Effect of PAH Specific Therapy on Pulmonary Hemodynamics and Six-Minute Walk Distance in Portopulmonary Hypertension: A Systematic Review and Meta-Analysis

Table 1

Underlying causes of portal hypertension in the included studies [10–21].

1st author and year		ALD	HBV or HCV	Cryptogenic	ALD + HCV	Miscellaneous
Krowka 1999	14	1	1	3	3	1 HCC + HCV, 1 sarcoidosis,; 2 PBC, 1 Budd Chiari, 1 AIH
Hoeper 2005	11	7	1	0	2	1 biliary atresia
Reichenberger 2006	14	7	3			3 PBC, 1 Budd Chiari
Sussman 2006	8	4	1	2	1
Fix 2007	35	7	9	3	10	1 HIV, 2 HCC, 2 PBC, 1 AIH
Hoeper 2007 (bosentan)	18	11	3	1	0	1 PVT, 2 AIH
Hoeper 2007 (iloprost)	13	6	2	0	0	1 biliary duct atresia, 4 AIH
Gough and White 2009	11	4	2	0	4	1 ALD + NASH
Hemnes 2009	13	1	8	0	0	1 PBC
Melgosa 2010	13	2	7	0	0	1 hemochromatosis, 2 PBC, 1 portal vein thrombosis
Halank 2011	13	8	1	3	0	1 PBC
Hollatz 2012	11	5	1	0	3	1 ALD + NASH + PBC, 1 biliary atresia
Savale 2013	34	20	4		3	6 PVT, 1 AIH
Total		83 (40%)	43 (20%)	12 (5%)	26 (12%)	41 (20%)

ALD: alcoholic liver disease; HBV: hepatitis B; HCV: hepatitis C; HCC: hepatocellular cancer; PBC: primary biliary cirrhosis; AIH: autoimmune hepatitis; HIV: human immunodeficiency virus; PVT: portal vein thrombosis; NASH: nonalcoholic steatohepatitis.