Foster E
Adult Congenital Heart Disease Service, University of California, San Francisco (UCSF), School of Medicine 94143-0214, USA.
West J Med. 1995 Nov;163(5):492-8.
Patients reaching adulthood with unoperated and operated congenital heart disease require attention to issues of exercise, antibiotic prophylaxis, contraception, and pregnancy. A careful clinical history is important to establish the degree of a person's disability, if any, and the symptoms responsible for the disability, whether due to heart failure, cyanosis, or both. The findings of a physical examination and a noninvasive evaluation, including electrocardiogram, chest x-ray film, and echocardiography, are often sufficient to establish a diagnosis and to assess the adequacy of a previous operation. Transesophageal echocardiography and magnetic resonance imaging are adjunctive procedures that are indicated when routine transthoracic echocardiography is limited. Cardiac catheterization may be necessary when the noninvasive data are ambiguous and to assess coronary artery disease (congenital and acquired) in patients considered for surgical therapy. Cardiac catheterization is increasingly therapeutic (such as percutaneous pulmonary balloon valvuloplasty) as well as diagnostic. Primary surgical repair or additional surgical palliative procedures should be considered in symptomatic adults. A patient with Eisenmenger's syndrome--severe pulmonary hypertension--is a special case that may be amenable only to transplantation.
患有未经手术和接受过手术治疗的先天性心脏病并达到成年期的患者需要关注运动、抗生素预防、避孕和妊娠等问题。仔细的临床病史对于确定一个人的残疾程度（如果有的话）以及导致残疾的症状很重要，这些症状可能是由于心力衰竭、紫绀或两者兼而有之。体格检查和非侵入性评估（包括心电图、胸部X光片和超声心动图）的结果通常足以做出诊断并评估先前手术的充分性。当常规经胸超声心动图受限的时候，经食管超声心动图和磁共振成像则作为辅助检查手段。当非侵入性数据不明确且需要评估考虑接受手术治疗患者的冠状动脉疾病（先天性和后天性）时，可能需要进行心导管检查。心导管检查越来越多地用于治疗（如经皮肺动脉球囊瓣膜成形术）以及诊断。有症状的成年人应考虑进行一期手术修复或额外的手术姑息治疗。艾森曼格综合征患者——严重肺动脉高压——是一种特殊情况，可能仅适合进行移植手术。
