With respect to pulmonary hypertension patients with congenital heart disease:a. Pregnancy is well tolerated, making counselling on the topic unnecessary
Pulmonary hypertension is an important complication in congenital heart disease. At the most extreme end of the spectrum is Eisenmenger syndrome, defined by reversal of a left to right shunt due to the development of pulmonary hypertension. Venesection for patients with Eisenmenger syndrome is now avoided. A sinus venosus defect (and aberrant pulmonary venous drainage) should be considered in patients with a dilated right heart and PH but no other explanation. A transoesophageal echocardiogram can visualize the upper inter-atrial septum well. The development of PAH can make pregnancy hazardous, and the oral contraceptive pill can interact with bosentan. Therefore family planning is vital. The six-minute walk test has recognized limitations. Therefore a number of factors are looked at when trying to prognosticate.