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389 Detection and Grading of Coronary Allograft Vasculopathy in Children Using Late Gadolinium Enhancement MRI
Abstracts 386 Donors Characteristics and Impact on Outcomes in Pediatric Heart Transplant Recipients J. Conway,1 C. Chin,2 M. Kemna,3 M. Burch,6 A. Barnes,4 M.A. Tresler,7 J.N. Scheel,8 D.C. Naftel,7 K. Beddow,5 T. Allain-Rooney,1 A.I. Dipchand.1 1Labatt Family Heart Centre, University of Toronto, Toronto, ON, Canada; 2Division of Pediatric Cardiology, Stanford University, Standford, CA; 3Division of Pediatric Cardiology, Seattle Children’s Hospital, Seattle, WA; 4Division of Cardiology, Children’s Medical Center, Dallas, TX; 5Pediatric Heart Transplant, Children’s Hospital of New York Presbyterian, New York, NY; 6Division of Cardiology, Great Ormond Street Hospital for Children, London, United Kingdom; 7Department of Surgery, University of Alabama at Birmingham, Birmingham, AL; 8Division of Pediatric Cardiology, Johns Hopkins Hospital, Baltimore, MD. Purpose: Organ availability and acceptability limit pediatric heart transplantation (HTx). There is a lack of literature on pediatric donors, and it remains unclear what defines a marginal pediatric donor. The purpose of this study was to define the characteristics of pediatric donors and determine the donor risk factors that affect recipient survival. Methods and Materials: Data from the PHTS, a prospective multicenter study that includes 35 institutions, was used to examine the impact of donor factors on the outcomes of patients listed ⬍ 18 yrs old who received a HTx between 1993-2009. Results: Donor data was available for 3122/3132 HTx (99.6%). Median donor age was 4.2 years (0-53). Donors were male (57%), white race (68.4%) and blood group O (59%). Head trauma (55%) and anoxia (30%) were the main causes of death. Normal echocardiogram (echo) was reported in 96% with 69% on inotropes. Mean ischemic time (IT) was 222 mins (27-627), and longer IT significantly impacted survival (p⫽0.03). Cause of death & need for inotropes or CPR did not effect outcomes (pⱖ0.05). Combination of any of the latter 4 donor factors did not impact survival (p⫽0.7). In univariate analysis, donor risk factors for early death post HTx were younger age (for those Tx at ⬍6m of age;p⫽0.008), older age (for those Tx at ⬎10yrs,p⫽0.0003), body surface area (p⬍0.0001) and IT (p⬍0.0001). Multivariate analysis (adjusted for recipient factors) identified younger donor age (p⫽0.03), inotropic support (p⫽0.03) and longer IT (p⫽0.04) as risk factors for early death. When stratified by recipient age at HTx, the only significant risk factors identified in those ⬍6 m were a non-normal donor echo (p⫽0.03) and younger donor age (p⫽0.02). No donor risk factors for early death were identified by multivariate analysis in those Tx ⬎10 years of age. Conclusions: This analysis of the largest cohort of pediatric donors ever reported, shows that factors that we traditionally use to consider a donor as high risk may be age dependant and may not adequately define a marginal pediatric donor. 387 WITHDRAWN 388 Outcomes after Percutaneous Coronary Artery Revascularization Procedures for Allograft Vasculopathy in Pediatric Heart Transplant Recipients: A Multi-Institutional Experience A. Jeewa,1 C. Chin,2 E. Pahl,3 A.M. Atz,4 M.P. Carboni,5 M.A. Tresler,6 D.C. Naftel,6 R.J. Rodriguez,7 T. Allain-Rooney,1 A.I. Dipchand.1 1The Hospital for Sick Children, Toronto, Canada; 2Stanford University, Stanford; 3Children’s Memorial Hospital, Chicago; 4MUSC Children’s Hospital, Charleston; 5Duke University Medical Center, Durham; 6 University of Alabama at Birmingham, Birmingham; 7Morgan Stanley Children’s Hospital of New York Presbyterian, New York. Purpose: Coronary allograft vasculopathy (CAV) is a major contributor to long term graft loss after heart transplant (HTx). In adult recipients, percutaneous revascularization procedures (PRP) have variable success with high restenosis rates and no impact on graft survival. Limited experience is reported in children. The aim of this study was to examine the multicenter experience with PRP in pediatric HTx recipients.
S133 Methods and Materials: Data from the Pediatric Heart Transplant Study, a prospective multicenter study that includes 35 institutions, was used to examine PRP and their impact on post-HTx outcomes in patients listed {18 yrs old who received a first HTx between 1993-2009 (n⫽3,156). Results: A total of 46 PRPs were done in 22 patients at 14/35 centers. Mean recipient age at time of HTx was 8.6 yrs (⫾6.9); mean donor age was 15.9 yrs (0.7-48.7). Mean time to first PRP was 5.3 years (⫾2.9). Vessels involved were left anterior descending artery (46%), right coronary artery (20%), circumflex artery(17%) or other coronary branches (17%); and PRPs consisted of 15 (33%) balloon angioplasties; 29 (63%) stent implantations and 2 other PRPs. Freedom from graft loss post-PRP was 86%, 67% and 48% at 1,3 and 12 mos (Fig 1).[figure1]
Conclusions: In the largest cohort reported in the literature to date, PRPs for CAV in pediatric HTx recipients were rare, likely related to procedural feasibility and amenability of the lesions to intervention. Despite technically successful interventions, graft loss was found to occur in 52% within 1 year post-procedure. This data supports a role for concurrent re-listing for heart transplant in these patients despite apparent procedural success. 389 Detection and Grading of Coronary Allograft Vasculopathy in Children Using Late Gadolinium Enhancement MRI T. Hussain,1 G. Greil,1 A. Taylor,2 V. Muthurangu,2 M. Fenton,2 R. Botnar,1 M. Burch.2 1Imaging Sciences, King’s College London, London, United Kingdom; 2Great Ormond Street Hospital, London, United Kingdom. Purpose: Coronary Allograft Vasculopathy (CAV) is the leading cause of late death after heart transplantation in children. It is poorly detected by conventional angiography (XA). Intravascular ultrasound (IVUS) is invasive, costly and is not feasible in small children. The purpose here is to show that MRI late gadolinium enhancement (LGE) of the coronary vessel wall can detect and grade CAV. Methods and Materials: Participants underwent coronary angiography, IVUS and MRI. IVUS was performed in the left anterior descending (LAD) artery. Maximal intimal thickness (MIT) and mean intimal index (MII) was recorded. MRI included coronary magnetic resonance angiogram (CMRA) and LGE vessel wall imaging. The volume of enhancement was quantified on LGE images fused with CMRA as a roadmap. Results: 11 children (5 male) participated (mean age of 16yrs (12-17 yrs)). The mean follow up was 4.5 years post-transplant (3 months-12 years). One IVUS failed due to technical reasons. MIT was 0.54 ⫾0.31mm and MII was 18.4 ⫾5.8%. Mean volume of vessel wall LGE was 39 ⫾72mm3/cm of vessel imaged. Correlation of LGE with MIT using Pearson’s coefficient was 0.91 (p⬍0.01) and with MII was 0.60 (p⫽0.069).
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.
391
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]
S133 Methods and Materials: Data from the Pediatric Heart Transplant Study, a prospective multicenter study that includes 35 institutions, was used to examine PRP and their impact on post-HTx outcomes in patients listed {18 yrs old who received a first HTx between 1993-2009 (n⫽3,156). Results: A total of 46 PRPs were done in 22 patients at 14/35 centers. Mean recipient age at time of HTx was 8.6 yrs (⫾6.9); mean donor age was 15.9 yrs (0.7-48.7). Mean time to first PRP was 5.3 years (⫾2.9). Vessels involved were left anterior descending artery (46%), right coronary artery (20%), circumflex artery(17%) or other coronary branches (17%); and PRPs consisted of 15 (33%) balloon angioplasties; 29 (63%) stent implantations and 2 other PRPs. Freedom from graft loss post-PRP was 86%, 67% and 48% at 1,3 and 12 mos (Fig 1).[figure1]
Conclusions: In the largest cohort reported in the literature to date, PRPs for CAV in pediatric HTx recipients were rare, likely related to procedural feasibility and amenability of the lesions to intervention. Despite technically successful interventions, graft loss was found to occur in 52% within 1 year post-procedure. This data supports a role for concurrent re-listing for heart transplant in these patients despite apparent procedural success. 389 Detection and Grading of Coronary Allograft Vasculopathy in Children Using Late Gadolinium Enhancement MRI T. Hussain,1 G. Greil,1 A. Taylor,2 V. Muthurangu,2 M. Fenton,2 R. Botnar,1 M. Burch.2 1Imaging Sciences, King’s College London, London, United Kingdom; 2Great Ormond Street Hospital, London, United Kingdom. Purpose: Coronary Allograft Vasculopathy (CAV) is the leading cause of late death after heart transplantation in children. It is poorly detected by conventional angiography (XA). Intravascular ultrasound (IVUS) is invasive, costly and is not feasible in small children. The purpose here is to show that MRI late gadolinium enhancement (LGE) of the coronary vessel wall can detect and grade CAV. Methods and Materials: Participants underwent coronary angiography, IVUS and MRI. IVUS was performed in the left anterior descending (LAD) artery. Maximal intimal thickness (MIT) and mean intimal index (MII) was recorded. MRI included coronary magnetic resonance angiogram (CMRA) and LGE vessel wall imaging. The volume of enhancement was quantified on LGE images fused with CMRA as a roadmap. Results: 11 children (5 male) participated (mean age of 16yrs (12-17 yrs)). The mean follow up was 4.5 years post-transplant (3 months-12 years). One IVUS failed due to technical reasons. MIT was 0.54 ⫾0.31mm and MII was 18.4 ⫾5.8%. Mean volume of vessel wall LGE was 39 ⫾72mm3/cm of vessel imaged. Correlation of LGE with MIT using Pearson’s coefficient was 0.91 (p⬍0.01) and with MII was 0.60 (p⫽0.069).
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.
391
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]