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Semin Respir Crit Care Med 2023; 44(06): 762-770
DOI: 10.1055/s-0043-1770119
Review Article

Management of Acutely Decompensated Pulmonary Hypertension

Laurent Savale
1   INSERM UMR_S 999, Pulmonary Hypertension: Pathophysiology and Novel Therapies, Hôpital Marie Lannelongue, Le Plessis-Robinson, France
2   Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France
3   Service de Pneumologie et Soins Intensifs Respiratoires, Assistance Publique - Hôpitaux de Paris (AP-HP), Hôpital Bicêtre, Le Kremlin-Bicêtre, France
4   European Reference Network on Rare Pulmonary Diseases (ERN-LUNG), Germany
,
Mithum Kularatne
5   Division of Respiratory Medicine, Department of Medicine, University of Calgary, Calgary, Canada
,
Anne Roche
1   INSERM UMR_S 999, Pulmonary Hypertension: Pathophysiology and Novel Therapies, Hôpital Marie Lannelongue, Le Plessis-Robinson, France
2   Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France
3   Service de Pneumologie et Soins Intensifs Respiratoires, Assistance Publique - Hôpitaux de Paris (AP-HP), Hôpital Bicêtre, Le Kremlin-Bicêtre, France
4   European Reference Network on Rare Pulmonary Diseases (ERN-LUNG), Germany
,
Jérémie Pichon
1   INSERM UMR_S 999, Pulmonary Hypertension: Pathophysiology and Novel Therapies, Hôpital Marie Lannelongue, Le Plessis-Robinson, France
2   Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France
3   Service de Pneumologie et Soins Intensifs Respiratoires, Assistance Publique - Hôpitaux de Paris (AP-HP), Hôpital Bicêtre, Le Kremlin-Bicêtre, France
4   European Reference Network on Rare Pulmonary Diseases (ERN-LUNG), Germany
,
Audrey Baron
1   INSERM UMR_S 999, Pulmonary Hypertension: Pathophysiology and Novel Therapies, Hôpital Marie Lannelongue, Le Plessis-Robinson, France
2   Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France
3   Service de Pneumologie et Soins Intensifs Respiratoires, Assistance Publique - Hôpitaux de Paris (AP-HP), Hôpital Bicêtre, Le Kremlin-Bicêtre, France
4   European Reference Network on Rare Pulmonary Diseases (ERN-LUNG), Germany
,
Athenaïs Boucly
1   INSERM UMR_S 999, Pulmonary Hypertension: Pathophysiology and Novel Therapies, Hôpital Marie Lannelongue, Le Plessis-Robinson, France
2   Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France
3   Service de Pneumologie et Soins Intensifs Respiratoires, Assistance Publique - Hôpitaux de Paris (AP-HP), Hôpital Bicêtre, Le Kremlin-Bicêtre, France
4   European Reference Network on Rare Pulmonary Diseases (ERN-LUNG), Germany
,
Olivier Sitbon
1   INSERM UMR_S 999, Pulmonary Hypertension: Pathophysiology and Novel Therapies, Hôpital Marie Lannelongue, Le Plessis-Robinson, France
2   Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France
3   Service de Pneumologie et Soins Intensifs Respiratoires, Assistance Publique - Hôpitaux de Paris (AP-HP), Hôpital Bicêtre, Le Kremlin-Bicêtre, France
4   European Reference Network on Rare Pulmonary Diseases (ERN-LUNG), Germany
,
Marc Humbert
1   INSERM UMR_S 999, Pulmonary Hypertension: Pathophysiology and Novel Therapies, Hôpital Marie Lannelongue, Le Plessis-Robinson, France
2   Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France
3   Service de Pneumologie et Soins Intensifs Respiratoires, Assistance Publique - Hôpitaux de Paris (AP-HP), Hôpital Bicêtre, Le Kremlin-Bicêtre, France
4   European Reference Network on Rare Pulmonary Diseases (ERN-LUNG), Germany
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Abstract

Pulmonary arterial hypertension is a severe life-threatening condition associated with increased pulmonary vascular resistance and resulting right heart dysfunction. Admission to intensive care unit with acutely decompensated right heart failure is a significant negative prognostic event with a high risk of multisystem organ dysfunction and death. Presentations are heterogenous and may combine signs of both diastolic and systolic dysfunction complicating management. Renal dysfunction is often present, but other organ systems can be involved resulting in findings such as acute hepatic dysfunction or bowel wall congestion and ischemia. The goals of therapy are to rapidly reverse ventriculo-arterial decoupling and reduce right ventricular afterload to prevent progression to refractory or irreversible right heart failure. Triggering events must be investigated for and addressed urgently if identified. Volume status management is critical and both noninvasive and invasive testing can aid in prognostication and guide management, including the use of inotropes and vasopressors. In cases of refractory right heart dysfunction, consideration of urgent lung transplantation and mechanical circulatory support is necessary. These patients should be managed at expert centers in an intensive care setting with a multidisciplinary team of practitioners experienced in the management of right heart dysfunction given the high short- and long-term mortality resulting from acute decompensated right heart failure.

Keywords

pulmonary hypertension - right heart failure - intensive care unit - lung transplantation - vasopressor


Publication History

Article published online:
27 June 2023

© 2023. Thieme. All rights reserved.

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