Dhingra Nitish K, Mazer C David, Connelly Kim A, Verma Subodh
Division of Cardiac Surgery.
Department of Anesthesia.
Curr Opin Cardiol. 2023 Mar 1;38(2):82-87. doi: 10.1097/HCO.0000000000001011. Epub 2023 Jan 19.
A growing number of adult patients with congenital heart disease (ACHD) are entering the healthcare system as a result of advances in the diagnosis and management of congenital heart defects. Heart failure is a common final pathway for this diverse patient population, representing the leading cause of mortality in ACHD patients. Herein, we review present guideline-directed management of heart failure in ACHD patients.
There exists a dearth of data to guide management of ACHD-related heart failure. Given this gap, recent guidelines have been limited in the recommendations they can provide for this patient population, with practitioners being consequently forced to generalize findings from studies of acquired heart disease patients based on mechanistic plausibility. The small number of studies directly assessing ACHD patients have been largely limited in their clinical relevance through being negative, small, observational, limited to specific subsets of ACHD patients or assessing nonvalidated outcomes.
Despite the prevalence and impact of ACHD-related heart failure, there are limited evidence-based therapies for its management. Given the rising burden of this clinical problem, definitive trials assessing newer therapies are required to establish their potential role in heart failure amongst ACHD patients.
由于先天性心脏缺陷诊断和管理方面的进展，越来越多的成年先天性心脏病（ACHD）患者进入医疗保健系统。心力衰竭是这一多样化患者群体常见的最终转归途径，是ACHD患者死亡的主要原因。在此，我们综述目前针对ACHD患者心力衰竭的指南导向管理。
缺乏指导ACHD相关心力衰竭管理的数据。鉴于这一差距，近期指南在为该患者群体提供建议方面受到限制，从业者因此被迫根据机制合理性，将后天性心脏病患者的研究结果进行推广。少数直接评估ACHD患者的研究，大多因呈阴性、样本量小、为观察性研究、局限于ACHD患者的特定亚组或评估未经验证的结局，而在临床相关性方面受到限制。
尽管ACHD相关心力衰竭普遍存在且影响重大，但针对其管理的循证疗法有限。鉴于这一临床问题的负担不断加重，需要进行评估新疗法的确定性试验，以确定其在ACHD患者心力衰竭中的潜在作用。
