Vinod A Sebastian1, Javier Brenes2, Raghav Murthy3, Surendranath Veeram Reddy4, V Vivian Dimas4, Alan Nugent4, Thomas Zellers4, Rong Huang5, Kristine J Guleserian2, Joseph M Forbess2. 1. Division of Pediatric Cardiothoracic Surgery, University of Texas Southwestern Medical Center, Dallas, Texas. Electronic address: vinod.sebastian@utsouthwestern.edu. 2. Division of Pediatric Cardiothoracic Surgery, University of Texas Southwestern Medical Center, Dallas, Texas. 3. Department of Cardiovascular and Thoracic Surgery, University of Texas Southwestern Medical Center, Dallas, Texas. 4. Division of Pediatric Cardiology, University of Texas Southwestern Medical Center and Children's Medical Center, Dallas, Texas. 5. Research Department, Children's Medical Center Dallas, Dallas, Texas.
Abstract
BACKGROUND: Historic outcomes of patients with heterotaxy and pulmonary atresia or pulmonary stenosis (PA/PS) have been poor and in the current era are incompletely described. We reviewed our management of these patients and associated risk factors for death. METHODS: We retrospectively reviewed the records of all patients with heterotaxy and PA/PS treated in our institution from January 1, 2002, to August 31, 2012. Death data were also confirmed with the Social Security Death Index. The log-rank test was done to assess six risk factors for death. RESULTS: We identified 42 patients with heterotaxy and PA/PS. Median age at the first operation was 6.5 days, and median follow-up was 3.5 years. Death data were complete for all patients. Overall mortality was 19% (8 of 42). The 30-day, 1-year, and 5-year mortality estimation was 4.76%, 12.3%, and 19.1% respectively, as determined by the Kaplan-Meier method. The log-rank test showed total anomalous pulmonary venous return (TAPVR) (p<0.05) and obstructed TAPVR requiring an operation at less than 30 days (p=0.001) were significant risk factors for death. CONCLUSIONS: In the current era, surgical treatment of heterotaxy and PA/PS can result in good outcomes. Associated TAPVR and obstructed TAPVR requiring neonatal correction were noted to be risk factors for death.
BACKGROUND: Historic outcomes of patients with heterotaxy and pulmonary atresia or pulmonary stenosis (PA/PS) have been poor and in the current era are incompletely described. We reviewed our management of these patients and associated risk factors for death. METHODS: We retrospectively reviewed the records of all patients with heterotaxy and PA/PS treated in our institution from January 1, 2002, to August 31, 2012. Death data were also confirmed with the Social Security Death Index. The log-rank test was done to assess six risk factors for death. RESULTS: We identified 42 patients with heterotaxy and PA/PS. Median age at the first operation was 6.5 days, and median follow-up was 3.5 years. Death data were complete for all patients. Overall mortality was 19% (8 of 42). The 30-day, 1-year, and 5-year mortality estimation was 4.76%, 12.3%, and 19.1% respectively, as determined by the Kaplan-Meier method. The log-rank test showed total anomalous pulmonary venous return (TAPVR) (p<0.05) and obstructed TAPVR requiring an operation at less than 30 days (p=0.001) were significant risk factors for death. CONCLUSIONS: In the current era, surgical treatment of heterotaxy and PA/PS can result in good outcomes. Associated TAPVR and obstructed TAPVR requiring neonatal correction were noted to be risk factors for death.
Authors: Thomas G Saba; Gabrielle C Geddes; Stephanie M Ware; David N Schidlow; Pedro J Del Nido; Nathan S Rubalcava; Samir K Gadepalli; Terri Stillwell; Anne Griffiths; Laura M Bennett Murphy; Andrew T Barber; Margaret W Leigh; Necia Sabin; Adam J Shapiro Journal: Orphanet J Rare Dis Date: 2022-09-09 Impact factor: 4.303