Pulmonary Hypertension and Pregnancy

Pulmonary hypertension is a term used to describe a complex multifactorial group of conditions diagnosed by an elevated mean pulmonary artery pressure of 20 mm Hg or higher on right heart catheterization. The diagnosis of pulmonary hypertension in pregnancy is important, as it is associated with high rates of maternal morbidity and mortality, even with modern management. Diagnostic testing is important for establishing the diagnosis, type, and severity of pulmonary hypertension, which in turn, dictates treatment options. Echocardiographic assessment is the first step in diagnosis and the gold standard for monitoring right heart function in patients with pulmonary hypertension. Supportive therapy for pulmonary hypertension includes monitored exercise, vaccination, and avoidance of certain activities. Therapies for pulmonary hypertension are considered conventional or targeted. Conventional therapy includes preventative care, anticoagulation, and calcium channel blockers for appropriate patients. Targeted therapy is usually reserved for patients with World Health Organization group 1 pulmonary arterial hypertension (including idiopathic, heritable, drug-induced or associated with congenital heart disease) and involves different types of direct pulmonary vasodilators. Right heart failure is the end result of pulmonary hypertension and the options for management include medical optimization, support with extracorporeal membrane oxygenation, and combined heart-lung transplantation. With pregnancy, management must be individualized, and patients should be cared for as part of an experienced multidisciplinary team. There are few studies addressing, timing and mode of delivery, including anesthetic considerations. In this review, the natural history of pulmonary hypertension in pregnancy and outcomes are summarized and current evidence-based management is discussed.