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Echocardiographic characteristics in Fontan patients before the onset of protein-losing enteropathy or plastic
Florian Du Bois1, Brigitte Stiller1, Thomas Borth-Bruhns2
1University Heart Center Freiburg-Bad Krozingen, Department of Congenital Heart Disease and Pediatric Cardiology, Medical Center-University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany.
Insights
Fontan patients at risk for protein-losing enteropathy (PLE) or plastic bronchitis (PB) show altered abdominal artery flow. Early detection of elevated celiac artery resistance may predict these complications in Fontan patients.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Vascular Ultrasound
Background:
- The Fontan circulation presents unique challenges for abdominal organ perfusion.
- Protein-losing enteropathy (PLE) and plastic bronchitis (PB) are serious complications following Fontan procedures.
- Early identification of patients at risk for PLE/PB is crucial for timely intervention.
Purpose of the Study:
- To evaluate echocardiographic and Doppler ultrasound characteristics of abdominal arteries in Fontan patients.
- To assess flow patterns in Fontan patients before the onset of PLE or PB.
- To identify potential hemodynamic markers predictive of PLE or PB development.
Main Methods:
- Retrospective cohort study of 170 Fontan patients with echocardiographic and Doppler ultrasound examinations.
- Comparison between patients who developed PLE/PB and those who did not.
- Follow-up questionnaires to ascertain the occurrence of PLE or PB post-examination.
Main Results:
- Patients who later developed PLE/PB showed significantly slower diastolic flow velocities in the celiac and superior mesenteric arteries.
- Pre-manifestation elevated resistance indices in the celiac artery were observed in future PLE/PB patients.
- These hemodynamic differences were significant even before the clinical diagnosis of PLE or PB.
Conclusions:
- Elevated flow resistance in the celiac artery may be a precursor to PLE or PB in Fontan patients.
- Doppler ultrasound assessment of abdominal arteries could aid in predicting PLE/PB risk.
- These findings highlight the importance of monitoring mesenteric and celiac artery hemodynamics in Fontan survivors.
Background:
It was this study's objective to evaluate the echocardiographic characteristics and flow patterns in abdominal arteries of Fontan patients before the onset of protein-losing enteropathy (PLE) or plastic bronchitis (PB).
Design:
In this retrospective cohort investigation, we examined 170 Fontan patients from 32 different centers who had undergone echocardiographic and Doppler ultrasound examinations between June 2006 and May 2013. Follow-up questionnaires were completed by 105 patients a median of 5.3 (1.5-8.5) years later to evaluate whether one of the complications had occurred since the examinations.
Results:
A total of 91 patients never developed PLE or PB ("non-PLE/PB"); they were compared to 14 affected patients. Eight of the 14 patients had already been diagnosed with "present PLE/PB" when examined. Six "future PLE/PB" patients developed those complications later on and were identified on follow-up. The "future PLE/PB" patients presented significantly slower diastolic flow velocities in the celiac artery (0.1 (0.1-0.5) m/s vs 0.3 (0.1-1.0) m/s (P = .04) and in the superior mesenteric artery (0.0 (0.0-0.2) m/s vs 0.2 (0.0-0.6) m/s, P = .02) than the "non-PLE/PB" group. Median resistance indices in the celiac artery were significantly higher (0.9 (0.8-0.9) m/s vs 0.8 (0.6-0.9) m/s, (P = .01)) even before the onset of PLE or PB.
Conclusion:
An elevated flow resistance in the celiac artery may prevail in Fontan patients before the clinical manifestation of PLE or PB.
