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Abstract
Background The Fontan circulation is associated with a number of long term complications including supraventricular arrhythmia, liver disease and circulatory failure. As the population of Fontan patients expands and becomes older we expect to see a rise in the number of these complications.Within Yorkshire and the Humber, we have generated a protocol of investigations for adult Fontan patients with the aim of identifying clinical decline or the development of complications in a timely manner to enable instigating treatment and/or intervention. Our aim was to review the compliance with this protocol and assess for any trends in our Fontan patients in order to develop more streamlined follow up of complications. MethodsWe retrospectively reviewed a cohort of 116 adult Fontan patients from September 2021 to February 2022. As well as collecting baseline demographic data (Table 1) on age, gender, type of Fontan and years since Fontan completion, we assessed compliance with our current clinical protocol, which includes annual echocardiography and liver assessment (serology and imaging), and three yearly cardiopulmonary exercise testing (CPEX), cardiac MRI and ambulatory ECG monitoring. As part of the liver assessment we also reviewed patients who had an Enhanced Liver Fibrosis score (ELF).
Results Of our cohort, 83 (72%) had undergone total cavopulmonary connection (TCPC), 16 (13%) lateral tunnel (LT) and 17 (15%) atrio-pulmonary connection (AP). Chart 1 shows the proportion of patients who have undergone investigations as per our protocol. The mean VO2 Max for this cohort was 21.7 mL/kg/min (range 10.5 - 34.6 mL/kg/min) which was similar across all Fontan groups with 31% (n=19) falling between the 50th and 75th Brompton centile (2). 15 (13%) patients had a cardiac device in situ, 7 (8%) of TCPC, 3 (19%) of LT and 5 (29%) of AP. Abnormal liver serology was present in 57 (52%) patients. The mean ELF score was 9.2 (range 7.3 - 11.6) which was again similar across all Fontan groups. Liver imaging was performed in 101 (87%) patients, 93 (92%) with ultrasound and 8 (7%) with MRI. In total 57 (56%) had abnormal liver imaging with a greater proportion seen within the AP Fontan group (82%) compared to both LT (69%) and TCPC (47%) groups.
Association between Root Mean Square Voltage and AHI/Hour
Association between Mean Voltage of Terminal 40msec of filtered QRS and AHI/Hour
Discussion and Conclusions A number of factors played a part in the compliance of investigations performed in this cohort of patients. This included factors such as poor mobility, negating the ability to undergo CPEX, MRI incompatible devices, non-attendance to follow up and geographical factors. One outcome of interest is the greater proportion of abnormal liver imaging seen in patients with AP Fontan, although time since Fontan completion may largely explain this finding. This review has helped shape the planning of follow up for patients with Fontan circulation in Yorkshire and the Humber. Multicentre analysis would be useful in comparing cohorts and the standard of follow up for this growing population of patients.
Conflict of Interest Nil