Portopulmonary hypertension associated with congenital absence of the portal vein treated with bosentan

Internal Medicine

Volumn 48, Issue 8, 2009, Pages 597-600

  Hino, Tomoko ^a   Hayashida, Akihiro ^a   Okahashi, Noriko ^a   Wada, Nozomi ^a   Watanabe, Nozomi ^a   Obase, Kikuko ^a   Neishi, Yoji ^a   Kawamoto, Takahiro ^a   Okura, Hiroyuki ^a   Yoshida, Kiyoshi ^a  

^a KAWASAKI MEDICAL SCHOOL   (Japan)

Author keywords

Bosentan therapy; Congenital absence of the portal vein; Portopulmonary hypertension; Pulmonary hypertension

Indexed keywords

ALANINE AMINOTRANSFERASE; ALKALINE PHOSPHATASE; AMMONIA; ASPARTATE AMINOTRANSFERASE; BOSENTAN; GAMMA GLUTAMYLTRANSFERASE; ANTIHYPERTENSIVE AGENT; SULFONAMIDE;

AGENESIS; ALANINE AMINOTRANSFERASE BLOOD LEVEL; ALKALINE PHOSPHATASE BLOOD LEVEL; AMMONIA BLOOD LEVEL; ARTICLE; ASPARTATE AMINOTRANSFERASE BLOOD LEVEL; ASTHMA; CASE REPORT; CHILD; CHRONIC OBSTRUCTIVE LUNG DISEASE; CLINICAL FEATURE; COMPUTER ASSISTED TOMOGRAPHY; DISEASE ASSOCIATION; DISEASE SEVERITY; DRUG RESPONSE; DYSPNEA; ECHOCARDIOGRAPHY; ELECTROCARDIOGRAM; FOLLOW UP; GAMMA GLUTAMYL TRANSFERASE BLOOD LEVEL; HEART CATHETERIZATION; HEART RIGHT ATRIUM PRESSURE; HEART RIGHT VENTRICLE HYPERTROPHY; HEART RIGHT VENTRICLE PRESSURE; HEART VENTRICLE ENDDIASTOLIC PRESSURE; HEART VENTRICLE SEPTUM DEFECT; HUMAN; LIVER DYSFUNCTION; LUNG ARTERY PRESSURE; LUNG FUNCTION TEST; LUNG VASCULAR RESISTANCE; MALE; PORTAL VEIN AGENESIS; PORTOPULMONARY HYPERTENSION; PORTOSYSTEMIC ANASTOMOSIS; PULMONARY ARTERY; PULMONARY HYPERTENSION; PULMONARY VALVE INSUFFICIENCY; SCHOOL CHILD; THORAX RADIOGRAPHY; TRICUSPID VALVE REGURGITATION; CONGENITAL MALFORMATION; PORTAL HYPERTENSION; PORTAL VEIN; TREATMENT OUTCOME;

ANTIHYPERTENSIVE AGENTS; CHILD; HUMANS; HYPERTENSION, PORTAL; HYPERTENSION, PULMONARY; MALE; PORTAL VEIN; SULFONAMIDES; TREATMENT OUTCOME;

EID: 67649739164     PISSN: 09182918     EISSN: 13497235     Source Type: Journal    
DOI: 10.2169/internalmedicine.48.1715     Document Type: Article

References (16)

1. Hoeper MM, Halank M, Marx C, et al. Bosentan therapy for portopulmonary hypertension. Eur Respir J 25: 502-508, 2005.
2. Ohno T, Muneuchi J, Ihara K, et al. Pulmonary hypertension in patients with congenital portosystemic venous shunt: A previously unrecognized association. Pediatrics 121: e892-e899, 2008.
3. Benjaminov FS, Prentice M, Sniderman KW, Siu S, Liu P, Wong F. Portopulmonary hypertension in decompensated cirrhosis with refractory ascites. Gut 52: 1355-1362, 2003.
4. Hoeper MM, Krowka MJ, Strassburg CP. Portopulmonary hypertension and hepatopulmonary syndrome. The Lancet 363: 1461-1468, 2004.
5. Budhiraja R, Hassoun PM. Portopulmonary hypertension: a tale of two circulations. Chest 123 (2): 562-576, 2003.
6. Staehler G, Hunnius P. Successful treatment of portopulmonary hypertension with bosentan: case report. Eur J Clin Invest 36: 62-66, 2006.
7. Grander W, Eller P, Fuschelberger R, Tilg H. Bosentan treatment of portopulmonary hypertension related to liver cirrhosis owing to hepatitis C. Eur J Clin Invest 36: 67-70, 2006.
8. Kuntzen C, Gruelberg V, Gerbes AL. Use of a mixed endothelin receptor antagonist in portopulmonary hypertension: A safe and effective therapy? Gastroenterology 128: 164-168, 2005.
9. Grander W, Eller P, Fuschelberger R, Tilg H. Bosentan treatment of portopulmonary hypertension related to liver cirrhosis owing to hepatitis C. Eur J Clin Invest 36 (suppl.3): 67-70, 2006.
10. Hoeper MM, Halank M, Marx C, et al. Bosentan therapy for portopulmonary hypertension. Eur Respir J 25: 502-508, 2005.
11. Robalino BD, Moodie DS. Association between primary pulmonary hypertension and portal hypertension: analysis of its pathophysiology and clinical, laboratory and hemodynamic manifestations. J Am Coll Cardiol 17: 492-498, 1991.
12. Herré P, Lebrec D, Brenot F, et al. Pulmonary vascular disorders in portal hypertension. Eur Respir J 11: 1153-1166, 1998.
13. Marios D, van Heerden JA, Carpender HA, Sheedy PF. 2nd. Congenital absence of the portal vein. Mayo Clin Proc 54: 55-59, 1979.
14. Komatsu S, Nagino M, Hayakawa N, Yamamoto H, Nimura Y. Congenital absence of portal vein system associated with a large inferior mesenteric-caval shunt: a case report. Hepatogastroenterology 42: 286-290, 1995.
15. Olling S, Olsson R. Congenital absence of portal venous system in a 50-year-old woman. Acta Med Stand 196 (4): 343-345, 1974.
16. Morse SS, Taylor KJ, Strauss EB, Ramirez E, Seashore JH. Congenital absence of the portal vein in oculoauriculovertebral dysplasia (Goldenhar syndrome). Pediatr Radiol 16: 437-439, 1986.