title: Isolated pulmonary artery arising from a duct: a single-center review of diagnostic and therapeutic strategies.
abstract: OBJECTIVE
  Isolated pulmonary artery of ductal origin (IPADO) is a rare disease with diverse presentations. Diagnostic and therapeutic approaches vary widely given the low incidence. Reporting additional cases may help inform medical decision making.
  
  
  METHODS
  We reviewed diagnostic data--including echocardiography, catheterization, and MRI--and outcomes for all patients with IPADO at our institution.
  
  
  RESULTS
  Thirty-seven patients who met inclusion criteria were identified. The mean age at diagnosis was 3 months (range, 1 day-45 years). In 11 patients, the duct supplying the IPADO was patent (ie, patent ductus arteriosis [PDA]); the duct had closed (ligamentum) in the other 26 patients. When performed, catheterization delineated the anatomy in 90% (100% if PDA) versus 54% with magnetic resonance imaging. Patients with a PDA were more likely to undergo intervention (100% vs 58%, P=.02) and had earlier first intervention (1 vs 20 months; P<.001). Patients diagnosed at age≤6 months were more likely to undergo intervention (86% vs 50%; P=.03) and unifocalization (81% vs 44%; P=.04), and had greater IPADO flow at follow-up (40% vs 14%; P<.001). Patients who underwent any intervention had greater IPADO flow than those without intervention (38% vs 0%).
  
  
  CONCLUSIONS
  Early IPADO diagnosis is important in long-term outcome. However, successful interventions can be performed on older patients. Diagnosis relies on angiography but magnetic resonance imaging may play an increasingly important role. Although initial intervention depends on individual factors, the ultimate goal should be early unifocalization.
authors: Department of Cardiology, Boston Children's Hospital, Boston, Mass; Department of Pediatrics, Harvard Medical School, Boston, Mass; Division of Pediatric Cardiology, Batson Children's Hospital, Jackson, Miss; Department of Pediatrics, University of Mississippi Medical Center, Jackson, Miss. Electronic address: sbatlivala@umc.edu.
authors: Sarosh P
authors: SP
authors: Batlivala
authors: Department of Cardiology, Boston Children's Hospital, Boston, Mass; Department of Pediatrics, Harvard Medical School, Boston, Mass.
authors: Doff B
authors: DB
authors: McElhinney
authors: Department of Cardiac Surgery, Boston Children's Hospital, Boston, Mass; Department of Surgery, Harvard Medical School, Boston, Mass.
authors: Frank A
authors: FA
authors: Pigula
authors: Department of Cardiology, Boston Children's Hospital, Boston, Mass; Department of Pediatrics, Harvard Medical School, Boston, Mass.
authors: Audrey C
authors: AC
authors: Marshall
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D001900:Boston; D006328:Cardiac Catheterization; D006348:Cardiac Surgical Procedures / Q000009:adverse effects; D002648:Child; D002675:Child, Preschool; D004373:Ductus Arteriosus / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000601:surgery*; D004374:Ductus Arteriosus, Patent / Q000175:diagnosis / Q000628:therapy; D042241:Early Diagnosis; D004452:Echocardiography; D005260:Female; D006330:Heart Defects, Congenital / Q000175:diagnosis* / Q000503:physiopathology / Q000628:therapy*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000601:surgery*; D011652:Pulmonary Circulation; D011859:Radiography; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult