ALAT
: alanine aminotransferase
ASAT
: aspartate aminotransferase
APAH
: associated pulmonary arterial hypertension
BAS
: balloon atrial septostomy
BMPR2
: bone morphogenetic protein receptor 2
BNP
: brain natriuretic peptide
BPA
: balloon pulmonary angioplasty
BREATHE
: Bosentan

/pdf/2015-esc-ers-guidelines-for-the-diagnosis-and-treatment-of-y26yabnnom.pdf

2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT).

Published on behalf of the European Society of Cardiology. All rights reserved. & 2015 European Society of Cardiology & European Respiratory Society. This article is being published concurrently in the European Heart Journal (10.1093/eurheartj/ehv317) and the European Respiratory Journal (10.1183/13993003.01032-2015). The articles are identical except for minor stylistic and spelling differences in keeping with each journal’s style. Either citation can be used when citing this article. * Corresponding authors: Nazzareno Galiè, Department of Experimental, Diagnostic and Specialty Medicine–DIMES, University of Bologna, Via Massarenti 9, 40138 Bologna, Italy, Tel: +39 051 349 858, Fax: +39 051 344 859, Email: nazzareno.galie@unibo.it.

/pdf/special-article2015-esc-ers-guidelines-for-the-diagnosis-and-22zaj14m7s.pdf

Special article2015 ESC/ERS Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension

Nazzareno Galiè (ESC Chairperson), Marc Humbert (ERS Chairperson), Jean-Luc Vachiery, Simon Gibbs, Irene Lang, Adam Torbicki, Gérald Simonneau, Andrew Peacock, Anton Vonk Noordegraaf, Maurice Beghetti, Ardeschir Ghofrani, Miguel Angel Gomez Sanchez, Georg Hansmann, Walter Klepetko, Patrizio Lancellotti, Marco Matucci, Theresa McDonagh, Luc A. Pierard, Pedro T. Trindade, Maurizio Zompatori and Marius Hoeper The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and of the European Respiratory Society (ERS)

https://journals.viamedica.pl/kardiologia_polska/article/download/KP.2015.0242/57586

[2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension].

Guidelines summarize and evaluate all available evidence on a particular issue at the time of the writing process, with the aim of assisting health professionals in selecting the best management strategies for an individual patient with a given condition, taking into account the impact on outcome, as well as the risk–benefit ratio of particular diagnostic or therapeutic means. Guidelines and recommendations should help health professionals to make decisions in their daily practice. However, the final decisions concerning an individual patient must be made by the responsible health professional(s) in consultation with the patient and caregiver as appropriate.

https://erj.ersjournals.com/content/erj/46/4/903.full.pdf

Aprobada por la Association for European Paediatric and Congenital Cardiology (AEPC) y la International Society for Heart and Lung Transplantation (ISHLT) Autores/Miembros del grupo de trabajo: Nazzareno Galiè* (coordinador de la ESC) (Italia), Marc Humberta,* (coordinador de la ERS) (Francia), Jean-Luc Vachieryc (Bélgica), Simon Gibbs (Reino Unido), Irene Lang (Austria), Adam Torbicki (Poland), Gérald Simonneaua (Francia), Andrew Peacocka (Reino Unido), Anton Vonk Noordegraafa (Países Bajos), Maurice Beghettib (Suiza), Ardeschir Ghofrania (Alemania), Miguel Ángel Gómez Sánchez (España), Georg Hansmannb (Alemania), Walter Klepetkoc (Austria), Patrizio Lancellotti (Bélgica), Marco Matuccid (Italia), Theresa McDonagh (Reino Unido), Luc A. Pierard (Bélgica), Pedro T. Trindade (Suiza), Maurizio Zompatorie (Italia) y Marius Hoepera (Alemania)

/pdf/guia-esc-ers-2015-sobre-diagnostico-y-tratamiento-de-la-4e09adc1o3.pdf

Guía ESC/ERS 2015 sobre diagnóstico y tratamiento de la hipertensión pulmonar

Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPHTN) are the two major pulmonary vascular complications of liver disease. While HPS is characterized by low pulmonary vascular resistance, PPHTN is defined by the presence of elevated pulmonary vascular resistance. Given these seemingly opposing pathophysiologic mechanisms, these conditions were traditionally felt to be mutually exclusive. In this series, we present three patients with severe hepatopulmonary syndrome who had spontaneous resolution of their HPS with the subsequent development of PPHTN. To our knowledge, this is the largest case series presented of this phenomenon in nontransplanted patients. One proposed mechanism for the occurrence of this phenomenon involves dysregulation of the same vascular signaling pathway, which may lead to both pulmonary vascular dilatations and pulmonary arterial remodeling in the same patient. Another theory involves the possible differential binding of endothelin-1, a vasoactive signaling peptide that induces vasoconstriction when bound to receptor A and vasodilation when bound to receptor B. Although the mechanisms for this phenomenon remain unclear, it is important to be vigilant of this phenomenon as it may change the patient's overall treatment plan, especially in regard to appropriateness and timing of liver transplant.

https://downloads.hindawi.com/journals/cripu/2013/561870.pdf

Transition from Hepatopulmonary Syndrome to Portopulmonary Hypertension: A Case Series of 3 Patients

BACKGROUND AND AIMS Patients with acute liver failure have high rates of infections, likely from defects in immune function. Whether infections are independently associated with poor outcomes is unclear. We hypothesized that patients with acute liver injury who developed infections were at increased risk of adverse outcomes. METHODS We conducted a retrospective analysis of 150 critically ill adult patients admitted with acute liver dysfunction at a single academic institution between 2005 and 2011. We excluded patients with immunocompromised states, patients with chronic liver disease and patients who died or were discharged within 48 h of admission. Our primary endpoint was a 30-day event-free survival, with events defined as either death or liver transplantation. Our secondary endpoint was length of stay. Univariate and multivariate analyses were performed to determine associations between presence of infection and our primary and secondary endpoints. RESULTS Of our cohort of 150 patients, 62 (41%) were infected and 88 (59%) were not infected. Of the infected patients, 45% died or underwent transplantation, compared to 22% for the non-infected patients (P = 0.003). Univariate and multivariate analyses demonstrated that infections in patients with acute liver dysfunction were an independent predictor of poor outcome (i.e. death or transplantation). In addition, specific types of infection, including pneumonia, independently led to a 48% increase in length of stay (P = 0.002). CONCLUSIONS Infections in patients with acute liver dysfunction are associated with increased risk of death or transplant and increased hospital length of stay.

Radhika Zopey

Papers

Transition from Hepatopulmonary Syndrome to Portopulmonary Hypertension: A Case Series of 3 Patients

Prognostic significance of infections in critically ill adult patients with acute liver injury: a retrospective cohort study

Microscopic Polyangiitis Presenting with Eosinophilic Pleural Effusions