Abstract
BackgroundThere is no consensus on the clinical utility of ‘routine’ diagnostic cardiac catheterisation in patients with Fontan palliation in the absence of symptoms or haemodynamic lesions.ObjectiveWe sought to evaluate whether diagnostic cardiac catheterisation for a variety of indications led to a change in the clinical management of patients with a Fontan circulation.MethodsAll adult patients (≥16 years) with Fontan palliation undergoing diagnostic cardiac catheterisation at our institution from 2016 to 2019 were included retrospectively. Patients undergoing electrophysiological studies were excluded as haemodynamic measurements were not taken. Routine cardiac catheterisation at our institution is considered in adult patients who have not had a diagnostic cardiac catheter for more than 5 years.ResultsThirty-eight patients, mean age 27 ± 7 years, 60% NYHA I, 31% NYHA II, 8% NYHA III, at mean duration post Fontan of 20 ± 6 years, lateral tunnel (LT) n = 20, extracardiac (EC) n = 14 and atriopulmonary (AP) n = 4, underwent 41 diagnostic cardiac catheterisation procedures. Indication for cardiac catheterisation was as follows: haemodynamic lesion identified on cross-sectional imaging in 12; routine catheterisation in 9; cyanosis in 8; dyspnoea in 8; significant liver stiffness on ultrasound hepatic elastography in 2; and arrhythmia in 2. Of the 9 patients undergoing routine diagnostic catheterisation, 3 had not had any diagnostic catheterisation since their Fontan completion and, in the remaining six, the mean time lapsed since the last diagnostic catheter was 8 ± 3 years. The diagnostic catheterisation led to a recommended change in clinical management on 24 occasions (59%): catheter intervention in 17 (40%); surgery in 4 (10%); medication change in 3 (17%); and transplant referral in 2 (5%). The clinical indications that led to changes in clinical management were: cyanosis (8/8), dyspnoea (7/8), haemodynamic lesions on cross-sectional imaging (8/11) and arrhythmia (1/2). None of the 9 patients listed for routine diagnostic catheterisation or as a result of findings on ultrasound hepatic elastography had a recommended change in clinical management.ConclusionDiagnostic cardiac catheterisation frequently leads to changes in the clinical management of patients with Fontan palliation presenting with dyspnoea, cyanosis, and for further evaluation of potential haemodynamic lesions identified on cross-sectional imaging. Routine cardiac catheterisation in the absence of the above indications had limited impact on clinical management in our cohort.
Highlights
Most Fontan patients reach adulthood [1,2,3]; a standardised long-term follow-up strategy has not been agreed, partly reflecting the non-standardised management of these challenging patients across institutions
Diagnostic cardiac catheterisation frequently leads to changes in the clinical management of patients with Fontan palliation presenting with dyspnoea, cyanosis, and for further evaluation of potential haemodynamic lesions identified on cross-sectional imaging
Diagnostic catheterisation led to a recommended change in clinical management in 24 occasions (59%): catheter intervention in 17 (40%); surgery in 4 (10%); medication change in 3 (17%); and transplant referral in 2 (5%) (Fig. 1)
Summary
Most Fontan patients reach adulthood [1,2,3]; a standardised long-term follow-up strategy has not been agreed, partly reflecting the non-standardised management of these challenging patients across institutions. The risk and severity of these complications increases over time and with advancing age, and early identification and treatment of any haemodynamic drivers for the development of these complications is essential [3]. Previous studies suggested that these patients present a bimodal pattern, with a cumulative risk of death from thromboembolism increasing during the first 3 years after Fontan palliation and again 15 years after surgery [4, 5], reiterating the importance of surveillance. Recent findings indicate that the use of cardiac MRI for postFontan surveillance follows this bimodal trend [1], with significantly greater odds of a change in management following clinically indicated MRI after Fontan as compared to screening MRI. There is no consensus on the clinical utility of ‘routine’ diagnostic cardiac catheterisation in patients with Fontan palliation in the absence of symptoms or haemodynamic lesions
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