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Clinical Manifestations and Long-Term Follow-Up in Pediatric Patients Living at Altitude With Isolated Pulmonary Artery of Ductal Origin

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Abstract

This study’s aim was to define the clinical manifestations and long-term outcome of pediatric patients living at altitude with isolated pulmonary artery (PA) of ductal origin (IPADO). This was a retrospective cohort study of 17 consecutive cases of IPADO at a single center. All patients lived at modest altitude (median 2050 m [range 1700 m to 3050 m]). Fifteen children (88%) were symptomatic at presentation. High-altitude pulmonary edema was present in 2 patients (12%) at diagnosis, and only 1 patient had episodes of hemoptysis during follow-up. Fourteen patients (82%) demonstrated evidence of pulmonary arterial hypertension (PAH). Among 14 patients with PAH, 11 patients had surgical interventions. PAH resolved in 5 of 11 patients (45%) undergoing surgical rehabilitation. One patient died during follow-up, and 7 patients are receiving oral vasodilator therapies due to residual PAH; 14 patients remained asymptomatic. Our study showed that early intervention in patients with IPADO at modest altitude can potentially rehabilitate the isolated PA and reverse PAH. Whether surgery is indicated for patients with this disorder in the absence of PAH is unknown.

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Acknowledgements

This study was supported by the Jayden DeLuca Foundation, the Leah Bult Foundation, UL1 RR025780 Colorado Clinical Translational Science Institute, National Center for Research Resources, and National Institutes of Health. D. D. I. serves as a member of the Gilead Sciences Research Scholars Program. The University of Colorado Denver receives fees from Actelion, Gilead, Pfizer, and United Therapeutics for D. D. I. consultant work.

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Authors and Affiliations

  1. Section of Cardiology, Department of Pediatrics, Children’s Hospital Colorado, University of Colorado Denver, Aurora, CO, USA

    Shinichi Takatsuki, Jeffrey R. Darst, Thomas E. Fagan, Robert Wolfe & David Dunbar Ivy

  2. Section of Cardiology, Department of Pediatrics, Children’s Medical Center, University of Texas Southwestern, Dallas, TX, USA

    Bibhuti B. Das

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  1. Shinichi Takatsuki
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  2. Jeffrey R. Darst
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  3. Bibhuti B. Das
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  4. Thomas E. Fagan
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  5. Robert Wolfe
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  6. David Dunbar Ivy
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Correspondence to Shinichi Takatsuki.

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Takatsuki, S., Darst, J.R., Das, B.B. et al. Clinical Manifestations and Long-Term Follow-Up in Pediatric Patients Living at Altitude With Isolated Pulmonary Artery of Ductal Origin. Pediatr Cardiol 33, 775–781 (2012). https://doi.org/10.1007/s00246-012-0213-z

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  • DOI: https://doi.org/10.1007/s00246-012-0213-z

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