Lymphatic Decompression Concomitant With Fontan/Kreutzer Procedure: Early Experience

Abstract

Objective: To present a strategy for identifying patients at risk of lymphatic failure in the setting of planned Fontan/Kreutzer completion, allowing a tailored surgical approach. Methods: Since January 2017, clinical evaluation before performance of the Fontan/Kreutzer procedure included T2-weighted magnetic resonance imaging (MRI) lymphangiography. Thoracic lymphatic abnormalities were categorized using a scale of I to IV according to progression of severity. Patients with severe lymphatic abnormalities (types III and IV) underwent Fontan/Kreutzer with lymphatic decompression via connection of the left jugular–subclavian junction containing the thoracic duct to the systemic atrium (group A). Results: Thirteen patients were enrolled. Magnetic resonance imaging showed type I abnormalities in four cases (30.7%), II in four (30.7%), III in two (15.3%), and IV in three (23.3%). Patients in types III and IV underwent a Fontan/Kreutzer with lymphatic decompression (group A, n = 5), while patients in types I and II underwent a fenestrated extracardiac Fontan/Kreutzer procedure without lymphatic decompression (group B, n = 8). Preoperatively, there were no differences in age, weight, ventricular dominance (right vs left), superior vena cava pressure, incidence of chylothorax after previous superior cavopulmonary anastomosis (Glenn), or need for concomitant procedures at Fontan/Kreutzer completion. There were no differences in procedural times between the groups, nor were there differences in mortalities and Fontan/Kreutzer takedowns. There were no statistically significant differences in early and late morbidity between the two groups with the exception of total volume of effusions output postoperatively. At median follow-up of 18 months (range, 4-28 months), all patients in group A are in New York Heart Association class 1 with no differences between groups in arterial oxygen saturation. Conclusions: Lymphatic decompression during Fontan/Kreutzer procedure was successfully performed in patients identified by MRI as predisposed to lymphatic failure. A larger cohort of patients and longer follow-up are required to determine the efficacy of this approach in preventing early- and long-term Fontan/Kreutzer failure.

Lymphatic Disorders in Patients With Single Ventricle Heart Disease

“…Most often, minimally invasive procedures have replaced the surgical occlusive procedures and are the interventional modality of choice, if available. Surgical procedures to decompress the thoracic duct include the Hraska procedure or innominate vein turndown (21)(22)(23)(24). While the right to left shunting is of concern in both the minimally invasive and surgical decompression procedures, this often is not an issue and patients tolerate the procedures well.…”

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

“…Prophylactic InV turn-down may also be considered at time of the Fontan operation for patients with thoracic lymphangiectasia type 3 or 4 pattern at pre-Fontan MRI lymphangiography. 9 Further studies are needed to determine the appropriate indication for this procedure.

Figure 5 Decision-making tree.

…”

“… 1 , 2 , 3 , 4 Novel interventional and surgical approaches have been developed to treat post-Fontan lymphatic complications with promising outcomes. 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 …”

Lymphatic Decompression for Fontan/Kreutzer Procedures—Indeed, a Clever Concept, But Will It Truly Work in Practice?

“…The article by Christian Kreutzer’s group published in this issue of the World Journal for Pediatric and Congenital Heart Surgery 1 represents a serious effort to extend the use of lymphatic system decompression to remove extra fluid, proteins, and cells and by doing so to reduce venous stasis. They used several versions of the lymphatic decompression procedure first described by Hraska in 2013.…”

“…The work by Kreutzer and colleagues 1 must be acknowledged as an attempt to decide individually, on an earlier basis, that is, after achieving partial cavopulmonary connection (superior cavopulmonary anastomosis) but prior to Fontan completion, and based on imaging lymphatics, which patients would be at risk for developing secondary lymphatic dysfunction. Based on this decision, they assign them to either a drainage procedure or a traditional cavopulmonary pathway fenestration.…”