Hipertensão pulmonar persistente do recém-nascido: revisão de conceitos
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A hipertensão pulmonar persistente do recém-nascido (HPPN) é uma síndrome clínica, causada pela persistência da elevada resistência vascular pulmonar após o nascimento. A falha na transição da circulação fetal para neonatal mantém resistências vasculares pulmonares altas, causando hipoxemia e cianose nos recém-nascidos. Em 2018, foi proposta uma nova classificação para a hipertensão pulmonar e, devido a suas particularidades anatômicas e fisiológicas, a HPPN foi movida para uma subcategoria separada (grupo 1; subgrupo 1.6). No entanto, na prática clínica, a classificação mais utilizada é a de hipertensão pulmonar primária ou idiopática e secundária, quando associada a doenças parenquimatosas pulmonares. O diagnóstico inclui história completa dos fatores de risco, exame físico, medição da saturação de oxigênio pré- e pós-ductal, gasometria arterial, raio X de tórax e ecocardiograma. O entendimento do caráter multifatorial da doença tem levado a pesquisas do papel de mediadores inflamatórios e mecanismos genéticos na fisiopatologia da doença. Além do tratamento tradicional, incluindo ventilação mecânica “gentil”, manutenção da temperatura corporal, controle de eletrólitos, hidratação, analgesia e sedação, novas estratégias baseadas nas diferentes vias inflamatórias têm sido cada vez mais utilizadas na prática clínica.
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Referências
1.Sankaran D, Lakshminrusimha S. Pulmonary hypertension in the newborn - etiology and pathogenesis. Semin Fetal Neonatal Med. 2022 Aug;27(4):101381. doi: 10.1016/j.siny.2022.101381
2. Martinho S, Adão R, Leite-Moreira AF and Brás-Silva C. Persistent Pulmonary Hypertension of the Newborn: Pathophysiological Mechanisms and Novel Therapeutic Approaches. Front Pediatr 2020; 8: 342
3. Steurer MA, Jelliffe-Pawlowski LL, Baer RJ, Partridge JC, Rogers EE, Keller RL. Persistent Pulmonary Hypertension of the Newborn in Late Preterm and Term Infants in California. Pediatrics. 2017 1;139(1):e20161165
4. Rasanen J, Wood DC, Weiner S, Ludomirski A, Huhta JC. Role of the pulmonary circulation in the distribution of human fetal cardiac output during the second half of pregnancy. Circulation 1996;94:1068-73.
5. Rudolph AM. Aortopulmonary transposition in the fetus: speculation on pathophysiology and herapy. Pediatr Res 2007;61:375-80.
6. Lakshminrusimha S, Steinhorn RH. 155 - pathophysiology of persistent pulmonary hypertension of the newborn. In: Polin RA, Abman SH, Rowitch DH, Benitz WE, Fox WW, editors. Fetal and neonatal physiology. fifth ed. Elsevier; 2017. 1576-1588.e4.
7. Lakshminrusimha S, KeszlerM. Persistent pulmonary hypertension of the newborn. NeoReviews 2015;16:e680-92.
8. Sharma V, Berkelhamer S, Lakshminrusimha S. Persistent pulmonary hypertension of the newborn. Matern Health Neonatol Perinatol 2015;1:14.
9. Van Marter LJ, Hernandez-Diaz S, Werler MM, Louik C, Mitchell AA. Nonsteroidal antiinflammatory drugs in late pregnancy and persistent pulmonary hypertension of the newborn.Pediatrics 2013;131:79-87.
10. Chandrasekharan PK, Rawat M, Madappa R, Rothstein DH, Lakshminrusimha S. Congenital Diaphragmatic hernia – a review. Matern Health Neonatol Perinatol 2017;3:6.
11. Kosiński P, Wielgoś M. Congenital diaphragmatic hernia: pathogenesis, prenatal diagnosis and management - literature review. Ginekol Pol. 2017;88(1):24-30. doi: 10.5603/GP.a2017.0005
12. Hedrick HL, Danzer E, Merchant A, Bebbington MW, Zhao H, Flake AW et al. Liver position and lung-to-head ratio for prediction of extracorporeal membrane oxygenation and survival in isolated left congenital diaphragmatic hernia. Am JObstet Gynecol 2007;197:422. e1-. e4.
13. Atkinson C, Stewart S, Upton PD, Machado R, Thomson JR, Trembath RC, Morrell NW. Primary pulmonary hypertension is associated with reduced pulmonary vascular expression of type II bone morphogenetic protein receptor. Circulation 105: 1672 1678, 2002.
14. Deng Z, Morse JH, Slager SL, Cuervo N, Moore KJ, Venetos G, Kalachikov S, Cayanis E, Fischer SG, Barst RJ, Hodge SE, Knowles JA. Familial primary pulmonary hypertension (gene PPH1) is caused by mutations in the bone morphogenetic protein receptor-II gene. Am J Hum Genet 67: 737-744, 2000.
15. Kinsella JP. Inhaled nitric oxide therapy in premature newborns. Curr Opin Pediatr 18: 107-111, 2006.
16. Levy M, Eyries M, Szezepanski I, Ladouceur M, Nadaud S, Bonnet D, Soubrier F. Genetic analyses in a cohort of children with pulmonary hypertension. Eur Respir J 48: 1118-1126,2016.
17. Rabinovitch M Molecular pathogenesis of pulmonary arterial hypertension. J Clin Invest 122: 4306-4313, 2012.
18. West J, Austin E, Fessel JP, Loyd J, Hamid R. Rescuing the BMPR2 signaling axis in pulmonary arterial hypertension. Drug Discov Today 19: 1241-1245, 2014.
19. Ruopp NF, Cockrill BA. Diagnosis and Treatment of Pulmonary Arterial Hypertension: A Review. JAMA. 2022 Apr 12;327(14):1379 1391. doi: 10.1001/jama.2022.4402. Erratum in: JAMA. 2022 Sep 6;328(9):892. doi: 10.1001/jama.2022.13696.
20. Mukherjee D, Konduri GG. Pediatric Pulmonary Hypertension: Definitions, Mechanisms, Diagnosis, and Treatment. Compr Physiol. 2021 Jun 30;11(3):2135-2190. doi: 10.1002/cphy.c200023. PMID: 34190343
21. Singh Y, Lakshminrusimha S. Pathophysiology and Management of Persistent Pulmonary Hypertension of the Newborn. Clin Perinatol. 2021 Aug;48(3):595-618. doi: 10.1016/j.clp.2021.05.009
22. Siefkes HM, Lakshminrusimha S. Management of systemic hypotension in term infants with persistent pulmonary hypertension of the newborn: an illustrated review. Arch Dis Child Fetal Neonatal Ed. 2021 Jul;106(4):446-455. doi: 10.1136/ archdischild-2020-319705. Epub 2021 Jan 21.
23. Humbert M, Kovacs G, Hoeper MM, Badagliacca R, Berger RMF, Brida M, Carlsen J, Coats AJS, Escribano-Subias P, Ferrari P, Ferreira DS, Ghofrani HA, Giannakoulas G, Kiely DG, Mayer E, Meszaros G, Nagavci B, Olsson KM, Pepke-Zaba J, Quint JK, Rådegran G, Simonneau G, Sitbon O, Tonia T, Toshner M, Vachiery JL, Vonk Noordegraaf A, Delcroix M, Rosenkranz S; ESC/ERS Scientific Document Group. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J. 2023 Jan 6;61(1):2200879. doi: 10.1183/13993003.00879-2022
24. Mandell E, Kinsella JP, Abman SH. Persistent pulmonary hypertension of the newborn. Pediatr Pulmonol. 2021 Mar;56(3):661-669. doi: 10.1002/ppul.25073
25. Kim YJ, Shin SH, Kim EK, Kim HS. Short term effect of intravenous treprostinil in term and preterm infants with pulmonary hypertension. BMC Pediatric. 2024 Jan 29;24(1):83. doi: 10.1186/ s12887-023-04501-4
26. Cookson MW, Abman SH, Kinsella JP, Mandell EW. Pulmonary vasodilator strategies in neonates with acute hypoxemic respiratory failure and pulmonary hypertension. Semin Fetal Neonatal Med. 2022 Aug;27(4):101367. doi: 10.1016/j. siny.2022.101367. Epub 2022 Jun 3
27. Yıldırım Ş. Inhaled iloprost is an effective alternative therapy for persistent pulmonary hypertension in newborns. Pulm Circ. 2023 Jul 18;13(3):e12268. doi: 10.1002/pul2.12268.
28. Martinho S, Adão R, Leite-Moreira AF, Brás-Silva C. Persistent Pulmonary Hypertension of the Newborn: Pathophysiological Mechanisms and Novel Therapeutic Approaches. Front Pediatr. 2020 Jul 24;8:342. doi: 10.3389/fped.2020.00342.
29. Olicker AL, Raffay TM, Ryan RM. Neonatal Respiratory Distress Secondary to Meconium Aspiration Syndrome. Children (Basel). 2021 Mar 23;8(3):246. doi:10.3390/ children8030246
30. Kim F, Bernbaum J, Connelly J, Gerdes M, Hedrick HL, Hoffman C, Rintoul NE, Ziolkowski K, DeMauro SB. Survival and Developmental Outcomes of Neonates Treated with Extracorporeal Membrane Oxygenation: A 10-Year Single-Center Experience. J Pediatr. 2021 Feb;229:134-140.e3. doi: 10.1016/j.jpeds.2020.10.011. Epub 2020 Oct 13