Prevalence of Subclinical Enteric Alpha-1-Antitrypsin Loss in Children with Univentricular Circulation Following Total Cavopulmonary Connection.

Protein-Losing Enteropathy post Fontan palliation is associated with significant morbidity and mortality. To date, very little research has been carried out to improve early identification of enteric protein loss in these patients. We hypothesise that subclinical enteric protein loss may occur in patients post Fontan surgery. A cross-sectional study was performed on 43 patients post Fontan surgery. We collected specimens of stool and blood from patients with no symptoms of protein-losing enteropathy post Fontan. Stool samples were assessed for alpha one antitrypsin. The stool samples of two patients were discarded, leaving 41 stool samples. Blood samples were also collected to review albumin, C-reactive protein, liver and renal function. Twenty-eight (65%) of those enrolled were male. The median (IQR) age between Fontan and collection of study specimens was 3.5 (2-7) years. Two (5%) patients had elevated levels of alpha-1-antitrypsin. There was no correlation between blood biochemistry and elevated stool alpha-1-antitrypsin. Subclinical protein loss is rare in asymptomatic children after Fontan procedure with only 5% of patients having elevated stool alpha-1-antitrypsin but no other symptoms. These findings may relate to our small cohort size and the time to testing post cardiac surgery. Future longitudinal follow-up studies should assess the ability of alpha-1-antitrypsin to provide earlier detection of protein-losing enteropathy in asymptomatic patients post Fontan. Given the serious prognosis of protein-losing enteropathy in this patient group, further work is warranted.

Outcome of shared care for pediatric cardiac transplantation between two nations with different health care systems.

BACKGROUND: No data are available for the outcome of children undergoing cardiac transplantation with shared care programs in different countries. We sought to investigate the outcome of a shared care transplant program between 2 countries given the complex immunologic, cardiac, and psychologic needs of these young people. METHODS: We investigated the results of a shared care program for children who underwent cardiac transplantation between our center in the Republic of Ireland and 2 centers in the United Kingdom over 2 decades. RESULTS: Between 1990 and 2013, 22 patients underwent 23 cardiac transplants. The median age at transplant was 3.2 years (range, 0.3-13.3 years), median age at listing was 30 months (range, 0.1-13.3 years), and the median waiting list time was 2.8 months (range, 0.3-14 months). The median time to return to the referral center from the time of transplant was 3 weeks (range, 2-8 weeks). The referral center treated 4 of 5 late rejection episodes. Angiography was undertaken in the transplant center at annual or biannual review. Outcomes for rejection, coronary vasculopathy, and survival were comparable between the referral and transplant centers. CONCLUSIONS: This report of shared care for pediatric transplant patients between 2 sovereign nations demonstrates good results, with comparable outcomes to the specialist transplant center. These data may encourage liberalization of follow-up in other centers.