Frontiers in Fontan failure: Innovation and improving outcomes: A conference summary

Abstract

The initial "Frontiers in Fontan Failure" conference in 2015 in Atlanta, Georgia, provided an opportunity for experts in the field of pediatric cardiology and adult congenital heart disease to focus on the etiology, physiology, and potential interventions for patients with "Failing Fontan" physiology. Four types of "Fontan Failure" were described and then published by Dr Book et al. The acknowledgment that even Dr Fontan himself realized that the Fontan procedure "imposed a gradually declining functional capacity and premature late death after an initial period of often excellent palliation." The purpose of the second "Frontiers in Fontan Failure" was to further the discussion regarding new data and technologies as well as novel interventions. The 2017 "Frontiers in Fontan Failure: Innovation and Improving Outcomes" was sponsored by Children's Healthcare of Atlanta, Sibley Heart Center Cardiology, and Emory University School of Medicine. Future directions in the management of Fontan failure include further investigations into the risk of sudden cardiac death and how to properly prevent it, achievable interventions in modifying the Fontan physiology to treat or prevent late complications, and improved and refined algorithms in Fontan surveillance. Finally, further research into the interventional treatment of lymphatic-related complications hold the promise of marked improvement in the quality of life of advanced Fontan failure patients and as such should be encouraged and contributed to. K E Y W O R D S failure, Fontan, innovation, outcomes | 129 EphrEm Et al.

Long-term management of Fontan patients: The importance of a multidisciplinary approach

“…The anatomical changes brought by the subversion of the heart anatomy lead to a predisposition to arrhythmias, which are an important contributor to morbidity. Furthermore, arrhythmias can increase the risk of thromboembolic events (TEs), which have a mortality rate of 25% ( 23 , 24 ). On the other hand, all patients with Fontan have per se a higher risk of TE related to the low-flow state, atrial stasis, and increased central venous pressure; in particular, the latter causes hepatic dysfunction and therefore coagulation abnormalities ( 23 , 25 ).…”

“…These findings suggest the possibility of a simpler antithrombotic regimen, for example, starting with warfarin in the first 3–6 months, then switching to aspirin ( 32 ). At the moment, no risk stratification scores for TE are available for patients with Fontan and the standard risk stratification scores, such as CHADS2/CHADS2-VASc scores, seem to be not predictive in patients with congenital heart disease, as evidenced in a relevant multicenter study ( 24 , 34 ). More studies are needed to develop a risk stratification tool to guide antithrombotic therapy and to compare the different treatment strategies.…”

“…Routine surveillance of oxygen saturation, chest X-ray, and pulmonary function testing are the best options to evaluate pulmonary status. MR lymphography based on heavily T2-weighted sequences can visualize the vascular architecture and eventually map abnormal networks in the chest ( 24 ) and, when performed in conjunction with endoscopy, can show leakage into the bowels ( 37 ). The goal of treatment, both in PLE and PB, is to optimize systemic circulation and reduce the overload with diuretic therapy.…”

Neutrophil-to-Lymphocyte and Platelet-to-Lymphocyte Ratio in Univentricular Patients From Birth to Follow-Up After Fontan—Predicting Lymphatic Abnormalities

“…Treatment and care of patients with univentricular heart defects has undeniably made great progress in recent years. Despite the therapeutic achievements, Fontan patients continue to experience high complication rates after Fontan surgery including the development of the clinically challenging protein-losing enteropathy (PLE) and plastic bronchitis (4)(5)(6)(7). The pathophysiological changes leading to Fontan failure and PLE are multifactorial and cannot yet be answered completely.…”

Lymphatic Decompression Concomitant With Fontan/Kreutzer Procedure: Early Experience

“…[16][17][18][19] The new lymphatic-based diagnosis tools, particularly T2 MRI, contrast dynamic magnetic resonance (MR) lymphangiography, and innovative procedures, both surgical or via lymphatic intervention, may provide the promise of significant positive impact on long-term outcomes, reducing morbidity and mortality and improving quality of life. 20 The aim of this study was to assess the feasibility of a new MRI protocol for pre-Fontan/Kreutzer procedure lymphatic risk classification combined with the introduction of lymphatic decompression (LD) in the Fontan/Kreutzer completion operation for patients with severe lymphatic thoracic abnormalities.…”