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Cardiopulmonary, vascular and sudden death are the reasons for the majority of young adult mortality in SCD. To better understand the clinical importance of multi\u2010level vascular dysfunction, in 2009 we assessed cardiac function including tricuspid regurgitant jet velocity (TRV), tissue velocity in systole(S\u2032) and diastole (E\u2032), inflammatory, rheologic and hemolytic biomarkers as predictors of mortality in patients with SCD. With up to 9\u2009years of follow up, we determined survival in 95 children, adolescents and adults with SCD. Thirty\u2010eight patients (40%) were less than 21\u2009years old at initial evaluation. Survival and Cox proportional\u2010hazards analysis were performed. There was 19% mortality in our cohort, with median age at death of 35\u2009years. In the pediatric subset, there was 11% mortality during the follow up period. The causes of death included cardiovascular and pulmonary complications in addition to other end\u2010organ failure. On Cox proportional\u2010hazards analysis, our model predicts that a 0.1 m\/s increase in TRV increases risk of mortality 3%, 1 cm\/s increase in S\u2032 results in a 91% increase, and 1 cm\/s decrease in E\u2032 results in a 43% increase in mortality. While excluding cardiac parameters, higher plasma free hemoglobin was significantly associated with risk of mortality (p=.049). In conclusion, elevated TRV and altered markers of cardiac systolic and diastolic function predict mortality in a cohort of adolescents and young adult patients with SCD. These predictors should be considered when counseling cardiovascular risk and therapeutic optimization at transition to adult providers.<\/jats:p>","DOI":"10.1002\/ajh.26003","type":"journal-article","created":{"date-parts":[[2020,9,18]],"date-time":"2020-09-18T05:45:45Z","timestamp":1600407945000},"page":"31-39","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["Tricuspid regurgitant jet velocity and myocardial tissue Doppler parameters predict mortality in a cohort of patients with sickle cell disease spanning from pediatric to adult age groups \u2010 revisiting this controversial concept after 16\u2009years of additional evidence"],"prefix":"10.1002","volume":"96","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5211-6169","authenticated-orcid":false,"given":"Payal","family":"Shah","sequence":"first","affiliation":[{"name":"General Pediatrics Children's Hospital Los Angeles  Los Angeles California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silvie","family":"Suriany","sequence":"additional","affiliation":[{"name":"Hematology\/Oncology Children's Hospital Los Angeles  Los Angeles California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roberta","family":"Kato","sequence":"additional","affiliation":[{"name":"Pulmonology Children's Hospital Los Angeles  Los Angeles California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adam M.","family":"Bush","sequence":"additional","affiliation":[{"name":"Radiology Stanford University  Palo Alto California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9023-7903","authenticated-orcid":false,"given":"Patjanaporn","family":"Chalacheva","sequence":"additional","affiliation":[{"name":"Biomedical Engineering University of Southern California  Los Angeles California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Saranya","family":"Veluswamy","sequence":"additional","affiliation":[{"name":"Hematology\/Oncology Children's Hospital Los Angeles  Los Angeles California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3070-1775","authenticated-orcid":false,"given":"Christopher C.","family":"Denton","sequence":"additional","affiliation":[{"name":"Hematology\/Oncology Children's Hospital Los Angeles  Los Angeles California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kelly","family":"Russell","sequence":"additional","affiliation":[{"name":"Hematology\/Oncology Children's Hospital Los Angeles  Los Angeles California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maha","family":"Khaleel","sequence":"additional","affiliation":[{"name":"Hematology\/Oncology Children's Hospital Los Angeles  Los Angeles California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Henry J.","family":"Forman","sequence":"additional","affiliation":[{"name":"University of Southern California  Los Angeles California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael C. 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