Portopulmonary hypertension.
It has been widely accepted that development of porto-pulmonary hypertension (POPH) is independent of the cause of portal hypertension. The degree of hepatic damage and liver function do not correlate with predisposition to POPH or its severity. However, portal hypertension has been confirmed as a prerequisite for developing pulmonary hypertension. Transthoracic echocardiography is the best screening test for the presence of POPH, but a diagnosis of POPH can be established only by right heart catheterization. Randomized controlled trials comparing the efficacy and safety of different pharmacologic strategies are lacking in patients with POPH. The general management includes diuretics and oxygen supplementation. Notably, moderate to severe POPH predisposes candidates for orthotopic liver transplantation to a higher risk of perioperative mortality. Vasomodulating pharmacologic agents are used in patients with moderate to severe POPH to decrease pulmonary arterial hypertension, thereby permitting liver transplantation to be performed safely. Epo-prostenol is the best-studied medication, and bosentan appears promising.
Nayak
Ravi P
RP
Division of Pulmonary, Critical Care and Sleep Medicine, 1402 South Grand Boulevard, St. Louis, MO 63104, USA. nayakrp@slu.edu
Li
Dechun
D
Matuschak
George M
GM
D000959:Antihypertensive Agents / Q000627:therapeutic use; D000077300:Bosentan; D006328:Cardiac Catheterization; D004452:Echocardiography; D011464:Epoprostenol / Q000031:analogs & derivatives / Q000627:therapeutic use; D006801:Humans; D006975:Hypertension, Portal / Q000175:diagnosis / Q000628:therapy; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000628:therapy; D016285:Iloprost / Q000627:therapeutic use; D016031:Liver Transplantation; D010879:Piperazines / Q000627:therapeutic use; D011379:Prognosis; D011687:Purines / Q000627:therapeutic use; D000068677:Sildenafil Citrate; D013449:Sulfonamides / Q000627:therapeutic use; D013450:Sulfones / Q000627:therapeutic use; D014665:Vasodilator Agents / Q000627:therapeutic use
D000959:Antihypertensive Agents; D010879:Piperazines; D011687:Purines; D013449:Sulfonamides; D013450:Sulfones; D014665:Vasodilator Agents; D000068677:Sildenafil Citrate; D011464:Epoprostenol; D016285:Iloprost; D000077300:Bosentan; C427248:treprostinil