Subscribe to RSS
![](/products/assets/desktop/img/oa-logo.png)
DOI: 10.1055/s-0040-1722178
Association of Lymphatic Abnormalities with Early Complications after Fontan Operation
![](https://www.thieme-connect.de/media/thoracic/2021S03/lookinside/thumbnails/10-1055-s-0040-1722178_205970pcc-1.jpg)
Abstract
Background Increased central venous pressure is inherent in Fontan circulation but not strongly related to Fontan complication. Abnormalities of the lymphatic circulation may play a crucial role in early Fontan complications.
Methods This was a retrospective, single-center study of patients undergoing Fontan operation from 2008 to 2015. The primary outcome was significant early Fontan complication defined as secondary in-hospital treatment due to peripheral edema, ascites, pleural effusions, protein-losing enteropathy, or plastic bronchitis. All patients received T2-weighted magnetic resonance images to assess abdominal and thoracic lymphatic perfusion pattern 6 months after Fontan completion with respect to localization, distribution, and extension of lymphatic perfusion pattern (type 1–4) and with application of an area score (0–12 points).
Results Nine out of 42 patients developed early Fontan complication. Patients with complication had longer chest tube drainage (mean 28 [interquartile range [IQR]: 13–60] vs. 13 [IQR: 2–22] days, p = 0.01) and more often obstructions in the Fontan circuit 6 months after surgery (56 vs. 15%, p = 0.02). Twelve patients showed little or no abnormalities of lymphatic perfusion (lymphatic perfusion pattern type 1). Most frequently magnetic resonance imaging showed lymphatic congestion in the supraclavicular region (24/42 patients). Paramesenteric lymphatic congestion was observed in eight patients. Patients with early Fontan complications presented with higher lymphatic area score (6 [min–max: 2–10] vs. 2 [min–max: 0–8]), p = 0.001) and greater distribution and extension of thoracic lymphatic congestion (type 3–4: n = 5/9 vs. n = 1/33, p = 0.001).
Conclusion Early Fontan complication is related to hemodynamic factors such as circuit obstruction and to the occurrence and extent of lymphatic congestion.
Keywords
congenital heart disease - Fontan operation - lymphatic perfusion pattern - cardiovascular magnetic resonance imagingPublication History
Received: 09 July 2020
Accepted: 30 October 2020
Article published online:
31 December 2020
© 2020. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)
Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany
-
References
- 1 Marshall KH, D'Udekem Y, Sholler GF. et al. Health-related quality of life in children, adolescents, and adults with a Fontan circulation: a meta-analysis. J Am Heart Assoc 2020; 9 (06) e014172
- 2 Schleiger A, Ovroutski S, Peters B. et al. Treatment strategies for protein-losing enteropathy in Fontan-palliated patients. Cardiol Young 2020; 30 (05) 698-709
- 3 Mertens L, Hagler DJ, Sauer U, Somerville J, Gewillig M. Ple Study Group. Protein-losing enteropathy after the Fontan operation: an international multicenter study. PLE study group. J Thorac Cardiovasc Surg 1998; 115 (05) 1063-1073
- 4 Meadows J, Jenkins K. Protein-losing enteropathy: integrating a new disease paradigm into recommendations for prevention and treatment. Cardiol Young 2011; 21 (04) 363-377
- 5 Gewillig M, Brown SC. The Fontan circulation after 45 years: update in physiology. Heart 2016; 102 (14) 1081-1086
- 6 Rychik J. Protein-losing enteropathy after Fontan operation. Congenit Heart Dis 2007; 2 (05) 288-300
- 7 Ovroutski S, Sohn C, Barikbin P. et al. Analysis of the risk factors for early failure after extracardiac Fontan operation. Ann Thorac Surg 2013; 95 (04) 1409-1416
- 8 Rychik J, Atz AM, Celermajer DS. et al; American Heart Association Council on Cardiovascular Disease in the Young and Council on Cardiovascular and Stroke Nursing. Evaluation and management of the child and adult with Fontan circulation: a scientific statement from the American Heart Association. Circulation 2019; •••: CIR0000000000000696
- 9 Alsaied T, Possner M, Brown N. et al. Lymphopenia in adults after the Fontan operation: prevalence and associations. Cardiol Young 2020; 30 (05) 641-648
- 10 Udink Ten Cate FE, Hannes T, Germund I. et al. Towards a proposal for a universal diagnostic definition of protein-losing enteropathy in Fontan patients: a systematic review. Heart 2016; 102 (14) 1115-1119
- 11 Feldt RH, Driscoll DJ, Offord KP. et al. Protein-losing enteropathy after the Fontan operation. J Thorac Cardiovasc Surg 1996; 112 (03) 672-680
- 12 John AS, Johnson JA, Khan M, Driscoll DJ, Warnes CA, Cetta F. Clinical outcomes and improved survival in patients with protein-losing enteropathy after the Fontan operation. J Am Coll Cardiol 2014; 64 (01) 54-62
- 13 Ghosh RM, Griffis HM, Glatz AC. et al. Prevalence and cause of early Fontan complications: does the lymphatic circulation play a role?. J Am Heart Assoc 2020; 9 (07) e015318
- 14 Book WM, Gerardin J, Saraf A, Marie Valente A, Rodriguez III F. Clinical phenotypes of Fontan failure: implications for management. Congenit Heart Dis 2016; 11 (04) 296-308
- 15 Biko DM, DeWitt AG, Pinto EM. et al. MRI evaluation of lymphatic abnormalities in the neck and thorax after Fontan surgery: relationship with outcome. Radiology 2019; 291 (03) 774-780
- 16 Rychik J, Dodds KM, Goldberg D. et al. Protein losing enteropathy after Fontan operation: glimpses of clarity through the lifting fog. World J Pediatr Congenit Heart Surg 2020; 11 (01) 92-96
- 17 Glöckler M, Severin T, Arnold R. et al. First description of three patients with multifocal lymphangiomatosis and protein-losing enteropathy following palliation of complex congenital heart disease with total cavo-pulmonary connection. Pediatr Cardiol 2008; 29 (04) 771-774
- 18 Wang Y, Moss J, Thisted R. Predictors of body surface area. J Clin Anesth 1992; 4 (01) 4-10
- 19 Gill CC, Moodie DS, McGoon DC. Staged surgical management of pulmonary atresia with diminutive pulmonary arteries. J Thorac Cardiovasc Surg 1977; 73 (03) 436-442
- 20 Nakata S, Imai Y, Takanashi Y. et al. A new method for the quantitative standardization of cross-sectional areas of the pulmonary arteries in congenital heart diseases with decreased pulmonary blood flow. J Thorac Cardiovasc Surg 1984; 88 (04) 610-619
- 21 Rompel O, Janka R, May MS. et al. Cardiac MRI in children and adolescents who have undergone surgical repair of right-sided congenital heart disease: automated left ventricular volumes and function analysis and effects of different manual adjustments. RoFo Fortschr Geb Rontgenstr Nuklearmed 2015; 187 (12) 1099-1107
- 22 Meadows J, Gauvreau K, Jenkins K. Lymphatic obstruction and protein-losing enteropathy in patients with congenital heart disease. Congenit Heart Dis 2008; 3 (04) 269-276
- 23 Aspelund A, Robciuc MR, Karaman S, Makinen T, Alitalo K. Lymphatic system in cardiovascular medicine. Circ Res 2016; 118 (03) 515-530
- 24 Kovacikova L, Krasnanova V, Skrak P. et al. Immune abnormalities in patients with single ventricle circulation precede the Fontan procedure. World J Pediatr Congenit Heart Surg 2017; 8 (06) 672-682
- 25 Magdo HS, Stillwell TL, Greenhawt MJ. et al. Immune abnormalities in Fontan protein-losing enteropathy: a case-control study. J Pediatr 2015; 167 (02) 331-337
- 26 Mattes M, Connor J, Kelly SS, Schwartz MC. Lymphopenia in patients with single-ventricle heart disease after the Fontan operation. Congenit Heart Dis 2016; 11 (03) 270-275