Abstract：
OBJECTIVE:Protein-losing enteropathy is an infrequent but severe condition occurring after Fontan procedure. The multifactorial pathogenesis remains unclear and no single proposed treatment strategy has proven universally successful. Therefore, we sought to describe different treatment strategies and their effect on clinical outcome and mortality. MATERIAL AND METHODS:We performed a retrospective observational study. From the total cohort of 439 Fontan patients treated in our institution during the study period 1986-2019, 30 patients (6.8%) with protein-losing enteropathy were identified. Perioperative, clinical, echocardiographic, laboratory, and invasive haemodynamic findings and treatment details were analysed. RESULTS:Median follow-up after disease onset was 13.1 years [interquartile range 10.6]. Twenty-five patients received surgical or interventional treatment for haemodynamic restrictions. Medical treatment, predominantly pulmonary vasodilator and/or systemic anti-inflammatory therapy with budesonide, was initiated in 28 patients. In 15 patients, a stable remission could be achieved by medical or surgical procedures (n = 3 each), by combined multimodal therapy (n = 8), or ultimately by cardiac transplantation (n = 1). Phrenic palsy, bradyarrhythmia, Fontan pathway stenosis, and absence of a fenestration were significantly associated with development of protein-losing enteropathy (p = 0.001-0.48). Ten patients (33.3%) died during follow-up; 5-year survival estimate was 96.1%. In unadjusted analysis, medical therapy with budesonide and pulmonary vasodilator therapy in combination was associated with improved survival. CONCLUSIONS:Protein-losing enteropathy is a serious condition limiting survival after the Fontan procedure. Comprehensive assessment and individual treatment strategies are mandatory to achieve best possible outcome. Nevertheless, relapse is frequent and long-term mortality substantial. Cardiac transplantation should be considered early as treatment option.
journal_name
Cardiol Youngjournal_title
Cardiology in the youngauthors
Schleiger A,Ovroutski S,Peters B,Schubert S,Photiadis J,Berger F,Kramer Pdoi
10.1017/S1047951120000864subject
Has Abstractpub_date
2020-05-01 00:00:00pages
698-709issue
5eissn
1047-9511issn
1467-1107pii
S1047951120000864journal_volume
30pub_type
杂志文章abstract：:With the advent of improved medical and surgical care in congenital heart disease, there has been an increase in the number of patients who survive into adulthood, giving rise to a new patient population 'Adults with congenital heart disease'. In the past, morbidity and mortality were the primary concerns for this gro...
journal_title：Cardiology in the young
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journal_title：Cardiology in the young
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