Division of Cardiology, University of Toronto, Hospital for Sick Children, 555 University Avenue, Toronto, ON M5G 1X8, Canada.
Heart Fail Clin. 2010 Oct;6(4):497-506, ix. doi: 10.1016/j.hfc.2010.06.002.
Up to 20% of all postoperative patients with repaired congenital heart disease later develop symptoms of heart failure, but slow disease progression makes patients difficult to study. The incidence is higher in the presence of lesions with a solitary or systemic right ventricle (RV). Several mechanisms cause the development of heart failure in this population, and the treatment options are not always aligned to conventional therapy for heart failure in a biventricular heart with cardiomyopathy. The pathogenesis and options for management of this complex problem in specific high-risk lesions are explored. Heart failure is commonly encountered in preoperative congenital heart disease and usually effectively dealt with by cardiac surgical or catheter-based interventions. Some lesions (single-ventricle physiology, tetralogy of Fallot, and systemic RV amongst others) are never completely repaired, and others may have persistent abnormal hemodynamic loading abnormalities; patients with these remain at risk of postoperative heart failure. Treatment is multifaceted and relies on focused surgical reintervention, timely medical therapy, and, occasionally, innovative measures, such as resynchronization therapy. The authors propose a construct whereby a primary underlying cause or substrate combined with additional remodeling stimuli results in the release of second messengers acting on classical heart failure signaling pathways. Together, these factors result in progressive heart failure in certain high-risk patients with congenital heart disease. Understanding this sequence leads to a logical application of treatments at the various stages of disease.
高达 20%的先天性心脏病修复术后患者随后会出现心力衰竭症状，但疾病进展缓慢使得患者难以研究。在存在单一或系统性右心室（RV）病变的情况下，发病率更高。多种机制导致该人群发生心力衰竭，治疗选择并不总是与双心室心肌病心力衰竭的传统治疗方法一致。本文探讨了特定高危病变中这种复杂问题的发病机制和管理选择。心力衰竭在术前先天性心脏病中很常见，通常可以通过心脏手术或导管介入治疗有效地处理。一些病变（单心室生理、法洛四联症和系统性 RV 等）从未完全修复，其他病变可能持续存在异常的血液动力学负荷异常；这些患者仍有术后心力衰竭的风险。治疗是多方面的，依赖于有针对性的再次手术干预、及时的药物治疗，以及偶尔的创新措施，如再同步治疗。作者提出了一种结构，其中主要的潜在原因或基质与其他重塑刺激相结合，导致释放作用于经典心力衰竭信号通路的第二信使。这些因素共同导致某些高危先天性心脏病患者发生进行性心力衰竭。了解这一序列可以使我们在疾病的各个阶段对治疗方法进行逻辑应用。
