**Author details**

thromboendarterectomy is the treatment of choice because it improves hemodynamics,

Elective surgery involves an increased risk in patients with PAH. The increased risk is proportionate to the severity of the disease. It is not clear which type of anesthesia is advisable, but probably local and regional anesthesia are better tolerated than general anesthesia. Surgery preferably is performed at referral centers with experienced anesthesia and pulmonary hypertension teams that can deal with potential complications.[36],[53] Anticoagulant treatment should be interrupted for as short a period as possible. In patients with CTEPH,

Although successful pregnancies have been reported in PAH patients, pregnancy and delivery in PAH patients are associated with an increased mortality rate of 30% to 50%, and pregnancy should be avoided or terminated. An appropriate method of birth control is highly recom‐ mended in all women with pulmonary hypertension who have childbearing potential. Unfortunately, there is no current consensus on the most appropriate birth control method in PAH patients. Because of the increased risk of thrombosis with estrogen-based contraception, some experts suggest the use of estrogen-free products, surgical sterilization, or barrier

PAH has no cure. However, the rate of progression is highly variable and depends upon the type and severity of the PAH. Untreated PAH leads to right-sided heart failure and death.

Prior to the 1990s, therapeutic options were limited. The emergence of prostacyclin analogues, endothelin receptor antagonists, phosphodiesterase-5 inhibitors, and other novel drug

For untreated IPAH, the estimated 3-year survival rate is approximately 41%. In one study of long-term continuous intravenous prostacyclin therapy, 3-year survival increased to approx‐ imately 63%. [54] With newer therapies, perhaps in combination, these figures are expected to

Less symptomatic patients in WHO class II/III, with normal right atrial and ventriclar size and pressure and can walk more than 400 meters on 6 minute walk distance (MWD) are considered lower risk group of patients for morbidity and mortality. While symptomatic patients in WHO

therapies has greatly improved the outlook for patients with PAH.

bridging with heparin is recommended to minimize the time off anticoagulation.

functional status, and survival. [35],[37]

**11.1. Surgery**

14 Pulmonary Hypertension

**11.2. Pregnancy**

methods.[24], [35]

improve further.

**12. Natural history and prognosis**

**11. Considerations for special populations**

Dr Saleem Sharieff MBBS, FCPS, FRCPC1,2

1 Grand River Hospital, Kitchener, ON, Canada

2 McMaster University Hospital, Hamilton, ON, Canada
