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Management of portopulmonary hypertension:New perspectives

World Journal of Gastroenterology 2013年第45期19卷 8252-8257页

作者： Luigi Mancuso Francesca Scordato Michela Pieri Eliana Valerio Andrea mancuso Department of Cardiologia Ospedale Cervello90100 Palermo Italy Cardiologia Clinica Candela 90100 Palermo Italy Epatologia e Gastroenterologia Ospedale Niguarda Cà Granda Milano 20162 Milano Italy Medicina Interna 1 ARNAS Civico 90100 Palermo Italy

Portopulmonary hypertension(PPHTN)is a known complication of cirrhosis.Moderate-to-severe PPHTN implies an extremely poor prognosis.It occurs in 5%-10%of patients referred for liver transplantation(LT),and probably with an higher incidence in patients with large portosystemic shunts.Patients with moderate-tosevere pulmonary hypertension have been previously excluded from LT because of the extremely high surgical risk and since the post-transplant outcome reported was poor.Recently,new perspectives in the management of patients with portopulmonary hypertension are emerging.In fact,some pulmonary vasoactive drugs have become routine in the treatment of patients with idiopathic pulmonary hypertension.These drugs,particularly epoprostenol,have been recently introduced in the treatment of patients with PPHTN,and have been shown to be effective in reducing pulmonary artery pressure as well as pulmonary vascular resistances.Furthermore,recent studies seem to demonstrate that treatment with pulmonary vasoactive drugs could allow liver transplantation with acceptable surgical risks and excellent survival.Although there are not large series nor prospective studies addressing this topic,the clinical scenario of patients with PPHTN seems to be positively changing.

关键词： Portopulmonary hypertension Cirrhosis Liver transplantation Management Epoprostenol

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为 Budd-Chiari 症候群的 caval 自体移植片由于劣等的静脉 cava 阻塞

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World Journal of Hepatology 2013年第5期5卷 292-295页

作者： Andrea mancuso luigi Martinelli Luciano De Carlis Antonio Gaetano Rampoldi Giovanni Magenta Aldo Cannata Luca Saverio Belli Epatologia e Gastroenterologia Ospedale Niguarda Cà GrandaPiazza Ospedale Maggiore 320162 MilanoItaly Cardiochirurgia Ospedale Niguarda Cà GrandaPiazza Ospedale Maggiore 320162 MilanoItaly Chirurgia Generale e dei Trapianti Ospedale Niguarda Cà GrandaPiazza Ospedale Maggiore 320162 MilanoItaly Radiologia Interventistica Ospedale Niguarda Cà GrandaPiazza Ospedale Maggiore 320162 MilanoItaly Cardiologia 3 Ospedale Niguarda Cà GrandaPiazza Ospedale Maggiore 320162 MilanoItaly

Transjugular intrahepatic portosystemic shunt (TIPS) is the standard treatment of Budd-Chiari syndrome (BCS) non responsive to medical therapy. However, patients with inferior vena cava (IVC) obstruction proximal to the atrium do not benefit from TIPS and a surgical approach is mandatory. We report the case of BCS due to intrapericardial IVC obstruction. We describe a novel surgical approach using a fresh caval homograft. An attempt to balloon dilatation of the IVC obstruction was complicated by right atrial disruption with tamponade and ventricular fibrillation. Lately, the patient successfully underwent a reconstruction of the cavo-atrial continuity by the interposition of a fresh caval homograft, a novel surgical approach never described before for BCS. Further follow-up revealed progressive reduction and resolution of ascites, and overall clinical improvement. IVC obstruction near to the atrium can be surgically approached with a new technique consisting in inferior vena cava resection and replacement with a caval homograft.

关键词： Budd-Chiari syndrome Inferior vena cava Occlusion Surgery Liver transplantation

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