Das Bibhuti, Deshpande Shriprasad, Akam-Venkata Jyothsna, Shakti Divya, Moskowitz William, Lipshultz Steven E
Department of Pediatrics, Division of Cardiology, University of Mississippi Medical Center, 2500 North State Street, Jackson, MS, 39216, USA.
Department of Pediatrics, Children's National Hospital, The George Washington University, Washington, DC, USA.
Pediatr Cardiol. 2023 Mar;44(3):513-529. doi: 10.1007/s00246-022-02960-7. Epub 2022 Aug 17.
Diastolic dysfunction (DD) refers to abnormalities in the mechanical function of the left ventricle (LV) during diastole. Severe LVDD can cause symptoms and the signs of heart failure (HF) in the setting of normal or near normal LV systolic function and is referred to as diastolic HF or HF with preserved ejection fraction (HFpEF). Pediatric cardiologists have long speculated HFpEF in children with congenital heart disease and cardiomyopathy. However, understanding the risk factors, clinical course, and validated biomarkers predictive of the outcome of HFpEF in children is challenging due to heterogeneous etiologies and overlapping pathophysiological mechanisms. The natural history of HFpEF varies depending upon the patient's age, sex, race, geographic location, nutritional status, biochemical risk factors, underlying heart disease, and genetic-environmental interaction, among other factors. Pediatric onset HFpEF is often not the same disease as in adults. Advances in the noninvasive evaluation of the LV diastolic function by strain, and strain rate analysis with speckle-tracking echocardiography, tissue Doppler imaging, and cardiac magnetic resonance imaging have increased our understanding of the HFpEF in children. This review addresses HFpEF in children and identifies knowledge gaps in the underlying etiologies, pathogenesis, diagnosis, and management, especially compared to adults with HFpEF.
舒张功能障碍(DD)是指左心室(LV)在舒张期机械功能出现异常。严重的左心室舒张功能障碍可在左心室收缩功能正常或接近正常的情况下引发症状及心力衰竭(HF)体征,被称为舒张性心力衰竭或射血分数保留的心力衰竭(HFpEF)。儿科心脏病专家长期以来一直在推测先天性心脏病和心肌病患儿中存在HFpEF。然而,由于病因异质性和病理生理机制重叠,了解儿童HFpEF的危险因素、临床病程以及可预测其预后的有效生物标志物具有挑战性。HFpEF的自然病程因患者的年龄、性别、种族、地理位置、营养状况、生化危险因素、潜在心脏病以及基因-环境相互作用等因素而异。儿童期起病的HFpEF往往与成人的情况不同。通过应变、斑点追踪超声心动图、组织多普勒成像和心脏磁共振成像进行左心室舒张功能的无创评估取得的进展,增进了我们对儿童HFpEF的了解。本综述探讨了儿童HFpEF,并确定了在潜在病因、发病机制、诊断和管理方面的知识空白,特别是与成人HFpEF相比。