Introduction A Fontan procedure is a palliative surgical approach for patients whose congenital heart defects mean they have a single ventricle. Lifelong, specialist follow up for these complex patients is crucial. The European Society of Cardiology (ESC) guidelines (2010) recommend an annual review carried out at a specialised Grown Up Congenital Heart (ACHD) centre that should include an ECG, echocardiogram and exercise testing, and blood tests covering haematology, serum albumin, renal function and liver function, as well as cardiac MRI and liver ultrasounds are carried out at appropriate intervals.
Methods A retrospective analysis of Fontan patients at a large quaternary Specialist ACHD centre over the past three years using patient notes was performed.
Results Data was collected on 214 patients; 45% (n = 96) were female and 55% (n = 118) male.
The mean age of the cohort was 28 +/- 9.27 years (range=17–68 years). 20 (9%) patients had died by the time of audit. 49% (n = 105) had a Modified AP Fontan, 41% (n = 87) had a TCPC Fontan and 10% (n = 22) had a Lateral Tunnel Fontan. The mean age at time of Fontan procedure was 8 +/- 7.69 years (range = 1–52 years).
In 2014, most patients, 81% (n = 158) of patients had a specialist review appointment.
69% (n = 134) had an ECG and over half, 55% (n = 107) had an echo.
Only 46 patients (29%) underwent CPEX testing. Only 26% of patients (n = 50) had a liver ultrasound.
Almost a third of patients, 29% (n = 48) had a cardiac MRI (27 patients contraindicated due to pacemakers/ICD).
53% (n = 102) had haematology bloods performed, 56% (n = 109) had serum creatinine, 56% (n = 108) had serum albumin and liver function tests and 42% (n = 81) had alpha-fetoprotein bloods. Over the 3 year period, 25 Fontan patients (13%) had abnormal liver function blood tests, but did not attend for a liver ultrasound.
Conclusions Although the guidelines are not being fulfilled, a number of patients do not attend for their recommended appointments. We propose an annual one-stop specialist Fontan clinic, to try and minimise appointments and improve adherence to guidelines.
We also propose that these recommendations should be re-assessed to see if they are realistic, such as the need for an annual echocardiogram in a clinically stable patient and how they could be adapted, especially in a young cohort, many of whom are in full time education or employment. Updating these Guidelines and a one-stop specialist clinic may accommodate this unique population and could improve patient care as well as reduce DNA rates for multiple appointments.
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