Abstract

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.