Erika B Rosenzweig1, Angela Bates2, Mary P Mullen3,4, Steven H Abman5, Eric D Austin6, Allen Everett7, Jeffrey Fineman8, Jeffery Feinstein9, Rachel K Hopper9, John P Kinsella10, Usha S Krishnan1, Minmin Lu3, Kenneth D Mandl11, J Usha Raj12, Nidhy Varghese13, Delphine Yung14, Stephanie S Handler15, Lynn A Sleeper3,4. 1. Division of Pediatric Cardiology, Department of Pediatrics, Columbia University Irving Medical Center, New York, New York. 2. Division of Cardiology, Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada. 3. Department of Cardiology, Boston Children's Hospital. 4. Department of Pediatrics, and. 5. Pediatric Heart Lung Center, Department of Pediatrics, and. 6. Department of Pediatrics, Monroe Carell Jr. Children's Hospital, Vanderbilt University Medical Center, Nashville, Tennessee. 7. Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, Maryland. 8. Division of Critical Care, Department of Pediatrics, University of California, San Francisco, San Francisco, California. 9. Department of Pediatrics, Lucile Packard Children's Hospital Stanford, Stanford University School of Medicine, Palo Alto, California. 10. Division of Neonatology, Department of Pediatrics, University of Colorado Denver Anschutz Medical Center and Children's Hospital Colorado, Aurora, Colorado. 11. Computational Health Informatics Program, Departments of Pediatrics and Biomedical Informatics, Harvard Medical School, Boston, Massachusetts. 12. Department of Pediatrics, University of Illinois at Chicago, Chicago, Illinois. 13. Department of Pediatrics, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas. 14. Department of Pediatrics, University of Washington, Seattle, Washington; and. 15. Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin.
Abstract
Rationale: Hemodynamic assessments direct care among children with pulmonary hypertension, yet the use of cardiac catheterization is highly variable, which could impact patient care and research. Objectives: We analyzed hemodynamic findings from right heart catheterization (RHC) and left heart catheterization and acute vasodilator testing (AVT) and the safety of catheterization in children with World Symposium on Pulmonary Hypertension (WSPH) group 1 and 3 subtypes in a large multicenter North American cohort. Methods: Of 1,475 children enrolled in the Pediatric Pulmonary Hypertension Network Registry (2014-2020), there were 1,383 group 1 and 3 patients, of whom 671 (48.5%) underwent RHC at diagnosis and were included for analysis. Results: Compared with those without diagnostic RHC, these children were older, less likely to be an infant or preterm, more often female, treated with targeted pulmonary hypertension medications at diagnosis, and had advanced World Health Organization functional class. Catheterization was performed without a difference in complication rates between WSPH groups. Pulmonary capillary wedge pressure was well correlated with left ventricular end-diastolic pressure and left atrial pressures. Results of AVT using three different methods were comparable; positive AVT results were observed in 8.0-11.8% of subjects, did not differ between WSPH groups 1 and 3, and were not associated with freedom from the composite endpoint of lung transplantation or death during follow-up. Conclusions: In a large pediatric pulmonary hypertension cohort, diagnostic RHC with or without left heart catheterization in WSPH group 1 and 3 patients was performed safely at experienced pediatric pulmonary hypertension centers. Hemodynamic differences were noted between group 1 and 3 subjects. Higher mean pulmonary arterial pressure and mean pulmonary arterial pressure/mean systemic arterial pressure ratio were associated with a higher risk of death/transplantation. Findings suggest overall safety and potential value of RHC as a standard diagnostic approach to guide pulmonary hypertension management in children.
Rationale: Hemodynamic assessments direct care among children with pulmonary hypertension, yet the use of cardiac catheterization is highly variable, which could impact patient care and research. Objectives: We analyzed hemodynamic findings from right heart catheterization (RHC) and left heart catheterization and acute vasodilator testing (AVT) and the safety of catheterization in children with World Symposium on Pulmonary Hypertension (WSPH) group 1 and 3 subtypes in a large multicenter North American cohort. Methods: Of 1,475 children enrolled in the Pediatric Pulmonary Hypertension Network Registry (2014-2020), there were 1,383 group 1 and 3 patients, of whom 671 (48.5%) underwent RHC at diagnosis and were included for analysis. Results: Compared with those without diagnostic RHC, these children were older, less likely to be an infant or preterm, more often female, treated with targeted pulmonary hypertension medications at diagnosis, and had advanced World Health Organization functional class. Catheterization was performed without a difference in complication rates between WSPH groups. Pulmonary capillary wedge pressure was well correlated with left ventricular end-diastolic pressure and left atrial pressures. Results of AVT using three different methods were comparable; positive AVT results were observed in 8.0-11.8% of subjects, did not differ between WSPH groups 1 and 3, and were not associated with freedom from the composite endpoint of lung transplantation or death during follow-up. Conclusions: In a large pediatric pulmonary hypertension cohort, diagnostic RHC with or without left heart catheterization in WSPH group 1 and 3 patients was performed safely at experienced pediatric pulmonary hypertension centers. Hemodynamic differences were noted between group 1 and 3 subjects. Higher mean pulmonary arterial pressure and mean pulmonary arterial pressure/mean systemic arterial pressure ratio were associated with a higher risk of death/transplantation. Findings suggest overall safety and potential value of RHC as a standard diagnostic approach to guide pulmonary hypertension management in children.
Authors: Robyn J Barst; D Dunbar Ivy; Aimee J Foreman; Michael D McGoon; Erika B Rosenzweig Journal: Am J Cardiol Date: 2013-10-04 Impact factor: 2.778
Authors: Dana B Harrar; Catherine L Salussolia; Patrick Vittner; Amy Danehy; Sonali Sen; Robert Whitehill; Jessica H Chao; Miya E Bernson-Leung; Michael J Rivkin Journal: Pediatr Neurol Date: 2019-07-19 Impact factor: 3.372
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Authors: Michael L O'Byrne; Kevin F Kennedy; Joshua P Kanter; John T Berger; Andrew C Glatz Journal: J Am Heart Assoc Date: 2018-02-28 Impact factor: 5.501
Authors: Erika B Rosenzweig; Steven H Abman; Ian Adatia; Maurice Beghetti; Damien Bonnet; Sheila Haworth; D Dunbar Ivy; Rolf M F Berger Journal: Eur Respir J Date: 2019-01-24 Impact factor: 16.671