390 Bronchiolitis Obliterans Syndrome (BOS) Is Not Specific for Bronchiolitis Obliterans (BO) in Pediatric Lung Transplant (LTx)

390 Bronchiolitis Obliterans Syndrome (BOS) Is Not Specific for Bronchiolitis Obliterans (BO) in Pediatric Lung Transplant (LTx)

S134 The Journal of Heart and Lung Transplantation, Vol 30, No 4S, April 2011 logic diagnosis it often mandates performing an open lung biopsy. BOS i...

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S134

The Journal of Heart and Lung Transplantation, Vol 30, No 4S, April 2011 logic diagnosis it often mandates performing an open lung biopsy. BOS is a clinical diagnosis based on spirometric data that is the accepted standard for staging chronic allograft dysfunction. The use of predicted values for pediatric recipients has not been validated. We determined the sensitivity, specificity, positive and negative predictive values (PPV and NPV) of the BOS stages for predicting BO in children. Methods and Materials: A chart review was conducted on the 139 open lung biopsies and 43 lung explants performed at our center from 1990 through June 2010 on pediatric LTx recipients. Data collected included date of LTx, age at LTx, gender, date of biopsy/explant, result of biopsy/explant, best 2 previous FEV1 percent predicted taken at least 3 weeks apart and simultaneous FEF 25-75 percent predicted, and 2 most recent FEV1 percent predicted taken at least 4 weeks apart and simultaneous FEF 25-75 percent predicted. Results were excluded from analysis if insufficient data existed to calculate a stable BOS stage prior to biopsy/explant. Sensitivity, specificity, PPV and NPV were then determined for patients meeting the minimum requirements for the BOS stages (i.e. patients in BOS stage 2 were also included in BOS stage 1 and 0p analysis). Results: 67 open lung biopsies and 31 lung explants met criteria for inclusion in the study of which 41 (61.2%) and 26 (83.9%) had BO respectively. Sensitivity, specificity, PPV and NPV are reviewed in Table 1.

Sensitivity Specificity PPV NPV

BOS 3

BOS 2

BOS 1

BOS 0p

49.3% 74.2% 80.5% 40.4%

65.7% 51.6% 74.6% 41.0%

91.0% 25.8% 72.6% 57.1%

97.0% 9.7% 69.9% 60.0%

Conclusions: We found that early declines in lung function are sensitive but not specific for BO. The low specificity for BOS stage to identify BO illustrates the challenge facing clinicians in determining the etiology of pulmonary decline following LTx.

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Figure 1. Left column: patient with mild CAV. Right column: patient with severe CAV Row (a) XA: normal Row (b) IVUS: (left) mild intimal thickening. (right) severe thickening Row (c) IVUS: segmentation of intima (green) Row (d) CMRA Row (e) Overlay of LGE (arrows) on CMRA Conclusions: Direct non-invasive CMRA and LGE in children with CAV is feasible. Correlation of MIT with LGE appears to be very good. These promising results warrant larger studies to confirm the utility of this technique. This approach may enable closer follow-up and better prevention of CAV.

390 Bronchiolitis Obliterans Syndrome (BOS) Is Not Specific for Bronchiolitis Obliterans (BO) in Pediatric Lung Transplant (LTx) C. Towe,1 A.C. Ogborn,2 T. Ferkol,1 S. Sweet,1 C. Huddleston,3 A. Faro.1 1Pediatrics, Washington University, St. Louis, MO; 2 Pediatrics, University of New Mexico, Albuquerque, NM; 3 Cardiothoracic Surgery, Washington Unviersity, St. Louis, MO. Purpose: BO is the leading cause of mortality beyond the first year after transplant in pediatric LTx recipients. Since BO is a histo-

Lung Transplantation Is a Viable Treatment Option in Patients with Congenital or Acquired Pulmonary Vein Stenosis A. Bharat,1 D.J. Epstein,1 A. Faro,2 P. Michelson,2 S.C. Sweet,2 C.B. Huddleston.1 1Cardiothoracic Surgery, Children’s Hospital, St Louis, MO; 2Pulmonary Medicine, Children’s Hospital, St Louis, MO. Purpose: Congenital or acquired pulmonary vein stenosis (PVS) is associated with high mortality (3-year survival ⬍50%) because surgical repair is usually not feasible or ineffective. Lung transplant has been proposed as an option but the long-term outcomes are unknown. Methods and Materials: A retrospective review of prospectively maintained database. Multivariate analysis was performed using SPSS software. Results: Between 1990 and 2010, we transplanted 20 patients with PVS. Nine had acquired PVS from prior repair for anomalous pulmonary return. The median wait time for transplant was 26 days. The mean age at transplant was 1.1⫾0.89 yrs, and gender F:M (12:8). Fifteen (75%) patients had either percutaneous or open intervention prior to transplant. All patients had bilateral transplant on cardiopulmonary bypass. Four (20%) patients were on ECMO pre-transplant and 3 (15%) continued to be on ECMO post-op. The mean ICU stay was 33.5⫾29.1 days and the hospital stay was 58.7⫾43.5 days. The 30-day mortality was 10%. Three (15%) patients required re-transplant. ECMO in the perioperative period was the only predictor of 30-day and 1-yr mortality (HR 3.6 & 5.6, p⫽0.01). Overall 5-year survival was 59.8% (Congenital 67.3% Vs Acquired 50.7%).[figure1]