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

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.

scholarly journals Development of nephropathy in an adult patient after Fontan palliation for cyanotic congenital heart disease

2021 ◽  
Author(s):  
Kaori Hayashi ◽  
Akinori Hashiguchi ◽  
Masako Ikemiyagi ◽  
Hirobumi Tokuyama ◽  
Shu Wakino ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Adult Patient ◽  
Congenital Heart ◽  
Cyanotic Congenital Heart Disease ◽  
Fontan Palliation

scholarly journals IMPACT Registry and National Pediatric Cardiology Quality Improvement Collaborative: Contributions to Quality in Congenital Heart Disease

2019 ◽  
Vol 10 (1) ◽  
pp. 72-80 ◽  
Author(s):  
Gerard R. Martin ◽  
Jeffrey B. Anderson ◽  
Robert N. Vincent
Keyword(s):  
Congenital Heart Disease ◽  
Quality Improvement ◽  
Heart Disease ◽  
Congenital Heart ◽  
Pediatric Cardiology ◽  
Device Closure ◽  
Stent Angioplasty ◽  
Quality Improvement Collaborative ◽  
The Common ◽  
The Impact

The IMproving Pediatric and Adult Congenital Treatments (IMPACT) Registry and the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) are two efforts initiated to improve outcomes in the congenital heart disease field. The IMPACT Registry is focused on evaluating the use, risks, adverse events (AEs), and outcomes associated with diagnostic and common interventional catheterization procedures in all children and adults with congenital heart disease. Utilizing a modular approach, the common procedures include diagnostic cardiac catheterization, atrial septal defect device closure, patent ductus arteriosus device closure, pulmonary valvuloplasty, aortic valvuloplasty, balloon and stent angioplasty of coarctation of the aorta, pulmonary artery balloon stent angioplasty, transcatheter pulmonary valve replacement, and electrophysiology procedures including radiofrequency ablation. To date, important observations on the common procedures have been made and a risk stratification methodology has been created to allow comparisons between centers in AEs and quality improvement activity. The registry is open to international participation. The NPC-QIC was developed to reduce mortality and improve the quality of life of infants with Hypoplastic Left Heart Syndrome (HLHS) during the interstage period between discharge from the Norwood operation and admission for the bidirectional Glenn procedure. Mortality in the interstage has been reduced by 44%. The IMPACT Registry and the NPC-QIC have demonstrated value to the congenital heart disease community. The IMPACT Registry, however, has not yet demonstrated an impact on patient outcomes. The NPC-QIC, which combines both a registry with a learning collaborative with specific aims, key drivers, and change strategies, has made more significant gains with reductions in variation, growth failures, and mortality.

Chylothorax after surgery for congenital heart disease in children

1998 ◽  
Vol 8 (2) ◽  
pp. 199-204 ◽  
Author(s):  
Simone Rolim Fernandes Fontes Pedra ◽  
Carlos Augusto Cardoso Pedra ◽  
Valmir Fernandes Fontes ◽  
Maria Virgínia Tavares Santana ◽  
Paulo Paredes Paulista
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Fontan Procedure ◽  
Bidirectional Glenn ◽  
Surgical Data ◽  
Considerable Morbidity ◽  
Classical Means ◽  
Hypercaloric Diet ◽  
High Output

AbstractWe reviewed retrospectively the clinical and surgical data from 9 children with postoperative chylothorax secondary to cardiac surgery for congenital heart disease seen over a 3 year period. Mean age was 26 months and 6 patients were female. The procedures performed were 2 Blalock-Taussig shunts, 6 Bidirectional Glenn anastomoses and 1 modified Fontan procedure. Diagnosis of chylothorax was made by classical means. The overall incidence of chylothorax was 0.8%, with the relative incidences being 2.5% for Blalock-Taussig, 2.7% for Fontan, and 11.7% for the Glenn procedures. All patients were initially treated conservatively with chest tube drainage and hypercaloric diet suplemented with medium chain triglycerides. Parenteral nutrition was needed in 2 patients, one because of sepsis and the other because of progressive malnourishment. High output fistulas and long periods of drainage were noted after cavo-pulmonary procedures. Up to 12% weight loss was observed in 6 patients, one patient was successfully treated of pneumonia and another died ofPseudomonas aeruginosasepsis. Two patients required chemical pleurodesis because of unabated drainage, with the others being successfully treated conservatively. Chylothorax is a common complication after bidirectional Glenn procedures, and is associated with considerable morbidity and mortality. Earlier operative intervention may be required in these patients. Pleurodesis is a simple, safe, and effective procedure to stop chylous flow.

scholarly journals Diagnosis and Management of Lymphatic Disorders in Congenital Heart Disease

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.

scholarly journals Reversal of Protein-Losing Enteropathy with Heparin Therapy in an Adult Patient with Congenital Heart Disease

Digestion ◽  
2006 ◽  
Vol 74 (3-4) ◽  
pp. 206-207 ◽  
Author(s):  
Takao Tsuzuki ◽  
Hiroyuki Okada ◽  
Ryuta Takenaka ◽  
Yoshiro Kawahara ◽  
Jun Kato ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Adult Patient ◽  
Congenital Heart ◽  
Heparin Therapy ◽  
Protein Losing Enteropathy

scholarly journals Congenital heart disease protein 5 associates with CASZ1 to maintain myocardial tissue integrity

Development ◽  
2014 ◽  
Vol 141 (15) ◽  
pp. 3040-3049 ◽  
Author(s):  
S. Sojka ◽  
N. M. Amin ◽  
D. Gibbs ◽  
K. S. Christine ◽  
M. S. Charpentier ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Myocardial Tissue ◽  
Tissue Integrity ◽  
Disease Protein
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