Clinical Manifestations and Long-Term Follow-Up in Pediatric Patients Living at Altitude With Isolated Pulmonary Artery of Ductal Origin
- Resource Type
- Original Paper
- Authors
- Takatsuki, Shinichi; Darst, Jeffrey R.; Das, Bibhuti B.; Fagan, Thomas E.; Wolfe, Robert; Ivy, David Dunbar
- Source
- Pediatric Cardiology. June 2012 33(5):775-781
- Subject
- Isolated pulmonary artery of ductal origin
Unilateral absence of pulmonary artery
Altitude
High-altitude pulmonary edema
Pulmonary arterial hypertension
- Language
- English
- ISSN
- 0172-0643
1432-1971
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.