Elevated Right Ventricular Pressures Are Not a Contraindication to Liver Transplantation in Alagille Syndrome

Ovaert, Caroline; Germeau, Catherine; Barrea, Cathy; Moniotte, St phane; Obbergh, Luc Van; Sokal, Étienne; Reding, Raymond; Otte, Jean-Bernard; Sluysmans, Thierry

doi:10.1097/00007890-200107270-00034

Cited by 30 publications

(28 citation statements)

References 11 publications

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“…AGS patients universally have varying degrees of pulmonary hypertension and elevated right ventricular systolic pressures. As a result, the most common complication during surgical transplantation has been refractory hypotension following caval clamping [24,25]. Based on transplant series, hemodynamic changes are expected to occur when the right ventricle to systemic pressure ratio exceeds 0.5 [6].…”

Section: Discussionmentioning

confidence: 99%

“…Based on transplant series, hemodynamic changes are expected to occur when the right ventricle to systemic pressure ratio exceeds 0.5 [6]. The addition of phosphodiesterase inhibitors has been described to treat transplant patients with portopulmonary hypertension [25]. The benefit of decreasing pulmonary hypertension in AGS patients undergoing major liver resection is unknown and phosphodiesterase inhibitors should not be routinely administered to AGS patients.…”

Section: Discussionmentioning

confidence: 99%

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Management of Large Hepatocellular Carcinoma in Adult Patients with Alagille Syndrome: A Case Report and Review of Literature

et al. 2010

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Management of Large Hepatocellular Carcinoma in Adult Patients with Alagille Syndrome: A Case Report and Review of Literature

et al. 2010

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Section: Cholestatic Diseasesmentioning

confidence: 75%

“…Liver replacement should be considered in cases of poor quality of life related to jaundice, pruritus not responding to medical therapy, multiple xanthomas, or, more rarely, because of complications of liver cirrhosis and portal hypertension. Contrary to early reports, high right ventricular pressures do not contraindicate liver transplantation [59]. Homozygous familial hypercholesterolemia is cured by liver transplantation [65].The time to proceed is dictated by the response to medical treatment (statins) but should precede the onset of cardiovascular complications.…”

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confidence: 99%

Paediatric liver transplantation-a review based on 20 years of personal experience

Otté¹

2004

Transplant Int

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The natural history of most liver diseases requiring liver replacement in children is well known, and the potential of this therapy has been ascertained regarding life expectancy, which currently exceeds 90% in the long term. The timing of liver transplantation must be anticipated, to reduce the physical, psychological and mental impact of chronic liver diseases. Several studies show evidence that the best long-term results with regard to patient and graft survival are obtained with grafts procured from relatively young donors. Since the shortage of post-mortem liver donors will most likely worsen, further development of live, relateddonor transplantation can be expected. The main progress to come will concern immunosuppression, taking advantage of the immunological privilege of the liver. Protocols are under development for induction of operational tolerance.Keywords Children . Liver transplantation * Indications . Survival . Medical complications * Immunosuppression HistoryThe history of paediatric liver transplantation cannot be dissociated from the name of Thomas E. Starzl, whose pioneering and ever-continuing efforts contributed more than anyone else's to what has become a routinely successful procedure [ 1, 21. I had the unique privilege to be his research fellow during my 3rd-year surgical residency (1965)(1966).His first attempt, performed in 1963 in a child with biliary atresia, failed [3]. Four of the eight children that received transplants in 1967 survived beyond 1 year; one is still alive, in perfect clinical condition, off immunosuppression for > 17 years (T.E. Starzl, personal communication). Cyclosporine, introduced in 1980, allowed the long-term survival of the majority of children that had received transplants [4]. After its transfer to the University of Pittsburgh in 1981, the liver programme of T.E. Starzl expanded impressively, with 808 children ( < 18 years) receiving a transplant between March 198 1 and April 1998 [5].In Europe, liver transplantation in a 10-month-old child with biliary atresia was attempted in Cambridge, UK, by Sir Roy Calne [6] in June 1968; the child died during surgery.In continental Europe, we performed, in March 1971, the first successful liver transplantation in an 18-monthold child with biliary atresia [7]. Our programme really started in 1984, on a multidisciplinary paediatric basis, after the transfer of our university hospital from Leuven to Brussels. The first four children are still alive, having reached 20 years of post-transplant survival. From 1984 to 2003 our group performed liver transplantation in 593 children ( < 15 years), including 120 patients who re-563 ceived a live, related-donor transplant. Most of those children were referred from abroad. Prof. Daniel Alagille [8] was the first to put his trust in our group. In 1985 we started a 3-year collaboration while awaiting the implementation of a liver programme in HBpitalBicgtre, Paris. We were honoured by the collaboration with this distinguished paediatric hepatologist, who was an internatio...

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“…Clinicians argue that depending on the degree of severity of cardiovascular disease and/or liver disease, these patients with ESLD needing LT are too sick and will not tolerate the required operation. Several reports have been published attesting to the safety of LT for patients with congenital heart disease, particularly, in Alagille syndrome (11)(12)(13)(14)(15). Early recognition of the cardiac abnormality and patient preparation are of paramount importance in all the reports.…”

Section: Discussionmentioning

confidence: 99%

Living Donor Liver Transplantation in Children With Congenital Heart Disease

Concejero

Chen²,

Liang³

et al. 2007

Transplantation

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Elevated Right Ventricular Pressures Are Not a Contraindication to Liver Transplantation in Alagille Syndrome

Abstract: Liver transplantation can be performed safely in children with Alagille syndrome, even in the presence of elevated right ventricular pressure.

Cited by 30 publications

References 11 publications

Management of Large Hepatocellular Carcinoma in Adult Patients with Alagille Syndrome: A Case Report and Review of Literature

Management of Large Hepatocellular Carcinoma in Adult Patients with Alagille Syndrome: A Case Report and Review of Literature

Paediatric liver transplantation-a review based on 20 years of personal experience

Living Donor Liver Transplantation in Children With Congenital Heart Disease

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