Frigiola, A; (2012) Right ventricular outflow tract dysfunction following repair of congenital heart disease: Surgical solutions and clinical implications. Doctoral thesis , UCL (University College London).

Abstract

The dilemma of when to treat patients with free pulmonary incompetence, presenting late after repair of right ventricular outflow tract obstruction (in particular treated tetralogy of Fallot), is one that faces all congenital heart disease clinicians. Though we have learnt that in the long-term pulmonary incompetence is detrimental, leading to an increased incidence of adverse events (death, sustained arrhythmias, increasing symptoms), the conventional thinking has been that the benefit of treating free pulmonary incompetence is outweighed by the potential risks of surgical pulmonary valve replacement, and the lack of longevity of this treatment (conduit dysfunction within 10-15 years that exposes patients to multiple operations over their life). Thus PVR has often been delayed till patients develop symptoms, even though at this point in time symptomatic improvement following surgery may be limited due to the fact that the right ventricle has been chronically exposed to pulmonary incompetence. More recently, there has been a shift in this risk/benefit continuum: Operative mortality and morbidity are now small with modern operative methods and postoperative care, and conduit life can now be extended using new non-invasive percutaneous approaches to treat conduit dysfunction. Thus, the timing of treatment can be based more on the patients’ normalization of parameters in terms of subjective and quantitative measures of outcome and physiological response to pulmonary valve replacement. A good understanding of right ventricular pathophysiology is essential in order to aid this decision making process with regard to pulmonary valve replacement. Furthermore, as at present there is no clinically available non-invasive parameter of intrinsic myocardial function that is a simple and direct measure of systolic and diastolic function and therefore able to predict the outcome of surgery. With this research, I aim to improve the understanding of this difficult topic by systematically assessing patients with severe pulmonary regurgitation using currently available, clinical, non-invasive tools such as echocardiography, cardiopulmonary exercise testing and magnetic resonance imaging. I will also compare the results obtained from the same investigations performed before and after surgical pulmonary valve replacement in order to identify possible predictors of outcome.

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