Fontan-Associated Liver Disease: Pathophysiology, Staging, and Management

Téllez, Luís; Santiago, Enrique Rodríguez de; Albillos, Agustı́n

doi:10.1055/s-0041-1732355

Cited by 7 publications

(3 citation statements)

References 107 publications

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“…35 Patients with Fontan physiology are known to be at risk for bleeding because of their chronically elevated central venous pressures, associated liver disease, and known coagulation abnormalities. 36 At the same time, children with cyanotic HD, single-ventricle palliation, or implanted ventricular assist devices are known to be at risk for thrombotic complications. 37 Patients with GORE-TEX shunts, especially modified Blalock-Thomas-Taussig shunts, are at risk for shunt thrombosis when anticoagulation is stopped and the inflammatory response, hemodynamic changes, and fluid shifts related to surgery are encountered.…”

Section: Preoperative Evaluation Before Noncardiac Surgerymentioning

confidence: 99%

Perioperative Considerations for Pediatric Patients With Congenital Heart Disease Presenting for Noncardiac Procedures: A Scientific Statement From the American Heart Association

Nasr¹,

Markham²,

Clay³

et al. 2023

Circ: Cardiovascular Quality and Outcomes

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Continuous advances in pediatric cardiology, surgery, and critical care have significantly improved survival rates for children and adults with congenital heart disease. Paradoxically, the resulting increase in longevity has expanded the prevalence of both repaired and unrepaired congenital heart disease and has escalated the need for diagnostic and interventional procedures. Because of this expansion in prevalence, anesthesiologists, pediatricians, and other health care professionals increasingly encounter patients with congenital heart disease or other pediatric cardiac diseases who are presenting for surgical treatment of unrelated, noncardiac disease. Patients with congenital heart disease are at high risk for mortality, complications, and reoperation after noncardiac procedures. Rigorous study of risk factors and outcomes has identified subsets of patients with minor, major, and severe congenital heart disease who may have higher-than-baseline risk when undergoing noncardiac procedures, and this has led to the development of risk prediction scores specific to this population. This scientific statement reviews contemporary data on risk from noncardiac procedures, focusing on pediatric patients with congenital heart disease and describing current knowledge on the subject. This scientific statement also addresses preoperative evaluation and testing, perioperative considerations, and postoperative care in this unique patient population and highlights relevant aspects of the pathophysiology of selected conditions that can influence perioperative care and patient management.

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Section: Preoperative Evaluation Before Noncardiac Surgerymentioning

confidence: 99%

Perioperative Considerations for Pediatric Patients With Congenital Heart Disease Presenting for Noncardiac Procedures: A Scientific Statement From the American Heart Association

Nasr¹,

Markham²,

Clay³

et al. 2023

Circ: Cardiovascular Quality and Outcomes

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show abstract

“…It is not until the more advanced stages of fibrosis and cirrhosis that serological markers are markedly affected with an additional decrease in platelet count as a sign of PHTN and splenic consumption (70). In the case of HCC development in the Fontan circulation, an increase in α-fetoprotein may be seen in 74%-80% of patients (57,59).…”

Section: Diagnosismentioning

confidence: 99%

Fontan-associated liver disease: Diagnosis, surveillance, and management

2023

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The Fontan operation is a lifesaving procedure for patients with functional single-ventricle congenital heart disease, where hypoplastic left heart syndrome is the most frequent anomaly. Hemodynamic changes following Fontan circulation creation are now increasingly recognized to cause multiorgan affection, where the development of a chronic liver disease, Fontan-associated liver disease (FALD), is one of the most important morbidities. Virtually, all patients with a Fontan circulation develop liver congestion, resulting in fibrosis and cirrhosis, and most patients experience childhood onset. FALD is a distinctive type of congestive hepatopathy, and its pathogenesis is thought to be a multifactorial process driven by increased nonpulsatile central venous pressure and decreased cardiac output, both of which are inherent in the Fontan circulation. In the advanced stage of liver injury, complications of portal hypertension often occur, and there is a risk of developing secondary liver cancer, reported at young age. However, FALD develops with few clinical symptoms, a surprisingly variable degree of severity in liver disease, and with little relation to poor cardiac function. The disease mechanisms and modifying factors of its development are still not fully understood. As one of the more important noncardiac complications of the Fontan circulation, FALD needs to be diagnosed in a timely manner with a structured monitoring scheme of disease development, early detection of malignancy, and determination of the optimal time point for transplantation. There is also a clear need for consensus on the best surveillance strategy for FALD. In this regard, imaging plays an important role together with clinical scoring systems, biochemical workups, and histology. Patients operated on with a Fontan circulation are generally followed up in cardiology units. Ultimately, the resulting multiorgan affection requires a multidisciplinary team of healthcare personnel to address the different organ complications. This article discusses the current concepts, diagnosis, and management of FALD, with special emphasis on the role of different imaging techniques in the diagnosis and monitoring of disease progression, as well as current recommendations for liver disease surveillance.

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“…However, exercise intolerance in patients after ECF is well known ( 10 , 11 ). Long-term Fontan-associated liver disease (FALD), a serious consequence after Fontan operation, has recently recognized after the Fontan operation ( 12 – 14 ). Fontan fenestration is expected to reduce pressure in the Fontan circuit and increase cardiac output by increasing the preload ( 9 ).…”

Section: Introductionmentioning

confidence: 99%

The long-term effects of the fenestration in patients with extracardiac Fontan circulation—a multicenter Korean cohort study based on national Fontan registry

Ko,

Song,

Chi

et al. 2024

Front. Cardiovasc. Med.

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IntroductionThe long-term effects of fenestration in patients with Fontan circulation remain unclear. We aim to evaluate the fenestration impact on early and late outcomes in patients with extracardiac Fontan (ECF) using a propensity score matching analysis.MethodsWe performed an extensive retrospective multicenter clinical data review of the Korean Fontan registry and included 1,233 patients with surgical ECF (779 fenestrated, 454 non-fenestrated). Demographics, baseline, and follow-up data were collected and comprehensively analyzed. Patients were divided into two groups according to the baseline presence or absence of surgical fenestration. Subsequently, patients were sub-divided according to the fenestration status at the last follow-up. Propensity-score matching was performed to account for collected data between the 2 groups using a multistep approach. The primary outcomes were survival and freedom from Fontan failure (FFF). We also looked at postoperative hemodynamics, cardiopulmonary exercise test results, oxygen saturations, and functional status.ResultsAfter propensity-score matching (454 matched pairs), there was no difference in survival or FFF between the 2 groups. However, ECF patients with baseline fenestration had significantly lower oxygen saturation (p = 0.001) and lower functional status (p < 0.001). Patients with fenestration had significantly longer bypass times, higher postoperative central venous pressure, higher postoperative left atrial pressure, and less prolonged pleural effusion in the early postoperative period. The propensity score matching according to the fenestration status at the last follow-up (148 matched pairs) showed that patients with a persistent fenestration had significantly lower oxygen saturation levels (p < 0.001). However there were no intergroup differences in the functional status, survival and FFF.ConclusionsOur results showed no long-term benefits of the Fenestration in terms of survival and FFF. Patients with persistent fenestration showed oxygen desaturation but no difference in exercise intolerance was shown between the 2 groups.

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Fontan-Associated Liver Disease: Pathophysiology, Staging, and Management

Cited by 7 publications

References 107 publications

Perioperative Considerations for Pediatric Patients With Congenital Heart Disease Presenting for Noncardiac Procedures: A Scientific Statement From the American Heart Association

Perioperative Considerations for Pediatric Patients With Congenital Heart Disease Presenting for Noncardiac Procedures: A Scientific Statement From the American Heart Association

Fontan-associated liver disease: Diagnosis, surveillance, and management

The long-term effects of the fenestration in patients with extracardiac Fontan circulation—a multicenter Korean cohort study based on national Fontan registry

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