PDF(Pubmed)
Abstract：
UNASSIGNED: Systemic right ventricle (RV) dysfunction is associated with lower transplant-free survival (TFS) in hypoplastic left heart syndrome (HLHS), but the likelihood of functional improvement and utility of heart failure (HF) medications is not understood.
UNASSIGNED: The authors aimed to describe TFS, HF medication use, and surgical interventions in HLHS patients with RV dysfunction with and without subsequent improvement in function.
UNASSIGNED: The SickKids HF Database is a retrospective cohort that includes all pediatric HLHS patients with RV dysfunction lasting >30 days. We compared TFS, HF medications, and surgical interventions in HLHS patients with and without functional normalization.
UNASSIGNED: Of 99 patients with HLHS and RV dysfunction, 52% had normalized function for ≥30 days. TFS at 2 years after dysfunction onset was lower in those without normalization (14% vs 78%, P < 0.001). Patients without normalization were less likely to reach target dosing (TD) of HF medications (27% vs 47% on 1 medication at TD, P < 0.001) and undergo Fontan completion (7% vs 53%, P < 0.001). Clinical factors associated with improved TFS were normalization of function for ≥30 days, onset of dysfunction after bidirectional Glenn, and exposure to ACE inhibition.
UNASSIGNED: Our cohort of HLHS patients with systemic RV dysfunction demonstrated a novel finding of improved TFS in those with functional normalization for ≥30 days. Achieving TD of HF medications was associated with improved outcomes. This may reflect patient stability and tolerance for HF medication more than its therapeutic effect, but it can help inform decisions to proceed with surgical palliation or list for transplant.
UNASSIGNED: The authors aimed to describe TFS, HF medication use, and surgical interventions in HLHS patients with RV dysfunction with and without subsequent improvement in function.
UNASSIGNED: The SickKids HF Database is a retrospective cohort that includes all pediatric HLHS patients with RV dysfunction lasting >30 days. We compared TFS, HF medications, and surgical interventions in HLHS patients with and without functional normalization.
UNASSIGNED: Of 99 patients with HLHS and RV dysfunction, 52% had normalized function for ≥30 days. TFS at 2 years after dysfunction onset was lower in those without normalization (14% vs 78%, P < 0.001). Patients without normalization were less likely to reach target dosing (TD) of HF medications (27% vs 47% on 1 medication at TD, P < 0.001) and undergo Fontan completion (7% vs 53%, P < 0.001). Clinical factors associated with improved TFS were normalization of function for ≥30 days, onset of dysfunction after bidirectional Glenn, and exposure to ACE inhibition.
UNASSIGNED: Our cohort of HLHS patients with systemic RV dysfunction demonstrated a novel finding of improved TFS in those with functional normalization for ≥30 days. Achieving TD of HF medications was associated with improved outcomes. This may reflect patient stability and tolerance for HF medication more than its therapeutic effect, but it can help inform decisions to proceed with surgical palliation or list for transplant.
摘要：
■系统性右心室（RV）功能障碍与左心发育不全综合征（HLHS）的无移植生存率（TFS）降低有关，但心力衰竭（HF）药物的功能改善和效用的可能性尚不清楚。
■作者旨在描述TFS，HF药物使用，和手术干预的HLHS患者的RV功能障碍有或没有随后的功能改善。
SickKidsHF数据库是一个回顾性队列研究，包括所有RV功能障碍持续>30天的儿童HLHS患者。我们比较了TFS，HF药物,以及有和没有功能正常化的HLHS患者的手术干预。
■在99例HLHS和RV功能障碍患者中，52%的正常功能≥30天。在未正常化的患者中，功能障碍发作后2年的TFS较低(14%vs78%，P<0.001)。未正常化的患者不太可能达到目标剂量（TD）的HF药物（27％vs47％的1种药物在TD，P<0.001）并进行Fontan完成（7%vs53%，P<0.001)。与改善TFS相关的临床因素是功能正常化≥30天，双向Glenn后出现功能障碍，和暴露于ACE抑制。
■我们的具有系统性RV功能障碍的HLHS患者队列显示了在功能正常化≥30天的患者中改善的TFS的新发现。实现HF药物的TD与改善的结果相关。这可能反映了患者对HF药物的稳定性和耐受性超过其治疗效果，但它可以帮助告知决定进行手术缓解或移植清单。
■作者旨在描述TFS，HF药物使用，和手术干预的HLHS患者的RV功能障碍有或没有随后的功能改善。
SickKidsHF数据库是一个回顾性队列研究，包括所有RV功能障碍持续>30天的儿童HLHS患者。我们比较了TFS，HF药物,以及有和没有功能正常化的HLHS患者的手术干预。
■在99例HLHS和RV功能障碍患者中，52%的正常功能≥30天。在未正常化的患者中，功能障碍发作后2年的TFS较低(14%vs78%，P<0.001)。未正常化的患者不太可能达到目标剂量（TD）的HF药物（27％vs47％的1种药物在TD，P<0.001）并进行Fontan完成（7%vs53%，P<0.001)。与改善TFS相关的临床因素是功能正常化≥30天，双向Glenn后出现功能障碍，和暴露于ACE抑制。
■我们的具有系统性RV功能障碍的HLHS患者队列显示了在功能正常化≥30天的患者中改善的TFS的新发现。实现HF药物的TD与改善的结果相关。这可能反映了患者对HF药物的稳定性和耐受性超过其治疗效果，但它可以帮助告知决定进行手术缓解或移植清单。
