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

Satoshi Akagi, Kazufumi Nakamura, Soichiro Fuke, Kengo Fukushima Kusano, Hiroshi Itoh

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Abstract

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 452cm/s. Right heart catheterization demonstrated severe pulmonary hypertension [pulmonary arterial pressure (PAP) systolic/diastolic/mean=73/20/41mmHg and pulmonary vascular resistance (PVR)=509dynscm-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 480cm/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 (20mg) decreased PAP (93/30/55-77/27/44mmHg) and PVR (908-833dynscm-5), we added sildenafil (20mg, t.i.d.) to the prostacyclin analog. Tricuspid regurgitation velocity decreased to 403cm/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.

Original languageEnglish
JournalJournal of Cardiology Cases
Volume4
Issue number1
DOIs
Publication statusPublished - Aug 2011

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Tricuspid Valve Insufficiency
Hypertension
Lung
Cardiac Catheterization
Epoprostenol
Pulmonary Hypertension
Vascular Resistance
Arterial Pressure
Therapeutics
Doppler Echocardiography
Portal Hypertension
Heart Atria
Liver Cirrhosis
Pharmaceutical Preparations
Heart Ventricles
Echocardiography
Heart Failure
Sildenafil Citrate

Keywords

  • Acute pulmonary vasoreactivity test
  • Portopulmonary hypertension
  • Sildenafil

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

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title = "Usefulness of acute pulmonary vasoreactivity test of sildenafil in treatment of portopulmonary hypertension. A case report",
abstract = "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 452cm/s. Right heart catheterization demonstrated severe pulmonary hypertension [pulmonary arterial pressure (PAP) systolic/diastolic/mean=73/20/41mmHg and pulmonary vascular resistance (PVR)=509dynscm-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 480cm/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 (20mg) decreased PAP (93/30/55-77/27/44mmHg) and PVR (908-833dynscm-5), we added sildenafil (20mg, t.i.d.) to the prostacyclin analog. Tricuspid regurgitation velocity decreased to 403cm/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.",
keywords = "Acute pulmonary vasoreactivity test, Portopulmonary hypertension, Sildenafil",
author = "Satoshi Akagi and Kazufumi Nakamura and Soichiro Fuke and Kusano, {Kengo Fukushima} and Hiroshi Itoh",
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T1 - Usefulness of acute pulmonary vasoreactivity test of sildenafil in treatment of portopulmonary hypertension. A case report

AU - Akagi, Satoshi

AU - Nakamura, Kazufumi

AU - Fuke, Soichiro

AU - Kusano, Kengo Fukushima

AU - Itoh, Hiroshi

PY - 2011/8

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N2 - 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 452cm/s. Right heart catheterization demonstrated severe pulmonary hypertension [pulmonary arterial pressure (PAP) systolic/diastolic/mean=73/20/41mmHg and pulmonary vascular resistance (PVR)=509dynscm-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 480cm/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 (20mg) decreased PAP (93/30/55-77/27/44mmHg) and PVR (908-833dynscm-5), we added sildenafil (20mg, t.i.d.) to the prostacyclin analog. Tricuspid regurgitation velocity decreased to 403cm/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.

AB - 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 452cm/s. Right heart catheterization demonstrated severe pulmonary hypertension [pulmonary arterial pressure (PAP) systolic/diastolic/mean=73/20/41mmHg and pulmonary vascular resistance (PVR)=509dynscm-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 480cm/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 (20mg) decreased PAP (93/30/55-77/27/44mmHg) and PVR (908-833dynscm-5), we added sildenafil (20mg, t.i.d.) to the prostacyclin analog. Tricuspid regurgitation velocity decreased to 403cm/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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