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Case Reports
. 2025 Aug;29(5):e70129.
doi: 10.1111/petr.70129.

Prostacyclin Therapy as Adjunctive Treatment for Pulmonary Hypertension in Pediatric Heart Transplantation: A Case Report

Anusha Konduri  1 , Aly J Sonnen  1 , Heang M Lim  1 , Kurt R Schumacher  1 , David M Peng  1 , Vikram Sood  1 , Mary K Olive  1 , Amanda D McCormick  1
Affiliations
Case Reports

Prostacyclin Therapy as Adjunctive Treatment for Pulmonary Hypertension in Pediatric Heart Transplantation: A Case Report

Anusha Konduri et al. Pediatr Transplant. 2025 Aug.

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 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.

Keywords: pediatric heart transplantation; prostacyclin; pulmonary hypertension.

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Conflict of interest statement

The authors have nothing to report.

References

    1. Ofori‐Amanfo G., Hsu D., Lamour J. M., et al., “Heart Transplantation in Children With Markedly Elevated Pulmonary Vascular Resistance: Impact of Right Ventricular Failure on Outcome,” Journal of Heart and Lung Transplantation 30, no. 6 (2011): 659–666, 10.1016/j.healun.2010.12.007. - DOI - PubMed
    1. Liden H., Haraldsson A., Ricksten S. E., Kjellman U., and Wiklund L., “Does Pretransplant Left Ventricular Assist Device Therapy Improve Results After Heart Transplantation in Patients With Elevated Pulmonary Vascular Resistance?,” European Journal of Cardio‐Thoracic Surgery 35, no. 6 (2009): 1029–1034, 10.1016/j.ejcts.2008.12.024. - DOI - PubMed
    1. Alba A. C., Rao V., Ross H. J., et al., “Impact of Fixed Pulmonary Hypertension on Post‐Heart Transplant Outcomes in Bridge‐To‐Transplant Patients,” Journal of Heart and Lung Transplantation 29, no. 11 (2010): 1253–1258, 10.1016/j.healun.2010.06.002. - DOI - PubMed
    1. Perez‐Villa F., Farrero M., Sionis A., Castel A., and Roig E., “Therapy With Sildenafil or Bosentan Decreases Pulmonary Vascular Resistance in Patients Ineligible for Heart Transplantation Because of Severe Pulmonary Hypertension,” Journal of Heart and Lung Transplantation 29, no. 7 (2010): 817–818, 10.1016/j.healun.2010.02.004. - DOI - PubMed
    1. Pons J., Leblanc M. H., Bernier M., et al., “Effects of Chronic Sildenafil Use on Pulmonary Hemodynamics and Clinical Outcomes in Heart Transplantation,” Journal of Heart and Lung Transplantation 31, no. 12 (2012): 1281–1287, 10.1016/j.healun.2012.09.009. - DOI - PubMed

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