Abdominal imaging findings in adult patients with Fontan circulation

Kim, Tae-Hyung; Yang, Hyun Kyoung; Jang, Hyun‐Jung; Yoo, Shi‐Joon; Khalili, Korosh; Kim, Tae Kyoung

doi:10.1007/s13244-018-0609-2

Cited by 34 publications

(23 citation statements)

References 89 publications

(111 reference statements)

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“…Irregular parenchymal fatty infiltration of a perivascular distribution can be seen on ultrasound. Contrast-enhanced ultrasound (CEUS) shows heterogeneous and decreased enhancement of the liver in the portal venous phase 25 . Hepatic vein waveforms assessed by doppler ultrasound change in accordance with liver fibrosis progression 26 .…”

Section: Introductionmentioning

confidence: 99%

Fontan-associated liver disease and hepatocellular carcinoma in adults

Kogiso

Tokushige

2020

Sci Rep

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The Fontan operation creates a unique circulation, and is a palliative therapy for patients with single-ventricle congenital heart disease. Increased venous pressure and decreased cardiac output and hepatic venous drainage result in sinusoidal dilatation around the central veins. This causes congestion and hypoxia in the liver, leading to Fontan-associated liver disease (FALD). Non-invasive and invasive markers enable diagnosis and evaluation of the fibrosis status in chronic liver disease; however, these markers have not been validated in FALD. Additionally, regenerative nodules such as focal nodular hyperplasia (FNH) are frequently found. The severity of fibrosis correlates with the duration of the Fontan procedure and the central venous pressure. Cirrhosis is a risk factor for hepatocellular carcinoma (HCC), the annual risk of which is 1.5–5.0%. HCC is frequently difficult to diagnose and treat because of cardiac complications, coagulopathy, and congenital abnormalities. The mortality rate of FALD with liver cirrhosis and/or FALD-HCC was increased to ~ 29.4% (5/17 cases) in a nationwide survey. Although there is no consensus on the surveillance of patients with FALD, serial monitoring of the alpha fetoprotein level and imaging at 6-month intervals is required in patients with cirrhosis.

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Section: Introductionmentioning

confidence: 99%

Fontan-associated liver disease and hepatocellular carcinoma in adults

Kogiso

Tokushige

2020

Sci Rep

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“…The coexistence of CHD and P-PGL has already been reported in previous studies and a causal link between the two conditions has been postulated [ 5 , 6 ].…”

Section: Introductionmentioning

confidence: 82%

“…We present a case of pheochromocytoma in a young patient affected with congenital tricuspid atresia treated by Fontan surgery. Several cases of cooccurrence of pheochromocytoma and CCHD have been described in the literature [ 5 ]. It has been hypothesized that chronic hypoxia plays a fundamental role in these cases.…”

Section: Discussionmentioning

confidence: 99%

Pheochromocytoma in Congenital Cyanotic Heart Disease

Aresta

Butera

Tufano

et al. 2018

Case Reports in Endocrinology

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Studies on genome-wide transcription patterns have shown that many genetic alterations implicated in pheochromocytoma-paraganglioma (P-PGL) syndromes cluster in a common cellular pathway leading to aberrant activation of molecular response to hypoxia in normoxic conditions (the pseudohypoxia hypothesis). Several cases of P-PGL have been reported in patients with cyanotic congenital heart disease (CCHD). Patients affected with CCHD have an increased likelihood of P-PGL compared to those affected with noncyanotic congenital heart disease. One widely supported hypothesis is that chronic hypoxia represents the determining factor supporting this increased risk. We report the case of a 23-year-old woman affected with congenital tricuspid atresia surgically by the Fontan procedure. The patient was admitted to hospital with hypertensive crisis and dyspnea. Chest computed tomography revealed, incidentally, a 6-cm mass in the left adrenal lodge. Increased levels of noradrenaline (NA) and its metabolites were detected (plasma NA 5003.7 pg/ml, n.v.<480; urinary NA 1059.5 µg/24 h, n.v.<85.5; urinary metanephrine 489 µg/24 h, n.v.<320). The patient did not report any additional symptom related to catecholamine excess. The left adrenal tumor showed abnormal accumulation when 131I-metaiodobenzylguanidine scintigraphy was performed. A 18F-fluorodeoxyglucose positron emission tomography showed no significant metabolic activity in the left adrenal gland but intense uptake in the supra- and subdiaphragmatic brown adipose tissue, probably due to noradrenergic-stimulated glucose uptake. The patient underwent left open adrenalectomy after preconditioning with α- and β-blockers and histopathological examination confirmed the diagnosis of pheochromocytoma (Ki-67<5%). Screening for germline mutations did not show any genes mutation (investigated mutations: RET, TMEM127, MAX, SDHD, SDHC, SDHB, SDHAF2, SDHA, and VHL). Clinicians should consider P-PGL when an unexplained clinical deterioration occurs in CCHD patients, even in the absence of typical paroxysmal symptoms.

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“…However, the diagnosis of liver disease and HCC is challenging, as the clinical presentation is extremely heterogeneous in this patient cohort. Abnormal hepatic blood flow may influence the typical arterial enhancing pattern of HCC on CT imaging [8] , necessitating biopsy to confirm histologically the diagnosis. HCC development was a relatively rare event (1.3%), being associated with moderately increased AFP [5] .…”

Section: Discussionmentioning

confidence: 99%

Transarterial chemoembolization for hepatocellular carcinoma in Fontan surgery patient

Orlacchio¹,

Gasparrini²,

Lenci

et al. 2020

Radiology Case Reports

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We describe the case of a 41‐year‐old woman who developed a liver neoplasm due to previous Fontan surgery for a single ventricle anomaly and pacemaker implantation. She was admitted to our hospital for moderate ascites and she was affected by hepatocellular carcinoma treated by trans-arterial chemoembolization (TACE). Computed tomography showed features of chronic liver disease and 4 cm hepatic nodules with arterial enhancement. Laboratory analyses documented preserved liver function and increased levels of alpha-fetoprotein. TACE was performed obtaining complete necrosis at 4 weeks of follow up and significant reduction of alpha-fetoprotein after 2 months. The patient is currently in follow-up, being evaluated for further treatments and/or combined liver/heart transplantation. TACE is a therapeutic option for the treatment of patients with unresectable HCC and with severe heart disease, like those submitted to FS.

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Abdominal imaging findings in adult patients with Fontan circulation

Cited by 34 publications

References 89 publications

Fontan-associated liver disease and hepatocellular carcinoma in adults

Fontan-associated liver disease and hepatocellular carcinoma in adults

Pheochromocytoma in Congenital Cyanotic Heart Disease

Transarterial chemoembolization for hepatocellular carcinoma in Fontan surgery patient

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