Combination Chemotherapy in Severe Pulmonary Vein Stenosis—A Case Series

Krivenko, Gabriel; Iacono, Karen; Nykanen, David; Keen, Robyn; Sutphin, Robert; Farias, Michael

doi:10.3390/children10020364

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Combination Chemotherapy in Severe Pulmonary Vein Stenosis—A Case Series

by

Gabriel Krivenko

¹

,

Karen Iacono

²,

David Nykanen

²,

Robyn Keen

²,

Robert Sutphin

³

and

Michael Farias

^2,4,*

¹

School of Medicine, University of Central Florida, Orlando, FL 32837, USA

²

The Heart Center, Orlando Health Arnold Palmer Hospital for Children, Orlando, FL 32806, USA

³

Division of Hematology/Oncology, Orlando Health Arnold Palmer Hospital for Children, Orlando, FL 32806, USA

⁴

Department of Cardiology, Boston Children’s Hospital, Boston, MA 02115, USA

^*

Author to whom correspondence should be addressed.

Children 2023, 10(2), 364; https://doi.org/10.3390/children10020364

Submission received: 17 December 2022 / Revised: 20 January 2023 / Accepted: 9 February 2023 / Published: 11 February 2023

(This article belongs to the Special Issue Pulmonary Vein Stenosis in Neonates and Infants: Etiology, Diagnosis and Management)

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Abstract

Pulmonary vein stenosis results from a proliferative process that leads to the progressive obstruction of venous return to the left atrium. It is often resistant to catheterization and surgical based interventions and is frequently fatal when encountered in its severe form. Here, we describe three patients with severe, primary pulmonary vein stenosis that was progressing despite aggressive conventional management strategies. All three patients were initiated on combination chemotherapy with imatinib and sirolimus, drugs which have been previously shown to independently have potential benefit against PVS. Soon after the initiation of these therapies, all three patients experienced a stabilization of their disease process and clinical improvement. All three patients remain alive, with tolerable side effects from the medications. Although early in our experience and with only a small number of patients, combination chemotherapy with imatinib and sirolimus shows promise and merits further investigation as a therapeutic option for this aggressive disease.

Keywords: pulmonary vein stenosis; children; imatinib; sirolimus

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MDPI and ACS Style

Krivenko, G.; Iacono, K.; Nykanen, D.; Keen, R.; Sutphin, R.; Farias, M. Combination Chemotherapy in Severe Pulmonary Vein Stenosis—A Case Series. Children 2023, 10, 364. https://doi.org/10.3390/children10020364

AMA Style

Krivenko G, Iacono K, Nykanen D, Keen R, Sutphin R, Farias M. Combination Chemotherapy in Severe Pulmonary Vein Stenosis—A Case Series. Children. 2023; 10(2):364. https://doi.org/10.3390/children10020364

Chicago/Turabian Style

Krivenko, Gabriel, Karen Iacono, David Nykanen, Robyn Keen, Robert Sutphin, and Michael Farias. 2023. "Combination Chemotherapy in Severe Pulmonary Vein Stenosis—A Case Series" Children 10, no. 2: 364. https://doi.org/10.3390/children10020364

APA Style

Krivenko, G., Iacono, K., Nykanen, D., Keen, R., Sutphin, R., & Farias, M. (2023). Combination Chemotherapy in Severe Pulmonary Vein Stenosis—A Case Series. Children, 10(2), 364. https://doi.org/10.3390/children10020364

Note that from the first issue of 2016, this journal uses article numbers instead of page numbers. See further details here.

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Combination Chemotherapy in Severe Pulmonary Vein Stenosis—A Case Series

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