Abstract
Portopulmonary hypertension (POPH) is a part of group 1 pulmonary hypertension (pulmonary hypertension associated with portal hypertension). Liver transplantation (LTx) may be curative, but is usually restricted to patients with mild-to-moderate POPH. The presence of severe POPH may be a contraindication to transplantation because of the elevated risk of peritransplantation and post-transplantation morbidity and mortality. This report describes a series of seven patients with onset of moderate (two patients) or severe (five patients) POPH before LTx, of whom six were treated with oral vasodilator therapy for POPH. Although previous studies recommend aggressive parenteral prostacyclin therapy (epoprostenol), we describe the opportunity to treat cases of severe POPH with an oral phosphodiesterase type 5 inhibitor (sildenafil) and/or an endothelin receptor antagonist (bosentan/ambrisentan) as a bridge to successful LTx in selected patients.
MeSH terms
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Administration, Oral
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Adult
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Antihypertensive Agents / administration & dosage
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Antihypertensive Agents / therapeutic use*
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Bosentan
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Combined Modality Therapy
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Exercise Test / methods
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Female
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Humans
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Hypertension, Portal / complications
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Hypertension, Portal / drug therapy*
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Hypertension, Portal / surgery
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Hypertension, Pulmonary / complications
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Hypertension, Pulmonary / drug therapy*
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Hypertension, Pulmonary / surgery
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Liver Transplantation*
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Male
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Middle Aged
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Phenylpropionates / administration & dosage
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Phenylpropionates / therapeutic use
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Phosphodiesterase 5 Inhibitors / administration & dosage
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Phosphodiesterase 5 Inhibitors / therapeutic use
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Pyridazines / administration & dosage
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Pyridazines / therapeutic use
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Sulfonamides / administration & dosage
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Sulfonamides / therapeutic use
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Vasodilator Agents / administration & dosage
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Vasodilator Agents / therapeutic use*
Substances
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Antihypertensive Agents
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Phenylpropionates
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Phosphodiesterase 5 Inhibitors
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Pyridazines
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Sulfonamides
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Vasodilator Agents
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ambrisentan
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Bosentan