Fontan-Sprechstunde

Condition(s) studied

Investigational drug(s) / intervention(s)

Univentricular Hearts: Prevention when factors are recognized

Study summary

After successful Fontan surgery, the risk of mortality in childhood is only low. Unfortunately, some of the patients suffer from Fontan-typical long-term complications in the long-term course, whereby protein loss neuropathy must be mentioned in particular, which is described in the literature with an incidence of 3-14% (1, 2) and still has a 5-year risk of death of 6-12% today (2, 3). Protein loss tereopathy leads to loss of protein in the intestine and subsequently to diarrhea and edema. Other problems concern the liver, which can develop cirrhosis due to chronic congestion (4-6). Cardiac can lead to heart failure and arrhythmias.

The registry study described in this protocol is intended to identify factors that influence the treatment outcome of patients in the Fontan circulation in the long term through systematic prospective documentation of the data from our standardized and guideline-oriented treatment.

Eligibility

Primary outcome measure(s)

• Reducing mortality and morbidity and improving quality of life in long-term care for Fontan patients — 50 Jahre
  1. Fontan Tunnel Obstruction 2. Fontan-typical klin. Symp. defined in points 5.+6. o. Presence of edema, ascites, susceptibility to infections, reduced resilience, cyanosis, lack of size and weight development, diarrhea 3. Heart or heart valve insufficiency defined as end-diastolic volume/BSA (EDP corrected) above the norm, ventricle ejection fraction (EF) \<50%, atrioventricular heart valve insufficiency (AVI) \>grade 2, aortic valve insufficiency \>grade 2 4. Pathological lymphatic vessel imaging defined as lymphatic vasodilation (LAE) type ≥ 3 according to Biko et al. (abnormal supraclavicular lymphatic vasodilation with infiltration of the mediastinum) (7). Presence of mesenteric lymphatic vascular congestion (8) 5. Hypoproteinaemia defined as serum albumin \<3.4 mg/dl (4), total protein (TP) \<50 g/l 6. protein-losing enteropathy (PLE) defined as (3, 4) 7. Failing Fontan defined as Operative Fontan Take-down, Listing for Heart Transplantation and/or Death

Trial sites (1)

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