Pregnancy and Pulmonary Hypertension

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Physiology

The physiologic changes of pregnancy are poorly tolerated by women with PAH. During pregnancy, cardiac output increases by 50% from baseline. This is accomplished by several mechanisms. Blood volume increases in the early stages, leading to an increase in stroke volume. This is further enhanced by a reduction in afterload secondary to decreased peripheral vascular resistance.2 Later in pregnancy, cardiac output is augmented by an increase in heart rate. Under normal conditions, there is a

Classification of PH

According to the latest World Health Organization criteria, PAH is defined by a mean pulmonary artery (PA) pressure greater than 25 mm Hg at rest, a normal PA occlusion pressure (≤15 mm Hg), and increased PVR.5 Current categorization of PAH includes patients with idiopathic or heritable PH, PH associated with congenital heart disease, as well as PH in the setting of connective tissue disease, human immunodeficiency virus (HIV), portal hypertension, anorexigen use, or pulmonary venoocclusive

Diagnosis and screening

The availability and improved technique of transthoracic echocardiography (TTE) has led to its widespread use as a screening tool for PH. Good correlation has been demonstrated between PA pressures measured by right heart catheterization (RHC) and the right ventricular systolic pressure measured by TTE, but many factors, such as advanced lung disease, premature ventricular contractions, and inaccurate estimates of right atrial pressure in the modified Bernoulli equation, can lead to

Pregnancy outcomes and mortality

PH complicates a relatively small number of pregnancies. The true incidence of PH in pregnancy has not been reported, but studies have attempted to estimate the effect of PH on maternal outcomes. Between 2002 and 2004, there were an estimated 14 million hospitalizations related to pregnancy, based on extrapolations from the Nationwide Inpatient Sample database.10 Of these, 407 (0.003%) were to the result of a diagnosis of idiopathic pulmonary arterial hypertension (IPAH). In this study, PAH was

PH developing during pregnancy

The onset of PH during pregnancy can be a first manifestation of previously undiagnosed or asymptomatic PH, which becomes unmasked under the stress of pregnancy. PH can also develop acutely during the course of pregnancy. The presence of severe dyspnea, syncope, or chest pain during pregnancy should prompt immediate investigation. The diagnosis of acute pulmonary embolism should always be suspected in such patients. If excluded, a TTE should be done to look for cardiac disease, including PH. If

Conception counseling

Despite possible improvements in pregnancy outcomes in woman with PH, the risk of death is still high enough to warrant a recommendation to avoid pregnancy. Contraception is indicated in women of child-bearing age with PH, but the modality must be chosen carefully. Combined hormonal contraceptives (oral, implanted, or transdermal) increase the risk of thrombosis, and are therefore contraindicated in woman with PH in whom this could represent a life-threatening complication. In patients with

Management of PH during pregnancy

For many different reasons, neither contraception nor termination of pregnancy is an option for some patients. Although it is unclear to what extent current strategies used to manage PH in pregnancy have had an effect on mortality, successful outcomes have been described with a variety of medical and surgical approaches. When a woman with known PH becomes pregnant, or if PH is newly discovered during pregnancy, the patient should be referred to a center that has expertise in managing PH.

Summary and recommendations

The diagnosis of PH by TTE is not uncommon in pregnancy. Because of the high output state of pregnancy, PH on a TTE should never be misconstrued as diagnostic of PAH. In approximately one-third of cases, the TTE finding represents a false-positive. Those with true arteriopathy and PAH are at increased risk for mortality as a result of the physiologic stresses of pregnancy, labor, and delivery. Recently published data that take newer therapies into account suggest that the mortality of PH in

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