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Predictors of protein losing enteropathy after Fontan completion: An 8-year retrospective study at Sheikh Khalifa
Antoine Fakhry AbdelMassih1,2, Laszlo Kiraly3, Hazem El Badaoui4
1Pediatric Cardiology unit, Department of Pediatrics, Faculty of Medicine, Cairo University, Cairo, Egypt.
Insights
Predictors of protein-losing enteropathy (PLE) after the Fontan procedure include elevated superior vena cava pressure and atrio-ventricular valve regurgitation. Low oxygen saturation before the Fontan procedure also predicts PLE development in patients with univentricular hearts.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease Surgery
- Cardiovascular Physiology
Background:
- The Fontan procedure is a critical palliative surgery for univentricular heart conditions like hypoplastic left heart syndrome (HLHS).
- Fontan physiology can lead to diminished cardiac output and elevated systemic venous pressure, causing venous congestion.
- This congestion is a known risk factor for developing protein-losing enteropathy (PLE).
Purpose of the Study:
- To identify predictors of protein-losing enteropathy (PLE) in patients who underwent the Fontan procedure.
- To analyze clinical, laboratory, echocardiographic, and hemodynamic data to find correlations with PLE development.
- To evaluate the effectiveness of pre-Fontan parameters in predicting post-Fontan PLE.
Main Methods:
- Retrospective analysis of medical records from 48 patients undergoing Fontan completion between 2012 and 2020.
- Patients were categorized into two groups: those who developed PLE and those who did not.
- Data collected included demographics, pre-operative oxygen saturation, serum albumin, echocardiographic findings (valve regurgitation, ventricular function), and hemodynamic pressures (SVC, pulmonary artery).
Main Results:
- 13 out of 48 patients (25%) developed protein-losing enteropathy (PLE).
- Multivariate regression identified superior vena cava (SVC) mean pressure (P=0.012) and atrio-ventricular valve regurgitation (P=0.013) as significant predictors of PLE.
- Pre-operative oxygen saturation below 83% demonstrated 92% sensitivity in predicting post-Fontan PLE.
Conclusions:
- Elevated SVC pressure (higher than 11 mmHg) and moderate-to-severe atrio-ventricular valve regurgitation are predictors of Fontan failure and subsequent PLE.
- The study observed a higher prevalence of PLE and lower predictive SVC pressure cut-offs in their cohort.
- This may be attributed to the high proportion of hypoplastic left heart syndrome (HLHS) patients, reflecting the center's specialization in complex pediatric cardiac surgeries.
Background:
The Fontan procedure is the final stage of a three-stage palliation process in patients born with a univentricular heart as part of hypoplastic left heart syndrome (HLHS) or other pathologies with a univentricular heart. As essential as this procedure has proven to be for such cases, the Fontan physiology diminishes cardiac output and expands systemic venous pressure, which then leads to venous congestion that can be complicated by protein-losing enteropathy (PLE). This retrospective study aimed to identify the predictors of such complications in all patients who underwent completion of the Fontan procedure at our center (Sheikh Khalifa Medical City/SKMC) in the past eight years.
Methods:
This study examined the medical records of patients who underwent completion of Fontan repair at our center since the inauguration of the cardiac surgery program of SKMC in the United Arab Emirates (UAE) - 01 Jan 2012 to 31 Dec 2020. Exclusion criteria included the absence of any of the undermentioned data in patient files. Patients were divided into two groups: those who developed PLE and those who did not. For each group, the following data were collected: demographics data (current age and age at completion of Fontan), clinical and laboratory data (oxygen saturation, serum albumin), echocardiographic data (classification of original cardiac diagnosis, degree of atrio-ventricular valve regurgitation, ventricular functions), hemodynamic data (mean pressures of superior vena cava and pulmonary arteries before Fontan completion), operative data (type of initial palliation, type of Fontan, presence of fenestrations and its size) and the need for any cardiac intervention prior to Fontan completion, such as atrio-ventricular valve repair, peripheral pulmonary stenting and arch balloon dilatation.
Results:
Of the 48 included patients,13 (25%) developed PLE. Multivariate regression analysis proved that the best predictors of PLE were superior vena cava mean pressure (P = 0.012) and the degree of atrio-ventricular valve regurgitation (P = 0.013). An oxygen saturation <83% prior to Fontan completion was 92% sensitive in predicting PLE after Fontan completion.
Conclusion:
This is a single-center study of the predictors of PLE after Fontan procedure and, as expected from similar studies, SVC pressure higher than 11 mmHg and moderate-to-severe atrio-ventricular valve regurgitation were predictors of Fontan failure. The higher prevalence of PLE in our cohort, as well as lower cut-offs of SVC pressure that can predict complications, may be related to the predominance of hypoplastic left heart in the operated patients, which has been the main referral center for cardiac surgeries in UAE in the last decade.
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