MRI of Lymphatic Abnormalities After Functional Single-Ventricle Palliation Surgery

Dori, Yoav; Keller, Marc S.; Fogel, Mark A.; Rome, Jonathan J.; Whitehead, Kevin K.; Harris, Matthew A.; Itkin, Maxim

doi:10.2214/ajr.13.11797

Cited by 129 publications

(109 citation statements)

References 14 publications

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“…Having significant gastrointestinal pathology may also, when compounded with single ventricle physiology, lead to abnormal stool A1AT samples, as the one subject with surgical repair of trachoesophageal fistula and necrotizing enterocolitis had an abnormal stool A1AT sample. Our findings support the hypothesis that children with single ventricle type of congenital heart disease may have inherently altered intestinal physiology, perhaps due to decreased cardiac output and splanchnic perfusion or due to abnormal intestinal lymphatics either as part of the congenital malformation itself or acquired during early palliative intervention (13). We hypothesize that children with single ventricle type of congenital heart disease remain in a state of acute compensation, even before Fontan operation.…”

Section: Commentsupporting

confidence: 85%

Early Impact of Fontan Operation on Enteric Protein Loss

Patel

Loomes

Goldberg

et al. 2016

The Annals of Thoracic Surgery

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Background Protein losing enteropathy (PLE) is a challenging complication after Fontan operation. Subclinical enteric protein loss may precede development of overt PLE. We evaluated the acute effects of Fontan circulation on enteric protein loss and mesenteric vascular resistance. Methods A prospective cohort study was performed evaluating enteric protein loss in children undergoing Fontan operation. Stool alpha-1-antitrypsin (A1AT) concentration was measured in the pre-operative, early post-operative, and intermediate post-operative (3–9 months) periods. The intestinal circulation was characterized by Doppler-derived resistance indices of the superior mesenteric artery, and serum albumin and protein levels were obtained. Results We enrolled 33 subjects at a median age at operation of 3.0 (2.5–3.3) years. No clinical PLE was observed. Six of the 93 stool A1AT samples obtained were elevated (>54 mg/dl), with two abnormal samples at each of the three time points. Two of the five subjects with elevated stool A1AT values had significant hemodynamic disturbances requiring intervention (junctional bradycardia or tricuspid stenosis). There was no difference in superior mesenteric artery resistance in the pre-operative versus early post-operative period (p=0.9). Serum albumin levels were lower in the early post-operative period compared to the pre-operative period (3.2 mg/dl [IQR 2.9–3.5] vs. 4.1 mg/dl [IQR 3.4–4.5], p=0.01) but did not correlate with abnormal stool A1AT concentration or superior mesenteric artery resistance indices. Conclusions The Fontan operation does not commonly result in acute development of increased enteric protein loss. However, increased enteric protein loss may occur in children before or after Fontan operation, particularly when hemodynamic disturbances are present.

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Section: Commentsupporting

confidence: 85%

Early Impact of Fontan Operation on Enteric Protein Loss

Patel

Loomes

Goldberg

et al. 2016

The Annals of Thoracic Surgery

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“…4 Dynamic contrast-enhanced magnetic resonance lymphangiogram (DCMRL) has recently been described as a method of imaging the central lymphatic system. 15, 16 Dori et al 16,17 have recently reported on the use of this technique and T2-weighted MRI to characterize lymphatic abnormalities in patients with PB.…”

Section: Clinical Perspective On P 1170mentioning

confidence: 99%

Percutaneous Lymphatic Embolization of Abnormal Pulmonary Lymphatic Flow as Treatment of Plastic Bronchitis in Patients With Congenital Heart Disease

et al. 2016

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“…We presented one patient with resolution of symptoms after catheter-based oil and glue embolization of lymphatic collaterals originating from the thoracic duct [25]. Increased understanding of the Fontan lymphatic system and its alterations under elevated central venous pressure is necessary and may be possible with novel MRI imaging techniques [26]. A strategy that combines relief of anatomic obstruction in the Fontan pathway and vigorous pulmonary vasodilation with catheter-based occlusion of well-mapped, isolated sites of lymphatic leakage into the airways may ultimately be the ideal management plan.…”

Section: Commentmentioning

confidence: 99%

A Multifaceted Approach to the Management of Plastic Bronchitis After Cavopulmonary Palliation

Avitabile¹,

Goldberg²,

Dodds³

et al. 2014

The Annals of Thoracic Surgery

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Background Plastic bronchitis is a rare, potentially life-threatening complication after Fontan operation. Hemodynamic alterations (elevated central venous pressure and low cardiac output) likely contribute to the formation of tracheobronchial casts composed of inflammatory debris, mucin, and fibrin. Pathologic studies of cast composition support medical treatment with fibrinolytics such as inhaled tissue plasminogen activator (t-PA). Methods Retrospective case series of medical, surgical, and catheter-based management of patients with plastic bronchitis after cavopulmonary palliation. Results Fourteen patients (86% male, 93% white) were included. Median age at Fontan operation was 2.7 years (range 1.2–4.1) with median interval to plastic bronchitis presentation of 1.5 years (9 days–15.4 years). Cast composition was available for 11 patients (79%) and included fibrin deposits in 7. All patients were treated with pulmonary vasodilators. Thirteen patients (93%) were treated with inhaled t-PA. Hemodynamically significant lesions in the Fontan pathway were addressed via catheter-based (n=9) and surgical (n=3) interventions. Three patients (21%) underwent heart transplantation. Median length of follow-up was 2.7 years (0.6–8.7). Symptoms improved such that six of thirteen (46%) patients were weaned off t-PA. Rare or episodic casts are successfully managed with outpatient t-PA in the majority of other patients. Of the 3 patients who underwent heart transplant, two are asymptomatic and one has recurrent casts in the setting of elevated filling pressures and rejection. Conclusions A systematic step-wise algorithm, which includes optimization of hemodynamics, aggressive pulmonary vasodilation and inhaled t-PA, is an effective treatment strategy for patients with plastic bronchitis after cavopulmonary connection.

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MRI of Lymphatic Abnormalities After Functional Single-Ventricle Palliation Surgery

Abstract: Lymphatic abnormalities are found in many patients after functional single-ventricle palliation. T2-weighted unenhanced MRI is capable of anatomic assessment of the lymphatic system in this patient population and has promise for guiding treatment in the future.

Cited by 129 publications

References 14 publications

Early Impact of Fontan Operation on Enteric Protein Loss

Early Impact of Fontan Operation on Enteric Protein Loss

Percutaneous Lymphatic Embolization of Abnormal Pulmonary Lymphatic Flow as Treatment of Plastic Bronchitis in Patients With Congenital Heart Disease

A Multifaceted Approach to the Management of Plastic Bronchitis After Cavopulmonary Palliation

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