Successful single-stage embolization of the source of lymphorrhea from the thoracic duct and balloonplasty of its ostium in a patient with fontan circulation

The article presents the first case of simultaneous embolization of the source of lymphorrhea from the thoracic duct and balloon plasty of its ostium for the treatment of plastic bronchitis in a patient with Fontan circulation in the Republic of Belarus.

The main complications of the lymphatic system in patients with Fontan circulation, including plastic bronchitis (PB), protein-losing enteropathy (PLE) and chylothorax (CT), cause high morbidity and mortality in this group of patients, the therapeutic options for such patients have remained limited for many years. The techniques developed in recent years, such as transnodal lymphangiography, dynamic contrast magnetic resonance lymphangiography, transhepatic lymphography, have allowed us to obtain good visualization of the lymphatic system and identify the main pathophysiological mechanisms leading to disruption of normal lymphatic circulation. These mechanisms include 2 factors: an increased flow of lymphatic fluid due to elevated venous pressure and the presence of anatomical features of the development of lymphatic vessels in which they are located extremely close to the serous (pleural cavity in chylothorax) or mucosal (plastic bronchitis and protein-losing enteropathy) layers. New minimally invasive interventional techniques such as thoracic duct embolization, interstitial embolization and embolization of the hepatic lymphatic vessels allow blocking abnormal lymphatic vessels, which leads to the elimination of symptoms. Single-stage embolization of the source of lymph leakage and improvement of venous outflow during balloon angioplasty of the ostium of the thoracic duct (TD) potentially improve the immediate and long-term outcome of treatment.

1. Fontan F., Baudet E. Surgical repair of tricuspid atresia. Thorax, 1971, vol. 26, pp. 240–248.

2. Rychik J., Atz A.M., Celermajer D.S., et al. Evaluation and Management of the child and adult with Fontan circulation: A scientific statement from the American Heart Association. Circulation, 2019, vol. 140. doi:10.1161/cir.0000000000000696.

3. Schilling C., Dalziel K., Nunn R., et al. The Fontan epidemic: Population projections from the Australia and New Zealand Fontan Registry. Int J Cardiol, 2016, vol. 219, pp. 14–19. doi:10.1016/j.ijcard.2016.05.035.

4. Schumacher K.R., Singh T.P., Kuebler J., et al: Risk factors and outcome of Fontan-associated plastic bronchitis: a case-control study. J Am Heart Assoc, 2014. doi: 10.1161/JAHA.114.000865.

5. Caruthers R.L., Kempa M., Loo A., et al. Demographic characteristics and estimated prevalence of Fontan-associated plastic bronchitis. Pediatr Cardiol, 2013, vol. 34, pp. 256–261. doi:10.1007/s00246-012-0430-5.

6. Avitabile C.M., Goldberg D.J., Dodds K., et al. A multifaceted approach to the management of plastic bronchitis after cavopulmonary palliation. Ann Thorac Surg, 2014, vol. 98, pp. 634–640. doi:10.1016/j.athoracsur.2014.04.015.

7. Schumacher K.R., Stringer K.A., Donohue J.E., et al: Fontan-Associated Protein-Losing Enteropathy and Plastic Bronchitis. J Pediatr, 2015, vol. 166(4), pp. 970–977. doi:10.1016/j.jpeds.2014.12.068.

8. John A.S., Johnson J.A., Khan M., et al. Clinical outcomes and improved survival in patients with protein-losing enteropathy after the Fontan operation. J Am Coll Cardiol, 2014, vol. 64, pp. 54–62. doi:10.1016/j.jacc.2014.04.025.

9. Goldberg D.J., Dodds K., Avitabile C.M., et al. Children with protein-losing enteropathy after the Fontan operation are at risk for abnormal bone mineral density. Pediatr Cardiol, 2012, vol. 33(8), pp. 1264–1268. doi:10.1007/s00246-012-0290-z.

10. Mertens L., Hagler D.J., Sauer U., et al. Protein-losing enteropathy after the Fontan operation: An international multicenter study. PLE study group. J Thorac Cardiovasc Surg, 1998, vol. 115, pp. 1063–1073.

11. Atz A.M., Zak V., Mahony L., et al. Longitudinal outcomes of patients with single ventricle after the fontan procedure. J Am Coll Cardiol, 2017, vol. 69, pp. 2735–2744. doi:10.1016/j.jacc.2017.03.582.

12. Tanaka M., Iwakiri Y. The Hepatic Lymphatic Vascular System: Structure,Function, Markers, and Lymphangiogenesis. Cell Mol Gastroenterol Hepatol, 2016:1-17. doi:10.1016/j.jcmgh.2016.09.002

13. Kelly B., Mohanakumar S., Hjortdal V.E. Diagnosis and Management of Lymphatic Disorders in Congenital Heart Disease. Current Cardiology Reports, 2020, vol. 22(12), pp. 164. doi:10.1007/s11886-020-01405-y.

14. Languepin J., Scheinmann P., Mahut B., et al. Bronchial casts in children with cardiopathies: the role of pulmonary lymphatic abnormalities. PediatrPulmonol, 1999, vol. 28(5), pp. 329–336. doi: 10.1002/(sici)1099-0496(199911)28:53.0.co;2-k.

15. Shah S.S.A., Drinkwater D.C., Christian K.G. Plastic bronchitis: is thoracic duct ligation a real surgical option. Ann Thorac Surg, 2006, vol. 81, pp. 2281–2283. doi:10.1016/j.athoracsur.2005.07.004.

16. Dori Y., Keller M.S., Rome J., et al. Percutaneous lymphatic embolization of abnormal pulmonary lymphatic flow as treatment of plastic bronchitis in patients with congenital heart disease. Circulation, 2016, vol. 133, pp. 1160–1170. doi: 10.1161/CIRCULATIONAHA.115.019710.

17. Dori Y., Keller M.S.M.S., Rychik J., et al: Successful treatment of plastic bronchitis by selective lymphatic embolization in a Fontan patient. Pediatrics, 2014, vol. 134, pp. e590–e595. doi:10.1542/peds.2013-3723.

18. Cope C. Diagnosis and treatment of postoperative chyle leakage via percutaneous transabdominal catheterization of the cisterna chyli: a preliminary study. J Vasc Interv Radiol, 1998, vol. 9(5), pp. 727–734. doi: 10.1016/S1051-0443(98)70382-3.

19. Blanding W.M., Denlinger C.E. Restoring the soul. J Thorac Cardiovasc Surg, 2018, vol. 156, pp. 836–837. doi:10.1016/j.jtcvs.2018.04.032.

20. Nadolski G.J., Itkin M., Dori Y., et al. Lymphangiography and thoracic ductembolization following unsuccessful thoracic duct ligation: Imaging findings and outcomes. J Vasc Interv Radiol, 2018, vol. 27, pp. S177. doi:21.1016/j.jvir.2015.12.458.

21. DePopas E.M., Veress L.A., Ahmed F., et al. Percutaneous thoracic duct intervention to treat plastic bronchitis related to Fontan palliation. Pediatr Pulmonol, 2017, vol. 52, pp. E97–E101. doi:10.1002/ppul.23793.

22. Chung A., Gill A.E., Rahman F.N., et al: Retrograde thoracic duct embolization in a pediatric patient with total cavopulmonary connection and plastic bronchitis. J Vasc Interv Radiol, 2015, vol. 26, pp. 1743–1746. doi: 10.1016/j.jvir.2015.07.023.

23. Gray M., Kovatis K.Z., Stuart T., et al: Treatment of congenital pulmonary lymphangiectasia using ethiodized oil lymphangiography. J Perinatol, 2014, vol. 34, pp. 720–722. doi:10.1038/jp.2014.71.

24. Nadolski G.J., Itkin M. Feasibility of ultrasound-guided intranodal lymphangiogram for thoracic duct embolization. J Vasc Interv Radiol, 2012, vol. 23, pp. 613–616. doi:10.1016/j.jvir.2012.01.078.

25. Nadolski G.J., Itkin M., Dori Y., et al. Lymphangiography and thoracic duct embolization following unsuccessful thoracic duct ligation: Imaging findings and outcomes. J Vasc Interv Radiol, 2018, vol. 27, pp. S177. doi:10.1016/j.jvir.2015.12.458.

26. Chung A., Gill A.E., Rahman F.N., et al. Retrograde Thoracic Duct Embolization in a Pediatric Patient with Total Cavopulmonary Connection and Plastic Bronchitis. J Vasc Interv Radiol, 2015, vol. 26, pp. 1743–1746. doi:10.1016/j.jvir.2015.07.023.