Hepatocellular Carcinoma with Right Atrial Extension in a Young Patient with Congenitally Unguarded Tricuspid Orifice

Barberis, Vassilis I.; Brili, Stella; Dimopoulou, Maria; Barbetseas, John; Stefanadis, Christodoulos

doi:10.1111/j.1540-8175.2006.00226.x

Cited by 7 publications

(4 citation statements)

References 6 publications

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“…Aside from the literature on FALD, there have been few reports, there have been few reports that liver tumors were associated with congenital heart disease [29,30,77]. In regard to FALD, the collected data from 6 studies showed that, 10 to 15 years after Fontan operation, hypervascular nodules were developed in approximately 20% to 30% of patients (Table 1) [17,68,[78][79][80][81].…”

Section: Liver Tumors Associated With Congenital Heart Diseasementioning

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: Liver Tumors Associated With Congenital Heart Diseasementioning

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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“…As of 2006, only five cases of hepatocellular carcinoma had been reported in the setting of cardiac cirrhosis, including only one in a Fontan patient. 30,[51][52][53][54] In their autopsy study, Ghaferi and Hutchins described a patient who had died at the age of 24 years, 18 years after his Fontan surgery. At autopsy, a 4-cm liver mass with overlying hemorrhage was found protruding from the posteroinferior aspect of the left lobe.…”

Section: Prognosismentioning

confidence: 99%

“…Also concerning are case reports of hepatocellular carcinoma in patients with Fontan circulation and liver disease that are appearing with increasing frequency. As of 2006, only five cases of hepatocellular carcinoma had been reported in the setting of cardiac cirrhosis, including only one in a Fontan patient 30,51–54 . In their autopsy study, Ghaferi and Hutchins described a patient who had died at the age of 24 years, 18 years after his Fontan surgery.…”

Section: Prognosismentioning

confidence: 99%

Liver Disease in the Patient with Fontan Circulation

Ukomadu²,

Odze

et al. 2011

Congenital Heart Disease

128

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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“…[127][128][129][130][131] MR imaging with a hepatocyte-specific contrast agent has emerged as the preferred imaging modality for characterization of liver masses but may not be an option in the substantial subgroup of patients with CHD with pacemakers or defibrillators and may be limited in those with coils, vascular plugs, or other metallic devices. 132 Newer contrast-enhanced ultrasonography techniques recently have been approved for use in the United States and may increase the diagnostic accuracy of ultrasound for characterizing focal liver lesions.…”

Section: Managementmentioning

confidence: 99%

Diagnosis and Management of Noncardiac Complications in Adults With Congenital Heart Disease: A Scientific Statement From the American Heart Association

Lui¹,

Saïdi²,

Bhatt³

et al. 2017

Circulation

176

136

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ABSTRACT:Life expectancy and quality of life for those born with congenital heart disease (CHD) have greatly improved over the past 3 decades. While representing a great advance for these patients, who have been able to move from childhood to successful adult lives in increasing numbers, this development has resulted in an epidemiological shift and a generation of patients who are at risk of developing chronic multisystem disease in adulthood. Noncardiac complications significantly contribute to the morbidity and mortality of adults with CHD. Reduced survival has been documented in patients with CHD with renal dysfunction, restrictive lung disease, anemia, and cirrhosis. Furthermore, as this population ages, atherosclerotic cardiovascular disease and its risk factors are becoming increasingly prevalent. Disorders of psychosocial and cognitive development are key factors affecting the quality of life of these individuals. It is incumbent on physicians who care for patients with CHD to be mindful of the effects that disease of organs other than the heart may have on the well-being of adults with CHD. Further research is needed to understand how these noncardiac complications may affect the long-term outcome in these patients and what modifiable factors can be targeted for preventive intervention.

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Hepatocellular Carcinoma with Right Atrial Extension in a Young Patient with Congenitally Unguarded Tricuspid Orifice

Cited by 7 publications

References 6 publications

Liver disease secondary to congenital heart disease in children

Liver disease secondary to congenital heart disease in children

Liver Disease in the Patient with Fontan Circulation

Diagnosis and Management of Noncardiac Complications in Adults With Congenital Heart Disease: A Scientific Statement From the American Heart Association

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