Heart and heart–liver transplantation in adults with failing Fontan physiology

Reardon, L.; DePasquale, E.C.; Tarabay, Jana; Cruz, Daniel; Laks, Hillel; Biniwale, Reshma; Busuttil, Ronald W.; Kaldas, Fady M.; Saab, Sammy; Venick, Robert S.; Lin, Jeannette P.; Nsair, Ali; Deng, Mario C.; Ardehali, A.; Caderias, Martin; Iygengar, Amit; Aboulhosn, Jamil

doi:10.1111/ctr.13329

Cited by 52 publications

(29 citation statements)

References 19 publications

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“…Before cardiac hepatopathy reaches an advanced stage, patients should be considered for heart transplantation [61][62][63]. Combined heart and liver transplantation is an uncommon procedure in failing Fontan patients [61,147,148]. However, if extended hepatic fibrosis is accompanied by HCC or portal hypertension, combined heart and liver transplantation is considered [100].…”

Section: Treatmentmentioning

confidence: 99%

Liver disease secondary to congenital heart disease in children

Komatsu

Inui

Kishiki

et al. 2019

Expert Review of Gastroenterology & Hepatology

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Introduction: Hepatic fibrosis and hepatocellular carcinoma (HCC) can develop in children with congenital heart disease. Although hepatic fibrosis and HCC are prone to develop after the Fontan operation, they can also develop in patients suffering from congenital heart disease who have not undergone Fontan operation. Area covered: The history of cardiac hepatopathy including Fontan-associated liver disease is described. Patient characteristics, liver histology, imaging examinations and blood tests are reviewed to elucidate the mechanism of cardiac hepatopathy. In addition, a flowchart for the follow-up management of cardiac hepatopathy in children with congenital heart disease is proposed. Expert opinion: Congestion and low cardiac output are the main causes of cardiac hepatopathy. Advanced hepatic fibrosis is presumed to be associated with HCC. HCC can develop in both adolescents and young adults. Regardless of whether the Fontan operation is performed, children with a functional single ventricle and chronic heart failure should be regularly examined for cardiac hepatopathy. There is no single reliable laboratory parameter to accurately detect cardiac hepatopathy; hepatic fibrosis indices and elastography have shown inconsistent results for detection of this disease. Further studies using liver specimen-confirmed patients and standardization of evaluation protocols are required to clarify the pathogenesis of cardiac hepatopathy.

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Section: Treatmentmentioning

confidence: 99%

Liver disease secondary to congenital heart disease in children

Komatsu

Inui

Kishiki

et al. 2019

Expert Review of Gastroenterology & Hepatology

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“…Impaired hepatic function is always present in patients suffering from failing Fontan circulation and has a negative influence on outcomes following heart transplantation (46,47). If hepatic cirrhosis is confirmed, heart-liver transplantation must be considered (48).…”

Section: Surgical Options For Failing Fontanmentioning

confidence: 99%

Current spectrum, challenges and new developments in the surgical care of adults with congenital heart disease

Hörer

2018

Cardiovasc. Diagn. Ther

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Today, more than two thirds of patients with congenital heart disease (CHD) are adults. Cardiac surgery plays an essential role in restoring and maintaining cardiac function, aside from evolving medical treatment and catheter-based interventions. The aim of the present publication was to describe the spectrum of operations performed on adults with CHD (ACHD) by reviewing current literature. Currently, surgery for ACHD is predominantly valve surgery, since valvular pathologies are often either a part of the basic heart defect or develop as sequelae of corrective or palliative surgery. Surgical techniques for valve repair, established in patients with acquired heart disease (non-ACHD), can often be transferred to ACHD. New valve substitutes may help to reduce the number of redo operations. Most of valve operations yield good results in terms of survival and quality of life, with the precondition that the ventricular function is preserved. Heart failure due to end-stage CHD is the most frequent cause of mortality in ACHD. However, surgical treatment by means of mechanical circulatory support (MCS) is still uncommon and the mortality exceeds the one following other operations in ACHD. Currently, different devices are used and new technical developments are in progress. However, there still is no ideal assist device available. Therefore, heart transplantation remains the only valid option for end-stage CHD. Despite higher early mortality following heart transplantation in ACHD compared to non-ACHD, the long-term survival compares favorably to non-ACHD. There is room for improvement by refining the indications, the time of listing, and the perioperative care of ACHD transplant patients. Sudden death is the second most frequent cause of mortality in ACHD. Ventricular tachycardia is the most frequent cause of sudden death followed by coronary artery anomaly. Due to the increasing awareness of physicians and the improved imaging techniques, coronary artery anomalies are coming more into the focus of cardiac surgeons. However, the reported experience is limited and it is currently difficult to provide a standardized and generally applicable recommendation for the indication and the adequate surgical technique. With the increasing age and complexity of ACHD, treatment of rhythm disturbances by surgical ablation, pacemaker or implantable cardioverter defibrillator (ICD) implantation and resynchronisation gains importance. A risk score specifically designed for surgery in ACHD is among the newest developments in predicting the outcome of surgical treatment of ACHD. This evidence-based score, derived from and validated with data from the Society of Thoracic Surgeons Congenital Heart Surgery Database, enables comparison of risk-adjusted performance of the whole spectrum of procedures performed in ACHD and helps in understanding the differences in surgical outcomes. The score is thus a powerful tool for quality control and quality improvement. In conclusion, new developments in surgery for ACHD are currently made with regard to ...

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“…Liver disease has gained attention in transplant evaluation for patients with Fontan; however, accurately predicting the risk of FALD that adds to cardiac transplantation is difficult . For Fontan patients with both advanced heart failure and progressive FALD, combined heart–liver transplantation has been successfully pursued, though the potential for perioperative morbidity and mortality is significant and long‐term outcomes remain unknown. These challenges place emphasis on early detection of clinically significant liver dysfunction to aid in timing of transplant consideration.…”

Section: Discussionmentioning

confidence: 99%

More than the heart: Hepatic, renal, and cardiac dysfunction in adult Fontan patients

Byrne

Weingarten

Clark

et al. 2019

Congenital Heart Disease

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Setting Fontan‐associated liver disease universally affects adults with single ventricle heart disease. Chronic kidney disease is also highly prevalent in adult Fontan patients. In this study, we evaluate the relationship of Fontan hemodynamics invasively and noninvasively with extra‐cardiac dysfunction as measured by MELD and MELD‐XI. Objective We hypothesize that invasive and noninvasive measures of Fontan circuit congestion and ventricular dysfunction are associated with increased MELD and MELD‐XI scores. Design Single‐center data from adults with Fontan palliation who had ongoing care, including cardiac catheterization, were retrospectively collected. Hemodynamic data from cardiac catheterization and echocardiographic assessment of ventricular and atrioventricular valve function were tested for association with serum creatinine, MELD, and MELD‐XI. Linear regression was used to perform multivariable analysis in the echocardiogram cohort. Results Fifty‐seven patients had congruent lab and catheterization data for analysis. Sixty‐three and sixty‐nine patients had congruent lab and echocardiogram data for MELD and MELD‐XI, respectively. Of the hemodynamic variables analyzed, only decreased systemic oxygen saturation had significant correlation with elevated MELD and MELD‐XI (P = .045). Patients with moderately or severely reduced ejection fraction by echocardiogram had significantly higher MELD and MELD‐XI scores compared to those with normal or mildly depressed systolic ventricular function (P = .008 and P < .001 for MELD and MELD‐XI, respectively). Significant differences in creatinine were also found among the ventricular dysfunction groups (P = .02). Conclusions In adults following Fontan palliation, systolic ventricular dysfunction and decreased oxygen saturation were associated with hepatic and renal dysfunction as assessed by elevated serum creatinine, MELD, and MELD‐XI scores.

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Heart and heart–liver transplantation in adults with failing Fontan physiology

Cited by 52 publications

References 19 publications

Liver disease secondary to congenital heart disease in children

Liver disease secondary to congenital heart disease in children

Current spectrum, challenges and new developments in the surgical care of adults with congenital heart disease

More than the heart: Hepatic, renal, and cardiac dysfunction in adult Fontan patients

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