Innominate vein turn-down procedure: Killing two birds with one stone

Abstract

See Commentaries on pages 261 and 263.Central MessageInnominate vein turn-down procedure improves lymphatic drainage by decompressing the thoracic duct to the lower-pressure system, with concomitant increase of systemic preload in Fontan circulation. See Commentaries on pages 261 and 263. Innominate vein turn-down procedure improves lymphatic drainage by decompressing the thoracic duct to the lower-pressure system, with concomitant increase of systemic preload in Fontan circulation. Feature Editor Note—In an effort to design a perfect third-stage palliation for our single-ventricle patients, our specialty has been making tremendous efforts to compensate for the well-known disadvantages of a circulatory setting involving a lifelong state of high systemic venous pressure. While we aim to excel at each stage of single-ventricle palliation to prevent a suboptimal Fontan circulation, our focus has indeed sharpened to also understand the variations and impact of the lymphatic system on the outcome of patients before or following third-stage palliation. Novel diagnostic techniques using magnetic resonance imaging aid in visualizing the problems in lymphatic drainage in a standardized manner, whereas novel targeted interventional and surgical techniques by experienced teams offer invaluable solutions for one of the conundrums of the Fontan circulation. Dr Hraśka and expert colleagues graciously contribute to this issue of the Journal with a multidisciplinary invited expert review of their current surgical and interventional strategies to address lymphatic system disturbances in Fontan circulation. The article overviews a clear diagnostic classification of lymphatic pathologies, leading to a decision-making tree. Medical, interventional, and surgical options are discussed in detail. The authors offer an updated cohort with characteristics and outcome of patients who were treated with the innovative innominate vein turn-down procedure. This excellent contribution is accompanied by images and illustrations. The congenital editors thank the authors for this invaluable presentation of their experience. Can Yerebakan, MD While systemic venous hypertension is the most prominent hemodynamic disturbance in the Fontan circulation, recent work has identified the consequent lymphatic hypertension and impaired drainage as pathophysiologic in the development of pleural effusions, edema, ascites, plastic bronchitis (PB), and protein-losing enteropathy (PLE).1Rychik R. Atz A.M. Celermajer D.S. Deal B.J. Gatzoulis M.A. Gewillig M.H. et al.Evaluation and management of the child and adult with Fontan circulation: a scientific statement from the American Heart Association.Circulation. 2019; 139: e1-e51PubMed Google Scholar, 2Hraska V. Mitchell M.E. Woods R.K. Hoffman G.M. Kindel S.J. Ginde S. Innominate vein turn-down procedure for failing Fontan circulation.Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann. 2020; 23: 34-40Abstract Full Text Full Text PDF PubMed Scopus (5) Google Scholar, 3Kreutzer C. Kreutzer G. The lymphatic system: the Achilles heel of the Fontan-Kreutzer circulation.World J Pediatr Congenit Heart Surg. 2017; 8: 613-623Crossref PubMed Scopus (18) Google Scholar, 4Gosh R.M. Dori Y. Prevalence and cause of early Fontan complications: does the lymphatic circulation play a role?.J Am Heart Assoc. 2020; 9: e015318PubMed Google Scholar Novel interventional and surgical approaches have been developed to treat post-Fontan lymphatic complications with promising outcomes.2Hraska V. Mitchell M.E. Woods R.K. Hoffman G.M. Kindel S.J. Ginde S. Innominate vein turn-down procedure for failing Fontan circulation.Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann. 2020; 23: 34-40Abstract Full Text Full Text PDF PubMed Scopus (5) Google Scholar, 3Kreutzer C. Kreutzer G. The lymphatic system: the Achilles heel of the Fontan-Kreutzer circulation.World J Pediatr Congenit Heart Surg. 2017; 8: 613-623Crossref PubMed Scopus (18) Google Scholar, 4Gosh R.M. Dori Y. Prevalence and cause of early Fontan complications: does the lymphatic circulation play a role?.J Am Heart Assoc. 2020; 9: e015318PubMed Google Scholar, 5Dori Y. Keller M.S. Rychik J. Itkin M. Successful treatment of plastic bronchitis by selective lymphatic embolization in a Fontan patient.Pediatrics. 2014; 134: e590-e595Crossref PubMed Scopus (88) Google Scholar, 6Dori Y. Keller M.S. Rome J.J. Gillepsie M.J. Glatz A.C. Dodds K. et al.Percutaneous lymphatic embolization of abnormal pulmonary lymphatic flow as treatment of plastic bronchitis in patients with congenital heart disease.Circulation. 2016; 22: 1160-1170Crossref Scopus (150) Google Scholar, 7Hraska V. Decompression of thoracic duct: new approach for the treatment of failing Fontan.Ann Thorac Surg. 2013; 96: 709-711Abstract Full Text Full Text PDF PubMed Scopus (41) Google Scholar, 8Smith C.L. Hoffman T.M. Dori Y. Rome J.J. Decompression of the thoracic duct: a novel transcatheter approach.Catheter Cardiovasc Interv. 2020; 95: E56-E61Crossref PubMed Scopus (12) Google Scholar, 9Kreutzer C. Lymphatic decompression concomitant with Fontan/Kreutzer procedure: early experience.World J Pediatr Congenit Heart Surg. 2020; 11: 284-292Crossref PubMed Google Scholar Under normal physiological conditions, an estimated 2 to 3 L of protein-rich fluid are filtered out from the blood circulation and into the interstitial space and returned to the blood circulation by the lymphatic vessels. The lymphatic vasculature is a ubiquitous bodily net functioning as a unidirectional drainage and transport system that is responsible for returning all interstitial fluid back to the blood circulation. The lymphatic vessels contract to propel the fluid forward, with contractile properties comparable with those of heart ventricles. Similar to the heart and intestines, pacemaker cells have been proposed to uphold frequency and secure continuous movement through the system,10Boedtkjer D.B. Rumessen J. Baandrup U. Skov Mikkelsen M. Telinius N. Pilegaard H. et al.Identification of interstitial Cajal-like cells in the human thoracic duct.Cells Tissues Organs. 2013; 197: 145-158Crossref PubMed Scopus (20) Google Scholar with ion channels required for depolarization.11Mohanakumar S. Majgaard J. Telinius N. Katballe N. Pahle E. Hjortdal V. et al.Spontaneous and a-adrenoreceptor-induced contractility in human collecting lymphatic vessels require chloride.Am J Physiol Heart Circ Physiol. 2018; 315: H389-H401Crossref PubMed Scopus (8) Google Scholar In addition, both increased tension in the vessel wall and adrenergic innervation have been shown to increase contraction frequency, enabling an estimated 10-fold increase in fluid removal if needed. The thoracic duct (TD) has a critical role in lymphatic transport—draining approximately 85% of lymph production from the entire body except for the right hemithorax, right head and neck, and right arm. The majority of TD flow comes from the liver and intestine, and in the majority of cases the TD drains at the posterior aspect of the left internal jugular and subclavian vein confluence and, when present, the right TD drains at the right subclavian jugular vein confluence. A valve prior to its drainage prevents blood flow into the TD. There are 2 distinct pressure elements that substantially determine the TD pressure—a forward pressure from lymph formation and a backpressure from the venous pressure at the lymph drainage point.2Hraska V. Mitchell M.E. Woods R.K. Hoffman G.M. Kindel S.J. Ginde S. Innominate vein turn-down procedure for failing Fontan circulation.Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann. 2020; 23: 34-40Abstract Full Text Full Text PDF PubMed Scopus (5) Google Scholar,12James H. Witte M.H. Bernas M. Barber B. Proposal for prevention or alleviation of protein/lymph-losing enteropathy (PLE/LLE) after Fontan circulation treatment of univentricular hearts: Restoration of lymph balance with a “lymphatic right-to-left shunt.”.Lymphology. 2016; 49: 114-127PubMed Google Scholar In the failing Fontan circulation, the central venous pressure (CVP) often exceeds 18 to 20 mm Hg. There is experimental evidence that such elevation of CVP leads to a significant increase of lymphatic production in the hepatosplanchnic region, resulting in greater pressure and flow in the TD. At the same time, the transport capacity of the TD is limited by increasing outflow pressure (backpressure exerted by elevated CVP), demonstrated by a clear breakpoint in the lymph flow–outflow pressure curve. At outflow pressures above the breakpoint, lymph flow decreases linearly with outflow pressure and ceases at an outflow pressure of 24 mm Hg.2Hraska V. Mitchell M.E. Woods R.K. Hoffman G.M. Kindel S.J. Ginde S. Innominate vein turn-down procedure for failing Fontan circulation.Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann. 2020; 23: 34-40Abstract Full Text Full Text PDF PubMed Scopus (5) Google Scholar,13Brace R.A. Valenzuela G.J. Effects of outflow pressure and vascular volume loading on thoracic duct lymph flow in adult sheep.Am J Physiol. 1990; 258: R240-R244PubMed Google Scholar In the Fontan circulation, the transport capacity of the TD is further limited by dilatation of the TD, resulting in incompetence of the lymphatic valves and stasis, and by lack of “diastolic suctioning” of lymph. In addition, lymphovenous communications, either to systemic or pulmonary veins, are not open and thus cannot decompress the lymphatic system.3Kreutzer C. Kreutzer G. The lymphatic system: the Achilles heel of the Fontan-Kreutzer circulation.World J Pediatr Congenit Heart Surg. 2017; 8: 613-623Crossref PubMed Scopus (18) Google Scholar An altered lymphatic architecture with vessels adjacent to a lower pressure lumen may lead to symptomatic lymphatic failure. In patients with PLE, lymphatic congestion and flow obstruction result in decompression of the lymphatic system through a route of lower resistance in the duodenal wall, with protein-rich lymphatic fluid spilling out into the low-pressure environment of the intestines.14Biko D.M. DeWitt A.G. Pinto E.M. Morrison M.E. Johnstone J.A. Griffis H. et al.MRI evaluation of lymphatic abnormalities in the neck and thorax after Fontan surgery: relationship with outcome.Radiology. 2019; 291: 774-780Crossref PubMed Scopus (35) Google Scholar,15Biko D.M. Smith C.L. Otero H.J. Saul D. White A.M. DeWitt A. et al.Intrahepatic dynamic contrast MR lymphangiography: initial experience with a new technique for the assessment of liver lymphatics.Eur Radiol. 2019; 29: 5190-5196Crossref PubMed Scopus (20) Google Scholar In the lungs, increased afterload resistance and increased lymphatic production may cause lymphatic breakthrough to the pulmonary airways, causing the devastating condition PB. The long-term effect of the lymphatic congestion on the contractional capacity is due to functional impairment with losing the ability to generate a greater pressure and increase contraction frequency. The contraction frequency and strength of the peripheral lymphatic system can be measured with near-infrared fluorescent imaging.11Mohanakumar S. Majgaard J. Telinius N. Katballe N. Pahle E. Hjortdal V. et al.Spontaneous and a-adrenoreceptor-induced contractility in human collecting lymphatic vessels require chloride.Am J Physiol Heart Circ Physiol. 2018; 315: H389-H401Crossref PubMed Scopus (8) Google Scholar,16Mohanakumar S. Telinius N. Kelly B. Lauridsen H. Boedtkjer D. Pedersen M. et al.Morphology and function of the lymphatic vasculature in patients with a Fontan circulation.Circ Cardiovasc Imaging. 2019; 12: e008074Crossref PubMed Scopus (24) Google Scholar Magnetic resonance imaging (MRI) is the imaging modality of choice for screening patients with thoracic lymphatic disorders and for imaging lymphatic anatomy and flow in patients with lymphatic failure.5Dori Y. Keller M.S. Rychik J. Itkin M. Successful treatment of plastic bronchitis by selective lymphatic embolization in a Fontan patient.Pediatrics. 2014; 134: e590-e595Crossref PubMed Scopus (88) Google Scholar,14Biko D.M. DeWitt A.G. Pinto E.M. Morrison M.E. Johnstone J.A. Griffis H. et al.MRI evaluation of lymphatic abnormalities in the neck and thorax after Fontan surgery: relationship with outcome.Radiology. 2019; 291: 774-780Crossref PubMed Scopus (35) Google Scholar,15Biko D.M. Smith C.L. Otero H.J. Saul D. White A.M. DeWitt A. et al.Intrahepatic dynamic contrast MR lymphangiography: initial experience with a new technique for the assessment of liver lymphatics.Eur Radiol. 2019; 29: 5190-5196Crossref PubMed Scopus (20) Google Scholar,17Dori Y. Smith C.L. DeWitt A.G. Srinivasan A. Krishnamurthy G. Escobar F.A. et al.Intramesenteric dynamic contrast pediatric MR lymphangiography: initial experience and comparison with intranodal and intrahepatic MR lymphangiography.Eur Radiol. 2020; 30: 5777-5784Crossref PubMed Scopus (4) Google Scholar MRI has been applied to visualize the central lymphatic architecture using heavily T2-weighted images in patients with single-ventricle physiology. T2 MRI has also been shown to correlate with acute Fontan outcomes and should be used as a screening tool in all patients undergoing single-ventricle palliation before undergoing the Fontan operation (Figure 1).14Biko D.M. DeWitt A.G. Pinto E.M. Morrison M.E. Johnstone J.A. Griffis H. et al.MRI evaluation of lymphatic abnormalities in the neck and thorax after Fontan surgery: relationship with outcome.Radiology. 2019; 291: 774-780Crossref PubMed Scopus (35) Google Scholar A new classification of lymphatic thoracic abnormalities has been introduced using a scale of 1 to 4 according to progression of severity (type 1: little or no abnormalities; type 2: abnormalities in the supraclavicular region; type 3: abnormalities in the mediastinum; type 4: abnormalities extending in to the mediastinum and the lungs). For patients with thoracic lymphatic disorders such as chylothorax or PB, intranodal and preferably also intrahepatic dynamic-contrast magnetic resonance lymphangiography (DCMRL) should be performed for interventional planning and to determine whether the patient has duodenal involvement, which could indicate risk for PLE (Figure 2).15Biko D.M. Smith C.L. Otero H.J. Saul D. White A.M. DeWitt A. et al.Intrahepatic dynamic contrast MR lymphangiography: initial experience with a new technique for the assessment of liver lymphatics.Eur Radiol. 2019; 29: 5190-5196Crossref PubMed Scopus (20) Google Scholar In addition, TD outlet patency should be confirmed. This can be done using contrast ultrasound lymphangiography or direct lymphangiography.16Mohanakumar S. Telinius N. Kelly B. Lauridsen H. Boedtkjer D. Pedersen M. et al.Morphology and function of the lymphatic vasculature in patients with a Fontan circulation.Circ Cardiovasc Imaging. 2019; 12: e008074Crossref PubMed Scopus (24) Google Scholar For patients with abdominal lymphatic abnormalities, such as PLE or ascites, intrahepatic and intramesenteric DCMRL are the imaging modality of choice for understanding the etiology and for interventional planning (Figure 2).15Biko D.M. Smith C.L. Otero H.J. Saul D. White A.M. DeWitt A. et al.Intrahepatic dynamic contrast MR lymphangiography: initial experience with a new technique for the assessment of liver lymphatics.Eur Radiol. 2019; 29: 5190-5196Crossref PubMed Scopus (20) Google Scholar,17Dori Y. Smith C.L. DeWitt A.G. Srinivasan A. Krishnamurthy G. Escobar F.A. et al.Intramesenteric dynamic contrast pediatric MR lymphangiography: initial experience and comparison with intranodal and intrahepatic MR lymphangiography.Eur Radiol. 2020; 30: 5777-5784Crossref PubMed Scopus (4) Google ScholarFigure 2A, Coronal MIP image of intranodal DCMRL in a patient with PB showing bilateral pulmonary perfusion (arrows). B, Coronal MIP image of intrahepatic DCMRL in a patient with PLE showing a duodenal leak (arrow). MIP, Magnetic imaging picture; DCMRL, dynamic contrast magnetic resonance lymphangiography; PB, plastic bronchitis; PLE, protein-losing enteropathy.View Large Image Figure ViewerDownload (PPT) The contractile properties, comparable with those of the heart, hold promise for the development of pharmacologic ways to optimize the lymphatic function. Norepinephrine increases the frequency of contractions as well as pressures generated in isolated human lymphatic vessels, and dopamine infusion has been reported to reduce symptoms in a small series of patients.18Mejia E. Otero H. Smith C. Shipman M. Liu M. Pinto E. et al.Use of contrast-enhanced ultrasound to determine thoracic duct patency.J Vasc Interv Radiol. 2020; 31: 1670-1674Abstract Full Text Full Text PDF PubMed Scopus (5) Google Scholar In all patients with a lymphatic dysfunction, cardiac evaluation, including cardiac catheterization, should be performed to determine whether there are reversible cardiac causes that could be treated, such as pulmonary artery stenosis or superior vena cava obstruction. Medical and conservative treatments should also be optimized. Lymphatic interventions can be divided into those meant to decompress the lymphatic system, such as lymphovenous anastomosis and percutaneous or surgical innominate vein (InV) turn-down, and those that are meant to reroute lymphatic flow away from abnormal lymphatic networks, such as selective lymphatic duct embolization (SLDE) or TD embolization. The decision on the therapeutic approach depends on the underlying disease and lymphatic anatomy. SLDE is the preferred interventional approach, as it maintains TD patency and flow, preserving the option of TD-decompression procedures. This is especially important in patients with multicompartment lymphatic failure or those with thoracic abnormalities and concerning findings on intrahepatic DCMRL, such as duodenal perfusion or leak. Complete TD embolization should be reserved for cases in which the TD outlet is occluded and lymphovenous anastomosis is not an option, there are decompressing vessels to the TD SLDE the TD is not or when the patient is and of TD flow is needed. The TD, which in the majority of patients the circulation at the left vein is by the to the V. Decompression of thoracic duct: new approach for the treatment of failing Fontan.Ann Thorac Surg. 2013; 96: 709-711Abstract Full Text Full Text PDF PubMed Scopus (41) Google Scholar The procedure is and The have been V. Mitchell M.E. Woods R.K. Hoffman G.M. Kindel S.J. Ginde S. Innominate vein turn-down procedure for failing Fontan circulation.Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann. 2020; 23: 34-40Abstract Full Text Full Text PDF PubMed Scopus (5) Google V. Decompression of thoracic duct: new approach for the treatment of failing Fontan.Ann Thorac Surg. 2013; 96: 709-711Abstract Full Text Full Text PDF PubMed Scopus (41) Google Scholar and the following the procedure is performed as a of Fontan operation on the is to the left venous one should of the posterior aspect of the confluence and of the TD and lymphatic (4) all venous of the jugular and subclavian vein are (5) of the to the and determine the for the turn-down by the of the of the and of central the at the with the right internal jugular vein and can be with vascular and the and determine whether there is of the for turn-down or if an is and the is and the are to an to the (Figure The aspect of the anastomosis may be by a is a on of anastomosis to the and an in the and the to a of approximately 3 to 4 it with a and in a technique (Figure have that not all are of with a heart can be an is we the technique most of the and could be done V. Mitchell M.E. Woods R.K. Hoffman G.M. Kindel S.J. Ginde S. Innominate vein turn-down procedure for failing Fontan circulation.Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann. 2020; 23: 34-40Abstract Full Text Full Text PDF PubMed Scopus (5) Google Scholar a right TD to be an is used from the internal jugular and subclavian vein confluence to the and a of venous system is required innominate vein is with right using an and the technique V. Mitchell M.E. Woods R.K. Hoffman G.M. Kindel S.J. Ginde S. Innominate vein turn-down procedure for failing Fontan circulation.Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann. 2020; 23: 34-40Abstract Full Text Full Text PDF PubMed Scopus (5) Google with from in and Large Image Figure ViewerDownload (PPT) A important issue is the to which the jugular vein should be TD to balance right to left this as on the of in the 2 to 3 cardiac is well the left jugular vein is through a on the neck and is either V. Mitchell M.E. Woods R.K. Hoffman G.M. Kindel S.J. Ginde S. Innominate vein turn-down procedure for failing Fontan circulation.Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann. 2020; 23: 34-40Abstract Full Text Full Text PDF PubMed Scopus (5) Google C. R. R. G. duct decompression and jugular vein treatment option for protein-losing enteropathy and plastic bronchitis in failing Fontan circulation: a Heart J 2020; 4: PubMed Google Scholar or if patency of venous drainage and should be in a by the left internal jugular vein and contrast patients with failing Fontan have the turn-down procedure at and Heart The procedure as the in and of patients who an turn-down surgical valve and pulmonary turn-down normal and normal turn-down and after with of PB at from due to pulmonary with no further symptoms of PLE or with of at 3 percutaneous of at with of PB at 3 percutaneous of of PB of at and required of at of at of have PLE and from bilateral dysfunction, heart risk for with to Innominate PLE, protein-losing PB, plastic bronchitis; valve right to right pulmonary with pulmonary left heart left vena superior vena left superior vena left in a new Innominate PLE, protein-losing PB, plastic bronchitis; valve right to right pulmonary with pulmonary left heart left vena superior vena left superior vena left new imaging techniques have on the etiology and of lymphatic complications such as PLE, PB, and is into understanding the and in patients with single-ventricle and lymphatic failure. In contrast to which has been leading to the development of interventional and the lymphatic system is in its and there are no lymphatic targeted the development of new TD-decompression it is that TD there is to SLDE of the to be the of as this the of therapeutic as pressure in a leak may be lower that in the decompression may not the R.K. V. Lymphatic fluid in both in the same Thorac Cardiovasc Surg. 2020; of Full Text Full Text PDF Scopus Google Scholar The turn-down procedure is to impaired lymphatic drainage in Fontan circulation. to the Fontan circulation, in which the TD is into the Fontan circulation, turn-down of the to the the TD to the lower-pressure system with “diastolic suctioning” of lymph. At the same time, the preload of the is increased at the of the right-to-left which as a with This an for or An is the to the development of which be by turn-down may be for and post-Fontan lymphatic complications of PB, and protein-losing enteropathy (Figure TD outlet or are occluded and to one should a lymphovenous anastomosis to vein (Figure turn-down may also be at of the Fontan operation for patients with thoracic type 3 or 4 at MRI C. Lymphatic decompression concomitant with Fontan/Kreutzer procedure: early experience.World J Pediatr Congenit Heart Surg. 2020; 11: 284-292Crossref PubMed Google Scholar are to determine the for this Complete of innominate vein in a patient with chylothorax after drainage is the vein (arrows). B, duct is with vein (arrow). the TD which prevents venous blood from the TD. TD, Large Image Figure ViewerDownload (PPT) The authors reported no of The Journal editors and to of and to or for which may have a of The editors and of this article have no of

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