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  • Airway Characteristics of Patients With 22q11 Deletion Undergoing Pulmonary Artery Reconstruction Surgery: Retrospective Cohort Study. Pediatric critical care medicine : a journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies Sganga, D., Meister, K., Sidell, D. R., Wise-Faberowski, L., Shek, J., Ma, M., Martin, E., Hanley, F. L., McElhinney, D., Asija, R. 2022

    Abstract

    OBJECTIVES: We have previously shown that patients with a chromosome 22q11 microdeletion are at risk for prolonged respiratory failure after pulmonary artery reconstruction surgery compared with those with normal genotype. We sought to describe preexisting airway abnormalities in this patient population and examine relationships between airway abnormalities and outcomes.DESIGN: Single-center retrospective chart review from Society of Thoracic Surgery and Pediatric Cardiac Critical Care Consortium databases and the electronic medical record.SETTING: Lucile Packard Children's Hospital at Stanford from September 2017 to February 2019.PATIENTS: All patients undergoing pulmonary artery reconstruction surgery were considered for inclusion.INTERVENTIONS: We identified 127 patients meeting study inclusion criteria. Thirty-nine patients met specific criteria and underwent screening preoperative bronchoscopy including microdirect laryngoscopy and lower airway examination. Postoperative bronchoscopy was performed at the discretion of the intensive care team.MEASUREMENTS AND MAIN RESULTS: Airway abnormalities were detected in 25/26 of children (96%) with a chromosome 22q11 deletion who underwent preoperative bronchoscopy. Upper and lower airway pathologies were found in 19/25 (73%) and 21/25 (81%) patients, respectively, and it was common for patients to have more than one abnormality. Presence of 22q11 deletion was associated with longer duration of mechanical ventilation (9.1 vs 4.3 d; p = 0.001), use of noninvasive positive pressure support (13 vs 6 d; p = 0.001), and longer hospital stays (30 vs 14 d; p = 0.002). These outcomes were worse when compared with patients with known airway abnormalities who did not have 22q11 deletion.CONCLUSIONS: Preexisting upper and lower airway pathologies are common in patients with a chromosome 22q11 deletion who undergo pulmonary artery reconstruction surgery. Despite similar postoperative hemodynamics and outcomes as their counterparts without 22q11 deletion, 22q11 deletion is associated with more postoperative respiratory complications not entirely explained by preexisting airway abnormalities.

    View details for DOI 10.1097/PCC.0000000000002921

    View details for PubMedID 35213412

  • Response to JHLT-D-21-00302 "Failing Fontan - heart or heart-liver transplant: The jury is (still) out?" JOURNAL OF HEART AND LUNG TRANSPLANTATION Chen, S., Bensen, R., Hollander, S. A., Sganga, D. 2021; 40 (10): 1248-1249
  • Response to JHLT-D-21-00302 "Failing Fontan - heart or heart-liver transplant: The jury is (still) out?" The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation Chen, S., Bensen, R., Hollander, S. A., Sganga, D. 2021

    View details for DOI 10.1016/j.healun.2021.06.015

    View details for PubMedID 34391657

  • Comparison of combined heart‒liver vs heart-only transplantation in pediatric and young adult Fontan recipients. The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation Sganga, D., Hollander, S. A., Vaikunth, S., Haeffele, C., Bensen, R., Navaratnam, M., McDonald, N., Profita, E., Maeda, K., Concepcion, W., Bernstein, D., Chen, S. 2020

    Abstract

    BACKGROUND: Indications for a heart‒liver transplantation (HLT) for Fontan recipients are not well defined. We compared listing characteristics, post-operative complications, and post-transplant outcomes of Fontan recipients who underwent HLT with those of patients who underwent heart-only transplantation (HT). We hypothesized that patients who underwent HLT have increased post-operative complications but superior survival outcomes compared with patients who underwent HT.METHODS: We performed a retrospective review of Fontan recipients who underwent HLT or HT at a single institution. Characteristics at the time of listing, including the extent of liver disease determined by laboratory, imaging, and biopsy data, were compared. Post-operative complications were assessed, and the Kaplan‒Meier survival method was used to compare post-transplant survival. Univariate regression analyses were performed to identify the risk factors for increased mortality and morbidity among patients who underwent HT.RESULTS: A total of 47 patients (9 for HLT, 38 for HT) were included. Patients who underwent HLT were older, were more likely to be on dual inotrope therapy, and had evidence of worse liver disease. Whereas ischemic time was longer for the group who underwent HLT, post-operative complications were similar. Over a median post-transplant follow-up of 17 (interquartile range: 5-52) months, overall mortality for the cohort was 17%; only 1 patient who underwent HLT died (11%) vs 7 patients who underwent HT (18%) (p = 0.64). Among patients who underwent HT, cirrhosis on pre-transplant imaging was associated with worse outcomes.CONCLUSIONS: Despite greater inotrope need and more severe liver disease at the time of listing, Fontan recipients undergoing HLT have post-transplant outcomes comparable with those of patients undergoing HT. HLT may offer a survival benefit for Fontan recipients with liver disease.

    View details for DOI 10.1016/j.healun.2020.12.008

    View details for PubMedID 33485775