Salaets Thomas, Gewillig Marc, Van De Bruaene Alexander, Mertens Luc L
Division of Pediatric Cardiology, University Hospitals Leuven, Leuven, Belgium.
The Labatt Family Heart Centre, Division of Cardiology, The Hospital for Sick Children, Department of Paediatrics, University of Toronto, Toronto, Canada.
JACC Adv. 2024 Nov 20;3(12):101411. doi: 10.1016/j.jacadv.2024.101411. eCollection 2024 Dec.
The right ventricle (RV) in hypoplastic left heart syndrome (HLHS) becomes the systemic ventricle pumping against systemic afterload. It also has to adapt to an initially increased volume load followed by a decrease in volume load after Fontan completion. Anatomical HLHS subtype, therapeutic strategy, tricuspid valve regurgitation, recoarctation, and genetics influence RV size and function. The resulting remodeling process can be maladaptive and lead to ventricular systolic and diastolic dysfunction. While systolic dysfunction is a strong predictor for mortality before Fontan, there is increasing evidence for the impact of progressive diastolic dysfunction after Fontan. This comprehensive review summarizes the (recent) empirical observations that increased understanding of RV remodeling and function in HLHS. It aims at clinicians and researchers wishing to increase their understanding of the physiology of this disease. It highlights the potential for future scientific work on the assessment and preservation of myocardial health throughout the palliation.
在左心发育不全综合征（HLHS）中，右心室（RV）成为对抗体循环后负荷进行泵血的体循环心室。它还必须适应最初增加的容量负荷，随后在Fontan手术完成后容量负荷降低。解剖学HLHS亚型、治疗策略、三尖瓣反流、再缩窄和遗传学影响右心室大小和功能。由此产生的重塑过程可能是适应不良的，并导致心室收缩和舒张功能障碍。虽然收缩功能障碍是Fontan手术前死亡率的强有力预测指标，但越来越多的证据表明Fontan手术后进行性舒张功能障碍的影响。这篇综述总结了（近期）实证观察结果，这些结果增进了对HLHS中右心室重塑和功能的理解。它针对希望增进对该疾病生理学理解的临床医生和研究人员。它强调了未来在整个姑息治疗过程中评估和保护心肌健康的科学工作潜力。
