Isolated pulmonary artery arising from a duct: a single-center review of diagnostic and therapeutic strategies.

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.