Hemodynamic Characteristics of Portopulmonary Hypertension : An Evaluation of Five Patients

We retrospectively studied the hemodynamic characteristics of liver cirrhosis complicated by idiopathic pulmonary hypertension (PH), and compared the results with those of chronic hepatitis and liver cirrhosis without PH. Etiology of liver cirrhosis was excess alcohol intake in 2, hemochromatosis in...

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Published inJapanese Journal of Portal Hypertension Vol. 5; no. 1; pp. 67 - 72
Main Authors Sekiyama, Tatsuya, Terada, Hideto, Katsuta, Yasumi, Zhang, Xuejun, Satomura, Katsuaki, Aramaki, Takumi, Nagano, Tomoo, Komeichi, Hirokazu, Shimizu, Shuji, Ohsuga, Masaru
Format Journal Article
LanguageJapanese
Published The Japan Society for Portal Hypertension 1999
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ISSN1344-8447
2186-6376
DOI10.11423/jsph1999.5.1_67

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Abstract We retrospectively studied the hemodynamic characteristics of liver cirrhosis complicated by idiopathic pulmonary hypertension (PH), and compared the results with those of chronic hepatitis and liver cirrhosis without PH. Etiology of liver cirrhosis was excess alcohol intake in 2, hemochromatosis in 1, autoimmune hepatitis in 1 and hepatitis C in 1 case. The age of diagnosis ranged between 42 and 64 years. Prevalence of PH ascertained by right cardiac catheterization was 2% (5 cases) among 250 patients with liver cirrhosis. In these 5 patients, mean pressure of the pulmonary artery was 42.4±12.1 mmHg (mean±SD). Mean vascular resistance of the pulmonary artery was 832±400 dynes·sec·cm-5/m2 and the cardiac index (CI) was 3.13±0.66 l/ min/m2 CI in 41 patients without PH was 4.26±0.90 l/min/m2, and significantly higher than that of the patients with PH (p<0.01). Mean systemic vascular resistance was significantly higher, and circulating plasma volume was significantly smaller in patients with PH than patients without PH (2438±743 vs 757±228 dynes·sec·cm-5/m2, p<0.01 and 1.71±0.36 vs 2.07±0.29 l/min/m2, p<0.05, respectively). These results suggest that hyperdynamic systemic circulation commonly seen in patients with cirrhosis is normalized by the complication of PH.
AbstractList We retrospectively studied the hemodynamic characteristics of liver cirrhosis complicated by idiopathic pulmonary hypertension (PH), and compared the results with those of chronic hepatitis and liver cirrhosis without PH. Etiology of liver cirrhosis was excess alcohol intake in 2, hemochromatosis in 1, autoimmune hepatitis in 1 and hepatitis C in 1 case. The age of diagnosis ranged between 42 and 64 years. Prevalence of PH ascertained by right cardiac catheterization was 2% (5 cases) among 250 patients with liver cirrhosis. In these 5 patients, mean pressure of the pulmonary artery was 42.4±12.1 mmHg (mean±SD). Mean vascular resistance of the pulmonary artery was 832±400 dynes·sec·cm-5/m2 and the cardiac index (CI) was 3.13±0.66 l/ min/m2 CI in 41 patients without PH was 4.26±0.90 l/min/m2, and significantly higher than that of the patients with PH (p<0.01). Mean systemic vascular resistance was significantly higher, and circulating plasma volume was significantly smaller in patients with PH than patients without PH (2438±743 vs 757±228 dynes·sec·cm-5/m2, p<0.01 and 1.71±0.36 vs 2.07±0.29 l/min/m2, p<0.05, respectively). These results suggest that hyperdynamic systemic circulation commonly seen in patients with cirrhosis is normalized by the complication of PH. 特発性肺高血圧を合併した肝硬変5例の血行動態を, 非合併肝硬変および慢性肝炎のそれと比較した.肝硬変の成因はアルコール2例, ヘモクロマトーシス1例, 自己免疫性肝炎1例およびC型肝炎1例であった.右心カテーテル検査を実施した肝硬変250例中の肺高血圧合併頻度は2%であった.肺動脈平均圧, 肺動脈血管抵抗および心係数の平均値はそれぞれ42.4 ± 12.1mmHg (平均値±標準偏差), 832 ± 400 dynes・sec・cm-5/m2および3.13 ± 0.66l/min/m2であった.肺高血圧非合併肝硬変41例の心係数は4.26±0.90l/min/m2と肺高血圧合併例よりも有意に高かった (p<0.01).肺高血圧合併肝硬変例の総末梢血管抵抗は非合併肝硬変例よりも有意に高く, 循環血漿量は有意に少なかった (それぞれ2438±743 vs 757±228dynes・sec・cm-5/m2, p<0.01, 1.71±0.36 vs 2.07±0.29 l/min/m2, p<0.05).これらの成績から肝硬変患者にしばしばみられる全身循環亢進は肺高血圧の合併により減弱されることが示唆された.
We retrospectively studied the hemodynamic characteristics of liver cirrhosis complicated by idiopathic pulmonary hypertension (PH), and compared the results with those of chronic hepatitis and liver cirrhosis without PH. Etiology of liver cirrhosis was excess alcohol intake in 2, hemochromatosis in 1, autoimmune hepatitis in 1 and hepatitis C in 1 case. The age of diagnosis ranged between 42 and 64 years. Prevalence of PH ascertained by right cardiac catheterization was 2% (5 cases) among 250 patients with liver cirrhosis. In these 5 patients, mean pressure of the pulmonary artery was 42.4±12.1 mmHg (mean±SD). Mean vascular resistance of the pulmonary artery was 832±400 dynes·sec·cm-5/m2 and the cardiac index (CI) was 3.13±0.66 l/ min/m2 CI in 41 patients without PH was 4.26±0.90 l/min/m2, and significantly higher than that of the patients with PH (p<0.01). Mean systemic vascular resistance was significantly higher, and circulating plasma volume was significantly smaller in patients with PH than patients without PH (2438±743 vs 757±228 dynes·sec·cm-5/m2, p<0.01 and 1.71±0.36 vs 2.07±0.29 l/min/m2, p<0.05, respectively). These results suggest that hyperdynamic systemic circulation commonly seen in patients with cirrhosis is normalized by the complication of PH.
Author Sekiyama, Tatsuya
Aramaki, Takumi
Shimizu, Shuji
Terada, Hideto
Katsuta, Yasumi
Ohsuga, Masaru
Komeichi, Hirokazu
Zhang, Xuejun
Satomura, Katsuaki
Nagano, Tomoo
Author_FL 荒牧 琢己
大須賀 勝
清水 秀治
古明地 弘和
勝田 悌実
張 雪君
寺田 秀人
長野 具雄
関山 達也
里村 克章
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References 16) Mark N, Elizabeth K, Thomas RN, et al : Neurohormonal activation in patients with right ventricular failure from pulmonary hypertension : relation to hemodynamic variables and endothelin levels. J Am Coll Cardiol 1995; 26 : 1581-1585
6) Ratner JR, Bartschi JP, Niedermann R, et al : Plexogenic pulmonary arteriopathy and liver cirrhosis. Thorax 1980; 35 : 133-136
11) Kuo PC, Plotkin JS, Johnson LB, et al : Distinctive clinical features of portopulmonary hypertension. Chest 1997; 112 : 980-986
5) McDonnell PJ, Toye PA, Hutchins GM : Primary pulmonary hypertension and cirrhosis : Are they related? Am Rev Respir Dis 1983; 127 : 437-441
15) 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 1991; 17 : 492-498
4) Lebrec D, Capron JP, Dhumeaux D, et al : Pulmonary hypertension complicating portal hypertension. Am Rev Respir Dis 1979; 120 : 849-856
14) Michael JK, Michael DM, Rochester M : Portopulmonary hypertension the next step. Chest 1997; 112 : 869-870
12) Hadengue A, Benhayoun MK, Lebrec D, et al : Pulmonary hypertension complicating portal hypertension : prevalence and relation to splanchnic hemodynamics. Gastroenterology 1991; 100 : 520-528
1) Murray JF, Dawson AM, Sherlock S : Circulatory changes in chronic liver disease. Am J Med 1958; 32 : 358-367
3) Abenhaim L, Moride Y, Brenor F, et al : Appetitesuppressant drugs and the risk of primary pulmonary hypertension. N Engl J Med 1996; 335 : 609-616
2) 関山達也, 長戸孝道, 吉田明弘, 他 : 門脈圧亢進症における肺循環に関する検討.日門食会誌1997;3 : 209-213
7) Yoshida EM, Erb SR, Pflugfelder PW, et al : Single lung versus liver transplantation for the treatment of porto-pulmonary hypertension : a comparison of two patients. Transplantation 1993 : 55 : 688-690
8) 荒牧琢己, 福間長知, 滝田孝之, 他 : 原発性肺高血圧症を合併した肝硬変の1例および本邦報告例の集計成積.肝臓1986; 27 : 976-981
13) Matsubara O, Nakamura T, Uehara T : Histometrical investigation of the pulmonary artery in severe hepatic disease. J Pathol 1984; 143 : 31-37
9) Lewis JR : Primary pulmonary hypertension. N Engl J Med 1997; 336 : 111-117
10) Kanemoto N, Constant J : Present status of primary hypertension. Tokai J Exp Med 1989; 14 : 257-280
References_xml – reference: 15) 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 1991; 17 : 492-498
– reference: 6) Ratner JR, Bartschi JP, Niedermann R, et al : Plexogenic pulmonary arteriopathy and liver cirrhosis. Thorax 1980; 35 : 133-136
– reference: 8) 荒牧琢己, 福間長知, 滝田孝之, 他 : 原発性肺高血圧症を合併した肝硬変の1例および本邦報告例の集計成積.肝臓1986; 27 : 976-981
– reference: 3) Abenhaim L, Moride Y, Brenor F, et al : Appetitesuppressant drugs and the risk of primary pulmonary hypertension. N Engl J Med 1996; 335 : 609-616
– reference: 16) Mark N, Elizabeth K, Thomas RN, et al : Neurohormonal activation in patients with right ventricular failure from pulmonary hypertension : relation to hemodynamic variables and endothelin levels. J Am Coll Cardiol 1995; 26 : 1581-1585
– reference: 14) Michael JK, Michael DM, Rochester M : Portopulmonary hypertension the next step. Chest 1997; 112 : 869-870
– reference: 2) 関山達也, 長戸孝道, 吉田明弘, 他 : 門脈圧亢進症における肺循環に関する検討.日門食会誌1997;3 : 209-213
– reference: 10) Kanemoto N, Constant J : Present status of primary hypertension. Tokai J Exp Med 1989; 14 : 257-280
– reference: 5) McDonnell PJ, Toye PA, Hutchins GM : Primary pulmonary hypertension and cirrhosis : Are they related? Am Rev Respir Dis 1983; 127 : 437-441
– reference: 7) Yoshida EM, Erb SR, Pflugfelder PW, et al : Single lung versus liver transplantation for the treatment of porto-pulmonary hypertension : a comparison of two patients. Transplantation 1993 : 55 : 688-690
– reference: 9) Lewis JR : Primary pulmonary hypertension. N Engl J Med 1997; 336 : 111-117
– reference: 4) Lebrec D, Capron JP, Dhumeaux D, et al : Pulmonary hypertension complicating portal hypertension. Am Rev Respir Dis 1979; 120 : 849-856
– reference: 13) Matsubara O, Nakamura T, Uehara T : Histometrical investigation of the pulmonary artery in severe hepatic disease. J Pathol 1984; 143 : 31-37
– reference: 1) Murray JF, Dawson AM, Sherlock S : Circulatory changes in chronic liver disease. Am J Med 1958; 32 : 358-367
– reference: 12) Hadengue A, Benhayoun MK, Lebrec D, et al : Pulmonary hypertension complicating portal hypertension : prevalence and relation to splanchnic hemodynamics. Gastroenterology 1991; 100 : 520-528
– reference: 11) Kuo PC, Plotkin JS, Johnson LB, et al : Distinctive clinical features of portopulmonary hypertension. Chest 1997; 112 : 980-986
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Snippet We retrospectively studied the hemodynamic characteristics of liver cirrhosis complicated by idiopathic pulmonary hypertension (PH), and compared the results...
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SubjectTerms circulating plasma volume
portal hypertension
portopulmonary hypertension
pulmonary hypertension
systemic hemodynamics
Title Hemodynamic Characteristics of Portopulmonary Hypertension : An Evaluation of Five Patients
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