Hepatogenic Pulmonary Angiodysplasia Theated with Coil-Spring Embolization

Felt, Richard W.; Kozak, B.; Rösch, Josef; Duell, Barton P.; Barker, Alan F.

doi:10.1378/chest.91.6.920

Cited by 25 publications

(6 citation statements)

References 10 publications

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“…23 A single report by Felt et al 24 showed partial success of vascular embolization of the diffuse HPS type I lesion. More recently, Poterucha et al 25 reported successful embolization of the focal pulmonary arteriovenous malformations characteristic of type II HPS lesions in a patient with persistent hypoxemia after liver transplantation.…”

Section: Discussionmentioning

confidence: 99%

Retrospective analysis of the results of liver transplantation for adults with severe hepatopulmonary syndrome

Collisson

Nourmand

Fraiman

et al. 2002

Liver Transplantation

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The hepatopulmonary syndrome (HPS), consisting of elevated alveolar-arterial oxygen gradient and intrapulmonary vascular abnormalities in the presence of advanced liver disease, is associated with high mortality. Liver transplantation (LT) has been used for the treatment of HPS; however, the success of LT for the treatment of HPS is not uniformly documented. We reviewed our experience over a 5-year period and identified eight adult patients with incapacitating respiratory symptoms compatible with HPS. Inclusion criteria included hypoxemia, normal lung volumes, reduced oxygen diffusing capacity (D L CO), and the presence of intrapulmonary shunting. Underlying liver disease was caused by hepatitis C (2 patients), primary biliary cirrhosis (1 patient), cryptogenic cirrhosis (1 patient), alcohol (2 patients), and hepatitis C with alcohol P atients with chronic advanced liver disease frequently show abnormalities in numerous organs. For example, renal insufficiency (hepatorenal syndrome) and peripheral arteriovenous shunting are widely recognized sequelae of cirrhosis. Less often recognized are the defects in pulmonary gas exchange observed with liver disease. A hepatopulmonary syndrome (HPS) has been described and encompasses a specific group of patients with severe liver disease and without cardiorespiratory disease who develop impaired gas exchange because of intrapulmonary vasodilatation (IPVD).In the past, hypoxemia was considered an absolute contraindication to liver transplantation (LT) 1 because of the operative and perioperative complications that arise in patients with gas exchange abnormalities. As the recognition of and experience with HPS has increased, concepts regarding indications LT for transplantation have come full circle, and it has been suggested that, in the face of otherwise stable liver disease, symptomatic HPS in and of itself may constitute an indication for LT. The role for transplantation in HPS is supported by the observation that the metabolic defects responsible for the pulmonary manifestations of HPS clearly are hepatic in origin, progressive, and, at present, refractory to medical management. However, the success of LT for the treatment of HPS is not uniformly documented.In the current study, we reviewed our single-center experience of eight patients with diagnosed HPS evaluated for LT, and report their long-term outcome and complications. Experimental Procedures Preoperative EvaluationAll patients were referred to UCLA for liver transplantation (LT) routinely undergo pulmonary function

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Section: Discussionmentioning

confidence: 99%

Retrospective analysis of the results of liver transplantation for adults with severe hepatopulmonary syndrome

Collisson

Nourmand

Fraiman

et al. 2002

Liver Transplantation

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“…Embolotherapy of diffuse IPVDs has been shown to improve oxygenation in HPS, likely also through a mechanism of pulmonary blood flow redistribution 38, 39, 40, 41. This is further supported by a large series reporting similar improvements in patients with the diffuse form of Hereditary Hemorrhagic Telangiectasia, in which patients have diffuse, basilar‐predominant IPVDs which are morphologically similar to those seen in HPS 42.…”

Section: Rationale and Considerations For Included Therapiesmentioning

confidence: 76%

Proposed Management Algorithm for Severe Hypoxemia After Liver Transplantation in the Hepatopulmonary Syndrome

Nayyar

Man

Granton

et al. 2015

American Journal of Transplantation

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The hepatopulmonary syndrome (HPS) is defined as the triad of liver disease, intrapulmonary vascular dilatation, and abnormal gas exchange, and is found in 10–32% of patients with liver disease. Liver transplantation is the only known cure for HPS, but patients can develop severe posttransplant hypoxemia, defined as a need for 100% inspired oxygen to maintain a saturation of ≥85%. This complication is seen in 6–21% of patients and carries a 45% mortality. Its management requires the application of specific strategies targeting the underlying physiologic abnormalities in HPS, but awareness of these strategies and knowledge on their optimal use is limited. We reviewed existing literature to identify strategies that can be used for this complication, and developed a clinical management algorithm based on best evidence and expert opinion. Evidence was limited to case reports and case series, and we determined which treatments to include in the algorithm and their recommended sequence based on their relative likelihood of success, invasiveness, and risk. Recommended therapies include: Trendelenburg positioning, inhaled epoprostenol or nitric oxide, methylene blue, embolization of abnormal pulmonary vessels, and extracorporeal life support. Availability and use of this pragmatic algorithm may improve management of this complication, and will benefit from prospective validation.

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“…Feeding arterial vessels were successfully occluded, and arterial oxygenation improved in a patient described by Felt et al 66 Over a 5-week period, three embolization procedures were conducted in an attempt to occlude multiple vascular abnormalities; the Pa02 substantially improved (initial Pa02 of 38 mm Hg improved to 53 mm Hg after final embolization). Feeding arterial vessels were successfully occluded, and arterial oxygenation improved in a patient described by Felt et al 66 Over a 5-week period, three embolization procedures were conducted in an attempt to occlude multiple vascular abnormalities; the Pa02 substantially improved (initial Pa02 of 38 mm Hg improved to 53 mm Hg after final embolization).…”

Section: Vascular Embolizationmentioning

confidence: 96%