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Comparison of Clinical Profiles in Patients with Protein-Losing Enteropathy With and Without Fontan Circulation
Shin Ono1, Hideo Ohuchi2, Aya Miyazaki1
1Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, 5-7-1 Fujishiro-dai, Suita, Osaka, 565-8565, Japan.
Insights
Protein-losing enteropathy (PLE) after congenital heart disease (CHD) repair differs between Fontan and biventricular repair (BVR) patients. BVR patients show higher remission rates for PLE, but mortality remains high in both groups.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Gastroenterology
Background:
- Protein-losing enteropathy (PLE) is a serious complication after Fontan operation for single-ventricle congenital heart disease (CHD).
- PLE can also occur in patients with CHD after biventricular repair (BVR).
- Limited comparative data exists on PLE in Fontan versus BVR patients.
Purpose of the Study:
- To compare the clinical profiles, treatment outcomes, and survival rates of PLE in patients following Fontan operation versus BVR.
Main Methods:
- Retrospective review of clinical charts of 42 post-operative CHD patients diagnosed with PLE.
- Comparison of patient demographics, clinical characteristics, interventions, and outcomes between the BVR group (n=14) and Fontan group (n=28).
Main Results:
- Fontan patients developed PLE significantly sooner post-operatively than BVR patients (14±2 vs. 8±1 years).
- BVR patients had a higher prevalence of underlying structural lesions (93% vs. 50%) and higher ventricular end-diastolic pressure at PLE onset.
- Complete PLE remission was significantly higher in the BVR group (38% vs. 7%), but overall 5- to 10-year survival rates after PLE onset were similar (81% for BVR, 66% for Fontan).
Conclusions:
- While biventricular repair patients may have a better chance of PLE remission compared to Fontan patients, mortality remains substantial in both groups.
- PLE in CHD patients necessitates careful management regardless of the type of surgical repair.
Abstract:
Protein-losing enteropathy (PLE) is a life-threatening complication in patients following the Fontan operation. However, PLE also develops in some patients with congenital heart disease (CHD) after biventricular repair (BVR). This study compared clinical profiles of PLE patients following the Fontan operation with those after BVR. We retrospectively reviewed clinical charts of postoperative CHD patients with PLE. The study population comprised 42 PLE patients (14BVR, 28Fontan). Postoperative follow-up period until onset was significantly shorter in the Fontan group than in the BVR group (14 ± 2 vs. 8 ± 1 years, p = 0.02), while there was no difference in PLE onset age between groups. Furthermore, there were no differences in prevalence of clinically relevant arrhythmias, cardiac output, or central venous pressure between the two groups at PLE onset. Percentage of structural lesions (valve regurgitation and/or stenotic lesions) responsible for development of PLE and ventricular end-diastolic pressure were higher in the BVR group than in the Fontan group (93 vs. 50%, p < 0.01), (13.4 ± 6.3 vs. 7.5 ± 4.1, p < 0.0001). Catheter intervention was applied in 2Fontan and 6BVR patients, while surgical intervention was required in 8BVR and 7Fontan patients. Of these, catheter intervention was effective in 2 (25%, 1Fontan, 1BVR) and surgical intervention was effective in 4 (26.7%, 1Fontan, 3BVR). Only one patient (5.3%) improved without intervention. Complete PLE remission rate was higher in the BVR group than in the Fontan group (38 vs. 7%, p = 0.02). During follow-up, death of 2 BVR and 8 Fontan patients occurred. There were no group differences in 5- to 10-year survival rates after PLE onset (81 vs. 81%, BVR, 81 vs. 66%, Fontan). Although BVR patients may have greater chance of PLE remission when compared with those exhibiting Fontan pathophysiology, mortality in PLE-CHD patients was significantly high regardless of postoperative hemodynamics.
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