Specific sequelae after Fontan operation at mid- and long-term follow-up

Cromme‐Dijkhuis, Adri H.; Hess, John; Hählen, K.; Henkens, C. M. A.; Bink‐Boelkens, Margreet Th.E.; Eygelaar, A; Bos, Egbert

doi:10.1016/s0022-5223(19)33989-3

Cited by 162 publications

(68 citation statements)

References 23 publications

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“…30,31 In addition, Fontan's operation causes an increase in systemic venous pressure, and hepatic complications in some cases. 32 This systemic venous hypertension not only obstructs lymph drainage from the thoracic duct, but it also causes hepatic congestion, which significantly increases the volume of lymph in the thoracic duct. 33 This could explain the relatively large proportion (6/11) of cavopulmonary anastomoses complicated by plastic bronchitis among the published pediatric cases of bronchial casts with cardiopathy.…”

Section: Discussionmentioning

confidence: 99%

Bronchial casts in children with cardiopathies: The role of pulmonary lymphatic abnormalities

et al. 1999

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Expectoration of bronchial casts, a condition also called plastic bronchitis, is very rare in children. Bronchial casts may be associated with bronchopulmonary diseases associated with mucus hypersecretion, bronchopulmonary bacterial infections, congenital and acquired cardiopathies, or pulmonary lymphatic abnormalities. A classification based on anatomy and pathology has been proposed which identifies an “acellular” group associated with congenital cardiopathies and palliative surgery. We report on 3 cases with bronchial casts associated with cardiopathy. Observations suggest that the formation of bronchial casts may result from lymphatic leakage into the bronchi. The 3 cases on which we report were immunodeficient and had pulmonary lymphatic abnormalities. The bronchial casts contained lymphocytes and lipids, as determined by histologic examination. In the absence of congenital pulmonary or diffuse lymphatic dysplasia associated with cardiopathy, the principal factors resulting in the formation of bronchial casts appear to be surgical trauma to the lymphatic channels surrounding the bronchi, pleural adhesions, and high systemic venous blood pressure. The prognosis for these patients is poor, and possibilities for treatment are limited. Pediatr Pulmonol. 1999;28:329–336. © 1999 Wiley‐Liss, Inc.

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Section: Discussionmentioning

confidence: 99%

Bronchial casts in children with cardiopathies: The role of pulmonary lymphatic abnormalities

et al. 1999

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“…Abnormalities in liver enzymes have long been recognized in post-Fontan patients. [54][55][56] A cholestatic pattern of liver enzyme elevation, with mild elevation of alkaline phosphatase and total and direct bilirubin, is common, although the most prevalent abnormality is elevation of gamma-glutamyl transferase (GGT). [57][58][59] In the absence of PLE, serum albumin is preserved until the onset of decompensated cirrhosis.…”

Section: Laboratory Monitoring Post Fontanmentioning

confidence: 99%

Surveillance for liver complications after the Fontan procedure

Hilscher

Johnson

Cetta

et al. 2017

Congenital Heart Disease

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The physiological consequences of the Fontan circulation impose risk for hepatic dysfunction and may culminate in hepatic fibrosis, cirrhosis, and hepatocellular carcinoma. Consensus regarding appropriate surveillance modalities to diagnose liver disease in Fontan patients is lacking, in part due to the relative lack of strong evidence and prospective studies in this patient population. The goal of this paper is to critically review the current evidence and provide recommendations for the surveillance of hepatic complications in the post-Fontan patient population.

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“…With length of follow-up ranging from 1 to 21.8 years, 60-75% of subjects had abnormalities of liver enzymes, most characterized as mild. [4][5][6] Narkewicz et al determined functional hepatic mass by assessing galactose elimination 8 ; all 11 subjects had a demonstrable increase in measured galactose elimination half-life, indicating impairment of hepatic function. However, no histologic correlations were made as many of these subjects did not undergo liver biopsy.…”

Section: Clinical Findingsmentioning

confidence: 99%

“…The clinical significance of these findings, however, has remained poorly understood. [3][4][5][6][7][8] As Fontan survivors have increased in age and number, 9 we have begun to better recognize subclinical hepatic dysfunction and the contribution of liver disease to adverse outcomes in this population. The purpose of this review is to summarize the existing data pertaining to liver disease in the Fontan population and to identify some questions that will require further study.…”

Section: Introductionmentioning

confidence: 99%

Liver Disease in the Patient with Fontan Circulation

Ukomadu²,

Odze

et al. 2011

Congenital Heart Disease

129

127

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The Fontan procedure has undergone many modifications since first being performed on a patient with tricuspid valve atresia in 1968. It is now the procedure of choice for individuals born with single-ventricle physiology or for those in whom a biventricular repair is not feasible. Forty years of experience with the Fontan procedure have gradually revealed the shortfalls of such a circulatory arrangement. Sequelae related to the underlying congenital anomaly or to the altered physiology of passive, nonpulsatile flow through the pulmonary arterial bed can result in failure of the Fontan circulation over time. Liver abnormalities including abnormalities in the clotting cascade have been well documented in Fontan patients. The clinical significance of these findings, however, has remained poorly understood. As Fontan survivors have increased in age and number, we have begun to better recognize subclinical hepatic dysfunction and the contribution of liver disease to adverse outcomes in this population. The purpose of this review is to summarize the existing data pertaining to liver disease in the Fontan population and to identify some questions that have yet to be answered.

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Specific sequelae after Fontan operation at mid- and long-term follow-up

Cited by 162 publications

References 23 publications

Bronchial casts in children with cardiopathies: The role of pulmonary lymphatic abnormalities

Bronchial casts in children with cardiopathies: The role of pulmonary lymphatic abnormalities

Surveillance for liver complications after the Fontan procedure

Liver Disease in the Patient with Fontan Circulation

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