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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 (June 1, 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 vasodilating factors. The most common pulmonary symptom is exertional dyspnea; fatigue, chest pain and syncope occur more often at an advanced stage. Edema, ascites and prominent jugular veins are signs of both decompensated hepatic cirrhosis and right ventricular failure. Right heart catheterisation is the gold standard for the diagnosis and defines PPHT in mild disease with PAP less than 35 mmHg, moderate disease with PAP between 35 and 45 mmHg, and severe disease with PAP of 45 mmHg or higher. The medical treatment of portopulmonary hypertension is based on the treatment of other forms of pulmonary arterial hypertension, including vasomodulating pharmacologic agents. Liver transplantation is accompanied by high risk of mortality, generally due to acute right ventricular failure and cardiovascular collapse. The prognosis of PPHT is poor with mean survival of 15 months.
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3

Lv, Yong, Guohong Han, and Daiming Fan. "Portopulmonary hypertension." Scandinavian Journal of Gastroenterology 51, no. 7 (March 22, 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 (September 2011): 703–10. http://dx.doi.org/10.1097/mcg.0b013e31820656bd.

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5

Le Pavec, Jérôme, Rogério Souza, Philippe Herve, Didier Lebrec, Laurent Savale, Colas Tcherakian, Xavier Jaïs, et al. "Portopulmonary Hypertension." American Journal of Respiratory and Critical Care Medicine 178, no. 6 (September 15, 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 (October 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 candidates for liver transplantation (LT). Right heart catheterization remains mandatory to confirm the diagnosis and exclude all other causes of elevated pulmonary pressures. The management of PoPH includes PAH-targeted therapies although few studies have evaluated these medications in this specific indication. The impact of LT on PoPH outcome remains complex and must be specified by future collaborative investigation. Although uncontrolled PoPH is associated with higher risk of postoperative right heart failure and death, stabilization, improvement, or normalization of pulmonary hemodynamics after LT seem to be achievable goals in selected patients with PoPH.
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7

DuBrock, Hilary M., Reena J. Salgia, Norman L. Sussman, Sonja D. Bartolome, Zakiyah Kadry, David C. Mulligan, Sarah Jenkins, et al. "Portopulmonary Hypertension." Transplantation Direct 5, no. 6 (June 2019): e456. http://dx.doi.org/10.1097/txd.0000000000000900.

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8

Sakuma, Masahito, Shinko Souma, Osamu Kitamukai, Jun Demachi, Tohru Takahashi, Jun Suzuki, Jun Nawata, 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 (February 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 (September 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 (February 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 (January 17, 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 (October 16, 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 (December 8, 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 (August 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 (May 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 (April 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 (October 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, Darren Taichman, Raed A. Dweik, David B. Badesch, and Michael D. McGoon. "Portopulmonary Hypertension." Chest 141, no. 4 (April 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 (March 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 (December 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 (July 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 (June 2023): 605–14. http://dx.doi.org/10.1684/hpg.2023.2601.

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24

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 (May 17, 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 prognosis. Although Doppler echocardiography is the best initial screening tool for symptomatic patients and candidates for liver transplant, right heart catheterization remains the gold standard for disease diagnosis. Treatment of patients with portopulmonary hypertension is aimed at improving cardiac function, reducing pulmonary vascular resistance, and optimizing functional capacity. Pulmonary hypertension-specific therapy, which includes prostacyclin and its receptor agonists, endothelin receptor antagonists, phosphodiesterase inhibitors, and guanylate cyclase stimulators, plays a key role in the treatment of patients with portopulmonary hypertension. Small uncontrolled and recent single randomized controlled trials have reported promising results of vasodilator therapy in terms of clinical and hemodynamic improvement in patients, allowing certain patients to undergo liver transplantation. This review discusses the epidemiology, approach to diagnosis and treatment of patients with portopulmonary hypertension. We used MEDLINE database on the PubMed platform and the Cochrane library to search for literature sources using the keywords: portopulmonary hypertension, portal hypertension, pulmonary hypertension, liver cirrhosis, pulmonary complications.
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25

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

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26

Porres-Aguilar, Mateo, and Debabrata Mukherjee. "Portopulmonary hypertension: An update." Respirology 20, no. 2 (December 18, 2014): 235–42. http://dx.doi.org/10.1111/resp.12455.

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27

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

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28

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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29

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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30

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

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31

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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32

Safdar, Zeenat, Sonja Bartolome, and Norman Sussman. "Portopulmonary hypertension: An update." Liver Transplantation 18, no. 8 (July 24, 2012): 881–91. http://dx.doi.org/10.1002/lt.23485.

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33

Ruggiero, Rosechelle M., and Sonja D. Bartolome. "Portopulmonary Hypertension: A Review." Advances in Pulmonary Hypertension 21, no. 4 (October 1, 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 continues to evolve. This article provides updates on the definition, clinical course, and treatment of patients with POPH. We will also discuss the evolving data in treatment and liver transplantation in POPH.
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34

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

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35

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 therapy. A secondary evaluation investigated the effect on other hemodynamic measurements, 6 min walk distance, functional class, safety outcomes and survival.RESULTS: Of 1385 patients screened, 25 patients with portopulmonary hypertension were identified, of whom 20 received a phosphodiesterase-5 inhibitor. After six months, there was a significant decrease in pulmonary vascular resistance (−236 dyn·s·cm−5[95% CI −343 dyn·s·cm−5to −130 dyn·s·cm−5]; P<0.001), mean pulmonary artery pressure (−8.9 mmHg [95% CI −13.7 mmHg to −4.2 mmHg]; P=0.001) and an increase in Fick cardiac output (0.9 L/min [95% CI 0.1 L/min to 1.6 L/min]; P=0.02). There was no change in 6 min walk distance. The proportion of subjects with a WHO functional class III or IV was significantly reduced at six months compared with baseline (18% versus 61%; P=0.002). Safety outcomes did not reveal any adverse events.CONCLUSIONS: Phosphodiesterase-5 inhibitor therapy improved hemodynamics and functional class at six months in a cohort of patients with portopulmonary hypertension.
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36

Jasso-Baltazar, Erick A., Gonzalo A. Peña-Arellano, Jonathan Aguirre-Valadez, Isaac Ruiz, Bruno Papacristofilou-Riebeling, Jose Victor Jimenez, Cristian J. García-Carrera, et al. "Portopulmonary Hypertension: An Updated Review." Transplantation Direct 9, no. 8 (July 21, 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 in which, if elevated pulmonary pressures are shown, patients should undergo right heart catheterization to confirm the diagnosis. The prognosis is extremely poor in untreated patients; therefore, management includes pulmonary arterial hypertension therapies with the aim of improving pulmonary hemodynamics and moving patients to orthotopic liver transplantation (OLT). In this article, we review in detail the epidemiology, pathophysiology, process for diagnosis, and most current treatments including OLT and prognosis in patients with PoPH. In addition, we present a diagnostic algorithm that includes the current criteria to properly select patients with PoPH who are candidates for OLT.
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37

Akahane, Takemi. "Factors associated with portopulmonary hypertension." Hepatology Research 51, no. 12 (November 30, 2021): 1179–80. http://dx.doi.org/10.1111/hepr.13721.

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38

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

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39

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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40

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

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41

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

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42

Hoeper, M. M. "Bosentan therapy for portopulmonary hypertension." European Respiratory Journal 25, no. 3 (March 1, 2005): 502–8. http://dx.doi.org/10.1183/09031936.05.00080804.

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43

Reichenberger, F., R. Voswinckel, E. Steveling, B. Enke, A. Kreckel, H. Olschewski, F. Grimminger, W. Seeger, and H. A. Ghofrani. "Sildenafil treatment for portopulmonary hypertension." European Respiratory Journal 28, no. 3 (June 28, 2006): 563–67. http://dx.doi.org/10.1183/09031936.06.00030206.

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44

Ioachimescu, O. C., A. C. Mehta, and J. K. Stoller. "Hepatopulmonary syndrome following portopulmonary hypertension." European Respiratory Journal 29, no. 6 (March 1, 2007): 1277–80. http://dx.doi.org/10.1183/09031936.00140306.

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45

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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46

Condino, Adria A., D. Dunbar Ivy, Judith A. O'Connor, Michael R. Narkewicz, Sarah Mengshol, John R. Whitworth, Lori Claussen, Aimee Doran, and Ronald J. Sokol. "Portopulmonary Hypertension in Pediatric Patients." Journal of Pediatrics 147, no. 1 (July 2005): 20–26. http://dx.doi.org/10.1016/j.jpeds.2005.02.019.

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47

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

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48

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

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49

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

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50

Ghofrani, Hossein-Ardeschir. "Cardiopulmonary haemodynamics in portopulmonary hypertension." Lancet Respiratory Medicine 7, no. 7 (July 2019): 556–58. http://dx.doi.org/10.1016/s2213-2600(19)30143-2.

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