Prostacyclin Therapy as Adjunctive Treatment for Pulmonary Hypertension in Pediatric Heart Transplantation: A Case Report
Abstract
BACKGROUND: Elevated pulmonary vascular resistance is a significant risk factor for right-ventricle failure and poor outcomes following pediatric heart transplantation. Pulmonary vasodilator therapy can help manage symptoms and improve heart transplant candidacy. While phosphodiesterase type 5 inhibitors and endothelin receptor antagonists are commonly used, prostacyclin therapy remains less studied in this context.
CASE DESCRIPTION: A 15-year-old female with Shone's complex developed heart failure with preserved ejection fraction and severe pulmonary hypertension. Given her indexed pulmonary vascular resistance (15 indexed wood units with vasodilator testing) on initial cardiac catheterizations was felt to be prohibitive to heart transplant, she was treated stepwise with tadalafil, ambrisentan, and selexipag. Serial cardiac catheterizations showed an improvement in her pulmonary vascular resistance as well as vasoreactivity of her pulmonary vasculature. She was listed for heart transplant and underwent transplantation with perioperative support including inhaled prostacyclin. Post-operatively, her mean pulmonary artery pressure and pulmonary vascular resistance improved, though she later required a transition to subcutaneous treprostinil for recurrence of pulmonary hypertension with right-ventricle failure despite only a mildly elevated left ventricle end-diastolic pressure. Over the following year, her pulmonary pressures stabilized, and she was transitioned back to oral…
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