Causes of deterioration in patients with PAH
Common causes of acute deterioration in PAH patients are listed here:
- Infection
- Arrhythmia
- Discontinuation of disease-targeted therapy
- Bleeding
- Haemoptysis
- Pulmonary artery aneurysms, rupture, dissection and compression of different intrathoracic structures such as the left main coronary artery
- Anaemia, renal or liver failure
- Thyroid disease
- Pulmonary arterial thrombosis and/or infarction
- Psychological and/or social stress
- Extremes of temperature and humidity
- Co-morbidities
- Unprovoked progression of PAH
- Development of PVOD which could not be identified previously.
End-of-life issues in pulmonary hypertension
Patients with pulmonary hypertension may die suddenly, or progressive heart failure may lead to clinical deterioration and death. Cardiopulmonary resuscitation tends to have poor outcomes in those with severe pulmonary hypertension.1
At the time of the initial diagnosis, the prognosis needs to be discussed openly and sensitively according to what the patient wishes to know. When appropriate, “do not resuscitate” orders should be discussed with the patient.
Those approaching the end of life may need specialist palliative care.
Learning point
- Patient support systems must be established and made accessible to all patients throughout the course of their disease and especially towards the end of life
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References
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7 British Thoracic Society recommendations
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22 Galie N, Ghofrani HA, Torbicki A et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 2005;353:2148–57. http://dx.doi.org/10.1056/NEJMoa050010
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32 McLaughlin VV, Oudiz RJ, Frost A et al. Randomized study of adding inhaled iloprost to existing bosentan in pulmonary arterial hypertension. Am J Respir Crit Care Med 2006;174:1257–63. http://dx.doi.org/10.1164/rccm.200603-358OC
33 Simonneau G, Rubin L, Galie N et al, for the PACES study group. Addition of sildenafil to long-term intravenous epoprostenol therapy in patients with pulmonary arterial hypertension. Ann Intern Med 2008;149:521–30.
34 Galie N, Brundage B, Ghofrani A et al. Tadalafil therapy for pulmonary arterial hypertension. Circulation 2009;119:2894–903. http://dx.doi.org/10.1161/CIRCULATIONAHA.108.839274
35 Tongers J, Schwerdtfeger B, Klein G et al. Incidence and clinical relevance of supraventricular tachyarrhythmias in pulmonary hypertension. Am Heart J 2007;153:127–32.
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Suggested reading:
Ghofrani HA, Voswinckel R, Reichenberger G et al. Differences in hemodynamic and oxygenation responses to three different phosphodiesterase-5 inhibitors in patients with pulmonary arterial hypertension: a randomized prospective study. J Am Coll Cardiol 2004;44:1488–96.
Michelakis ED, Tymchak W, Noga M et al. Long-term treatment with oral sildenafil is safe and improves functional capacity and hemodynamics in patients with pulmonary arterial hypertension. Circulation 2003;108:2066–9. http://dx.doi.org/10.1161/01.CIR.0000099502.17776.C2
Keogh AM, Mayer E, Benza RL et al. Interventional and surgical modalities of treatment in pulmonary hypertension. J Am Coll Cardiol 2009;54:S67–S77. http://dx.doi.org/10.1016/j.jacc.2009.04.016
Christie JD, Edwards LB, Aurora P et al. Registry of the International Society for Heart and Lung Transplantation: twenty-fifth official adult lung and heart/lung transplantation report—2008. J Heart Lung Transplant 2008;27:957–69. http://dx.doi.org/10.1016/j.healun.2008.07.018
Toyoda Y, Thacker J, Santos R et al. Long-term outcome of lung and heart-lung transplantation for idiopathic pulmonary arterial hypertension. Ann Thorac Surg 2008;86:1116–22. http://dx.doi.org/10.1016/j.athoracsur.2008.05.049
Clapp JF III, Capeless E. Cardiovascular function before, during and after the first and subsequent pregnancies. Am J Cardiol 1997;80:1469–73. http://dx.doi.org/10.1016/S0002-9149(97)00738-8
Further reading
Pulido, et al. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med 2013;369:809–18. http://dx.doi.org/10.1056/NEJMoa1213917
Ghofrani, et al. Riociguat for the treatment for pulmonary arterial hypertension. N Eng J Med 2013;369:330–40. http://dx.doi.org/10.1056/NEJMoa1209655
Simonneau, et al. Addition of sildenafil to long term intravenous epoprostenol therapy in patients with pulmonary arterial hypertension. Ann Intern Med 2008;149:521–30.
Simonneau et al. Selexipag, an oral, selective prostacyclin receptor agonist for the treatment of pulmonary arterial hypertension. Eur Respir J 2012;40:874–80 . http://dx.doi.org/10.1183/09031936.00137511
McLaughlin et al. Effect of selexipag on morbidity/mortality in pulmonary arterial hypertension: results of the GRIPHON study. J Am Coll Cardiol 2015;65(Suppl A):A380. http://dx.doi.org/10.1016/S0735-1097(15)61538-8
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