Optimising the management of pulmonary arterial hypertension patients: emergency treatments

Abstract

Pulmonary arterial hypertension (PAH) is a rare and potentially fatal disease whose management is usually restricted to a few specialised centres. As patients do not necessarily live in the neighbourhood of these centres, daily care and emergencies have to be delegated to first and second lines. Treatment guidelines do not usually provide recommendations for acute emergency situations as evidence is scarce. This short review provides a description of our therapeutic protocols based on available data.A model of transmural organisation of care for PAH patients, currently applied in Belgium, is described. Thereafter, based on an analysis of the reasons of death in the PAH population, a review of the main emergencies is provided. Cardiac arrest and resuscitation, decompensated right heart failure, respiratory failure, arrhythmia, pericardial effusion, haemoptysis, surgery and drug-related adverse events will be discussed successively.Case reports showing the precariousness of PAH patients will enforce our thesis of the need for optimal patient management organisation.

Major bleeding with vitamin K antagonist anticoagulants in pulmonary hypertension

“…There was no difference in mean right atrial pressure between the different groups of patients, hence no hint of a predominance of liver congestion in any of these groups, which could otherwise have explained a higher risk of gastrointestinal bleeding. The occurrence of haemoptysis in patients with pulmonary hypertension is well known, but is hardly ever reported in patients using VKAs for atrial fibrillation or venous thromboembolism (table 7) [26]. Haemoptysis occurred predominantly in IPAH patients.…”

Complications Leading to Sudden Cardiac Death in Pulmonary Arterial Hypertension

“…3 Since the bronchial circulation is the major source of hemoptysis, in selected patients therapeutic embolization can be life-saving. 105 In order to embolize the responsible vessel, a detailed angiography of the bronchial and pulmonary vascular tree is required.…”

“…3 Summary PAH is a rare and severe disease characterized by pulmonary vascular remodeling, leading to right heart failure and premature death. LMCS must be taken into consideration in PAH patients with angina, as well as in those without symptoms but with high-risk anatomy, as in case of severe pulmonary artery dilatation.…”

“…In the American National Institute of Health registry, 106 deaths were reported in a cohort of 194 patients with idiopathic PAH, of which 26% were sudden. 3 Likewise, 99 out of 316 patients died in the Leuven database during follow-up, of whom 18 suddenly. 3 We review the etiology, preventive measures, and management of complications associated with sudden cardiac death among PAH patients.…”

Antithrombotics in pulmonary hypertension: more work needed before we turn to newer agents!

“…Moreover, patients receiving prostacyclins should be followed with caution because of its antiplatelet effects, particularly if they also have thrombocytopenia. In the context of haemoptysis complicating PAH, bronchial artery embolisation remains the treatment of choice and withdrawal of VKA should be proposed in absence of formal indication [21]. Secondly, evaluation of antithrombotic therapy modalities should be discussed on an individual basis, with clinicians experimenting in the usual field of antithrombotic prescription.…”