Author + information
- Received August 6, 2018
- Revision received November 15, 2018
- Accepted November 30, 2018
- Published online February 4, 2019.
- Gurumurthy Hiremath, MDa,∗ (, )
- Athar M. Qureshi, MDb,
- Lourdes R. Prieto, MDc,
- Lakshmi Nagaraju, MDd,
- Phillip Moore, MDe,
- Lisa Bergersen, MD, MPHf,
- Nathaniel W. Taggart, MDg and
- Jeffery Meadows, MDe
- aDepartment of Pediatrics, Masonic Children’s Hospital, University of Minnesota, Minneapolis, Minnesota
- bDepartment of Pediatrics, Texas Children's Hospital and Baylor College of Medicine, Houston, Texas
- cDepartment of Pediatrics, Nicklaus Children's Hospital, Miami, Florida
- dDepartment of Pediatrics, Children’s Hospital of San Antonio, San Antonio, Texas
- eDepartment of Pediatrics, UCSF Benioff Children's Hospital and the University of California, San Francisco, San Francisco, California
- fDepartment of Cardiology, Boston Children's Hospital, Boston, Massachusetts
- gDepartment of Pediatrics and Adolescent Medicine, Mayo Clinic, Rochester, Minnesota
- ↵∗Address for correspondence:
Dr. Gurumurthy Hiremath, Department of Pediatrics, Masonic Children’s Hospital, University of Minnesota, 2450 Riverside Avenue, East Building, Room MB555, Minneapolis, Minnesota 55454.
Objectives This study sought to determine whether pulmonary artery intervention in patients with unilateral proximal pulmonary artery stenosis (PAS) improves exercise capacity, abnormal ventilatory response to exercise, and symptoms.
Background Stenosis of the branch pulmonary arteries results in pulmonary blood flow maldistribution (PBFM). The resulting ventilation–perfusion mismatch is associated with an increased ventilatory response to exercise and decreased exercise capacity. It is unclear if technical success in relieving branch PAS translates to clinical improvement in exercise capacity and ventilatory response.
Methods Twenty patients with biventricular circulation and a minimum 10% PBFM who underwent transcatheter relief of PAS were enrolled in a multi-institutional prospective cohort study. Pre- and post-procedure assessment of the degree of PBFM, exercise capacity, ventilatory response to exercise, and subjective assessment of breathlessness were collected and analyzed.
Results Technical success was achieved in all patients with significant angiographic improvement in minimal lumen diameter (p = 0.001) and peak gradient (p = 0.001). Median PBFM improved (19.5% [range 12.0% to 31.0%] before vs. 7.0% [range 0% to 33.0%] after; p = 0.003). Exercise capacity was low at baseline and improved significantly post-intervention; percent predicted peak oxygen consumption improved from 70% (range 45% to 96%) to 83% (range 47% to 121%) (p = 0.02). Percent predicted oxygen pulse improved (p = 0.02). Ventilatory response to exercise improved; ventilatory equivalent of carbon dioxide slope post-intervention decreased to 29.3 versus 32.5 pre-intervention (p = 0.01). Subjective assessment of dyspnea improved. Five patients with minimal improvement in PBFM also showed minimal improvement in exercise parameters.
Conclusions Successful relief of unilateral branch PAS results in significant improvements in exercise capacity, ventilatory efficiency, and symptoms.
Dr. Qureshi has served as a consultant for WL Gore and Associates and Edwards Lifesciences. Dr. Bergersen has served as a consultant for 480 Biomedical Inc. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.
- Received August 6, 2018.
- Revision received November 15, 2018.
- Accepted November 30, 2018.
- 2019 American College of Cardiology Foundation
This article requires a subscription or purchase to view the full text. If you are a subscriber or member, click Login or the Subscribe link (top menu above) to access this article.