Pregnancy and pulmonary hypertension: a practical approach to management

Abstract

Pulmonary hypertension remains a major cause of cardiac maternal death in the developed world. Over the last two decades, effective therapies for pulmonary hypertension have been developed, improving symptoms and survival. Consequently, increasing numbers of women with pulmonary hypertension and childbearing potential exist, with a number considering pregnancy. Patients with pulmonary hypertension may also present for the first time during pregnancy or shortly following delivery. The last decade has seen increasing reports of women with pulmonary hypertension surviving pregnancy using a variety of approaches but there is still a significant maternal mortality at between 12% and 33%. Current recommendations counsel that patients with known pulmonary hypertension should be strongly advised to avoid pregnancy with the provision of clear contraceptive advice and termination of pregnancy should be considered in its eventuality. In patients who are fully informed and who have been counselled regarding the risks of continuing with pregnancy, there is growing evidence that a multi-professional approach with expert care in pulmonary hypertension centres may improve outlook, although the mortality remains high.

Keywords: Pulmonary hypertension; chronic thromboembolic pulmonary hypertension; maternal mortality; pregnancy.

Figure 1.

Overview of the classification of pulmonary hypertension reproduced from the BMJ, Kiely et al. Patients for whom specific therapies aimed at the pulmonary vasculature are known to improve outcome and are shown on the left hand side; in pulmonary arterial hypertension (PAH)-specific pharmacological therapies aimed at the pulmonary vasculature improves outcome; in chronic thromboembolic pulmonary hypertension (CTEPH), major surgery with pulmonary endarterectomy can be curative and for selected patients pharmacological therapies may improve outcome. For patients on the right hand side of the diagram, there is no current evidence that drug therapies will improve outlook and indeed this may worsen symptoms.

Figure 2.

Example of a chest X-ray (CXR) and electrocardiogram from a patient presenting with severe pulmonary arterial hypertension at our unit who was successfully delivered at 34 weeks. The CXR shows cardiomegaly with enlargement of the proximal pulmonary arteries. The electrocardiogram shows classic changes of severe pulmonary arterial hypertension with right axis deviation, dominance of the “R” wave in V1 and widespread ST depression/T wave inversion in the inferior and anterior leads.

Figure 3.

Example of a CT pulmonary angiogram from a patient with severe pulmonary arterial hypertension. Enlargement of the pulmonary artery (PA) compared to a normal size aorta (Ao) and dilation of the right atrium (RA) and right ventricle (RV) with posterior displacement of the inter-ventricular septum (IVS) and compression of the left ventricle (LV) can be seen.

Figure 4.

Overview of the management plan for patients with pulmonary hypertension presenting with suspected pulmonary hypertension at the authors’ institution published in BJOG.

Figure 5.

Example of a continuous tracing available from LiDCO from a patient with severe pulmonary arterial hypertension taken at the time of delivery. This can be used to monitor patients around delivery and in post-partum period. This allows measurement of cardiac output, which is a key parameter in the peri-partum period. LiDCO: lithium dilution cardiac output.