Prevalence and Cause of Early Fontan Complications: Does the Lymphatic Circulation Play a Role?

Abstract

Background Recent studies suggest that lymphatic congestion plays a role in development of late Fontan complications, such as protein‐losing enteropathy. However, the role of the lymphatic circulation in early post‐Fontan outcomes is not well defined. Methods and Results This was a retrospective, single‐center study of patients undergoing first‐time Fontan completion from 2012 to 2017. The primary outcome was early Fontan complication ≤6 months after surgery, a composite of death, Fontan takedown, extracorporeal membrane oxygenation, chest tube drainage >14 days, cardiac catheterization, readmission, or transplant. Complication causes were assigned to 1 of 4 groups: (1) Fontan circuit obstruction, (2) ventricular dysfunction or atrioventricular valve regurgitation, (3) persistent pleural effusions in the absence of Fontan obstruction or ventricular dysfunction, and (4) chylothorax or plastic bronchitis. T2‐weighted magnetic resonance imaging sequences were used to assess for lymphatic perfusion abnormality. The cohort consisted of 238 patients. Fifty‐eight (24%) developed early complications: 20 of 58 (34.5%) in group 1, 8 of 58 (14%) in group 2, 18 of 58 (31%) in group 3, and 12 of 58 (20%) in group 4. Preoperative T2 imaging was available for 126 (53%) patients. Patients with high‐grade lymphatic abnormalities had 6 times greater odds of developing early complications ( P =0.001). Conclusions There is substantial morbidity in the early post‐Fontan period. Half of those who developed early complications had lymphatic failure or persistent effusions unrelated to structural or functional abnormalities. Preoperative T2 imaging demonstrated that patients with higher‐grade lymphatic perfusion abnormalities were significantly more likely to develop early complications. This has implications for risk stratification and optimization of patients before Fontan palliation.

Association of Lymphatic Abnormalities with Early Complications after Fontan Operation

“…2,8 The rate of postoperatively thoracic lymphatic perfusion pattern abnormalities in our study is comparable to those reported by Ghosh et al before Fontan surgery. 13 Ghosh et al reported normal thoracic lymphatic perfusion pattern preoperatively in 32% of patients compared with 29% postoperatively in our cohort. While a minority of 15% developed early complications in the absence of preoperative abnormalities of thoracic lymphatic perfusion pattern, 13 none of our patients with normal lymphatic perfusion pattern postoperatively developed early Fontan complications.…”

Case Report: Disseminated Systemic Embolism of Lipiodol After Lymphography for Plastic Bronchitis After Fontan Repair

“…Systemic embolism is more likely to occur in the subgroup of Fontan patients with plastic bronchitis, but not in those with protein losing enteropathy. Indeed, such patients exhibit frequent lymphatic abnormalities even before Fontan completion (9). Very frequently Fontan patients with plastic bronchitis have an additional problem of the thoracic duct (obstruction, occlusion, absence).…”

Diagnosis and Management of Lymphatic Disorders in Congenital Heart Disease

“…The underlying pathophysiology behind PB in CHD patients is not fully understood. However, recent findings attribute a central role to a pressured and morphologically changed lymphatic system ultimately resulting in lymphatic leakage into the airways [95][96][97][98]. Similarly to PLE, the lymphatic system in patients with PB may be thought to be challenged on multiple fronts.…”

“…The elevated CVP impedes lymphatic return and causes lymphatic congestion. Additionally, the changed lymphatic architecture with the existence of multiple abnormal collaterals and larger more dilated lymphatic vessels may increase risk of leakage, with protein-rich fluid leaking into the low-pressure environment of the airways [30,31,98,99]. Following leakage, as the final step, an inflammatory reaction, preceded by concurrent respiratory infections or individual abnormalities in the inflammatory response causes the fibrin in the lymphatic fluid to cross-link, producing the solid casts pathognomonic of PB (Fig.…”

“…Future continued implementation of lymphatic interventions and increased experience with pre-operative lymphatic risk stratification may improve patient selection and reduce risk of postoperative development of lymphatic complications [ 98 ]. Targeted pharmacological treatment of lymphatic complications is wanting and drugs selectively targeting and increasing inotropic and chronotropic lymphatic function may be hypothesized to alleviate congestion and postpone or prevent the development of lymphatic complications, and their discovery should be pursued (Table 1 ).…”