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Portopulmonary HypertensionPortopulmonary Hypertension: Light at the End of the Tunnel?

Olivier Sitbon, MD, PhD; Dermot S. O’Callaghan, MD; Laurent Savale, MD, PhD
Author and Funding Information

From AP-HP, Centre de Référence de l’Hypertension Pulmonaire Sévère, Service de Pneumologie et Réanimation, Hôpital Antoine Béclère (Drs Sitbon, O’Callaghan, and Savale); Université Paris-Sud 11, Faculté de Médecine (Drs Sitbon and Savale), Le Kremlin-Bicêtre, France; and INSERM U999 (Hypertension Artérielle Pulmonaire: Physiopathologie et Innovation Thérapeutique), IPSIT, Centre Chirurgical Marie-Lannelongue (Drs Sitbon and Savale), Le Plessis-Robinson, France.

Correspondence to: Olivier Sitbon, MD, PhD, Service de Pneumologie, Hôpital Antoine Béclère, 157 rue de la Porte de Trivaux, F-92141 Clamart, France; e-mail: olivier.sitbon@abc.aphp.fr


Financial/nonfinancial disclosures: The authors have reported to CHEST the following conflicts of interest: Dr Sitbon has relationships with drug companies including Actelion, Bayer HealthCare, GlaxoSmithKline, Eli Lilly and Company, Pfizer, and United Therapeutics. In addition to being an investigator in trials involving these companies, relationships include consultancy service and membership on scientific advisory boards. Dr Savale has relationships with drug companies including Actelion, Bayer HealthCare, GlaxoSmithKline, Eli Lilly and Company, and Pfizer. In addition to being an investigator in trials involving these companies, relationships include consultancy service. Dr O’Callaghan has reported that no potential conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (http://www.chestpubs.org/site/misc/reprints.xhtml).


© 2012 American College of Chest Physicians


Chest. 2012;141(4):840-842. doi:10.1378/chest.11-2378
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Portopulmonary hypertension (PoPH) is defined as pulmonary arterial hypertension (PAH) associated with portal hypertension, with or without liver disease.1 As is the case for all forms of PAH, PoPH is a life-threatening disease characterized by a marked and sustained elevation of pulmonary vascular resistance, leading to increased pulmonary artery pressure, right ventricular failure, and ultimately death.2 Prospective hemodynamic studies have shown that 2% to 6% of patients with portal hypertension develop significant pulmonary hypertension. Furthermore, this risk is independent of the presence or the severity of associated liver disease.3,4 PoPH accounts for approximately 7% to 10% of the overall PAH population.5 However, with increasing awareness of the disorder, this proportion is gradually increasing. Indeed, PoPH represents approximately 20% of all PAH cases in the recent French PAH registry and it is the most common form of nonidiopathic PAH.6

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