Neonatal and pediatric terminal patients with congenital malformations of the circulatory system: lethality survey in Brazil and integrative review of palliative care in the ICU
DOI:
https://doi.org/10.33448/rsd-v12i3.40588Keywords:
Heart Defects, Congenital; Intensive Care Units, Neonatal; Intensive Care Units, Pediatric; Intensive Care Units, Pediatric.Abstract
Palliative care instigates the bioethical and technical discussion of intensive care in pediatric cardiology. The aim of the study was to outline an overview of neonatal and pediatric patients with congenital malformations of the circulatory system, evaluating mortality indicators in Brazil and palliative care in the ICU. An epidemiological study using the Datasus database raised the general hospital mortality rate or due to congenital malformations of the circulatory system in the age group of less than 1 year or between 1 and 4 years. An integrative review using the PubMed database retrieved original thematic publications between 2017 and 2022, adopting the methodological approach of PRISMA and JBI and evidence level 1B from Oxford or high from GRADE. Despite the decennial decline in hospital lethality rates due to congenital malformations of the circulatory system, the 1-year age group persists as more critical care than the 1- to 4-year age group, revealing regional asymmetries in the national context. Randomized multicenter studies on hypoplastic left heart syndrome in children with stage 1 to 2 palliation were more frequent, with great heterogeneity of interventions and clinical results applied to pediatric cardiology. The lack of experiences with critical neonates and family-centered palliative care call for future studies.
References
Agrawal, H., Petit, C. J., Miro, J., Miranda, C. D., Kenny, D. & Justino, H. (2018). Contralateral Pulmonary Hypertension Following Resuscitation of Unilateral Ductal Origin of a Pulmonary Artery: A Multi-institutional Review. Pediatric Cardiology, 39 (1), 71-78. https://doi.org/10.1007/s00246-017-1729-z.
Ahmed, H., Anderson, J. B., Bates, K. E., Natarajan, S., Ghanayem, N. S., Lannon, C. M., Brown, D. W. & National Pediatric Cardiology Quality Improvement Collaborative (2021). Characteristics of Interstage Death After Discharge from Stage I Palliation. Pediatric Cardiology, 42 (6), 1372-1378. https://doi.org/10.1007/s00246-021-02621-1.
Ahmed, H., Anderson, J. B., Bates, K. E., Fleishman, C. E., Natarajan, S., Ghanayem, N. S., Sleeper, L. A., Lannon, C. M., Brown, D. W. & National Pediatric Cardiology Quality Improvement Collaborative (2020). Development of a validated risk score for interstage death or transplant after stage I palliation for single-ventricle heart disease. The Journal of Thoracic and Cardiovascular Surgery, 160 (4), 1021-1030. https://doi.org/10.1016/j.jtcvs.2019.11.001.
Alves, S. C. F., Teixeira, F. A. O., Franco, G. A., Cenci, G. I., Pantoja, J. C., Jacob, L. S. A. S., Vieira, D. K. C., Rodrigues, L. M., Candido, J. S., & Carvalho, É. Q. (2022). Mortalidade por malformações congênitas em aparelho circulatório em menores de 1 ano na região Sudeste do Brasil entre 2014 e 2019. Research, Society and Development, 11 (5), e3111527993. https://doi.org/10.33448/rsd-v11i5.27993
Anderson, J. B., Brown, D. W., Lihn, S., Mangeot, C., Bates, K. E., Van Bergen, A. H., Rudd, N. A., Hanke, S., Tweddell, J. & Lannon, C. (2019). Power of a Learning Network in Congenital Heart Disease. World Journal for Pediatric and Congenital Heart Surgery, 10 (1), 66-71. https://doi.org/10.1177/2150135118815023.
Bentham, J. R., Zava, N. K., Harrison, W. J., Shauq, A., Kalantre, A., Derrick, G., Chen, R. H., Dhillon, R., Taliotis, D., Kang, S. L., Crossland, D., Adesokan, A., Hermuzi, A., Kudumula, V., Yong, S., Noonan, P., Hayes, N., Stumper, O. & Thomson, J. D. R. (2018). Duct Stenting Versus Modified Blalock-Taussig Shunt in Neonates With Duct-Dependent Pulmonary Blood Flow: Associations With Clinical Outcomes in a Multicenter National Study. Circulation, 137 (6), 581-588. https://doi.org/10.1161/CIRCULATIONAHA.117.028972.
Brasil (2014). Ministério da Saúde. Secretaria de Ciência, Tecnologia e Insumos Estratégicos. Departamento de Ciência e Tecnologia. Diretrizes metodológicas: Sistema GRADE – Manual de graduação da qualidade da evidência e força de recomendação para tomada de decisão em saúde / Ministério da Saúde, Secretaria de Ciência, Tecnologia e Insumos Estratégicos, Departamento de Ciência e Tecnologia. – Brasília: Ministério da Saúde, 2014. 72 p.: il. ISBN 978-85-334-2186-8. https://bvsms.saude.gov.br/bvs/publicacoes/diretrizes_metodologicas_sistema_grade.pdf
Brasil (2018). Ministério da Saúde. Gabinete do Ministro. Comissão Intergestores Tripartide. Resolução n. 41, de 31 de outubro de 2018. Dispõe sobre as diretrizes para a organização dos cuidados paliativos, à luz dos cuidados continuados integrados, no âmbito Sistema Único de Saúde (SUS). https://bvsms.saude.gov.br/bvs/saudelegis/cit/2018/res0041_23_11_2018.html
Breatnach, C. R., Aggarwal, V., Al-Alawi, K., McMahon, C. J., Franklin, O., Prendiville, T., Oslizlok, P., Walsh, K., Qureshi, A. M. & Kenny, D. (2019). Percutaneous axillary artery approach for ductal stenting in critical right ventricular outflow tract lesions in the neonatal period. Catheterization and Cardiovascular Interventions, 93 (7), 1329-1335. https://doi.org/10.1002/ccd.28302.
Buelow, M. W., Rudd, N., Tanem, J., Simpson, P., Bartz, P. & Hill, G. (2018). Reintervention following stage 1 palliation: A report from the NPC-QIC Registry. Congenital Heart Disease, 13 (6), 919-926. https://doi.org/10.1111/chd.12655.
DATASUS/TABNET (2023). Morbidade Hospitalar do SUS por local de internação a partir de 2008 – Notas Técnicas. Departamento de Informática do SUS. http://tabnet.datasus.gov.br/cgi/sih/Morb_geral_loc_int_2008.pdf
Galvão, T. F. & Pereira, M. G. (2015). Avaliação da qualidade da evidência de revisões sistemáticas. Epidemiologia e Serviços de Saúde, 24 (1), 173-175. http://scielo.iec.gov.br/scielo.php?script=sci_arttext&pid=S1679-49742015000100019&lng=pt.
Garros, D. (2003). Uma "boa" morte em UTI pediátrica: é isso possível?. Jornal de Pediatria, 79 (suppl 2), 243-254. https://doi.org/10.1590/S0021-75572003000800014
Geerdink, L. M., du Marchie-Sarvaas, G. J., Kuipers, I. M., Helbing, W. A., Delhaas, T., Ter-Heide, H., Rozendaal, L., de Korte, C. L., Singh, S. K., Ebels, T., Hazekamp, M. G., Haas, F., Bogers, A. J. & Kapusta, L. (2017). Surgical outcome in pediatric patients with Ebstein's anomaly: A multicenter, long-term study. Congenital Heart Disease, 12 (1), 32-39. https://doi.org/10.1111/chd.12404.
Hansen, J. E., Brown, D. W., Hanke, S. P., Bates, K. E., Tweddell, J. S., Hill, G. & Anderson, J. B. (2020). Angiotensin-Converting Enzyme Inhibitor Prescription for Patients With Single Ventricle Physiology Enrolled in the NPC-QIC Registry. Journal of the American Heart Association, 9 (10), e014823. https://doi.org/10.1161/JAHA.119.014823.
JBI (2020). Critical Appraisal Tools – Checklist for systematic reviews and research synthesis. Joanna Briggs Institute. https://jbi.global/critical-appraisal-tools.
Lago PM, Piva J, Garcia PC, Troster E, Bousso A, Sarno MO, Torreão L, Sapolnik R & Brazilian Pediatric Center of Studies on Ethics. (2008). End-of-life practices in seven Brazilian pediatric intensive care units. Pediatric Critical Care Medicine, 9 (1), 26-31. doi: 10.1097/01.PCC.0000298654.92048.BD.
Law, M. A., Glatz, A. C., Romano, J. C., Chai, P. J., Mascio, C. E., Petit, C. J., McCracken, C. E., Kelleman, M. S., Nicholson, G. T., Meadows, J. J., Zampi, J. D., Shahanavaz, S., Batlivala, S. P., Pettus, J., Pajk, A. L., Hock, K. M., Goldstein, B. H. & Qureshi, A. M. & Congenital Cardiac Research Collaborative (CCRC) Investigators. (2022). Palliation Strategy to Achieve Complete Repair in Symptomatic Neonates with Tetralogy of Fallot. Pediatric Cardiology, 43 (7), 1587-1598. https://doi.org/10.1007/s00246-022-02886-0.
Linhares, D. G., Siqueira, J. E., & Previdelli, I. T. S. (2013). Limitação do suporte de vida em unidade de terapia intensiva pediátrica. Revista Bioética, 21 (2), 291-297.
Lourenção, M. L., & Troster, E. J. (2020). Fim de vida em unidades de terapia intensiva pediátrica. Revista Bioética, 28 (3), 537-542. https://doi.org/10.1590/1983-80422020283418
Lowenstein, S., Macauley, R., Perko, K. & Ronai, C. (2020). Provider perspective on the role of palliative care in hypoplastic left heart syndrome. Cardiology in the Young, 30 (3), 377-382. https://doi.org/10.1017/S1047951120000128.
Marçola, L., Barbosa, S. M. M., Zoboli, I., Polastrini, R. T. V., & Ceccon, M. E. J. (2017). Análise dos óbitos e cuidados paliativos em uma unidade de terapia intensiva neonatal. Revista Paulista De Pediatria, 35 (2), 125-129. https://doi.org/10.1590/1984-0462/;2017;35;2;00012
Meza, J. M., Devlin, P. J., Overman, D. M., Gremmels, D., Baffa, G., Cohen, M. S., Quartermain, M. D., Caldarone, C. A., Pourmoghadam, K., DeCampli, W. M., Fackoury, C. T. & Mertens, L. (2019). The Congenital Heart Surgeon's Society Complete Atrioventricular Septal Defect Cohort: Baseline, Preintervention Echocardiographic Characteristics. Seminars in Thoracic and Cardiovascular Surgery, 31 (1), 80-86. https://doi.org/10.1053/j.semtcvs.2018.02.004.
Mille, F. K., Badheka, A., Yu, P., Zhang, X., Friedman, D. F., Kheir, J., van den Bosch, S., Cabrera, A. G., Lasa, J. J., Katcoff, H., Hu, P., Borasino, S., Hock, K., Huskey, J., Weller, J., Kothari, H. & Blinder, J. (2020). Red Blood Cell Transfusion After Stage I Palliation Is Associated With Worse Clinical Outcomes. Journal of the American Heart Association, 9 (10), e015304. https://doi.org/10.1161/JAHA.119.015304.
Moritz, R. D., Deicas, A., Capalbo, M., Forte, D. N., Kretzer, L. P., Lago, P., Pusch, R., Othero, J., Piva, J., Silva, N. B. da ., Azeredo, N. & Ropelato, R.. (2011). II Fórum do "Grupo de Estudos do Fim da Vida do Cone Sul": definições, recomendações e ações integradas para cuidados paliativos na unidade de terapia intensiva de adultos e pediátrica. Revista Brasileira De Terapia Intensiva, 23 (1), 24-29. https://doi.org/10.1590/S0103-507X2011000100005
Nicholson, G. T., Glatz, A. C., Qureshi, A. M., Petit, C. J., Meadows, J. J., McCracken, C., Kelleman, M., Bauser-Heaton, H., Gartenberg, A. J., Ligon, R. A., Aggarwal, V., Kwakye, D. B. & Goldstein, B. H. (2020). Impact of Palliation Strategy on Interstage Feeding and Somatic Growth for Infants With Ductal-Dependent Pulmonary Blood Flow: Results from the Congenital Catheterization Research Collaborative. Journal of the American Heart Association, 9 (1), e013807. https://doi.org/10.1161/JAHA.119.013807.
Page, M. J., McKenzie, J. E., Bossuyt, P. M., Boutron, I., Hoffmann, T. C., Mulrow, C. D., Shamseer, L., Tetzlaff, J. M., Akl, E. A., Brennan, S. E., Chou, R., Glanville, J., Grimshaw, J. M., Hróbjartsson, A., Lalu, M. M., Li, T., Loder, E. W., Mayo-Wilson, E., McDonald, S., McGuinness, L. A., Stewart, L. A., Thomas, J., Tricco, A. C., Welch, V. A., Whiting, P. & Moher, D. (2022). A declaração PRISMA 2020: diretriz atualizada para relatar revisões sistemáticas. Epidemiologia e Serviços de Saúde, 31(2), e2022107. https://doi.org/10.1590/s1679-49742022000200033.
Patel, M., Yu, S., Romano, J. C., Bates, K., Uzark, K., Schumacher, K., Balasubramanian, S. & Gelehrter, S. (2022). Birth Location in Infants with Prenatally Diagnosed Hypoplastic Left Heart Syndrome. Pediatric Cardiology, 43 (2), 301-307. https://doi.org/10.1007/s00246-021-02721-y.
Peña-Juárez, R. A. & Medina-Andrade, M. A. (2020). Current situation of congenital heart diseases in two public hospitals in the state of Jalisco. Archivos de Cardiology de México, 90 (2), 124-129. https://doi.org/10.24875/ACM.19000283
Pereira, A. S., Shitsuka, D. M., Parreira, F. J. & Shitsika, R. (2018). Metodologia da pesquisa científica. Santa Maria: UAB/NTE/UFSM. https://repositorio.ufsm.br/bitstream/handle/1/15824/Lic_Computacao_Metodologia-Pesquisa-Cientifica.pdf?sequence=1.
Piva, J. P., Garcia, P. C. R., & Lago, P. M. (2011). Dilemas e dificuldades envolvendo decisões de final de vida e oferta de cuidados paliativos em pediatria. Revista Brasileira de Terapia Intensiva, 23 (1), 78-86. https://doi.org/10.1590/S0103-507X2011000100013
Prodhan, P., Tang, X., Gossett, J., Beam, B., Simsic, J., Ghanayem, N. & ElHassan, N. O. (2018). Gastrostomy tube placement among infants with hypoplastic left heart syndrome undergoing stage 1 palliation. Congenital Heart Disease, 13 (4), 519-527. https://doi.org/10.1111/chd.12610.
Prodhan, P., Agarwal, A, ElHassan, N. O., Bolin, E. H., Beam, B., Garcia, X., Gaies, M. & Tang, X. (2017). Tracheostomy Among Infants With Hypoplastic Left Heart Syndrome Undergoing Cardiac Operations: A Multicenter Analysis. The Annals of Thoracic Surgery, 103 (4), 1308-1314. https://doi.org/10.1016/j.athoracsur.2016.09.016.
Ramachandran, P., King, E., Nebbia, A., Beekman, R. H. & Anderson, J. B. (2017). Variability of antithrombotics use in patients with hypoplastic left heart syndrome and its variants following first- and second-stage palliation surgery: a national report using the National Pediatric Cardiology Quality Improvement Collaborative registry. Cardiology in the Young, 27 (4), 731-738. https://doi.org/10.1017/S1047951116001189.
Salim, T. R., Soares, G. P., Klein, C. H., Oliveira, G. M. M. (2016). Mortalidade por Doenças e Malformações do Aparelho Circulatório em Crianças no Estado do Rio de Janeiro. Arquivos Brasileiros de Cardiologia, 106 (6), 464-473. https://www.scielo.br/j/abc/a/7WdyqsJmDwMnw7WzsbPxRTx/?format=pdf&lang=pt
Sampaio, L. F. D., Barreto, N. M. P. V., & Correia, H. F. (2021). Perfil das internações de crianças por malformações congênitas do aparelho circulatório no Brasil de 2010 a 2020. Revista de Ciências Médicas e Biológicas, 20 (3), 425-430. https://doi.org/10.9771/cmbio.v20i3.47085
Santos, C. M. C., Pimenta, C. A. M. & Nobre, M. R. C. (2007). A estratégia PICO para a construção da pergunta de pesquisa e busca de evidências. Revista Latino-americana de Enfermagem, 15 (3), 108-511. https://www.scielo.br/j/rlae/a/CfKNnz8mvSqVjZ37Z77pFsy/?format=pdf&lang=pt
Schramm, J. M. de A., & Szwarcwald, C. L. (2000). Diferenciais nas taxas de mortalidade neonatal e natimortalidade hospitalares no Brasil: um estudo com base no Sistema de Informações Hospitalares do Sistema Único de Saúde (SIH/SUS). Cadernos de Saúde Pública, 16 (4), 1031-1040. https://doi.org/10.1590/S0102-311X2000000400021
Silva, E. M. B., Silva, M. J. M., & Silva, D. M. (2019). Perception of health professionals about neonatal palliative care. Revista Brasileira de Enfermagem, 72 (6), 1707-1714. https://doi.org/10.1590/0034-7167-2018-0842
Tadphale, S. D., Tang, X., El-Hassan, N. O., Beam, B. & Prodhan, P. (2017). Cavopulmonary Anastomosis During Same Hospitalization as Stage 1 Norwood/Hybrid Palliative Surgery. The Annals of Thoracic Surgery, 103 (4), 1285-1291. https://doi.org/10.1016/j.athoracsur.2017.01.013.
Yimgang, D. P., Sorkin, J. D., Evans, C. F., Abraham, D. S. & Rosenthal, G. L. (2018). Angiotensin converting enzyme inhibitors and interstage failure in infants with hypoplastic left heart syndrome. Congenital Heart Disease, 13 (4), 533-540. https://doi.org/10.1111/chd.12622.
Downloads
Published
How to Cite
Issue
Section
License
Copyright (c) 2023 Jaqueline Alessandra Castro da Silva; Igor Iuco Castro-Silva
This work is licensed under a Creative Commons Attribution 4.0 International License.
Authors who publish with this journal agree to the following terms:
1) Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a Creative Commons Attribution License that allows others to share the work with an acknowledgement of the work's authorship and initial publication in this journal.
2) Authors are able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgement of its initial publication in this journal.
3) Authors are permitted and encouraged to post their work online (e.g., in institutional repositories or on their website) prior to and during the submission process, as it can lead to productive exchanges, as well as earlier and greater citation of published work.