Pregnancy and contraception in PAH
Pregnancy in women with pulmonary arterial hypertension is associated with maternal mortality estimated at between 20 and 55%. PAH is a contra-indication to pregnancy38 and it is recommended that early termination should be offered if a woman becomes pregnant. With better treatment, more women with PAH want to become pregnant. Women with PAH should be counselled about the risks of pregnancy, and effective contraception should be offered at the time of diagnosis.
The choice of contraceptive preparation should be managed by a family planning doctor who understands the issues of PAH. Progesterone-only contraceptive preparations are one favoured option.39 (Note that bosentan may reduce the efficacy of hormonal contraceptive agents.) The Mirena coil is effective but the vasovagal reaction that may occur with device insertion can be hazardous in these patients. Sterilisation is rarely used in these patients because of the risks of surgery. Barrier contraceptives are safe for the patient, but less effective.
When a woman with PAH chooses to continue her pregnancy, she will need to be managed closely in a multidisciplinary setting, incorporating both a complex pregnancy team and a pulmonary hypertension team. She will need frequent visits and likely hospitalisation during the third trimester40 with planned elective delivery. The optimal mode of delivery remains a subject of debate. The beneficial effects of disease-targeted therapies for PAH in this population are uncertain.
Most maternal deaths occur within the first month from delivery. The main cause of peripartum death has been reported as refractory right heart failure.
Learning points
- Maternal mortality is high among pregnant women with PAH
- Reliable contraception is strongly advised in women with PAH
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
- 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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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
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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
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