Caring for the orphan's orphan: treatment of patients with portopulmonary hypertension

Kawut, Steven M.

doi:10.1183/09031936.00113407

Cited by 4 publications

(2 citation statements)

References 13 publications

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“…However, it is not established whether patients with liver disease who are not transplant candidates warrant screening for this condition. Given the relatively low specificity of echocardiography (that is, high false‐positive rate) and the lack of well‐studied therapies for PPHTN with documented efficacy,23–25 it is likely premature to recommend screening for patients with liver disease who are not transplant candidates, even if they fall into the “high‐risk” subgroups in our study. On the other hand, clinicians should have a low threshold to obtain echocardiography in patients with portal hypertension with symptoms suggestive of PPHTN.…”

Section: Discussionmentioning

confidence: 99%

Clinical risk factors for portopulmonary hypertension†

Kawut¹,

Krowka²,

Trotter³

et al. 2008

Hepatology

250

166

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Portopulmonary hypertension affects up to 6% of patients with advanced liver disease, but the predictors and biologic mechanism for the development of this complication are unknown. We sought to determine the clinical risk factors for portopulmonary hypertension in patients with advanced liver disease. We performed a multicenter case-control study nested within a prospective cohort of patients with portal hypertension recruited from tertiary care centers. Cases had a mean pulmonary artery pressure > 25 mm Hg, pulmonary vascular resistance > 240 dynes ⅐ second ⅐ cm ؊5 , and pulmonary capillary wedge pressure < 15 mm Hg. Controls had a right ventricular systolic pressure < 40 mm Hg (if estimable) and normal right-sided cardiac morphology by transthoracic echocardiography. The study sample included 34 cases and 141 controls. Female sex was associated with a higher risk of portopulmonary hypertension than male sex (adjusted odds ratio ‫؍‬ 2.90, 95% confidence interval 1.20-7.01, P ‫؍‬ 0.018). Autoimmune hepatitis was associated with an increased risk (adjusted odds ratio ‫؍‬ 4.02, 95% confidence interval 1.14-14.23, P ‫؍‬ 0.031), and hepatitis C infection was associated with a decreased risk (adjusted odds ratio ‫؍‬ 0.24, 95% confidence interval 0.09-0.65, P ‫؍‬ 0.005) of portopulmonary hypertension. The severity of liver disease was not related to the risk of portopulmonary hypertension. Conclusion: Female sex and autoimmune hepatitis were associated with an increased risk of portopulmonary hypertension, whereas hepatitis C infection was associated with a decreased risk in patients with advanced liver disease. Hormonal and immunologic factors may therefore be integral to the development of portopulmonary hypertension. (HEPATOLOGY 2008;48:196-203.) See Editorial on Page 13 P ulmonary arterial hypertension (PAH) is a progressive disease which is characterized by elevated pulmonary vascular resistance, right heart failure, exercise limitation, and an increased risk of death. Histopathologic examination reveals intimal proliferation, medial hypertrophy, and adventitial fibrosis in the small muscular pulmonary arteries. Plexiform lesions and in situ thrombosis are also commonly seen. Most commonly idiopathic, PAH may also be associated with portal hypertension, termed portopulmonary hypertension (PPHTN

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Section: Discussionmentioning

confidence: 99%

Clinical risk factors for portopulmonary hypertension†

Kawut¹,

Krowka²,

Trotter³

et al. 2008

Hepatology

250

166

View full text Add to dashboard Cite

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“…Results of several studies on PoPH-specific therapies including intravenous administration of epoprostenol, bosentan, and sildenafil in patients with PoPH have been published [5][6][7]. Although there is no consensus on the treatment of PoPH [8], sildenafil is thought to have an advantage for the oral treatment of PoPH because there is a low risk of hepatotoxicity and the treatment is noninvasive. An acute pulmonary vasoreactivity test of sildenafil in PoPH has not been reported, although it has been reported in idiopathic pulmonary arterial hypertension [9].…”

Section: Discussionmentioning

confidence: 97%

Usefulness of acute pulmonary vasoreactivity test of sildenafil in treatment of portopulmonary hypertension. A case report

Akagi

Nakamura

Fuke

et al. 2011

Journal of Cardiology Cases

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KEYWORDSSildenafil; Portopulmonary hypertension; Acute pulmonary vasoreactivity test Summary A 50-year-old man diagnosed with liver cirrhosis type C was referred to our hospital because of right heart failure with pulmonary hypertension. Echocardiography revealed enlargement of the right atrium and ventricle with severe tricuspid regurgitation. The peak flow velocity of tricuspid regurgitation by continuous wave Doppler echocardiography was 452 cm/s. Right heart catheterization demonstrated severe pulmonary hypertension [pulmonary arterial pressure (PAP) systolic/diastolic/mean = 73/20/41 mmHg and pulmonary vascular resistance (PVR) = 509 dyn s cm -5 ] with portal hypertension. We diagnosed the patient as having portopulmonary hypertension (PoPH). Although we treated the patient with a prostacyclin analog, tricuspid regurgitation velocity was increased to 480 cm/s four years after the start of the therapy. To select drugs for the treatment of PoPH, we performed an acute vasoreactivity test of sildenafil during right heart catheterization. Since single administration of sildenafil (20 mg) decreased PAP (93/30/55-77/27/44 mmHg) and PVR (908-833 dyn s cm -5 ), we added sildenafil (20 mg, t.i.d.) to the prostacyclin analog. Tricuspid regurgitation velocity decreased to 403 cm/s one year after the addition of sildenafil. An acute vasoreactivity test of sildenafil during right heart catheterization was useful for the decision of the drug to be used in the treatment of PoPH.

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