Background: Patients with congenital heart disease are surviving into adulthood because of new surgeries developed over the recent decades. One surgery is the Fontan procedure, which is used in patients with only one functioning ventricle. Although it has been saving lives, in the recent years multiple complications have been noted.
Objective: To discuss the Fontan procedure and inform nurse practitioners in the primary care setting the importance of early screening and referral because of increasing number of liver complications in these patients. This case study highlights the dilemmas a patient with the Fontan procedure faces as a young adult.
Methods: Evidence-based articles from the PubMed and Embase databases were used to support the case study.
Results: Patients with the low-pressure Fontan circulation are under chronic passive congestion and have known liver complications such as liver fibrosis, cirrhosis, and protein-losing enteropathy. This can lead to a high-risk dual organ heart-liver transplant.
Conclusions: Early screening and referral to a hepatologist are critical in these patients.
Implications for nursing: Since patients with congenital heart disease often present to the primary care setting, nurse practitioners in this area should understand the Fontan procedure and its associated complications so they can effectively manage these patients.
Keywords: Fontan; Fontan procedure; Fontan-associated liver disease; congenital heart disease; single ventricle.
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