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Tetralogy/DORV surgery peri-operative pathway
This is an agreed pathway for the peri-operative management of patients undergoing primary repair of Fallot’s and DORV continuum. It may well be considered applicable to patients with analogous physiological substrate. It is not intended as a binding or detailed clinical guideline but rather represents an agreed consistent strategy. Similarly, it does not seek to cover every aspect of care, particularly where other guidelines apply.
This is a demanding and highly variable group of patients who have a significant mortality and morbidity risk and frequently have long PICU stays.
In 2000, 9.1% of the 261 operations to repair ToF in the UK, resulted in death within 1 year (ie 24 deaths in one year). In 2015 the 30 Day mortality for ToF was zero. The 1 year mortality data from 2015 is not yet available. The post-operative survival data in the UK and Ireland for ToF remains daunting. NICCOR data over its entire 16 year data set (4382 operations) shows a 30 Day Mortality is 1.3% and (3843 operations) 1 year mortality of 3.0%. For the most recent 5 years of data the 30 day mortality is 0.4% (2011-15inc, 1514 operations) and 1 year mortality is 1.9% (2009-13 inc, 1545 operations). Post-discharge mortality in this group is rare.
This means effectively that we would reasonably expect 49 in 50 patients in this group to survive surgical repair and the post-operative course. This risk burden can reasonably be assumed to be much higher in those patients with less favourable ToF anatomy and physiology but individual characterisation of risk in this group remains difficult and inexact.
RV dysfunction is insidious. The results of ECLS in this group are poor.
It is, of course, inevitable that deviation from the pathway will be necessary but that this will be done after cross-speciality discussion and clinical review of the patient.
Last reviewed: 15 October 2018
Next review: 15 October 2021
Author(s): Andrew McLean, Mark Davidson, Judith McEwen, Patrick Noonan
Approved By: Cardiac Risk & Quality Group