Abstract
Tetralogy of Fallot (ToF) is the most common type of cyanotic congenital heart disease. Since the first surgical repair in 1954, treatment has continuously improved. The treatment strategies currently used in the treatment of ToF result in excellent long-term survival (30 year survival ranges from 68.5% to 90.5%). However, residual problems such as right ventricular outflow tract obstruction, pulmonary regurgitation, and (ventricular) arrhythmia are common and often require re-interventions. Right ventricular dysfunction can be seen following longstanding pulmonary regurgitation and/or stenosis. Performing pulmonary valve replacement or relief of pulmonary stenosis before irreversible right ventricular dysfunction occurs is important, but determining the optimal timing of pulmonary valve replacement is challenging for several reasons. The biological mechanisms underlying dysfunction of the right ventricle as seen in longstanding pulmonary regurgitation are poorly understood. Different methods of assessing the right ventricle are used to predict impending dysfunction. The atrioventricular, ventriculo-arterial and interventricular interactions of the right ventricle play an important role in right ventricle performance, but are not fully elucidated. In this review we present a brief overview of the history of ToF, describe the treatment strategies currently used, and outline the long-term survival, residual lesions, and re-interventions following repair. We discuss important remaining challenges and present the current state of the art regarding these challenges.
Highlights
Tetralogy of Fallot (ToF), the most common type of cyanotic congenital heart disease (CHD), has an incidence of 0.34 per 1000 live births[1]
Patients with ToF have varying degrees of cyanosis depending on the severity of right ventricular outflow tract (RVOT) stenosis and pulmonary artery (PA) anatomy
This review focuses on the “classic” ToF, with right ventricular outflow stenosis, rather than atresia, and excluding double outlet right ventricle
Summary
Tetralogy of Fallot (ToF), the most common type of cyanotic congenital heart disease (CHD), has an incidence of 0.34 per 1000 live births[1]. Palliative balloon dilation of the pulmonary annulus can be used to increase oxygen saturation and promote growth of the pulmonary vasculature and as bridge to later complete repair in selected patients[45,46] Whether this strategy reduces TAP use or improves long-term outcomes remains controversial[45,46]. A recent study found no relationship between the presence of EDFF and other markers of diastolic dysfunction (that is, RV hypertrophy, atrial dilatation, reduced stroke volume, or reduced PR)[146] Different mechanisms, such as pulmonary arterial capacitance and atrial function[147], may play significant roles in the occurrence of EDFF. The recommendations in current guidelines are often based on long-term outcomes of studies in patients who have been operated at a much older age than has been the practice in the past 20 years. An approach using information from different sources, including history, physical examination, electrocardiogram, imaging techniques, exercise testing, and blood biomarkers, may be most useful[174]
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