Rehabilitation of pulmonary artery in congenital unilateral absence of intrapericardial pulmonary artery
Received 13 March 2009; received in revised form 12 July 2009; accepted 12 September 2009. published online 20 July 2010. Corrected Proof
Objective
We evaluated the efficacy of the early rehabilitation of remnant pulmonary artery in unilateral absent intrapericardial pulmonary artery and the factors affecting pulmonary artery growth.
Methods
We retrospectively reviewed the medical records and imaging modalities of 15 patients with unilateral absent intrapericardial pulmonary artery (7 left and 8 right; median age at diagnosis, 5 months) from 1991 to 2008.
Results
The remnant pulmonary artery was found in 12 patients (mean diameter, 2.6 ± 0.7 mm) at the hilum. Eleven patients underwent operation (main pulmonary artery flap angioplasty in 5 patients; tube graft interposition in 6 patients), and 4 patients were inoperable. Transcatheter balloon angioplasty or stent implantation was required for the remaining pulmonary artery stenosis in 6 patients (55%). The last ipsilateral lung perfusion proportion at lung perfusion scan was 39% (range, 15%–51%), and the Z value of the last ipsilateral pulmonary artery diameter was −0.5 (range, 4.2 to 2). The patients with a smaller initial remnant pulmonary artery required more interventions (P = .003). The final perfusion proportion of affected lung was higher in the patients treated early (≤6 months, n = 7) than in those treated late (>6 months, n = 4) (41.9% ± 8.5% vs 24.9% ± 10.7%, respectively, P = .024). The patients with graft interposition showed a lower perfusion proportion of affected lung than those with main pulmonary artery flap angioplasty (P = .017).
Conclusion
In patients with unilateral absent intrapericardial pulmonary artery, early and aggressive management of combined surgical reconstruction and transcatheter intervention improved pulmonary artery growth and lung perfusion.
aDepartment of Pediatrics, Seoul National University Children's Hospital, Seoul, South Korea
bDepartment of Pediatrics, Pusan National University Hospital, Busan, South Korea
cDepartment of Thoracic and Cardiovascular Surgery, Seoul National University Children's Hospital, Seoul, South Korea
Address for reprints: Eun Jung Bae, MD, PhD, Department of Pediatrics, Seoul National University Children's Hospital, 101 Daehang-Ro, Jongno-gu, Seoul 110-744, South Korea.
ABSTRACT