Current outcomes and treatment of tetralogy of Fallot

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.

Understanding and Recognition of the Right Ventricular Function and Dysfunction: A Numerical Study

“…Focusing on the LV PV loops, the computed shift is always downward/leftward in all the investigated cases i.e., LV work reduces. Obviously, since the left ventricle mechanical functionality is fully maintained, such a ventricular work reduction results from the entanglement between the LV and the suffering RV, which progressively drops the SV 50 . Notice also that the effect of RV systolic dysfunction on LV is more severe in the CD than in the SD case.…”

“…In particular, the computed EF behaviour suggests that both EFRV and EFLV have to be evaluated in right diseases (e.g., Tetralogy of Fallot). Indeed, van der Ven et al 50 recently pointed out that the role of LV in Tetralogy of Fallot can not be neglected, despite LV parameters are not considered in current guidelines for the assessment of the most appropriate timing of intervention 50 .…”

An Adult Patient With a Tetralogy of Fallot Case

“…ToF may present with different phenotypes ranging from mild to severe cases such as Fallot-type double outlet right ventricle or ToF with pulmonary atresia [3]. Management approaches differ based on severity.…”

Stenting of the right ventricular outflow tract with subsequent radical correction in a child with a tetralogy of Fallot: results of six-year follow-up

“…Современные стратегии лечения ТФ демонстрируют высокий уровень долгосрочной выживаемости (30-летняя выживаемость колеблется от 68,5% до 90,5%). Тем не менее, такие проблемы, как обструкция выводного отдела правого желудочка (ВОПЖ), регургитация на клапане легочной артерии (кЛА) и желудочковые аритмии достаточно распространены у данных пациентов и часто требуют повторных вмешательств [1]. Это подразумевает необходимость дальнейшего совершенствования тактических и технических аспектов хирургического лечения, включая внедрение гибридных методов лечения, что особо актуально в когорте маловесных новорожденных с тяжелой гипоксией.…”

“…На настоящий момент перспективной альтернативой паллиативному хирургическому вмешательству является эндоваскулярный метод в виде стентирования ВОПЖ, позволяющий добиться уменьшения выраженности гипоксического синдрома, нормализовать легочный кровоток и состояние ветвей легочной артерии (ЛА), подготовить левый желудочек к радикальной коррекции порока [1,3].…”