Close Follow-Up - Center of Excellence and Additional Therapies

Patients with pulmonary hypertension should undergo close follow-up. Since pulmonary arterial hypertension is a rare disease with a high morbidity and mortality, patients should be managed at or in collaboration with a pulmonary arterial hypertension center of excellence. They should undergo follow-up imaging studies, 6-minute walk tests and repeat right heart catheterization to assess the efficacy of medical therapy. None of these patients should be started on medical therapy without a right heart catheterization to determine hemodynamics.

On follow-up evaluation of our patient, she noted that her symptoms had gradually improved, making lung transplant listing unnecessary at this time. In addition, she was advised against futher pregnancies, as the stress of pregnency is poorly tolerated by women with idiopathic pulmonary arterial hypertension, and sudden deterioration, particularly in the postpartum period can be fatal. She was also advised against using oral contraceptives for birth control, since their use may exacerbate pulmonary hypertension.