Theisen D, Dalla Pozza R D, Malec E, Reiser M F
Institut für Klinische Radiologie, Klinikum der Ludwig-Maximilians-Universität München, Marchioninistr. 15, 81377, München, Deutschland.
Radiologe. 2011 Jan;51(1):44-51. doi: 10.1007/s00117-010-1995-8.
With prevalences ranging from 0.26 to 0.8‰ of all live births tetralogy of Fallot (TOF) is the most common congenital heart disease with primary cyanosis. Due to improvements in surgical techniques, nearly all patients can nowadays expect to reach adulthood. After surgical repair, pulmonary regurgitation (PR) occurs in almost every child and is an important contributing factor in long-term morbidity and mortality. Cardiac magnetic resonance imaging is well established for functional assessment and flow measurements and is an ideal tool for serial post-surgical follow-up examinations, as it is non-invasive and does not expose patients to ionizing radiation. The timing of pulmonary valve replacement is crucial as right ventricular (RV) volumes have only proven to normalize when preoperative end-diastolic volumes are <170 ml/m(2) and end-systolic volumes are <85 ml/m(2). After surgical repair up to 15% of patients have residual or recurrent pulmonary artery stenosis. Distal pulmonary branch stenosis can aggravate PR and lead to right heart failure due to combined pressure and volume overload. Therefore, it has to be diagnosed in time and treated by angioplasty with or without stenting.
法洛四联症(TOF)在所有活产婴儿中的患病率为0.26‰至0.8‰,是最常见的伴有原发性发绀的先天性心脏病。由于手术技术的改进,如今几乎所有患者都有望成年。手术修复后,几乎每个儿童都会出现肺动脉反流(PR),这是导致长期发病和死亡的重要因素。心脏磁共振成像在功能评估和血流测量方面已得到广泛应用,是术后系列随访检查的理想工具,因为它是非侵入性的,不会让患者暴露于电离辐射。肺动脉瓣置换的时机至关重要,因为只有在术前舒张末期容积<170 ml/m²且收缩末期容积<85 ml/m²时,右心室(RV)容积才会恢复正常。手术修复后,高达15%的患者存在残余或复发性肺动脉狭窄。远端肺分支狭窄可加重PR,并由于压力和容量超负荷共同作用导致右心衰竭。因此,必须及时诊断并通过血管成形术(有无支架置入)进行治疗。