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1

Saleemi, Sarfraz. "Portopulmonary hypertension." Annals of Thoracic Medicine 5, no. 1 (2010): 5. http://dx.doi.org/10.4103/1817-1737.58953.

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2

Hopps, Eugenia, Amelia Valenti, and Gregorio Caimi. "Portopulmonary Hypertension." Clinical & Investigative Medicine 34, no. 3 (2011): 111. http://dx.doi.org/10.25011/cim.v34i3.15182.

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Portopulmonary hypertension (PPHT) is a respiratory complication of portal hypertension, defined as an increase in mean pulmonary artery pressure (PAP) of > 25 mmHg with an increase in pulmonary vascular resistance of > 240 dyn.s/cm-5 and a normal pulmonary capillary wedge pressure ( < 15 mmHg), which often occurs in subjects with liver cirrhosis. Histopathological features of PPHT are endothelial and smooth-muscle cell proliferation and fibrosis leading to luminal obstruction in the resistance arteries. The pathogenesis of PPHT may result from an imbalance between vasoconstrictor and
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3

Lv, Yong, Guohong Han, and Daiming Fan. "Portopulmonary hypertension." Scandinavian Journal of Gastroenterology 51, no. 7 (2016): 795–806. http://dx.doi.org/10.3109/00365521.2016.1157895.

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4

Mukhtar, Nizar A., and Oren K. Fix. "Portopulmonary Hypertension." Journal of Clinical Gastroenterology 45, no. 8 (2011): 703–10. http://dx.doi.org/10.1097/mcg.0b013e31820656bd.

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5

Le Pavec, Jérôme, Rogério Souza, Philippe Herve, et al. "Portopulmonary Hypertension." American Journal of Respiratory and Critical Care Medicine 178, no. 6 (2008): 637–43. http://dx.doi.org/10.1164/rccm.200804-613oc.

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6

Watherald, Jason, Olivier Sitbon, and Laurent Savale. "Portopulmonary Hypertension." Seminars in Respiratory and Critical Care Medicine 38, no. 05 (2017): 651–61. http://dx.doi.org/10.1055/s-0037-1606251.

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AbstractPortal hypertension may have major consequences on the pulmonary vasculature due to complex pathophysiological interactions between liver and lungs. Portopulmonary hypertension (PoPH) is characterized by the association of portal hypertension and pulmonary arterial hypertension (PAH). As progressive elevation of right ventricular afterload can lead to right heart failure, PoPH is a serious complication of portal hypertension, affecting functional status and prognosis of patients. Early detection by transthoracic echocardiography must be performed in symptomatic patients and in candidat
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7

DuBrock, Hilary M., Reena J. Salgia, Norman L. Sussman, et al. "Portopulmonary Hypertension." Transplantation Direct 5, no. 6 (2019): e456. http://dx.doi.org/10.1097/txd.0000000000000900.

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8

Sakuma, Masahito, Shinko Souma, Osamu Kitamukai, et al. "Portopulmonary Hypertension." Circulation Journal 69, no. 11 (2005): 1386–93. http://dx.doi.org/10.1253/circj.69.1386.

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9

Krowka, Michael. "Portopulmonary Hypertension." Seminars in Respiratory and Critical Care Medicine 33, no. 01 (2012): 17–25. http://dx.doi.org/10.1055/s-0032-1301731.

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10

Sokol, Ronald J. "Portopulmonary Hypertension." Journal of Pediatric Gastroenterology and Nutrition 61, no. 3 (2015): 268–69. http://dx.doi.org/10.1097/mpg.0000000000000892.

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11

Budhiraja, Rohit, and Paul M. Hassoun. "Portopulmonary Hypertension*." Chest 123, no. 2 (2003): 562–76. http://dx.doi.org/10.1378/chest.123.2.562.

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12

Nayak, Ravi P., Dechun Li, and George M. Matuschak. "Portopulmonary hypertension." Current Gastroenterology Reports 11, no. 1 (2009): 56–63. http://dx.doi.org/10.1007/s11894-009-0009-3.

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13

Halank, Michael, Ralf Ewert, Hans-Juergen Seyfarth, and Gert Hoeffken. "Portopulmonary hypertension." Journal of Gastroenterology 41, no. 9 (2006): 837–47. http://dx.doi.org/10.1007/s00535-006-1879-x.

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14

Halank, Michael, Ralf Ewert, Hans-Juergen Seyfarth, and Gert Hoeffken. "Portopulmonary hypertension." Journal of Gastroenterology 41, no. 11 (2006): 1133. http://dx.doi.org/10.1007/s00535-006-1971-2.

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15

Passarella, Michael, Michael B. Fallon, and Steven M. Kawut. "Portopulmonary Hypertension." Clinics in Liver Disease 10, no. 3 (2006): 653–63. http://dx.doi.org/10.1016/j.cld.2006.08.023.

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16

Cartin-Ceba, Rodrigo, and Michael J. Krowka. "Portopulmonary Hypertension." Clinics in Liver Disease 18, no. 2 (2014): 421–38. http://dx.doi.org/10.1016/j.cld.2014.01.004.

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17

Sitbon, Olivier, Dermot S. O'Callaghan, and Laurent Savale. "Portopulmonary Hypertension." Chest 141, no. 4 (2012): 840–42. http://dx.doi.org/10.1378/chest.11-2378.

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18

Krowka, Michael J., and Michael D. McGoon. "Portopulmonary Hypertension." Chest 112, no. 4 (1997): 869–70. http://dx.doi.org/10.1378/chest.112.4.869.

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19

Krowka, Michael J., Dave P. Miller, Robyn J. Barst, et al. "Portopulmonary Hypertension." Chest 141, no. 4 (2012): 906–15. http://dx.doi.org/10.1378/chest.11-0160.

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20

Golbin, Jason M., and Michael J. Krowka. "Portopulmonary Hypertension." Clinics in Chest Medicine 28, no. 1 (2007): 203–18. http://dx.doi.org/10.1016/j.ccm.2006.11.004.

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21

Al-Naamani, Nadine, and Kari E. Roberts. "Portopulmonary Hypertension." Clinics in Chest Medicine 34, no. 4 (2013): 719–37. http://dx.doi.org/10.1016/j.ccm.2013.08.008.

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22

Wells, J. T., J. R. Runo, and M. R. Lucey. "Portopulmonary hypertension." Hepatology 48, no. 1 (2008): 13–15. http://dx.doi.org/10.1002/hep.22378.

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23

Thévenot, Thierry, Delphine Weil, Marie-France Seronde, Sarah Raevens, and Laurent Savale. "Portopulmonary hypertension." Hépato-Gastro & Oncologie Digestive 30, no. 6 (2023): 605–14. http://dx.doi.org/10.1684/hpg.2023.2601.

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24

Chernyaev, A. L., N. A. Tsareva, S. N. Avdeev, and M. V. Samsonova. "Portopulmonary hypertension." Journal of Respiratory Medicine 1, no. 2 (2025): 39. https://doi.org/10.17116/respmed2025102139.

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The article is devoted to the review of literature data on portopulmonary hypertension. The data on the occurrence of this pathology among people with liver diseases, as well as among patients with pulmonary hypertension are presented. Modern concepts of the pathogenesis of the disease, including genetic disorders, are described. Treatment strategies for portopulmonary hypertension at the present stage coincide with those for pulmonary arterial hypertension, but further studies are needed to assess the effectiveness of various drugs in this pathology. Mortality in portopulmonary hypertension i
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25

Chooklin, S. M., S. S. Chuklin, M. M. Posivnych, and S. A. Krystopchuk. "Portopulmonary hypertension: peculiarities of diagnosis and treatment." EMERGENCY MEDICINE 20, no. 3 (2024): 146–58. http://dx.doi.org/10.22141/2224-0586.20.3.2024.1686.

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Portopulmonary hypertension is defined as the development of pulmonary hypertension secondary to portal one. Its exact prevalence is difficult to determine due to the lack of routine screening in patients with portal hypertension. Hemodynamic changes associated with portal hypertension, including the hyperdynamic state, portosystemic shunts, and splanchnic vasodilation, cause significant disturbances in the pulmonary vasculature and play a key role in the pathogenesis of the disease. Without treatment, portopulmonary hypertension leads to progressive right ventricular failure with a poor progn
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26

VV, Potii, Kiriienko VT, Potii DA, Glukhova EI, and Kunickaya OS. "Portopulmonary Hypertension (Literature Review)." Acta Scientific Gastrointestinal Disorders 5, no. 1 (2022): 67–73. http://dx.doi.org/10.31080/asgis.2022.05.0371.

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27

Porres-Aguilar, Mateo, and Debabrata Mukherjee. "Portopulmonary hypertension: An update." Respirology 20, no. 2 (2014): 235–42. http://dx.doi.org/10.1111/resp.12455.

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28

Stauber, Rudolf E., and Horst Olschewski. "Portopulmonary hypertension: short review." European Journal of Gastroenterology & Hepatology 22, no. 4 (2010): 385–90. http://dx.doi.org/10.1097/meg.0b013e3283337130.

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29

Koek, G. "Portopulmonary hypertension; treatment experience." Journal of Hepatology 34 (April 2001): 54. http://dx.doi.org/10.1016/s0168-8278(01)80181-1.

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30

Koek, G. H., F. Yurd, E. Haagsma, A. Boonstra, M. Delcroix, and J. Pirenne. "Portopulmonary hypertension; treatment experience." Journal of Hepatology 34 (April 2001): 54. http://dx.doi.org/10.1016/s0168-8278(01)81056-4.

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31

Maruyama, Toru, Kohichi Ohsaki, Shinji Shimoda, Yoshikazu Kaji, and Mine Harada. "Thromboxane-dependent portopulmonary hypertension." American Journal of Medicine 118, no. 1 (2005): 93–94. http://dx.doi.org/10.1016/j.amjmed.2004.11.007.

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32

Perkins, James D. "Screening for portopulmonary hypertension." Liver Transplantation 13, no. 3 (2007): 463–67. http://dx.doi.org/10.1002/lt.21105.

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33

Safdar, Zeenat, Sonja Bartolome, and Norman Sussman. "Portopulmonary hypertension: An update." Liver Transplantation 18, no. 8 (2012): 881–91. http://dx.doi.org/10.1002/lt.23485.

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34

Ruggiero, Rosechelle M., and Sonja D. Bartolome. "Portopulmonary Hypertension: A Review." Advances in Pulmonary Hypertension 21, no. 4 (2022): 123–29. http://dx.doi.org/10.21693/1933-088x-21.4.123.

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Portopulmonary hypertension (POPH) is a rare complication of liver disease occurring when pulmonary arterial hypertension develops in the setting of portal hypertension. It increases the morbidity and mortality compared to patients with cirrhosis alone. POPH is classified in Group 1 pulmonary arterial hypertension, which has important implications on treatment. After aggressive treatment and in carefully selected patients, liver transplantation can be performed; this can be curative of not only their liver disease but also of their POPH. Treatment and patient selection for optimum results cont
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35

Gossage, James R. "Primary Pulmonary Hypertension or Portopulmonary Hypertension?" Chest 114, no. 4 (1998): 1224–25. http://dx.doi.org/10.1378/chest.114.4.1224-a.

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36

Fisher, Jolene H., Sindhu R. Johnson, Cathy Chau, Amie T. Kron, and John T. Granton. "Effectiveness of Phosphodiesterase-5 Inhibitor Therapy for Portopulmonary Hypertension." Canadian Respiratory Journal 22, no. 1 (2015): 42–46. http://dx.doi.org/10.1155/2015/810376.

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BACKGROUND: Portopulmonary hypertension is associated with significant morbidity and mortality. Phosphodiesterase-5 inhibitor therapy is efficacious in other causes of WHO group I pulmonary arterial hypertension.OBJECTIVE: To evaluate the efficacy and safety of phosphodiesterase-5 inhibitor therapy in patients with portopulmonary hypertension.METHODS: A single-centre retrospective cohort study that included patients with a diagnosis of portopulmonary hypertension was performed. The primary outcome was change in pulmonary vascular resistance after six months of phosphodiesterase-5 inhibitor the
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37

Jasso-Baltazar, Erick A., Gonzalo A. Peña-Arellano, Jonathan Aguirre-Valadez, et al. "Portopulmonary Hypertension: An Updated Review." Transplantation Direct 9, no. 8 (2023): e1517. http://dx.doi.org/10.1097/txd.0000000000001517.

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Portal hypertension may have major consequences on the pulmonary vasculature due to the complex pathophysiological interactions between the liver and lungs. Portopulmonary hypertension (PoPH), a subset of group 1 pulmonary hypertension (PH), is a serious pulmonary vascular disease secondary to portal hypertension, and is the fourth most common subtype of pulmonary arterial hypertension. It is most commonly observed in cirrhotic patients; however, patients with noncirrhotic portal hypertension can also develop it. On suspicion of PoPH, the initial evaluation is by a transthoracic echocardiogram
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38

Akahane, Takemi. "Factors associated with portopulmonary hypertension." Hepatology Research 51, no. 12 (2021): 1179–80. http://dx.doi.org/10.1111/hepr.13721.

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39

Restrepo, Ricardo, Esmé F. Singer, and Michael Baram. "Hepatopulmonary Syndrome and Portopulmonary Hypertension." Hospital Practice 41, no. 2 (2013): 62–71. http://dx.doi.org/10.3810/hp.2013.04.1049.

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40

Aldenkortt, Florence. "Portopulmonary hypertension and hepatopulmonary syndrome." World Journal of Gastroenterology 20, no. 25 (2014): 8072. http://dx.doi.org/10.3748/wjg.v20.i25.8072.

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41

Auzinger, Georg, and Julia A. Wendon. "Hepatopulmonary Syndrome and Portopulmonary Hypertension." Critical Care Medicine 33, no. 2 (2005): 470–71. http://dx.doi.org/10.1097/01.ccm.0000153598.84976.e9.

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42

Wiklund, Richard A. "Hepatopulmonary Syndrome and Portopulmonary Hypertension." Critical Care Medicine 33, no. 2 (2005): 471. http://dx.doi.org/10.1097/01.ccm.0000153601.32819.b0.

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43

Hoeper, M. M. "Bosentan therapy for portopulmonary hypertension." European Respiratory Journal 25, no. 3 (2005): 502–8. http://dx.doi.org/10.1183/09031936.05.00080804.

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44

Reichenberger, F., R. Voswinckel, E. Steveling, et al. "Sildenafil treatment for portopulmonary hypertension." European Respiratory Journal 28, no. 3 (2006): 563–67. http://dx.doi.org/10.1183/09031936.06.00030206.

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45

Ioachimescu, O. C., A. C. Mehta, and J. K. Stoller. "Hepatopulmonary syndrome following portopulmonary hypertension." European Respiratory Journal 29, no. 6 (2007): 1277–80. http://dx.doi.org/10.1183/09031936.00140306.

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46

KAMATH, PATRICK S. "Portopulmonary hypertension and hepatopulmonary syndrome." Journal of Gastroenterology and Hepatology 17 (December 2002): S253—S255. http://dx.doi.org/10.1046/j.1440-1746.17.s3.9.x.

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47

Condino, Adria A., D. Dunbar Ivy, Judith A. O'Connor, et al. "Portopulmonary Hypertension in Pediatric Patients." Journal of Pediatrics 147, no. 1 (2005): 20–26. http://dx.doi.org/10.1016/j.jpeds.2005.02.019.

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48

Krowka, Michael J. "Many Faces of Portopulmonary Hypertension." Journal of Pediatrics 147, no. 1 (2005): 3–4. http://dx.doi.org/10.1016/j.jpeds.2005.04.014.

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49

Medarov, Boris I., Amit Chopra, and Marc A. Judson. "Clinical aspects of portopulmonary hypertension." Respiratory Medicine 108, no. 7 (2014): 943–54. http://dx.doi.org/10.1016/j.rmed.2014.04.004.

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50

Umeda, Naoki, and Patrick S. Kamath. "Hepatopulmonary syndrome and portopulmonary hypertension." Hepatology Research 39, no. 10 (2009): 1020–22. http://dx.doi.org/10.1111/j.1872-034x.2009.00552.x.

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