Rehabilitation of pulmonary artery in congenital unilateral absence of intrapericardial pulmonary artery

Kim, Gi Beom; Ban, Ji Eun; Bae, Eun Jung; Noh, Chung Il; Kim, Woong Han; Lee, Jeong Ryul; Kim, Yong Jin

doi:10.1016/j.jtcvs.2009.09.072

« Previous Next »The Journal of Thoracic and Cardiovascular Surgery
Volume 141, Issue 1 , Pages 171-178, January 2011

Rehabilitation of pulmonary artery in congenital unilateral absence of intrapericardial pulmonary artery

Gi Beom Kim, MD
- Department of Pediatrics, Seoul National University Children's Hospital, Seoul, South Korea
Ji Eun Ban, MD
- Department of Pediatrics, Pusan National University Hospital, Busan, South Korea
Eun Jung Bae, MD, PhD
- Department of Pediatrics, 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.
Chung Il Noh, MD, PhD
- Department of Pediatrics, Seoul National University Children's Hospital, Seoul, South Korea
Woong Han Kim, MD, PhD
- Department of Thoracic and Cardiovascular Surgery, Seoul National University Children's Hospital, Seoul, South Korea
Jeong Ryul Lee, MD, PhD
- Department of Thoracic and Cardiovascular Surgery, Seoul National University Children's Hospital, Seoul, South Korea
Yong Jin Kim, MD, PhD
- Department of Thoracic and Cardiovascular Surgery, Seoul National University Children's Hospital, Seoul, South Korea

Received 13 March 2009; received in revised form 12 July 2009; accepted 12 September 2009. published online 20 July 2010.

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).

Conclusions

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.

CTSNet classification: 20, 21

Abbreviations and Acronyms: LPS, lung perfusion scan, NYHA, New York Heart Association, PA, pulmonary artery, TOF, tetralogy of Fallot, UAPA, unilateral absence of the intrapericardial pulmonary artery, VSD, ventricular septal defect