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The Surprise Question as a Trigger for Primary Palliative Care Interventions for Children with Advanced Heart Disease

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Abstract

There is significant uncertainty in describing prognosis and a lack of reliable entry criteria for palliative care studies in children with advanced heart disease (AHD). This study evaluates the utility of the surprise question—“Would you be surprised if this child died within the next year?”—to predict one-year mortality in children with AHD and assess its utility as entry criteria for future trials. This is a prospective cohort study of physicians and nurses caring for children (1 month–19 years) with AHD hospitalized ≥ 7 days. AHD was defined as single ventricle physiology, pulmonary vein stenosis or pulmonary hypertension, or any cardiac diagnosis with signs of advanced disease. Primary physicians were asked the surprise question and medical record review was performed. Forty-nine physicians responded to the surprise question for 152 patients. Physicians responded “No, I would not be surprised if this patient died” for 54 (36%) patients, 20 (37%) of whom died within 1 year, predicting one-year mortality with 77% sensitivity, 73% specificity, 37% positive predictive value, and 94% negative predictive value. Patients who received a “No” response had an increased 1-year risk of death (hazard ratio 7.25, p < 0.001). Physician years of experience, subspecialty, and self-rated competency were not associated with the accuracy of the surprise question. The surprise question offers promise as a bedside screening tool to identify children with AHD at high risk for mortality and help physicians identify patients who may benefit from palliative care and advance care planning discussions.

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Data Availability

Deidentified individual participant data (including data dictionaries) will be made available, in addition to study protocols, the statistical analysis plan, and the informed consent form. The data will be made available upon publication to researchers who provide a methodologically sound proposal for use in achieving the goals of the approved proposal. Proposals should be submitted to Elizabeth.blume@cardio.chboston.org.

Abbreviations

AHD:

Advanced heart disease

AUC:

Area under the curve

CICU:

Cardiac intensive care unit

HR:

Hazard ratio

IQR:

Interquartile range

NPV:

Negative predictive value

OR:

Odds ratio

PIM-3:

Pediatric index of mortality

PPV:

Positive predictive value

PRISM-III:

Pediatric risk of mortality III

VAD:

Ventricular assist device

References

  1. Cunningham RM, Walton MA, Carter PM (2018) The major causes of death in children and adolescents in the United States. N Engl J Med 379:2468–2475. https://doi.org/10.1056/nejmsr1804754

    Article  PubMed  PubMed Central  Google Scholar 

  2. Morell E, Moynihan K, Wolfe J, Blume ED (2019) Palliative care and paediatric cardiology: current evidence and future directions. Lancet Child Adolesc Heal 3:502–510. https://doi.org/10.1016/S2352-4642(19)30121-X

    Article  Google Scholar 

  3. Balkin EM, Kirkpatrick JN, Kaufman B et al (2017) Pediatric cardiology provider attitudes about palliative care: a multicenter survey study. Pediatr Cardiol 38:1324–1331. https://doi.org/10.1007/s00246-017-1663-0

    Article  PubMed  Google Scholar 

  4. Balkin EM, Wolfe J, Ziniel SI et al (2015) Physician and parent perceptions of prognosis and end-of-life experience in children with advanced heart disease. J Palliat Med 18:318–323. https://doi.org/10.1089/jpm.2014.0305

    Article  PubMed  Google Scholar 

  5. Morell E, Miller MK, Lu M et al (2021) Parent and physician understanding of prognosis in hospitalized children with advanced heart disease. J Am Heart Assoc. https://doi.org/10.1161/jaha.120.018488

    Article  PubMed  PubMed Central  Google Scholar 

  6. Mack JW, Cronin AM, Uno H et al (2020) Unrealistic parental expectations for cure in poor-prognosis childhood cancer. Cancer 126:416–424. https://doi.org/10.1002/cncr.32553

    Article  PubMed  Google Scholar 

  7. Rosenberg AR, Orellana L, Kang TI et al (2014) Differences in parent-provider concordance regarding prognosis and goals of care among children with advanced cancer. J Clin Oncol 32:3005–3011. https://doi.org/10.1200/JCO.2014.55.4659

    Article  PubMed  PubMed Central  Google Scholar 

  8. Moynihan KM, Ziniel SI, Johnston E et al (2021) A “Good Death” for children with cardiac disease. Pediatr Cardiol 2021:1–12. https://doi.org/10.1007/S00246-021-02781-0

    Article  Google Scholar 

  9. Lau F, Cloutier-Fisher D, Kuziemsky C et al (2007) A systematic review of prognostic tools for estimating survival time in palliative care. J Palliat Care 23:93–112

    Article  Google Scholar 

  10. White N, Kupeli N, Vickerstaff V, Stone P (2017) How accurate is the “Surprise Question” at identifying patients at the end of life? A systematic review and meta-analysis. BMC Med 15:139. https://doi.org/10.1186/s12916-017-0907-4

    Article  PubMed  PubMed Central  Google Scholar 

  11. Lynn J (2005) Living long in fragile health: the new demographics shape end of life care. Hastings Cent Rep 35:s14. https://doi.org/10.1353/HCR.2005.0096

    Article  Google Scholar 

  12. Ros MM, van der Zaag-Loonen HJ, Hofhuis JGM, Spronk PE (2021) Survival prediction in severely Ill patients study—the prediction of survival in critically Ill patients by ICU physicians. Crit Care Explor. https://doi.org/10.1097/CCE.0000000000000317

    Article  PubMed  PubMed Central  Google Scholar 

  13. Mudge AM, Douglas C, Sansome X et al (2018) Risk of 12-month mortality among hospital inpatients using the surprise question and SPICT criteria: a prospective study. BMJ Support Palliat Care 8:213–220. https://doi.org/10.1136/bmjspcare-2017-001441

    Article  PubMed  Google Scholar 

  14. Yen YF, Lee YL, Hu HY et al (2020) Early palliative care: the surprise question and the palliative care screening tool—better together. BMJ Support Palliat Care. https://doi.org/10.1136/bmjspcare-2019-002116

    Article  PubMed  Google Scholar 

  15. Aaronson EL, George N, Ouchi K et al (2019) The surprise question can be used to identify heart failure patients in the emergency department who would benefit from palliative care. J Pain Symptom Manage 57:944–951. https://doi.org/10.1016/j.jpainsymman.2019.02.007

    Article  PubMed  PubMed Central  Google Scholar 

  16. Hubbard G (2011) The “surprise question” in end-of-life care. Br J Community Nurs 16:109

    Article  Google Scholar 

  17. Hudson KE, Wolf SP, Samsa GP et al (2018) The surprise question and identification of palliative care needs among hospitalized patients with advanced hematologic or solid malignancies. J Palliat Med 21:789–795. https://doi.org/10.1089/jpm.2017.0509

    Article  PubMed  PubMed Central  Google Scholar 

  18. Pang WF, Kwan BCH, Chow KM et al (2013) Predicting 12-month mortality for peritoneal dialysis patients using the “surprise” question. Perit Dial Int 33:60–66. https://doi.org/10.3747/pdi.2011.00204

    Article  PubMed  PubMed Central  Google Scholar 

  19. Gonzalez-Jaramillo V, Arenas Ochoa LF, Saldarriaga C et al (2021) The ‘Surprise question’ in heart failure: a prospective cohort study. BMJ Support Palliat Care. https://doi.org/10.1136/bmjspcare-2021-003143

    Article  PubMed  Google Scholar 

  20. Straw S, Byrom R, Gierula J et al (2019) Predicting one-year mortality in heart failure using the ‘Surprise Question’: a prospective pilot study. Eur J Heart Fail 21:227–234. https://doi.org/10.1002/ejhf.1353

    Article  PubMed  Google Scholar 

  21. Gonzalez-Jaramillo V, Fernanda L, Ochoa A et al (2021) The “Surprise question” in heart failure: a prospective cohort study. BMJ Support Palliat Care. https://doi.org/10.1136/bmjspcare-2021-003143

    Article  PubMed  Google Scholar 

  22. Burke K, Coombes LH, Menezes A, Anderson AK (2018) The ‘surprise’ question in paediatric palliative care: a prospective cohort study. Palliat Med 32:535–542. https://doi.org/10.1177/0269216317716061

    Article  PubMed  Google Scholar 

  23. Brock KE, Steineck A, Twist CJ (2016) Trends in end-of-life care in pediatric hematology, oncology, and stem cell transplant patients. Pediatr Blood Cancer 63:516. https://doi.org/10.1002/PBC.25822

    Article  PubMed  Google Scholar 

  24. Harris PA, Taylor R, Thielke R et al (2009) Research electronic data capture (REDCap)-a metadata-driven methodology and workflow process for providing translational research informatics support. J Biomed Inform 42:377–381. https://doi.org/10.1016/j.jbi.2008.08.010

    Article  PubMed  Google Scholar 

  25. McHugh ML (2012) Interrater reliability: the kappa statistic. Biochem Med 22:276. https://doi.org/10.11613/bm.2012.031

    Article  Google Scholar 

  26. Downar J, Goldman R, Pinto R et al (2017) The “surprise question” for predicting death in seriously ill patients: a systematic review and meta-analysis. CMAJ 189:E484–E493. https://doi.org/10.1503/cmaj.160775

    Article  PubMed  PubMed Central  Google Scholar 

  27. Peng Y, Qi X, Guo X (2016) Child-pugh versus MELD score for the assessment of prognosis in liver cirrhosis a systematic review and meta-analysis of observational studies. Medicine (United States) 95:e2877

    Google Scholar 

  28. Aydoǧdu M, Topbaşi Sinanoǧlu N, Doǧan NÖ et al (2014) Wells score and pulmonary embolism rule out criteria in preventing over investigation of pulmonary embolism in emergency departments. Tuberk Toraks 62:12–21. https://doi.org/10.5578/tt.6493

    Article  PubMed  Google Scholar 

  29. Venkataraman R, Gopichandran V, Ranganathan L et al (2018) Mortality prediction using acute physiology and chronic health evaluation II and acute physiology and chronic health evaluation IV scoring systems: is there a difference? Indian J Crit Care Med 22:332–335. https://doi.org/10.4103/ijccm.IJCCM_422_17

    Article  PubMed  PubMed Central  Google Scholar 

  30. Balakrishnan G, Aitchison T, Hallworth D, Morton NS (1992) Prospective evaluation of the paediatric risk of mortality (PRISM) score. Arch Dis Child 67:196–200. https://doi.org/10.1136/adc.67.2.196

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  31. Ponce-Ponce De León AL, Romero-Gutiérrez G, Aldana CV, González-Bravo FE (2005) Simplified PRISM III score and outcome in the pediatric intensive care unit. Pediatr Int 47:80–83. https://doi.org/10.1111/j.1442-200x.2004.01997.x

    Article  Google Scholar 

  32. Abdelkader A, Shaaban M, Zahran M (2018) Using two scores for the prediction of mortality in pediatric intensive care units. Al-Azhar Assiut Med J 16:349. https://doi.org/10.4103/AZMJ.AZMJ_48_18

    Article  Google Scholar 

  33. White N, Reid F, Harris A et al (2016) A systematic review of predictions of survival in palliative care: how accurate are clinicians and who are the experts? PLoS ONE. https://doi.org/10.1371/journal.pone.0161407

    Article  PubMed  PubMed Central  Google Scholar 

  34. Keele L, Keenan HT, Sheetz J, Bratton SL (2013) Differences in characteristics of dying children who receive and do not receive palliative care. Pediatrics 132:72–78. https://doi.org/10.1542/peds.2013-0470

    Article  PubMed  Google Scholar 

  35. Wolfe J, Hinds P, Sourkes B (2011) Textbook of interdisciplinary pediatric palliative care. Elsevier, Amsterdam

    Google Scholar 

  36. Moynihan KM, Snaman JM, Kaye EC et al (2019) Integration of pediatric palliative care into cardiac intensive care: a champion-based model. Pediatrics. https://doi.org/10.1542/PEDS.2019-0160

    Article  PubMed  Google Scholar 

  37. Moynihan KM, Heith CS, Snaman JM et al (2021) Palliative care referrals in cardiac disease. Pediatrics. https://doi.org/10.1542/PEDS.2020-018580/77078

    Article  PubMed  Google Scholar 

  38. Kang TI, Munson D, Hwang J, Feudtner C (2014) Integration of palliative care into the care of children with serious illness. Pediatr Rev 35:318–326. https://doi.org/10.1542/pir.35-8-318

    Article  PubMed  Google Scholar 

  39. Arya B, Glickstein JS, Levasseur SM, Williams IA (2013) Parents of children with congenital heart disease prefer more information than cardiologists provide. Congenit Heart Dis 8:78–85. https://doi.org/10.1111/j.1747-0803.2012.00706.x

    Article  PubMed  Google Scholar 

  40. Gonzales M, Corbeil Y, Safabakhsh N et al (2019) Routinizing goals of care conversations—improving patient outcomes and satisfaction (TH336). J Pain Symptom Manage 57:381–382. https://doi.org/10.1016/j.jpainsymman.2018.12.066

    Article  Google Scholar 

  41. Nelson RM, Botkin JR, Kodish ED et al (2000) Palliative care for children. Pediatrics 106:351–357

    Article  Google Scholar 

  42. Wilkinson D (2009) The self-fulfilling prophecy in intensive care. Theor Med Bioeth 30:401–410. https://doi.org/10.1007/S11017-009-9120-6

    Article  PubMed  Google Scholar 

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Acknowledgements

We would like to thank Jeffrey Reichman for his contributions to creating and maintaining the Survey about Caring for Children with Heart Disease Database used in this study.

Funding

This study was supported, in part, by the Grousbeck Fazzalari Fund for Cardiac Research and the Advanced Cardiac Therapies Research and Education Fund at Boston Children’s Hospital. The funder/sponsor did not participate in the work.

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Authors and Affiliations

  1. Department of Cardiology, Boston Children’s Hospital, 300 Longwood Avenue, Boston, MA, 02115, USA

    Faraz Alizadeh, Kevin Hummel, Yunhong Wu, David Wypij, Danes Matthew, Paul Esteso, Katie Moynihan & Elizabeth D. Blume

  2. Department of Pediatrics, Harvard Medical School, Boston, MA, USA

    Faraz Alizadeh, Kevin Hummel, David Wypij, Paul Esteso, Katie Moynihan & Elizabeth D. Blume

  3. Division of Cardiology, Department of Pediatrics, UCSF Benioff Children’s Hospital, San Francisco, CA, USA

    Emily Morell

  4. Department of Pediatrics, University of California San Francisco, San Francisco, CA, USA

    Emily Morell

  5. Department of Biostatistics, Harvard T.H. Chan School of Public Health, Boston, MA, USA

    David Wypij

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  1. Faraz Alizadeh
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  9. Elizabeth D. Blume
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Contributions

All authors contributed to the study conception and design. Interpretation of the data was performed by FA, EM, KH, PE, KM, and EB. Data collection was performed by DM. Data Checks, development of statistical analysis plan, analysis of final data were performed by YW and DW. The first draft of the manuscript was written by FA and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Faraz Alizadeh.

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Conflict of interest

The authors have no conflict of interest or financial relationships relevant to this article to disclose.

Ethical Approval

Approval was obtained from the Institutional Review Board of Boston Children’s Hospital. The procedures used in this study adhere to the tenets of the Declaration of Helsinki.

Informed Consent

Informed consent was obtained from all individual participants included in the study.

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Alizadeh, F., Morell, E., Hummel, K. et al. The Surprise Question as a Trigger for Primary Palliative Care Interventions for Children with Advanced Heart Disease. Pediatr Cardiol 43, 1822–1831 (2022). https://doi.org/10.1007/s00246-022-02919-8

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  • DOI: https://doi.org/10.1007/s00246-022-02919-8

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