An Autopsied Case of Primary Pulmonary Hypertension Complicated by Hepatopulmonary Syndrome
A 57-year-old man, who had received a transfusion five years before, was admitted to our hospital complaining of worsening dyspnea on exertion. Cardiac catheterization was performed, and pulmonary hypertension was diagnosed. Liver dysfunction was also documented. We administered diuretics and observ...
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| Published in | Nihon Kyōbu Shikkan Gakkai zasshi Vol. 33; no. 1; pp. 90 - 94 |
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| Main Authors | , , , , , , , |
| Format | Journal Article |
| Language | Japanese |
| Published |
Japan
The Japanese Respiratory Society
1995
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| Subjects | |
| Online Access | Get full text |
| ISSN | 0301-1542 1883-471X |
| DOI | 10.11389/jjrs1963.33.90 |
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| Abstract | A 57-year-old man, who had received a transfusion five years before, was admitted to our hospital complaining of worsening dyspnea on exertion. Cardiac catheterization was performed, and pulmonary hypertension was diagnosed. Liver dysfunction was also documented. We administered diuretics and observed his clinical course. Gradually worsening hypoxemia and radioisotope accumulation in the kidney following a lung perfusion scintigram suggested the existence of an intrapulmonary shunt. The patient died seven years later due to exacerbation of heart failure secondary to pulmonary infection. Autopsy revealed remarkable hypertensive pulmonary arteriopathy as well as abnormal dilation of precapillary pulmonary atterioles. Esophageal varices suggested portal hypertension. Marked hypoxemia and intrapulmonary vascular dilation suggest the contribution of an hepatopulmonary syndrome. |
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| AbstractList | A 57-year-old man, who had received a transfusion five years before, was admitted to our hospital complaining of worsening dyspnea on exertion. Cardiac catheterization was performed, and pulmonary hypertension was diagnosed. Liver dysfunction was also documented. We administered diuretics and observed his clinical course. Gradually worsening hypoxemia and radioisotope accumulation in the kidney following a lung perfusion scintigram suggested the existence of an intrapulmonary shunt. The patient died seven years later due to exacerbation of heart failure secondary to pulmonary infection. Autopsy revealed remarkable hypertensive pulmonary arteriopathy as well as abnormal dilation of precapillary pulmonary arterioles. Esophageal varices suggested portal hypertension. Marked hypoxemia and intrapulmonary vascular dilation suggest the contribution of an hepatopulmonary syndrome. A 57-year-old man, who had received a transfusion five years before, was admitted to our hospital complaining of worsening dyspnea on exertion. Cardiac catheterization was performed, and pulmonary hypertension was diagnosed. Liver dysfunction was also documented. We administered diuretics and observed his clinical course. Gradually worsening hypoxemia and radioisotope accumulation in the kidney following a lung perfusion scintigram suggested the existence of an intrapulmonary shunt. The patient died seven years later due to exacerbation of heart failure secondary to pulmonary infection. Autopsy revealed remarkable hypertensive pulmonary arteriopathy as well as abnormal dilation of precapillary pulmonary arterioles. Esophageal varices suggested portal hypertension. Marked hypoxemia and intrapulmonary vascular dilation suggest the contribution of an hepatopulmonary syndrome.A 57-year-old man, who had received a transfusion five years before, was admitted to our hospital complaining of worsening dyspnea on exertion. Cardiac catheterization was performed, and pulmonary hypertension was diagnosed. Liver dysfunction was also documented. We administered diuretics and observed his clinical course. Gradually worsening hypoxemia and radioisotope accumulation in the kidney following a lung perfusion scintigram suggested the existence of an intrapulmonary shunt. The patient died seven years later due to exacerbation of heart failure secondary to pulmonary infection. Autopsy revealed remarkable hypertensive pulmonary arteriopathy as well as abnormal dilation of precapillary pulmonary arterioles. Esophageal varices suggested portal hypertension. Marked hypoxemia and intrapulmonary vascular dilation suggest the contribution of an hepatopulmonary syndrome. A 57-year-old man, who had received a transfusion five years before, was admitted to our hospital complaining of worsening dyspnea on exertion. Cardiac catheterization was performed, and pulmonary hypertension was diagnosed. Liver dysfunction was also documented. We administered diuretics and observed his clinical course. Gradually worsening hypoxemia and radioisotope accumulation in the kidney following a lung perfusion scintigram suggested the existence of an intrapulmonary shunt. The patient died seven years later due to exacerbation of heart failure secondary to pulmonary infection. Autopsy revealed remarkable hypertensive pulmonary arteriopathy as well as abnormal dilation of precapillary pulmonary atterioles. Esophageal varices suggested portal hypertension. Marked hypoxemia and intrapulmonary vascular dilation suggest the contribution of an hepatopulmonary syndrome. |
| Author | Tasaka, Sadatomo Kanazawa, Minoru Matsumoto, Michinaga Handa, Shunnosuke Mori, Masaaki Kuramochi, Shigeru Yamaguchi, Kazuhiro Nakamura, Hidetoshi |
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| References | 7) Eriksson, L. S., Soderman, C., Ericzon, B. G., Eleborg, L., Wahren, J. & HAedenstierna, G.: Normalization of ventilation/peefusion relationships after liver transplantation in patients with decompensated cirrhosis; Evidence for a hepatopulmonary syndrome. Hepatology, 12: 1350, 1990. 1) Kennedy, T. C. & Knudson, R. J.: Exerciseaggravated hypoxemia and orthodeoxia in cirrhosis. Chest, 72: 305, 1977. 10) 宮森亮子, 丸谷公一, 則末栄己, 福井俊夫, 半田俊之介, 細田泰弘: 肝硬変・肝癌に合併した肺高血圧症の1剖検例. 呼と循, 33: 93, 1985. 6) 司城博志, 佐々木悠, 清水正賀, 奥村旬, 坂田博道: 著名な低酸素血症を伴う肝硬変症 (hepatopulmonary syndrome) の臨床病理学的検討. 肝臓, 32: 817, 1991. 12) Edwards, B. S., Weir, E. K., Edwards, W. D., Ludwig, J., Dykoski, R. K. & Edwards, J. E.: Coexistent pulmonary and portal hypertension: Morphologic and clinical features. J. Am. Coll. Cardiol., 10: 1233, 1987. 9) 川越康博, 米沢美枝子, 永井厚志, 前島周一, 阿部澄子, 藤川晃成, 金野公郎, 滝沢敬夫: 肝硬変に合併した肺高血圧症の1例. 日胸疾会誌, 16: 58, 1978. 14) Ruttner, J. R., Bartschi, J. P., Niedermann, R. & Schneider,J.: Plexogenic pulmonary arteriopathy and liver cirrhosis. Thorax, 35: 133, 1980. 11) McDonnell, P. J., Toye, P. A. & Hutchins, G. M., Primary pulmonary hypertension and cirrhosis; Are they related? Am. Rev. Respir. Dis., 127: 437, 1983. 5) Genovesi, M. G., Tierney, D. F., Taplin, G. V. & Eisenberg, H.: An intravenous radionuclide method to evaluate hypoxemia caused by abnormal vessels: Limitation of conventional techniques. Am. Rev. Respir. Dis., 114: 59, 1876. 8) Rodriquez-Roisin, R., Agusti, A. G. N. & Roca, J.; The hepatopulmonary syndrome; New nama, old complexities. Thorax, 47: 897, 1992. 16) Fuster, V., Steele, P. M., Edwards, W. D., Gersh, B. J., McGoon, M. D. & Frye, R. L.: Primary pulmonary hypertension; Natural history and the importance of thrombosis. Circulation, 70: 580, 1984. 3) Agusti, A. G. N., Roca, J., Bosch, J. & Rodriquez-Roisin, R.: The lung in patients with cirrhosis. J. Hepatol., 10: 251, 1990. 4) Fluckiger, M.: Vorkommen von trommelschagelformigen fingerend phalagen ohne chronische veranderungen an der lungen oiler am herzen. Wein. Med. Wehnschr., 49: 1457, 1884. 2) Krowka, M. J. & Cortese, D. A.: Hepatopulmonary syndrome: An evolving perspective in the era of liver transplantation. Hepatology, 11: 138, 1990. 13) Robalino, B. D. & Moodie, D. S.: Association between primary pulmonary hypertension and portal hypertension; Analysis of its pathophysiology and clinical, laboratory and hemodynamic manifestations. J. Am. Coll. Cardiol., 17: 492, 1991. 15) Cohen, M. D., Rubin, L. J., Taylor, W. E. & Cuthbert, J. A.: Primary pulmonary hypertension; An unusual case associated with extrahepatic portal hypertension. Hepatology, 3; 588, 1983. |
| References_xml | – reference: 9) 川越康博, 米沢美枝子, 永井厚志, 前島周一, 阿部澄子, 藤川晃成, 金野公郎, 滝沢敬夫: 肝硬変に合併した肺高血圧症の1例. 日胸疾会誌, 16: 58, 1978. – reference: 5) Genovesi, M. G., Tierney, D. F., Taplin, G. V. & Eisenberg, H.: An intravenous radionuclide method to evaluate hypoxemia caused by abnormal vessels: Limitation of conventional techniques. Am. Rev. Respir. Dis., 114: 59, 1876. – reference: 13) Robalino, B. D. & Moodie, D. S.: Association between primary pulmonary hypertension and portal hypertension; Analysis of its pathophysiology and clinical, laboratory and hemodynamic manifestations. J. Am. Coll. Cardiol., 17: 492, 1991. – reference: 11) McDonnell, P. J., Toye, P. A. & Hutchins, G. M., Primary pulmonary hypertension and cirrhosis; Are they related? Am. Rev. Respir. Dis., 127: 437, 1983. – reference: 3) Agusti, A. G. N., Roca, J., Bosch, J. & Rodriquez-Roisin, R.: The lung in patients with cirrhosis. J. Hepatol., 10: 251, 1990. – reference: 1) Kennedy, T. C. & Knudson, R. J.: Exerciseaggravated hypoxemia and orthodeoxia in cirrhosis. Chest, 72: 305, 1977. – reference: 6) 司城博志, 佐々木悠, 清水正賀, 奥村旬, 坂田博道: 著名な低酸素血症を伴う肝硬変症 (hepatopulmonary syndrome) の臨床病理学的検討. 肝臓, 32: 817, 1991. – reference: 16) Fuster, V., Steele, P. M., Edwards, W. D., Gersh, B. J., McGoon, M. D. & Frye, R. L.: Primary pulmonary hypertension; Natural history and the importance of thrombosis. Circulation, 70: 580, 1984. – reference: 15) Cohen, M. D., Rubin, L. J., Taylor, W. E. & Cuthbert, J. A.: Primary pulmonary hypertension; An unusual case associated with extrahepatic portal hypertension. Hepatology, 3; 588, 1983. – reference: 4) Fluckiger, M.: Vorkommen von trommelschagelformigen fingerend phalagen ohne chronische veranderungen an der lungen oiler am herzen. Wein. Med. Wehnschr., 49: 1457, 1884. – reference: 8) Rodriquez-Roisin, R., Agusti, A. G. N. & Roca, J.; The hepatopulmonary syndrome; New nama, old complexities. Thorax, 47: 897, 1992. – reference: 14) Ruttner, J. R., Bartschi, J. P., Niedermann, R. & Schneider,J.: Plexogenic pulmonary arteriopathy and liver cirrhosis. Thorax, 35: 133, 1980. – reference: 7) Eriksson, L. S., Soderman, C., Ericzon, B. G., Eleborg, L., Wahren, J. & HAedenstierna, G.: Normalization of ventilation/peefusion relationships after liver transplantation in patients with decompensated cirrhosis; Evidence for a hepatopulmonary syndrome. Hepatology, 12: 1350, 1990. – reference: 10) 宮森亮子, 丸谷公一, 則末栄己, 福井俊夫, 半田俊之介, 細田泰弘: 肝硬変・肝癌に合併した肺高血圧症の1剖検例. 呼と循, 33: 93, 1985. – reference: 12) Edwards, B. S., Weir, E. K., Edwards, W. D., Ludwig, J., Dykoski, R. K. & Edwards, J. E.: Coexistent pulmonary and portal hypertension: Morphologic and clinical features. J. Am. Coll. Cardiol., 10: 1233, 1987. – reference: 2) Krowka, M. J. & Cortese, D. A.: Hepatopulmonary syndrome: An evolving perspective in the era of liver transplantation. Hepatology, 11: 138, 1990. |
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| SubjectTerms | Dilatation, Pathologic Hepatopulmonary syndrome Humans Hypertension, Pulmonary - complications Hypoxia - complications Liver Cirrhosis - complications Lung Diseases - complications Male Middle Aged Pulmonary Artery - pathology Syndrome |
| Title | An Autopsied Case of Primary Pulmonary Hypertension Complicated by Hepatopulmonary Syndrome |
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