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How to manage idiopathic pulmonary hypertension in an emergency

Session presentations
  • Pulmonary arterial hypertension with frequent syncope. Presented by J L Vachiery (Brussels, BE)See the slides
  • Emergency management of acute deterioration of pulmonary arterial hypertension. Presented by N Galie (Bologna, IT)
Pulmonary Hypertension

Pulmonary arterial hypertension (PAH) is a devastating disease characterized by a progressive occlusion of the pulmonary precapillary vascular bed,  with increased pulmonary artery pressure and resistance, leading to right ventricular failure and premature death. Evolution can be rapidly detrimental requiring rapid adequate therapeutic actions.

Dr Jean-Luc Vachiery presented the case of a young lady with mild dyspnoea and recurrent syncope at exercise. She underwent a diagnostic work up which concluded to idiopathic PAH and received an endothelin receptor antagonist as first line therapy. Risk stratification based on clinical, exercise and hemodynamic parameters was rather favourable except for the existence of syncope. Because of relapsing symptoms and poor hemodynamic evolution, a phosphodiesterase inhibitor and a parenteral prostacyclin analogue were successively added to the therapy. Patient was ultimately put on the lung transplantation waiting list and as a bridge to transplantation, an atrial septostomy was performed. Syncope is a presenting symptom in about 10% of the patients.  It may be more frequent in patients with reversible pulmonary hypertension and in children. Two important causes of syncope in patients with PAH, low cardiac output and arrhythmia, were reviewed. The most frequent arrhythmia’s faced by PAH patients are atrial flutter and atrial fibrillation observed in 20% of the patients. Both are ideally treated with cordarone or electric cardioversion. Severe precapillary pulmonary hypertension is responsible for a major dilation of the right ventricle, compromising left ventricle filling and output. The insufficient increase of cardiac output at exercise leads to a drop in systemic pressure and syncope. Atrial septostomy by restoring left ventricle filling is an efficient intervention to prevent syncope. The technical aspects and survival benefit of balloon dilation atrial septostomy were briefly discussed.

Dr Nazzareno Galie presented 3 short cases.
The first one was a  young female patient with rapid and progressive deterioration under mono-, double- and finally triple combination therapy presenting at the end with disastrous pulmonary hemodynamics (right atrial pressure of more than 15 mmHg, cardiac index lower than 2 L/min/m2). Patient had already been listed for transplantation at the start of the parenteral prostacyclin therapy.  In order to bridge her to transplantation a right ventricular assist device with oxygenator was placed with restoration of better left ventricular filling and output. Different assist strategies were briefly discussed.
The second case was a patient with portopulmonary hypertension consecutive to alcoholic cirrhosis. He presented with severe pulmonary hypertension contraindicating liver transplantation.  Both pulmonary hemodynamics and liver function dramatically improved under intravenous prostacyclin therapy.
The third case was a patient with an atypical presentation of very severe but partially reversible pulmonary hypertension under nitric oxide. During the reversibility testing pulmonary arterial pressure did not decrease under 40 mmHg, but right atrial pressure and cardiac output normalized. Calcium channel blockers were progressively uptitrated with further clinical and hemodynamic improvements. The session was highly interactive and feed-back from the audience confirmed the limited access to atrial septostomy and urgent lung transplantation in most of the countries.




How to manage idiopathic pulmonary hypertension in an emergency

The content of this article reflects the personal opinion of the author/s and is not necessarily the official position of the European Society of Cardiology.