Treatment of pulmonary hypertension secondary to connective tissue diseases

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Abstract
Patients displaying pulmonary hypertension have a restricted pulmonary circulation. Any increase in cardiac output can precipitate worsening of the pulmonary hypertension and right heart failure. Furthermore, the addition of hypoxic pulmonary hypertension will increase pulmonary artery pressures and therefore the load on the right heart. In this patient population exercise should be restricted and guided by symptoms. High altitude should be avoided because of hypoxic vasoconstriction. Warm baths should be taken carefully because the induced cutaneous vasodilation may dramatically decrease cardiac output. Pregnancy is contraindicated because haemodynamic physiological changes may precipitate the patient to fatal right sided heart failure.11 12 Contraception is therefore always recommended in women of childbearing age (usually mechanical or progestative contraception). Lastly, surgical procedures including open lung biopsies should be avoided as much as possible.