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Eur J Cardiothorac Surg. 2013 Mar;43(3):e49-57. doi: 10.1093/ejcts/ezs714.

Haemodynamic characteristics before and after the onset of protein losing enteropathy in patients after the Fontan operation.

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  • 1Departments of Pediatric Cardiology, National Cerebral and Cardiovascular Center, Osaka, Japan.



Determinant risk factors for developing protein losing enteropathy (PLE), including haemodynamics, remain unclear in patients after the Fontan operation.


Our purpose was to characterize the serial PLE haemodynamics before and after the onset and to determine the risk factors based on the cardiac catheterization-based analysis.


Of 354 Fontan survivors who had undergone postoperative cardiac catheterizations, we experienced 26 PLE patients during the follow-up. Non-left ventricular morphology systemic ventricle, functional one-lung pulmonary circulation and an early postoperative high central venous pressure (CVP) were associated with the PLE onset and the high CVP (odds ratio (OR) = 1.19 per 1 mmHg, 95% confidence interval (CI) 1.04-1.37, especially ≥12 mmHg, OR = 3.09, 95% CI 1.25-7.64, P < 0.05 for both) and one-lung pulmonary circulation (OR = 10.0-10.5, P < 0.001) independently predicted the onset. At the time of the PLE onset, a Fontan route stenosis/obstruction, arrhythmias, ventricular dysfunction/heart failure and pulmonary arterio-venous fistulae were demonstrated in 10 (38%), 8 (31%), 4 (15%) and 3 (12%) patients, respectively. When compared with 56 excellent Fontan survivors, the high CVP, ventricular end-diastolic pressure, and pulmonary artery resistance, and the low arterial oxygen saturation, systemic artery pressure, and ventricular ejection fraction characterized the pre-PLE Fontan haemodynamics (P < 0.05-0.0001). However, the following intensive treatments reduced the CVP, systemic artery pressure and cardiac output (P < 0.05-0.01), resulting in haemodynamics no different from those of the excellent survivors, except for the low systemic pressure (P < 0.0001).


The pre-PLE haemodynamics was characterized by several impaired haemodynamics, while those after PLE only by a low systemic pressure. A high early postoperative CVP was the only haemodynamic predictor for a new onset of PLE. Strict selective criteria for the operation and strategies to eliminate CVP-raising factors are mandatory to prevent a new onset of PLE.

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