First name
Christopher
Middle name
L
Last name
Smith

Title

Transcatheter Thoracic Duct Decompression for Multicompartment Lymphatic Failure After Fontan Palliation.

Year of Publication

2022

Number of Pages

e011733

Date Published

06/2022

ISSN Number

1941-7632

Abstract

BACKGROUND: Lymphatic embolization therapy has proven effective for Fontan failure from plastic bronchitis or protein-losing enteropathy but not when multiple lymphatic compartments are involved; furthermore, embolization does not alter the underlying pathophysiology of lymphatic dysfunction. A technique for transcatheter thoracic duct decompression (TDD), rerouting the thoracic duct to the pulmonary venous atrium to treat multicompartment lymphatic failure is described and early outcomes presented.

METHODS: Initially covered stents were used to channel the innominate vein flow inside of the cavopulmonary pathway into the pulmonary venous atrium. A modified approach was developed where covered stents redirected innominate vein directly to the left atrium via an extravascular course. Baseline and follow-up data on all patients undergoing TDD were reviewed.

RESULTS: Twelve patients underwent TDD between March 2018 and February 2021 at a median age of 12 (range: 2-22) years. Lymphatic failure occurred in median of 3 compartments per patient (protein-losing enteropathy, ascites, pleural effusions, plastic bronchitis); 10 patients had lymphatic embolizations before TDD. TDD method was intra-Fontan tunnel in 4, direct approach in 7, and other in 1. There were no major procedural complications; 6 patients underwent subsequent procedures, most commonly to treat endoleaks. Lymphatic failure resolved in 6 patients, improved in 2, and was unchanged in 4 at 6 (range: 1-20) months follow-up. One patient died after TDD from Fontan failure.

CONCLUSIONS: TDD is a promising new treatment for the failing Fontan physiology from multicompartment lymphatic failure. Additional work is needed to refine the technique and define optimal candidates.

DOI

10.1161/CIRCINTERVENTIONS.121.011733

Alternate Title

Circ Cardiovasc Interv

PMID

35708032

Title

State-of-the-art imaging for lymphatic evaluation in children.

Year of Publication

2022

Date Published

08/2022

ISSN Number

1432-1998

Abstract

The lymphatic system has been poorly understood and its importance neglected for decades. Growing understanding of lymphatic flow pathophysiology through peripheral and central lymphatic flow imaging has improved diagnosis and treatment options in children with lymphatic diseases. Flow dynamics can now be visualized by different means including dynamic contrast-enhanced magnetic resonance lymphangiography (DCMRL), the current standard technique to depict central lymphatics. Novel imaging modalities including intranodal, intrahepatic and intramesenteric DCMRL are quickly evolving and have shown important advances in the understanding and guidance of interventional procedures in children with intestinal lymphatic leaks. Lymphatic imaging is gaining importance in the radiologic and clinical fields and new techniques are emerging to overcome its limitations.

DOI

10.1007/s00247-022-05469-6

Alternate Title

Pediatr Radiol

PMID

35980463

Title

Outcomes of Operator-Directed Sedation and Anesthesiologist Care in the Pediatric/Congenital Catheterization Laboratory: A Study Utilizing Data From the IMPACT Registry.

Year of Publication

2021

Number of Pages

401-413

Date Published

2021 Feb 22

ISSN Number

1876-7605

Abstract

OBJECTIVES: The objective of this study was to assess contemporary use of operator directed sedation (ODS) and anesthesiologist care (AC) in the pediatric/congenital cardiac catheterization laboratory (PCCL), specifically evaluating whether the use of operator-directed sedation was associated with increased risk of major adverse events.

BACKGROUND: The safety of ODS relative to AC during PCCL procedures has been questioned.

METHODS: A multicenter, retrospective cohort study was performed studying procedures habitually performed with ODS or AC at IMPACT (Improving Adult and Congenital Treatment) registry hospitals using ODS for ≥5% of cases. The risks for major adverse events (MAE) for ODS and AC cases were compared, adjusted for case mix. Current recommendations were evaluated by comparing the ratio of observed to expected MAE for cases in which ODS was inappropriate (inconsistent with those guidelines) with those for similar risk AC cases, as well as those in which ODS or AC was appropriate.

RESULTS: Of the hospitals submitting data to IMPACT, 28 of 101 met inclusion criteria. Of the 7,042 cases performed using ODS at these centers, 88% would be inappropriate. Use of ODS was associated with lower likelihood of MAE both in observed results (p < 0.0001) and after adjusting for case-mix (odds ratio: 0.81; p = 0.006). Use of AC was also associated with longer adjusted fluoroscopy and procedure times (p < 0.0001 for both). The observed/expected ratio for ODS cases with high pre-procedural risk (inappropriate for ODS) was significantly lower than that for AC cases with comparable pre-procedural risk. Across a range of pre-procedural risks, there was no stratum in which risk for MAE was lower for AC than ODS.

CONCLUSIONS: Across a range of hospitals, ODS was used safely and with improved efficiency. Clinical judgment better identified cases in which ODS could be used than pre-procedural risk score. This should inform future guidelines for the use of ODS and AC in the catheterization laboratory.

DOI

10.1016/j.jcin.2020.10.054

Alternate Title

JACC Cardiovasc Interv

PMID

33602437

Title

Magnetic resonance lymphangiography in post-Fontan palliation patients with MR non-conditional cardiac electronic devices: An institutional experience.

Year of Publication

2022

Number of Pages

43-52

Date Published

2022 Feb 23

ISSN Number

1873-4499

Abstract

<p>Magnetic resonance imaging (MRI) is a routinely used imaging modality for the diagnosis and treatment planning of many health conditions in children and adults. Yet, its use has been limited in many institutions for patients with cardiac implantable electronic devices (CIEDs) due to safety concerns. Current evidence relates primarily to devices with transvenous leads. However, patients with complex cardiac anatomy and palliative surgery procedures often require epicardial pacemakers. To date, very few studies have addressed MRI safety considerations with non-conditional CIEDs or abandoned epicardial leads in infants, and to our knowledge, this is the first report that shows Fontan palliation patients who underwent Dynamic Contrast enhanced MR Lymphangiography (DCMRL) with these types of devices. We present our institutional experience with five cases where a DCMRL was safely performed in three children and two adults with Fontan palliation to evaluate their lymphatic anatomy and guide interventional procedures. Regarding our brief experience, we concluded that DCMRL may be considered in post-Fontan patients with non-conditional CIEDs, including epicardial leads, seeking the best diagnostic and treatment options available. Institutional protocols must be revised in advance to perform this technique in a controlled setting.</p>

DOI

10.1016/j.clinimag.2022.02.016

Alternate Title

Clin Imaging

PMID

35334301

Title

Dynamic contrast-enhanced MR lymphangiography: feasibility of using ferumoxytol in patients with chronic kidney disease.

Year of Publication

2022

Date Published

2022 Jan 10

ISSN Number

1432-1084

Abstract

<p><strong>PURPOSE: </strong>To assess the feasibility of direct intra-lymphatic administration of diluted ferumoxytol as a T1-positive contrast agent for dynamic contrast-enhanced MR lymphangiography (DCMRL) imaging of the central lymphatics in children with renal disease.</p>

<p><strong>METHODS: </strong>In vitro scan of dilute ferumoxytol was initially performed using time-resolved and high-resolution 3D gradient echo (GRE) sequences with short TE values (1 to 1.5&nbsp;ms). A ferumoxytol concentration of 0.25 to 0.40&nbsp;mg/mL was found to retain high signal in the T1-weighted sequences. DCMRL was then performed in 4 children with renal disease with the same 3D GRE sequences administrating diluted ferumoxytol via intra-mesenteric (IM), intra-hepatic (IH), and intra-nodal (IN) routes (6 to 9&nbsp;mL to each site; average total dose of 0.75&nbsp;mg/kg) by slow hand injection (0.5 to 1.0&nbsp;mL/min). The signal-to-noise ratio (SNR) of the lymphatics was measured for quantitative evaluation.</p>

<p><strong>RESULTS: </strong>Ferumoxytol-enhanced DCMRL was technically successful in all patients. Contrast conspicuity within the lymphatics was sufficient without subtraction. The mean SNR was significantly higher than the muscle (50.1 ± 12.2 vs 13.2 ± 2.8; t = 15.9; p &lt; .001). There were no short-term complications attributed to the administration of ferumoxytol in any of the four patients.</p>

<p><strong>CONCLUSION: </strong>Magnetic resonance lymphangiography using ferumoxytol via IN, IH, and IM access is a new method to directly visualize the central lymphatic system and can be applied safely in patients with renal failure based on our preliminary report of four cases. Ferumoxytol-enhanced DCMRL shows diagnostic image quality by using 3D GRE sequences with short TE values and appropriate dilution of ferumoxytol.</p>

<p><strong>KEY POINTS: </strong>• MR lymphangiography using ferumoxytol via intra-nodal, intra-hepatic, and intra-mesenteric access is a new method to directly visualize the central lymphatic system from the groin to the venous angle. • FDCMRL can be applied safely in patients with renal failure based on our preliminary report of four cases. • FDCMRL shows diagnostic image quality by using 3D GRE sequences with short TE values and appropriate dilution of the ferumoxytol.</p>

DOI

10.1007/s00330-021-08448-x

Alternate Title

Eur Radiol

PMID

35001156

Title

Post-operative Chylothorax in Patients with Repaired Transposition of the Great Arteries.

Year of Publication

2021

Date Published

2021 Nov 29

ISSN Number

1432-1971

Abstract

<p>Patients with dextro-transposition of the great arteries (d-TGA) require surgical repair as neonates. These patients are at risk for post-operative chylothorax. We sought to describe the presentation, imaging, and outcomes after intervention for patients with d-TGA with post-operative chylothorax. A retrospective chart review was performed in patients with repaired d-TGA who were referred from 1/1/2013 to 4/1/2020 for evaluation of chylothorax. Patient history, lymphatic imaging, and interventional data were collected. Impact of intervention on lymphatic drainage was evaluated with a student's t-test. Eight patients met inclusion criteria for this study. Five patients had a history of central venous thrombus leading to thoracic duct outlet occlusion. Five patients underwent intervention, two were managed conservatively, and one was not a candidate for intervention. Chylothorax resolved in six patients. There was a significant difference in output from 7&nbsp;days prior to first intervention (114&nbsp;mL/kg/day) compared to 28&nbsp;days following final intervention (27&nbsp;mL/kg/day, p = 0.034). There were no procedural complications. Chylothorax in patients with repaired transposition of the great arteries is often amenable to intervention. Early surveillance and management of central venous thrombosis may reduce the burden of lymphatic disease in these patients.</p>

DOI

10.1007/s00246-021-02774-z

Alternate Title

Pediatr Cardiol

PMID

34841467

Title

Impact of Transcatheter Pulmonary Artery Intervention Following Superior Cavopulmonary Connection on Pulmonary Artery Growth.

Year of Publication

2021

Number of Pages

635-642

Date Published

2021 Sep

ISSN Number

2150-136X

Abstract

<p><strong>INTRODUCTION: </strong>Balloon and stent angioplasty of the pulmonary arteries (PAs) are frequently performed following superior cavopulmonary connection (SCPC), not only to normalize the caliber of the affected PA but also in hopes of maximizing downstream growth over time. There are limited data on the impact on subsequent PA growth prior to total cavopulmonary connection (TCPC).</p>

<p><strong>METHODS: </strong>A single-center, retrospective cohort study was performed on children who underwent transcatheter (TC) PA intervention following SCPC between January 1, 2010, and December 31, 2018. Growth of treated and contralateral PAs was measured at the lobar bifurcation (distal branch PA [DBPA]) and in the proximal lower lobe (lower lobe branch [LLB]) on serial angiograms. Growth rate was evaluated using a mixed-effect model clustered by individual patient with an interaction term for treated PA and time to evaluate for differential growth rates between treated and contralateral PAs.</p>

<p><strong>RESULTS: </strong>Thirty-five patients underwent TC PA intervention following SCPC, at a median of 70 days (interquartile range: 19-297 days) postoperatively. Significant growth was seen at both DBPA and LLB for raw (0.8 mm/year, 95% CI: 0.6-1.0, &lt; .001 for both) and body surface area (BSA) adjusted measures (8.4mm/m/year, 95% CI: 5.6-11.2, &lt; .001; 7.9 mm/m/year, 95% CI: 5.5-10.2, &lt; .001). The growth rate of the treated vessel was not significantly different from that of the contralateral vessel at the DBPA or LLB positions for raw ( = .71, .70) or BSA-adjusted measurements ( = .86, .64).</p>

<p><strong>CONCLUSION: </strong>Transcatheter PA intervention was associated with normal distal PA growth rate relative to the untreated side.</p>

DOI

10.1177/21501351211033238

Alternate Title

World J Pediatr Congenit Heart Surg

PMID

34597205

Title

Intrahepatic Dynamic Contrast-Enhanced Magnetic Resonance Lymphangiography: Potential Imaging Signature for Protein-Losing Enteropathy in Congenital Heart Disease.

Year of Publication

2021

Number of Pages

e021542

Date Published

2021 Sep 25

ISSN Number

2047-9980

Abstract

<p>Background Protein-losing enteropathy (PLE) is a significant cause of morbidity and mortality in congenital heart disease patients with single ventricle physiology. Intrahepatic dynamic contrast-enhanced magnetic resonance lymphangiography (IH-DCMRL) is a novel diagnostic technique that may be useful in characterizing pathologic abdominal lymphatic flow in the congenital heart disease population and in diagnosing PLE. The objective of this study was to characterize differences in IH-DCMRL findings in patients with single ventricle congenital heart disease with and without PLE. Methods and Results This was a single-center retrospective study of IH-DCMRL findings and clinical data in 41 consecutive patients, 20 with PLE and 21 without PLE, with single ventricle physiology referred for lymphatic evaluation. There were 3 distinct duodenal imaging patterns by IH-DCMRL: (1) enhancement of the duodenal wall with leakage into the lumen, (2) enhancement of the duodenal wall without leakage into the lumen, and (3) no duodenal involvement. Patients with PLE were more likely to have duodenal involvement on IH-DCMRL than patients without PLE (&lt;0.001). Conclusions IH-DCMRL findings of lymphatic enhancement of the duodenal wall and leakage of lymph into the duodenal lumen are associated with PLE. IH-DCMRL is a useful new modality for characterizing pathologic abdominal lymphatic flow in PLE and might be useful as a risk-assessment tool for PLE in at-risk patients.</p>

DOI

10.1161/JAHA.121.021542

Alternate Title

J Am Heart Assoc

PMID

34569246

Title

Liver lymphatic anatomy and role in systemic lymphatic disease.

Year of Publication

2021

Date Published

2021 Jun 24

ISSN Number

1432-1084

Abstract

<p><strong>OBJECTIVES: </strong>To characterize hepatic to systemic lymphatic connections in patients with systemic lymphatic disease using intra-hepatic lymphangiography and to compare outcomes after lymphatic intervention.</p>

<p><strong>METHODS: </strong>In this retrospective study, patients with intra-hepatic lymphangiography from May 2014 - April 2019 at our institution were included. Imaging review was performed and hepatic lymphatic connections and flow patterns were characterized. Clinical data were reviewed and comparisons between patients undergoing lymphatic intervention with or without abnormal hepatic lymphatics were performed.</p>

<p><strong>RESULTS: </strong>During the study period, 105 patients underwent intra-hepatic lymphangiography. Primary clinical presentation included ascites (19/105), chylothorax (27/105), plastic bronchitis (PB) (17/105), and protein losing enteropathy (PLE) (42/105). Five categories of hepatic lymphatic connections and flow patterns were identified (%): normal (25%, 26/105), hepatoperitoneal (12%, 13/105), hepatopulmonary (10.5%, 11/105), hepatomesenteric (7.5%, 8/105), and hepatoduodenal (41%, 43/105) with four patients having more than one abnormal pattern. A comparison between clinical presentation and imaging category revealed an increased likelihood of having ascites with hepatoperitoneal (p &lt; .0001), chylothorax/PB with hepatopulmonary (p = .01), and PLE with hepatoduodenal (p &lt; .001) connections. Seventy-six patients had a lymphatic intervention, 24% with normal, and 76% with abnormal liver lymphatics. There was no difference in length of hospital stay or mortality between the two groups, but there was a prolonged time to symptom resolution (p = .006) and persistent symptoms after 6&nbsp;months (5% vs 44%, p = .002) in the group with abnormal liver lymphatics.</p>

<p><strong>CONCLUSION: </strong>We identified five liver lymphatic imaging categories with a substantial correlation to presenting lymphatic disease. Abnormal imaging patterns correlated with increased morbidity. Evaluation of liver lymphatics should be considered in patients with a systemic lymphatic disease if central lymphatic imaging is normal.</p>

<p><strong>KEY POINTS: </strong>• We identified five liver lymphatic imaging patterns: normal, hepatoperitoneal, hepatomesenteric, hepatopulmonary, and hepatoduodenal. • Imaging patterns were correlated with disease presentation (normal - chylothorax/PB, hepatoperitoneal - ascites/chylothorax, hepatopulmonary - chylothorax/PB, hepatoduodenal - PLE). • Abnormal imaging patterns correlated with increased morbidity.</p>

DOI

10.1007/s00330-021-08098-z

Alternate Title

Eur Radiol

PMID

34165621

Title

Dynamic contrast-enhanced magnetic resonance lymphangiography.

Year of Publication

2021

Date Published

2021 Apr 08

ISSN Number

1432-1998

Abstract

<p>Lymphatic flow disorders include a broad spectrum of abnormalities that can originate in the lymphatic or the venous system. The development of these disorders is multifactorial and is most commonly associated with congenital heart diseases and palliative surgeries that these patients undergo. Central lymphatic disorders might be secondary to traumatic leaks, lymphatic overproduction, conduction abnormalities or lymphedema, and they can progress to perfusion anomalies. Several imaging modalities have been used to visualize the lymphatic system. However, the imaging of central lymphatic flow has always been challenging. Dynamic contrast-enhanced magnetic resonance lymphangiography (DCMRL) allows for visualization of central lymphatic flow disorders and has been recently applied for the assessment of plastic bronchitis, protein-losing enteropathy, chylothorax and chylopericardium, among other lymphatic disorders. The hepatic and mesenteric accesses are innovative and promising techniques for better identification and understanding of these abnormalities. The main objectives of this review are to discuss the physiology and anatomy of the lymphatic system and review the current uses of DCMRL in the diagnosis and management of lymphatic flow disorders.</p>

DOI

10.1007/s00247-021-05051-6

Alternate Title

Pediatr Radiol

PMID

33830292

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