occupation) or well-being [e.g., quality of life (QoL)] outcome in adulthood (≥18-years).
Out of 1330 articles screened, we identified five systematic reviews of associations of CHD with adult outcomes. All but one (which studied QoL) explored health outcomes: one cardiovascular, two mental, and one mortality after transplant. CHD patients had a higher risk of stroke, coronary heart disease and heart failure, with the pooled relative risk (RR) for any outcome of 3.12 (95% CI: 3.01 to 3.24), with substantial heterogeneity (I = 99%) explained by the outcome being studied (stronger association for heart failure) and geography (stronger in Europe compared with other regions). CHD patients had a higher risk of anxiety (OR = 2.58 (1.45 to 4.59)], and higher mean scores for depression/anxiety symptoms (difference in means = -0.11 SD (-0.28 to 0.06), I = 94%)]. Compared with patients having a cardiac transplant for other (non-CHD) diseases, CHD patients had higher short-term mortality (RR at 30-days post-transplant = 2.18 [1.62 to 2.93)], with moderate heterogeneity (I = 41%) explained by previous surgery (higher mortality with prior Fontan/Glenn operation). All domains of QoL were lower in patients with Fontan's circulation than non-CHD adults.
Adults with CHD have poorer cardiovascular, mental health and QoL outcomes, and higher short-term mortality after transplant. The paucity of systematic reviews, in particular for outcomes such as education, occupation and lifestyles, highlights the need for this to be made a priority by funders and researchers.
[www.crd.york.ac.uk/prospero], identifier [CRD42020175034].
先天性心脏病（CHD）患者管理方面的进展使长期生存率得以提高，即使是那些有严重缺陷的患者。（针对患者、家庭和临床医生的）研究重点已从关注如何提高生存率转向探索CHD患者的长期预后。对现有证据进行全面评估可为最佳实践提供参考，以最大限度地促进健康和福祉，并找出研究差距，从而针对患者和临床需求指导进一步研究。我们旨在严格评估所有已发表的关于成年CHD患者健康和福祉结局的系统评价。
我们进行了一项汇总分析，纳入任何评估患有与未患有CHD与成年期（≥18岁）任何长期健康（身体或心理）、社会（如教育、职业）或福祉[如生活质量（QoL）]结局之间关联的系统评价。
在筛选的1330篇文章中，我们确定了5篇关于CHD与成年结局关联的系统评价。除一篇研究生活质量的外，其余均探讨了健康结局：一项心血管结局、两项心理结局和一项移植后死亡率结局。CHD患者发生中风、冠心病和心力衰竭的风险更高，任何结局的合并相对风险（RR）为3.12（95%CI：3.01至3.24），结局研究（心力衰竭的关联更强）和地理位置（欧洲比其他地区更强）导致了显著的异质性（I² = 99%）。CHD患者焦虑风险更高（OR = 2.58（1.45至4.59）]，抑郁/焦虑症状的平均得分更高（均值差异 = -0.11 SD（-0.28至0.06），I² = 94%）]。与因其他（非CHD）疾病进行心脏移植的患者相比，CHD患者短期死亡率更高（移植后30天的RR = 2.18 [1.62至2.93]），中度异质性（I² = 41%）可由既往手术解释（既往进行Fontan/Glenn手术的死亡率更高）。与非CHD成年人相比，接受Fontan循环的患者生活质量的所有领域均较低。
成年CHD患者的心血管、心理健康和生活质量结局较差，移植后的短期死亡率更高。系统评价的匮乏，尤其是关于教育、职业和生活方式等结局的评价，凸显了资助者和研究人员将此作为优先事项的必要性。
