Transvenous Hepatic Biopsy in Stable Fontan Patients Undergoing Cardiac Catheterization

Evans, William N.; Winn, Brody J.; Yumiaco, Noel S.; Galindo, Alvaro; Rothman, Abraham; Acherman, Ruben J.; Restrepo, Humberto

doi:10.1007/s00246-014-0928-0

Cited by 41 publications

(34 citation statements)

References 29 publications

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“…Previous reports describe portal and sinusoidal hepatic fibrosis even in asymptomatic Fontan patients [2,11]. Recently, we reported an association between hepatic total-fibrosis scores and postoperative Fontan patients with and without a history of obstructed pulmonary blood flow at birth [3].…”

Section: Introductionmentioning

confidence: 70%

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Fontan Outcomes and Pulmonary Blood Flow at Birth

et al. 2015

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We previously noted, in a small group of post-Fontan patients, a possible association between hepatic fibrosis scores and the status of pulmonary blood flow at birth. To further explore this observation, we examined data from all Fontan patients seen in our center from July 2010 to March 2015. We identified 200 patients for analysis. Of the 200 patients, 56 underwent transvenous-hepatic biopsy. Of the 200 patients, 13 (6.5%) had protein-losing enteropathy. We divided both the 56 biopsy patients and the entire cohort of 200 patients into 4 groups: (1) unobstructed pulmonary blood flow at birth with functional left ventricles, (2) unobstructed pulmonary blood flow at birth with functional right ventricles, (3) obstructed pulmonary blood flow at birth with functional left ventricles, and (4) obstructed pulmonary blood flow at birth with functional right ventricles. Analysis of the 56 liver-biopsy patient groups showed median hepatic total-fibrosis scores for the 4 groups of 2 (0-6), 2 (0-8), 3 (2-6), and 4 (1-8), respectively, with statistical significance between groups 4 and 1 (p = 0.031). For the entire cohort of 200 patients, we analyzed the incidence of protein-losing enteropathy for each of the four groups and found protein-losing enteropathy percent occurrences of 0, 2.9, 8.8, and 16.1, respectively, with statistical significance between groups 4 and 2 (p = 0.031) and between groups 4 and 1 (p = 0.025). A history of obstructed pulmonary blood flow at birth, coupled with a functional right ventricle, may predict a poorer long-term Fontan outcome.

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Section: Introductionmentioning

confidence: 70%

“…If the two pathologists scored a specimen feature differently, then they conferred to arrive at a final score. For each patient, we added the portal-fibrosis score to the sinusoidal fibrosis score for a total-fibrosis score (0-8) [2].…”

Section: Methodsmentioning

confidence: 99%

Fontan Outcomes and Pulmonary Blood Flow at Birth

et al. 2015

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“…Two pathologists specializing in hepatology (SG and TCS), who were blinded to patient data, independently reviewed liver biopsy specimens. Portal fibrosis was graded using the Ishtak (stages 0–6) and Scheuer staging (stages 0–4) systems; and sinusoidal fibrosis was determined using staging described by Schwartz et al 12,13 Total fibrosis score was calculated by combining the grades of staging of all three staging techniques.…”

Section: Methodsmentioning

confidence: 99%

Magnetic Resonance Elastography

Poterucha

Johnson

Qureshi

et al. 2015

Mayo Clinic Proceedings

105

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Objective To evaluate the utility of magnetic resonance elastography (MRE) in screening patients for hepatic fibrosis, cirrhosis, and hepatocellular carcinoma after Fontan operation. Patients and Methods Hepatic MRE was performed in conjunction with cardiac magnetic resonance imaging in patients with prior Fontan operation between 2010-2014. Liver stiffness was calculated using previously reported techniques. Comparisons to available clinical, laboratory, imaging, and histopathologic data were made. Results Overall, 50 Fontan patients aged 25 (21-33) years were evaluated. The median interval between Fontan creation and MRE was 22 (16-26) years. Mean liver stiffness values were increased; 5.5±1.4 kilopascals relative to normal subjects. Liver stiffness directly correlated with liver biopsy total fibrosis score, time since operation; mean Fontan pressure; γ-glutamyl transferase (GGT); MELD score; creatinine; and pulmonary vascular resistance index (PVR). Liver stiffness was inversely correlated with cardiac index (CI). All 3 subjects with hepatic nodules exhibiting decreased contrast uptake on delayed post-contrast imaging and elevated nodule stiffness had biopsy-proven hepatocellular carcinoma. Conclusion The association between hepatic stiffness and fibrosis scores, MELD scores, and GGT, suggests that MRE may be useful to detect (and possibly quantify) hepatic cirrhosis in Fontan patients. Correlation between stiffness and post-Fontan time interval, mean Fontan pressure, PVR and reduced CI suggests a role for long term hepatic congestion in creating these hepatic abnormalities. MRE was useful in detecting abnormal nodules ultimately diagnosed as hepatocellular carcinoma. The relationship between stiffness with advanced fibrosis and hepatocellular carcinoma provides a strong argument for additional study and broader application of MRE in these patients.

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“…However, the risk factors for hepatic fibrosis remain unclear. We previously reported results of hepatic biopsies in stable Fontan surgery patients, and we found that fibrosis scores tended to moderately correlate with time from Fontan surgery [6]. Others have suggested thrombocytopenia as a marker for significant liver pathology [23].…”

Section: Introductionmentioning

confidence: 55%

“…Others have suggested thrombocytopenia as a marker for significant liver pathology [23]. Nevertheless, there are few reports that have analyzed hepatic biopsy findings in cohorts of either symptomatic or asymptomatic Fontan patients [6,14,16,23,24]. In lieu of liver biopsies, noninvasive magnetic resonance imaging and elastography, computed tomography, and ultrasound elastography all have enhanced the understanding of liver pathology and have identified elevated Fontan pressure, decreased cardiac index, elevated ventricular end-diastolic pressure, decreased ejection fraction, and elevated pulmonary arterial wedge pressure as risk factors for hepatic stiffness, which correlates with hepatic fibrosis from biopsy [3,9,17,28,29].…”

Section: Introductionmentioning

confidence: 99%

Fontan Hepatic Fibrosis and Pulmonary Vascular Development

Evans

Acherman

Winn³

et al. 2014

Pediatr Cardiol

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Fontan patients are at risk for hepatic fibrosis; however, risk factors are unclear. We performed a multivariate analysis in a small cohort of 14 patients (7-24 years old, mean 15) with Fontan circulation, undergoing cardiac catheterization and transvenous liver biopsies, all demonstrating fibrosis. We found by stepwise regression analysis that the history of pulmonary atresia was a predictor of higher total hepatic fibrosis scores than a history of unobstructed pulmonary blood flow (p = 0.002). Other variables including age, time from Fontan, hemodynamic measurements, and laboratory values were not predictive of total fibrosis scores at p values <0.05. Hepatic fibrosis scores between those born with pulmonary atresia versus unrestricted pulmonary blood flow may reflect differences in pulmonary circulatory physiology, resulting from differences in pulmonary vascular development.

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Transvenous Hepatic Biopsy in Stable Fontan Patients Undergoing Cardiac Catheterization

Cited by 41 publications

References 29 publications

Fontan Outcomes and Pulmonary Blood Flow at Birth

Fontan Outcomes and Pulmonary Blood Flow at Birth

Magnetic Resonance Elastography

Fontan Hepatic Fibrosis and Pulmonary Vascular Development

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