Clinical Trial: Plastic Bronchitis and Protein Losing Enteropathy in Children With Single Ventricle Physiology
Study Status: Completed
Recruit Status: Completed
Study Type: Observational
Official Title: An Investigation Into The Potential Roles Of Vasoactive Intestinal Peptide And Substance P In The Pathophysiology Of Plastic Bronchitis And Protein Losing Enteropathy In C
Brief Summary: The investigators are studying what causes Plastic Bronchitis and Protein Losing Enteropathy. The investigators think that these problems are from too much of two small proteins called Vasoactive Intestinal Peptide (VIP) and Substance P. VIP and Substance P are important proteins in the body that normally tell the body to make small amounts of fluid and they help the intestines work. Normally, VIP and Substance P are made in the intestines and then destroyed in the lungs after they do their normal work. The investigators think that kids who have Plastic Bronchitis and/or Protein Losing Enteropathy who also had the Fontan surgery might have too much VIP and Substance P in their bodies. The investigators think this causes too much fluid to go in the lungs and too much protein in the intestines.
Detailed Summary:
Protein losing enteropathy (PLE) has emerged as an increasingly common complication of single ventricle palliation in children born with cyanotic congenital heart disease. This entity becomes manifest months or years after the modified Fontan operation and is thought to affect 5-15% of all patients with Fontan physiology. Patients with PLE often suffer from symptoms related to severe hypoproteinemia and intestinal malabsorption, including edema, ascites, pleural and pericardial effusions, chronic diarrhea and poor growth. Mortality is strikingly high, with only 50% of patients surviving for more than 5 years following diagnosis. The pathophysiology is poorly understood and several theories are presented as possible mechanisms which includes: 1) chronic low cardiac output and intestinal injury leading to loss of intestinal integrity, 2) chronic intestinal inflammation leading to loss of intestinal integrity, 3) chronic elevated portal venous pressure with intestinal injury leading to loss of intestinal integrity. Treatment is sporadically successful and has included 1) creating a fenestration between the Fontan pathway and pulmonary venous atrium which decreases Fontan pressure and increases cardiac output, 2) chronic use of unfractionated or low molecular weight heparin to reconstitute the intestinal basement membrane, 3) Use of oral steroids to reduce intestinal inflammation, 4) Use of pulmonary vasodilators to reduce Fontan pressure and increase cardiac output 5) implantation of pacemakers to treat sinus node dysfunction and improve cardiac output, 6) heart transplantation. Short of cardiac transplantation, no treatment of PLE, including efforts to change systemic and/or gastrointestinal hemodynamics, is routinely successful. Thus, it appears that the etiology of PLE is more complex than simply "high venous pressure in the gut" as has been previously believed.
Sponsor: Medical College of WisconsinCurrent Primary Outcome: Etiologic factors in the development of PLE and PB in patients with Fontan physiology [ Time Frame: During catheterization ]
Original Primary Outcome:
Current Secondary Outcome:
Original Secondary Outcome:
Information By: Medical College of Wisconsin
Dates:
Date Received: March 23, 2012
Date Started: March 2012
Date Completion:
Last Updated: October 28, 2013
Last Verified: October 2013
