Original Article

Pediatric Cardiology

, Volume 33, Issue 5, pp 775-781

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

  • Shinichi TakatsukiAffiliated withSection of Cardiology, Department of Pediatrics, Children’s Hospital Colorado, University of Colorado Denver Email author 
  • , Jeffrey R. DarstAffiliated withSection of Cardiology, Department of Pediatrics, Children’s Hospital Colorado, University of Colorado Denver
  • , Bibhuti B. DasAffiliated withSection of Cardiology, Department of Pediatrics, Children’s Medical Center, University of Texas Southwestern
  • , Thomas E. FaganAffiliated withSection of Cardiology, Department of Pediatrics, Children’s Hospital Colorado, University of Colorado Denver
  • , Robert WolfeAffiliated withSection of Cardiology, Department of Pediatrics, Children’s Hospital Colorado, University of Colorado Denver
  • , David Dunbar IvyAffiliated withSection of Cardiology, Department of Pediatrics, Children’s Hospital Colorado, University of Colorado Denver

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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.

Keywords

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