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Low pulmonary vascular compliance predisposes post-Fontan patients to protein-losing enteropathy
Jeong Jin Yu1, Tae-Jin Yun, Sung-Cheol Yun
1Division of Pediatric Cardiology, College of Medicine, University of Ulsan, Seoul, South Korea.
Insights
Protein-losing enteropathy (PLE) after Fontan surgery is a serious complication. Low pulmonary vascular compliance before surgery is a key risk factor for developing PLE after the extracardiac conduit Fontan operation.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Cardiovascular Surgery
Background:
- Protein-losing enteropathy (PLE) is a severe complication following the Fontan operation.
- The risk factors for developing PLE after the extracardiac conduit Fontan procedure remain poorly understood.
- This study investigates pre-operative predictors of PLE in patients undergoing this specific surgical approach.
Purpose of the Study:
- To identify pre-operative risk factors associated with protein-losing enteropathy (PLE).
- To determine the relationship between pre-operative variables and the development of PLE post-extracardiac conduit Fontan operation.
Main Methods:
- A cross-sectional retrospective study involving 235 patients who underwent an initial extracardiac conduit Fontan operation.
- Pre-operative and peri-operative data were collected through medical record reviews.
- Median age at operation was 3.5 years, with a median follow-up of 5 years.
Main Results:
- 5.1% of patients (12/234) developed PLE at a median of 2.2 years post-operation.
- Univariate analysis identified pulmonary vascular compliance (Cpv), central venous pressure, ICU stay, hospitalization duration, and chest tube indwelling as related factors.
- Multivariate analysis confirmed that only low pulmonary vascular compliance (Cpv) remained a significant independent predictor of PLE (p=0.0367).
Conclusions:
- Low pulmonary vascular compliance is significantly associated with the development of protein-losing enteropathy after the extracardiac conduit Fontan operation.
- Identifying patients with low Cpv pre-operatively may aid in risk stratification and management strategies for preventing PLE.
Background:
Protein-losing enteropathy (PLE) is a life-threatening and poorly understood complication after the Fontan operation. We sought to determine the pre-operative risk factors for PLE which developed after the extracardiac conduit Fontan operation.
Methods:
Two hundred thirty-five patients who underwent the extracardiac conduit Fontan operation as an initial Fontan type procedure (median age at operation: 3.5 years) were enrolled in this cross-sectional retrospective study. Pre-operative and peri-operative variables were surveyed through a review of medical records.
Results:
Within the median follow-up duration of 5 years, 12 patients developed PLE (12/234, 5.1%) at a median interval of 2.2 years after the Fontan procedure, and 4 died of PLE at a median interval of 1.2 years (range 0.21-7.62) after diagnosis. Factors found to be related to the time to the development of PLE on univariate analysis were pulmonary vascular compliance (Cpv) (p=0.0019), central venous pressure at postoperative 12 hours (p=0.0026), days of ICU stay (P=0.0449), days of hospitalization (p=0.0135), and days of chest tube indwelling (p=0.0493). Multivariate analysis, however, showed that only Cpv (p=0.0367) remained significant. The range of Cpv was 8.8-26.1 mm(2)/m(2)/mmHg (median 17.9) in patients with PLE, and 6.6-122.3 mm(2)/m(2)/mmHg (median 26.8) in patients without PLE.
Conclusions:
Low pulmonary vascular compliance is associated with the development of PLE after the extracardiac conduit Fontan operation.
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