Liver Disease in the Patient with Fontan Circulation

Wu, Fred; Ukomadu, Chinweike; Odze, Robert D.; Valente, Anne Marie; Mayer, John E.; Earing, Michael G.

doi:10.1111/j.1747-0803.2011.00504.x

Cited by 129 publications

(129 citation statements)

References 72 publications

(198 reference statements)

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“…In the latter, decreased cardiac index together with increased right atrial pressure (termed central venous hypertension), which results from direct attachment of the right atrium to the pulmonary artery, leads to liver damage and fibrosis [51]. Of note, one-third of those patients show hepatomegaly and abnormal liver function tests [53]. After 11.5 years, more than one-quarter present with liver cirrhosis [54].…”

Section: Role Of Hepatic Sinusoids In the Pathogenesis Of Congestive mentioning

confidence: 99%

Hepatic sinusoids in liver injury, inflammation, and fibrosis: new pathophysiological insights

2016

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Section: Role Of Hepatic Sinusoids In the Pathogenesis Of Congestive mentioning

confidence: 99%

Hepatic sinusoids in liver injury, inflammation, and fibrosis: new pathophysiological insights

2016

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“…In patients with complex heart anomalies, a need for improving quality of life in the ones who survive after operation has become prominent (16,17). All patients are exposed to psychological stress caused by having chronic using pediatric functional independence measurement.…”

Section: Discussionmentioning

confidence: 99%

Bebeklik döneminde siyanotik kalp hastalığı nedeniyle açık kalp cerrahisi uygulanmış ergenlerde kendilik algısı ve yaşam niteliği

Yıldız¹,

Zahmacioglu²,

Koca³

et al. 2011

tpa

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Aim:To assess the quality-of-life of the teenage patients who had undergone open heart surgery because of cyanotic heart disease during their infancy together with their parents and to compare their self-perception to that of the physically healthy control group. Material and Method:This study includes 53 patients who had undergone operation for cyanotic congenital heart disease in İstanbul University, Institute of Cardiology between 1989 and 1994. The relatives and the patients were informed with a consent form approved by İstanbul University, Cerrahpaşa Medical Faculty, Clinical Studies, Ethics Commitee (Decree no. D-005, Date: 11-10-2009). Group 1 was the healthy control group (15 patients, median age: 19 years 8 months), Group 2 included patients with single ventricle who had undergone Fontan procedure (20 patients, median age: 19 years 6 months; 10 of them had tricuspid atresia, 5 of them had pulmonary atresia with intact ventricular septum, 3 of them had double inlet left ventricle, 1 of them had double outlet right ventricle, 1 of them had ventricular septal defect and pulmonary atresia), group 3 included patients who had undergone operation for congenital cyanotic heart disease (33 patients, median age: 19 years 2 months; 18 of them had transposition of the great arteries, 10 of them had tetralogy of Fallot, 2 of them had complete atrioventricular canal defect and pulmonary stenosis, 2 of them had double outlet right ventricle, 1 of them had pentalogy of Fallot). In this study, the quality of life and self perception inventories were used. The quality of life inventory was completed by patients, healthy adolescents and their parents seperately. The self perception inventory was completed only by the patients and healthy adolescents. The comparison of age and self perception scores between the groups was carried out with unidirectional analysis of variance (ANOVA) and the multicomparisons with LSD (Least Significant Difference). Since the quality of life variable did not have a normal distribution, the comparions were carried out with Kruskal-Wallis nonparametric ANOVA test and the multicomparisons were carried out with Dunn's test. Results:There was not a significant difference between the groups in terms of age and sex. In the quality of life scale (QOLS), for the replies to related questions, a significant difference was determined between the groups. The replies of group 2 and 3 about quality of life of both the children and the parents were far more negative when compared to those of the control group. When the groups were compared via multiple comparison tests, there was no significant difference between group 2 and 3. The replies of the parents regarding their children's quality of life were statistically more negative than those of their children (p<0,001). Within the self perception scale, there were significant differences in terms of social acceptance, behaviours in relationships and general self perception (p=0.03, p=0.03 and p=0.01; repectively) between group 1 and 3. The most significant dif...

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“…It has been reported in chronic congestive hepatopathy resulting from Fontan palliation, repaired TOF, and TGA following Mustard repair [50][51][52]. HCC has not been systematically studied in ACHD patients, and current ACHD guidelines do not address screening.…”

Section: Cirrhosismentioning

confidence: 99%

“…These patients are predisposed to hepatic injury by pre-Fontan hypoxemia as well as chronically increased CVP and (often) depressed cardiac output following Fontan palliation [52,57]. The presentation of hepatic dysfunction following Fontan is similar to other congestive hepatopathies, but signs and symptoms may be muted due to chronicity [52]. Additionally, for unclear reasons, the hepatic venous pressure gradient is usually normal [57].…”

Section: T R E N D S I N C a R D I O V A S C U L A R M E D I C I N E mentioning

confidence: 99%

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Extra-cardiac manifestations of adult congenital heart disease

Gaeta

Ward

Krasuski

2016

Trends in Cardiovascular Medicine

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a b s t r a c tAdvancement in correction or palliation of congenital cardiac lesions has greatly improved the lifespan of congenital heart disease patients, resulting in a rapidly growing adult congenital heart disease (ACHD) population. As this group has increased in number and age, emerging science has highlighted the systemic nature of ACHD. Providers caring for these patients are tasked with long-term management of multiple neurologic, pulmonary, hepatic, renal, and endocrine manifestations that arise as syndromic associations with congenital heart defects or as sequelae of primary structural or hemodynamic abnormalities. In this review, we outline the current understanding and recent research into these extra-cardiac manifestations.Key words: Cardiovascular disease, Adult congenital heart disease, Pulmonary hypertension. Advances in the diagnosis and management of congenital heart disease (CHD) over the past several decades have greatly improved longevity and quality of life in the CHD population. Palliative or corrective interventions are now available for nearly every type of congenital cardiac lesion, and approximately 90% of affected children now survive to adulthood [1]. Adults with CHD (ACHD) already outnumber pediatric CHD patients, and this population is expected to grow by 5% per year [1,2]. Strikingly, the median age of severe CHD patients has increased from 11 years in 1985 to 25 years in 2010 [3]. Continued improvement in CHD interventions means that new (and more complex) populations will likely reach adulthood in the coming years.Practitioners who take care of adults with CHD are often charged with the delivery or coordination of comprehensive care for these complex patients, including the management of manifestations and complications that arise later in life. ACHD has many extra-cardiac effects, both as syndromic associations with congenital heart defects, or more commonly, as sequelae of primary structural or hemodynamic abnormalities. The role of genetic predilection remains undefined for nearly all extra-cardiac complications, though their incidence has clearly risen over the past decades. Indeed, more than a third of hospital admissions for ACHD patients are now for noncardiovascular reasons [4]. Optimal care of ACHD patients, therefore, mandates a thorough understanding of its multiorgan system manifestations. Herein, we will review current understanding of the most common manifestations of ACHD organized by the affected organ systems. Neurologic/neuropsychiatric Cerebrovascular complicationsA recent study quantified the strikingly high rates of stroke in ACHD [5]. Among nearly 30,000 ACHD patients, 8.9% of men http://dx

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Liver Disease in the Patient with Fontan Circulation

Cited by 129 publications

References 72 publications

Hepatic sinusoids in liver injury, inflammation, and fibrosis: new pathophysiological insights

Hepatic sinusoids in liver injury, inflammation, and fibrosis: new pathophysiological insights

Bebeklik döneminde siyanotik kalp hastalığı nedeniyle açık kalp cerrahisi uygulanmış ergenlerde kendilik algısı ve yaşam niteliği

Extra-cardiac manifestations of adult congenital heart disease

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