日本小児循環器学会雑誌 Pediatric Cardiology and Cardiac Surgery

Background: Protein-losing enteropathy (PLE) following the Fontan operation has been an extremely difficult complication to treat successfully. This study aimed to assess the prognosis and to establish appropriate treatment strategies for PLE.

Methods: From 1991 to 2014, 23 patients with PLE following the Fontan operation were identified from clinical database at Okayama University Hospital. Data were collected retrospectively.

Results: A total of 401 patients underwent Fontan operation at our institution during the study period. In this cohort, the 5.7％ incidence of PLE was relative to the total number of Fontan operations. Following PLE diagnosis, patients had a 68％ and 54％ survival rate at 5 and 10 years, respectively. The causes of death included sepsis, multiple organ failure, heart failure, and intracranial bleeding. In the treatment of PLE, 23 (100％) patients were treated with medication, 10 (43％) patients underwent catheter intervention and 15 (65％) patients underwent surgery. Patients who died were older at the time of PLE diagnosis and were associated with a longer interval between the Fontan operation and PLE diagnosis. Decreased survival was observed in patients with the following conditions post-PLE treatment: a cardiac index (CI)＜2.8 L/min/mm, decreased ventricular ejection fraction (vEF)＜50％, and high central venous pressure (meanCVP)≧15 mmHg.

Conclusion: Our current treatment for PLE was only partially successful. Strategies for reducing CVP and improving both CI and vEF may be effective. Further studies are necessary to determine better treatment strategies for the future.