Tuberc Respir Dis.  2007 May;62(5):421-426.

A Case of Portopulmonary Hypertension Associated with Primary Biliary Cirrhosis

• Kim SJ
• Lee EJ
• Jung KH
• Kang EH
• Lee SY
• Lim HE
• Yim HJ
• Lee SY
• Kim JH
• Shin C
• Shim JJ
• In KH
• Kang KH
• Yoo SH

Abstract

Portopulmonary hypertension (PPHTN) is a clinically and pathophysiologically distinct complication of advanced liver disease. PPHTN is characterized by the development of pulmonary arterial hypertension in association with advanced hepatic disease-related portal hypertension. A characteristic feature of PPHTN is an obstruction to the pulmonary artery flow caused by vasoconstriction, the proliferation of the endothelium and smooth muscle components of the vascular wall, as well as in situ thrombosis. This disorder is commonly underdiagnosed but the clinical implications are significant because it has substantial effects on survival and requires special treatment. We report a case of portopulmonary hypertension in a 53-year-old woman with primary biliary cirrhosis who presented with exertional dyspnea.

Keyword

Portal hypertension; Primary biliary cirrhosis; Pulmonary hypertension

MeSH Terms

Dyspnea
Endothelium
Female
Humans
Hypertension*
Hypertension, Portal
Hypertension, Pulmonary
Liver Cirrhosis, Biliary*
Liver Diseases
Middle Aged
Muscle, Smooth
Pulmonary Artery
Thrombosis
Vasoconstriction

Figure

• 

  Figure 1 The simple chest radiography on admission (A) showed cardiomegaly, prominent pulmonary trunk, and pneumonic infiltration in right lower lung field, which improved two months later (B).

• 

  Figure 2 Chest computed tomography scan on admission showed the consolidative pneumonic infiltration in right lower lobe (A) and dilatation of pulmonary trunk without evidences of pulmonary thromboembolism (B).

Reference

1. Budhiraja R, Hassoun PM. Portopulmonary hypertension: a tale of two circulations. Chest. 2003. 123:562–576.

2. Moller S, Henriksen JH. Cardiopulmonary complications in chronic liver disease. World J Gastroenterol. 2006. 12:526–538.

3. Hoeper MM, Krowka MJ, Strassburg CP. Portopulmonary hypertension and hepatopulmonary syndrome. Lancet. 2004. 363:1461–1468.

4. Rodriguez-Roisin R, Krowka MJ, Herve P, Fallon MB. Pulmonary-Hepatic vascular Disorders (PHD). Eur Respir J. 2004. 24:861–880.

5. Krowka MJ. Hepatopulmonary syndrome and portopulmonary hypertension: implications for liver transplantation. Clin Chest Med. 2005. 26:587–597.

6. Hadengue A, Benhayoun MK, Lebrec D, Benhamou JP. Pulmonary hypertension complicating portal hypertension: prevalence and relation to splanchnic hemodynamics. Gastroenterology. 1991. 100:520–528.

7. Galie N, Torbicki A, Barst R, Dartevelle P, Haworth S, Higenbottam T, et al. Guidelines on diagnosis and treatment of pulmonary arterial hypertension. The Task Force on Diagnosis and Treatment of Pulmonary Arterial Hypertension of the European Society of Cardiology. Eur Heart J. 2004. 25:2243–2278.

8. Simonneau G, Galie N, Rubin LJ, Langleben D, Seeger W, Domenighetti G, et al. Clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2004. 43:5S–12S.

9. Laleman W, Landeghem L, Wilmer A, Fevery J, Nevens F. Portal hypertension: from pathophysiology to clinical practice. Liver Int. 2005. 25:1079–1090.

10. Espiritu JD. Pulmonary embolism in a patient with coagulopathy from end-stage liver disease. Chest. 2000. 117:924–925.

11. Herve P, Lebrec D, Brenot F, Simonneau G, Humbert M, Stbon O, et al. Pulmonary vascular disorders in portal hypertension. Eur Respir J. 1998. 11:1153–1166.

12. Leuschner U. Primary biliary cirrhosis--presentation and diagnosis. Clin Liver Dis. 2003. 7:741–758.

13. Poupon R, Chazouilleres O, Corpechot C, Chretien Y. Development of autoimmune hepatitis in patients with typical primary biliary cirrhosis. Hepatology. 2006. 44:85–90.

14. Choi HS, Lee SD. Medical therapy for pulmonary arterial hypertension. Tuberc Respir Dis. 2006. 60:142–150.

15. Abraldes JG, Angermayr B, Bosch J. The management of portal hypertension. Clin Liver Dis. 2005. 9:685–713.