Article by: T. Everett Jones, M.D., Yong G. Peng, M.D., Ph.D., FASE, FASA
A 16-year-old girl with familial pulmonary arterial hypertension (PAH) presented to our
institution for consideration of bilateral lung transplant. She was small for her age because of
her chronic medical condition and had been taking pulmonary vasodilators for the past several
Concomitant severe PAH and RV systolic dysfunction present a significant clinical challenge for
cardiothoracic anesthesiologists, who should be aware of the potential side effects of various
PAH treatment options. A balanced anesthetic technique and appropriate vasoactive selection
are essential to the success of lung transplant in this patient population.