ACE INHIBITION IN SINGLE VENTRICLE/PULMON. HYPERTENSION

描述（由申请人提供） 本申请的总体目标是检查治疗方式， 可提高两组先天性心脏病患者的临床护理水平 疾病：婴儿出生时只有一个心室，为心脏供血。 肺和身体和儿童与肺动脉高压相关 先天性心脏病婴儿中的主要假设是 心室是慢性血管紧张素转换酶（ACE）抑制 有利地改变心室重构对容量超负荷的反应， 在生命的第一年改善心室功能。连续变化 将使用磁共振成像评估心室几何形状， 与收缩和舒张功能的测量结果相比， 压力/容量关系和Tei指数，以及临床结局指标 包括术后病程和罗斯的变化心力衰竭 分类.预期ACE抑制的有益效果会发生 双向Glenn容量卸载手术前后 分流术或半Fontan术先天性心脏病研究的基本假设 与肺动脉高压相关的疾病是长期的影响， 用口服前列环素类似物或口服内皮素受体治疗 阻滞剂对运动能力、寿命和运动质量有良好的影响。 生活还将确定这些患者中是否有任何人携带缺陷 原发性肺动脉高压-1基因。这些研究中的每一项都可以 潜在地导致预后的显著改善：在单个 心室组通过防止心室的长期恶化 功能和肺动脉高压患者通过提高质量 没有移植的生命和生存。

DESCRIPTION (provided by applicant) The overall goal of this application is to examine treatment modalities which may improve the clinical care of two groups of patients with congenital heart disease: infants born with a single ventricle supplying blood flow to the lungs and body and children with pulmonary hypertension associated with congenital heart disease. The primary hypothesis in infants with single ventricle is that chronic angiotensin converting enzyme (ACE) inhibition favorably modifies the ventricular remodeling response to volume overload and improves ventricular function over the first year of life. Serial changes in ventricular geometry will be assessed using magnetic resonance imaging and compared with measurements of systolic and diastolic function, including the pressure/volume relation and the Tei index, and clinical outcome measures including post-operative course and changes in the Ross? heart failure classification. The beneficial effect of ACE inhibition is expected to occur prior to and following volume unloading surgery with the bidirectional Glenn shunt or hemi-Fontan. The primary hypothesis of the study in congenital heart disease associated with pulmonary hypertension is that the effect of long-term treatment with an oral prostacyclin analogue or an oral endothelin receptor blocker has a salutory effect on exercise capacity, longevity, and quality of life. It will also be determined whether any of these patients carry a defect of the primary pulmonary hypertension-1 gene. Each of these studies could potentially lead to a significant improvement in prognosis: in the single ventricle group by preventing a long-term deterioration in ventricular function and in the pulmonary hypertension patients by improving quality of life and survival without transplantation.