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Semin Respir Crit Care Med 2013; 34(05): 700-713
DOI: 10.1055/s-0033-1356460
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Management of Severe Pulmonary Arterial Hypertension

John Granton
1   Division of Respirology and Interdepartmental Division of Critical Care, Mount Sinai Hospital, University Health Network, University of Toronto, Toronto, Ontario, Canada
2   Lung Transplant Program, Toronto General Hospital, University of Toronto, Toronto, Canada
,
Olaf Mercier
3   Department of Thoracic and Vascular Surgery and Heart—Lung Transplantation, Hôpital Marie-Lannelongue, Paris-Sud University, France
,
Marc De Perrot
2   Lung Transplant Program, Toronto General Hospital, University of Toronto, Toronto, Canada
4   Division of Thoracic Surgery, University Health Network, Department of Surgery, University of Toronto, Toronto, Ontario, Canada
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Publikationsdatum:
13. September 2013 (online)

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Abstract

Despite advances in medical therapies, pulmonary arterial hypertension (PAH), continues to cause significant morbidity and mortality. Although, the right ventricle can adapt to an increase in afterload, progression of the pulmonary vasculopathy that characterizes PAH causes many patients to develop progressive right ventricular (RV) failure. Furthermore, acute RV decompensation may develop from disorders that lead to either an acute increase in cardiac demand or an increase in ventricular afterload including interruptions in medical therapy, arrhythmia, or pulmonary embolism. The poor reserve of the right ventricle, RV ischemia, and adverse RV influence on left ventricular filling may lead to a global reduction in oxygen delivery and multiorgan failure. The authors present an approach to patients with advanced PAH focusing on both medical and surgical strategies to improve RV function based upon current evidence and physiological principles.

Keywords

pulmonary hypertension - right ventricular failure - intensive care - extracorporeal life support - lung transplantation

Note

All authors contributed equally to this work. They drafted parts of the article and worked together on the complete article.