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Prostacyclin Therapy as Adjunctive Treatment for Pulmonary Hypertension in Pediatric Heart Transplantation: A Case
Anusha Konduri1, Aly J Sonnen1, Heang M Lim1
1University of Michigan, Congenital Heart Center, Ann Arbor, Michigan, United States of America.
Insights
Aggressive pulmonary hypertension treatment, including prostacyclin therapy, can make high-risk pediatric heart transplant candidates eligible. This approach improves outcomes for children with congenital heart disease and elevated pulmonary vascular resistance.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Pulmonary Hypertension
Background:
- Elevated pulmonary vascular resistance (PVR) is a major risk factor for right-ventricle failure and poor outcomes in pediatric heart transplantation.
- Pulmonary vasodilator therapy is crucial for managing symptoms and improving transplant candidacy.
- Prostacyclin therapy's role in this context is less understood compared to other vasodilators.
Observation:
- A 15-year-old female with Shone's complex presented with heart failure and severe pulmonary hypertension, with PVR initially prohibitive for heart transplant.
- Stepwise treatment with tadalafil, ambrisentan, and selexipag, followed by inhaled prostacyclin perioperatively, improved PVR and pulmonary vasoreactivity.
- Post-transplant, recurrence of pulmonary hypertension necessitated subcutaneous treprostinil, but pressures later stabilized, allowing transition back to oral therapies.
Findings:
- Aggressive medical management, including prostacyclin therapy, enabled a high-risk pediatric patient to become eligible for heart transplantation.
- Pulmonary vascular resistance and vasoreactivity improved significantly with multi-drug vasodilator therapy.
- Tailored, ongoing management of pulmonary hypertension is critical for successful pediatric heart transplantation outcomes.
Implications:
- Elevated PVR, especially if responsive to vasodilators, should not be an absolute contraindication for pediatric heart transplantation.
- Prostacyclin therapy can be a valuable tool in managing complex pulmonary hypertension in pediatric heart transplant candidates.
- This case supports the use of aggressive medical strategies to improve outcomes in children with congenital heart disease and pulmonary hypertension undergoing transplantation.
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 therapies and continued to do well at 1 year post-transplantation.
Conclusion:
This case highlights that aggressive medical management, including prostacyclin therapy, can render even high-risk pediatric patients with elevated pulmonary vascular resistance eligible for heart transplant and improve post-operative outcomes. It supports emerging evidence that elevated pulmonary vascular resistance, particularly when responsive to vasodilators, should not be viewed as an absolute contraindication to heart transplantation and emphasizes the importance of tailored, ongoing pulmonary hypertension management in children with congenital heart disease.
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