Clinical manifestations and long-term follow-up in pediatric patients living at altitude with isolated pulmonary artery of ductal origin

Shinichi Takatsuki, Jeffrey R. Darst, Bibhuti B. Das, Thomas E. Fagan, Robert Wolfe, David Dunbar Ivy

Research output: Contribution to journalArticle

4 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)775-781
Number of pages7
JournalPediatric Cardiology
Volume33
Issue number5
DOIs
StatePublished - Jun 2012

Keywords

  • Altitude
  • High-altitude pulmonary edema
  • Isolated pulmonary artery of ductal origin
  • Pulmonary arterial hypertension
  • Unilateral absence of pulmonary artery

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Cardiology and Cardiovascular Medicine

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