INTRAHEPATIC DYNAMIC CONTRAST-ENHANCED LYMPHANGIOGRAPHY IN PATIENTS WITH CONGENITAL HEART DISEASE: A POTENTIAL IMAGING SIGNATURE FOR PROTEIN-LOSING ENTEROPATHY

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 ( P <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.

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Temporal correlation-based dynamic contrast-enhanced MR imaging improves assessment of complex pulmonary circulation in congenital heart disease

Magnetic Resonance in Medicine ◽

10.1002/mrm.21001 ◽

2006 ◽

Vol 56 (3) ◽

pp. 517-526 ◽

Cited By ~ 6

Author(s):

Shang-Yueh Tsai ◽

Ming-Ting Wu ◽

Yi-Ru Lin ◽

Kai-Sheng Hsieh ◽

Chu-Chuan Lin ◽

...

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Mr Imaging ◽

Pulmonary Circulation ◽

Congenital Heart ◽

Temporal Correlation ◽

Dynamic Contrast Enhanced ◽

Contrast Enhanced

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An unusual cause of edema in a child with congenital heart disease post Fontan procedure

International Journal of Research in Medical Sciences ◽

10.18203/2320-6012.ijrms20194623 ◽

2019 ◽

Vol 7 (11) ◽

pp. 4396

Author(s):

Minal Wade ◽

Shweta Shettiwar ◽

Ankita Shah

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Congenital Heart ◽

Rare Complication ◽

Fontan Procedure ◽

Protein Losing Enteropathy ◽

Common Causes ◽

Disease Protein ◽

The Common ◽

Fontan Palliation

Protein Losing Enteropathy Post Fontan procedure. Protein Losing Enteropathy (PLE) is an uncommon cause of edema in children with congenital heart disease. Protein-Losing Enteropathy may be defined as excessive loss of proteins across the intestinal mucosa and is due to either a primary gastrointestinal abnormality or secondary to cardiac disease. Protein-losing enteropathy (PLE) is a rare complication of the Fontan palliation for functional single-ventricle. Although PLE occurs in about 3.5% of patients post-Fontan, it confers marked morbidity and high mortality within 5 years of diagnosis. The pathogenesis of Fontan-related PLE is not completely understood, and it is unclear why it develops in some patients post-Fontan and not others. We describe a child with Double Inlet Right Ventricle who had undergone Fontan procedure, and presented to us with generalised oedema. The child had hypoproteinaemia, the common causes for which were ruled out and was diagnosed as Protein Losing Enteropathy (PLE) related to his surgical intervention. Though, not frequently encountered it should be kept in mind as one of the causes of anasarca.

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The Feasibility and Clinical Utility of Microsphere Contrast-enhanced Transthoracic Echocardiography in Adult Congenital Heart Disease

Congenital Heart Disease ◽

10.1111/chd.12254 ◽

2015 ◽

Vol 10 (5) ◽

pp. 428-436 ◽

Cited By ~ 2

Author(s):

David G. Platts ◽

Natalie F.A. Kelly ◽

Vishva A. Wijesekera ◽

Abhishek Sengupta ◽

Kylie Burns ◽

...

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Transthoracic Echocardiography ◽

Clinical Utility ◽

Congenital Heart ◽

Adult Congenital Heart Disease ◽

Contrast Enhanced ◽

Adult Congenital

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Diagnosis and Management of Lymphatic Disorders in Congenital Heart Disease

Current Cardiology Reports ◽

10.1007/s11886-020-01405-y ◽

2020 ◽

Vol 22 (12) ◽

Author(s):

Benjamin Kelly ◽

Sheyanth Mohanakumar ◽

Vibeke Elisabeth Hjortdal

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Congenital Heart ◽

Treatment Options ◽

Future Research ◽

Protein Losing Enteropathy ◽

Current Review ◽

Lymphatic Complications ◽

And Function ◽

Lymphatic Imaging

Abstract Purpose of Review Lymphatic disorders have received an increasing amount of attention over the last decade. Sparked primarily by improved imaging modalities and the dawn of lymphatic interventions, understanding, diagnostics, and treatment of lymphatic complications have undergone considerable improvements. Thus, the current review aims to summarize understanding, diagnostics, and treatment of lymphatic complications in individuals with congenital heart disease. Recent Findings The altered hemodynamics of individuals with congenital heart disease has been found to profoundly affect morphology and function of the lymphatic system, rendering this population especially prone to the development of lymphatic complications such as chylous and serous effusions, protein-losing enteropathy and plastic bronchitis. Summary Although improved, a full understanding of the pathophysiology and targeted treatment for lymphatic complications is still wanting. Future research into pharmacological improvement of lymphatic function and continued implementation of lymphatic imaging and interventions may improve knowledge, treatment options, and outcome for affected individuals.

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