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1.
Rev Mal Respir ; 37(2): 171-179, 2020 Feb.
Article Fr | MEDLINE | ID: mdl-32061440

Right ventricular failure (RVF) is a common cause of admission to the intensive care unit and its presence is a major prognostic factor in acute pulmonary embolism (PE) and chronic pulmonary hypertension (PH). RVF results from an incapacity of the RV to adapt to an increase in afterload so it can become critical in acute PE and chronic PH. The presence of RVF in cases of acute PE with haemodynamic instability is an indication for thrombolytic therapy. RVF represents the most common cause of death in chronic PH. Factors triggering RV failure in PH, such as infection, PE, arrhythmias, or unplanned withdrawal of pulmonary arterial hypertension (PAH)-targeted therapy, have to be considered and treated if identified. However, RVF may also represent progression to end-stage disease. The management of RVF in patients with PH requires expertise and consists of optimization of fluid balance (with diuretics), cardiac output (with inotropic support such as dobutamine), perfusion pressure (with norepinephrine), and reduction of RV afterload with PAH-targeted therapies. Extracorporeal life support, lung transplantation or heart-lung transplantation should be considered in cases of refractory RVF in eligible patients.


Hypertension, Pulmonary/therapy , Pulmonary Embolism/therapy , Vascular Diseases/therapy , Ventricular Dysfunction, Right/therapy , Acute Disease , Critical Care/methods , Extracorporeal Membrane Oxygenation , Heart Failure/epidemiology , Heart Failure/etiology , Heart Failure/therapy , Heart-Lung Transplantation , Humans , Hypertension, Pulmonary/complications , Hypertension, Pulmonary/epidemiology , Hypertension, Pulmonary/physiopathology , Intensive Care Units , Lung Transplantation , Pulmonary Circulation/physiology , Pulmonary Embolism/complications , Pulmonary Embolism/epidemiology , Pulmonary Embolism/physiopathology , Vascular Diseases/complications , Vascular Diseases/epidemiology , Vascular Diseases/physiopathology , Ventricular Dysfunction, Right/complications , Ventricular Dysfunction, Right/epidemiology , Ventricular Dysfunction, Right/physiopathology
2.
Rev Mal Respir ; 35(2): 160-170, 2018 Feb.
Article Fr | MEDLINE | ID: mdl-29501213

Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary hypertension (PH) characterized by preferential remodelling of pulmonary venules and angioproliferation. PVOD term includes idiopathic, heritable (biallelic mutations of EIF2AK4 gene), drugs and toxins induced (alkylating agents, organic solvents) and connectivite-associated forms (especially systemic-sclerosis associated form). PVOD and pulmonary arterial hypertension (PAH) share a similar clinical presentation. Lung biopsy is contraindicated in PVOD due to high risk of life-threatening bleeding. A noninvasive diagnostic approach, including oxygen parameters, low diffusing capacity for carbon monoxide and characteristic signs on high-resolution computed tomography of the chest, is used to support a diagnosis of PVOD. PVOD prognosis is worse than other forms of PAH. There is no evidence-based medical therapy for PVOD and life-threatening pulmonary edema may occur following PAH targeted therapy in PVOD. Lung transplantation remains the preferred definitive therapy for eligible patients.


Pulmonary Veno-Occlusive Disease , Animals , Diagnostic Imaging/methods , Disease Models, Animal , Humans , Pulmonary Veno-Occlusive Disease/diagnosis , Pulmonary Veno-Occlusive Disease/epidemiology , Pulmonary Veno-Occlusive Disease/therapy , Rare Diseases/diagnosis , Rare Diseases/epidemiology , Rare Diseases/therapy , Respiratory Function Tests/methods , Risk Factors
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