Kurt R Schumacher1, Sunkyung Yu2, Ryan Butts3, Chesney Castleberry4, Sharon Chen5, Erik Edens6, Justin Godown7, Jonathan Johnson8, Mariska Kemna9, Kimberly Lin10, Ray Lowery2, Kathleen Simpson4, Shawn West11, Ivan Wilmot12, Jeffrey G Gossett13. 1. University of Michigan Congenital Heart Center, Ann Arbor, Michigan, USA. Electronic address: kurts@med.umich.org. 2. University of Michigan Congenital Heart Center, Ann Arbor, Michigan, USA. 3. University of Texas-Southwestern Children's Medical Center Dallas, Dallas, Texas, USA. 4. Washington University, St. Louis Children's Hospital, St. Louis, Missouri, USA. 5. Stanford University, Lucile Packard Children's Hospital, Palo Alto, California, USA. 6. University of Iowa, Iowa City, Iowa, USA. 7. Vanderbilt University, Monroe Carell Chidren's Hospital, Nashville, Tennessee, USA. 8. Mayo Clinic College of Medicine, Rochester, Minnesota, USA. 9. Seattle Children's Hospital, Seattle, Washington, USA. 10. Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA. 11. Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania, USA. 12. Cincinnati Children's Medical Center, Cincinnati, Ohio, USA. 13. University of California‒San Francisco Benioff Children's Hospital, San Francisco, California, USA.
Abstract
BACKGROUND: The influence of Fontan-associated protein-losing enteropathy's (PLE) severity, duration, and treatment on heart transplant (HTx) outcomes is unknown. We hypothesized that long-standing PLE and PLE requiring more intensive therapy are associated with increased post-HTx mortality. METHODS: This 12-center, retrospective cohort study of post-Fontan patients with PLE referred for HTx from 2003 to 2015 involved collection of demographic, medical, surgical, and catheterization data, as well as PLE-specific data, including duration of disease, intensity/details of treatment, hospitalizations, and complications. Factors associated with waitlist and post-HTx outcomes and PLE resolution were sought. RESULTS: Eighty patients (median of 5 per center) were referred for HTx evaluation. Of 68 patients listed for HTx, 8 were removed due to deterioration, 4 died waiting, and 4 remain listed. In 52 patients undergoing HTx, post-HTx 1-month survival was 92% and 1-year survival was 83%. PLE-specific factors, including duration of PLE pre-HTx, pre-HTx hospitalizations, need for/frequency of albumin replacement, PLE therapies, and growth parameters had no association with post-HTx mortality. Immunosuppressant regimen was associated with mortality; standard mycophenolate mofetil immunotherapy was used in 95% of survivors compared with only 44% of non-survivors (p = 0.03). Rejection (53%) and infection (42%) post-HTx were common, but not associated with PLE-specific factors. PLE resolved completely in all but 1 HTx survivor at a median of 1 month (interquartile range 1 to 3 months); resolution was not affected by PLE-specific factors. CONCLUSIONS: PLE severity, duration, and treatment do not influence post-HTx outcome, but immunosuppressive regimen may have an impact on survival. PLE resolves in nearly all survivors.
BACKGROUND: The influence of Fontan-associated protein-losing enteropathy's (PLE) severity, duration, and treatment on heart transplant (HTx) outcomes is unknown. We hypothesized that long-standing PLE and PLE requiring more intensive therapy are associated with increased post-HTx mortality. METHODS: This 12-center, retrospective cohort study of post-Fontan patients with PLE referred for HTx from 2003 to 2015 involved collection of demographic, medical, surgical, and catheterization data, as well as PLE-specific data, including duration of disease, intensity/details of treatment, hospitalizations, and complications. Factors associated with waitlist and post-HTx outcomes and PLE resolution were sought. RESULTS: Eighty patients (median of 5 per center) were referred for HTx evaluation. Of 68 patients listed for HTx, 8 were removed due to deterioration, 4 died waiting, and 4 remain listed. In 52 patients undergoing HTx, post-HTx 1-month survival was 92% and 1-year survival was 83%. PLE-specific factors, including duration of PLE pre-HTx, pre-HTx hospitalizations, need for/frequency of albumin replacement, PLE therapies, and growth parameters had no association with post-HTx mortality. Immunosuppressant regimen was associated with mortality; standard mycophenolate mofetil immunotherapy was used in 95% of survivors compared with only 44% of non-survivors (p = 0.03). Rejection (53%) and infection (42%) post-HTx were common, but not associated with PLE-specific factors. PLE resolved completely in all but 1 HTx survivor at a median of 1 month (interquartile range 1 to 3 months); resolution was not affected by PLE-specific factors. CONCLUSIONS: PLE severity, duration, and treatment do not influence post-HTx outcome, but immunosuppressive regimen may have an impact on survival. PLE resolves in nearly all survivors.
Authors: Ja-Kyoung Yoon; Gi Beom Kim; Mi Kyoung Song; Sang Yun Lee; Seong Ho Kim; So Ick Jang; Woong Han Kim; Chang-Ha Lee; Kyung Jin Ahn; Eun Jung Bae Journal: Korean Circ J Date: 2022-03-16 Impact factor: 3.101