Abordagens atuais na Síndrome de Eisenmenger: fisiopatologia, manifestações clínicas e estratégias terapêuticas

Authors

  • Leandro de Oliveira Reckel
  • Lucas de Brito Machado
  • Carolina Vial Alves
  • Davi Sarcinelli Vasconcelos
  • Brenda Vicente Helmer
  • Franciellen Mariano do Nascimento
  • Livia Maria Goldner Massariol
  • Ana Lívia Ramalho Carretta
  • Dayra Fieni
  • Nayara Stefany Lacerda Brandão Fundeheller

DOI:

https://doi.org/10.34119/bjhrv7n4-227

Keywords:

Síndrome de Eisenmenger, hipertensão pulmonar, cardiopatia congênita, resistência vascular pulmonar, tratamento personalizado

Abstract

Este artigo realiza uma revisão detalhada das abordagens contemporâneas na Síndrome de Eisenmenger, uma condição complexa associada à hipertensão pulmonar sistêmica decorrente de defeitos cardíacos congênitos não corrigidos. Explora-se a fisiopatologia multifacetada da síndrome, incluindo a reversão do fluxo sanguíneo e as alterações vasculares, além das diversas manifestações clínicas que variam desde sintomas cardíacos até complicações sistêmicas. São discutidas as estratégias terapêuticas mais recentes, como antagonistas do receptor de endotelina e inibidores da fosfodiesterase-5, analisando criticamente as evidências científicas disponíveis e identificando lacunas no conhecimento. O artigo também destaca desafios clínicos significativos e fornece insights fundamentais para o desenvolvimento de novas intervenções terapêuticas, visando melhorar a prática clínica e promover cuidados mais eficazes e personalizados aos pacientes com Síndrome de Eisenmenger.

References

ARVANITAKI, A. et al. Eisenmenger syndrome: diagnosis, prognosis and clinical management. Heart, v. 106, n. 21, p. 1638-1645, 2020.

ARVANITAKI, A. et al. Eisenmenger Syndrome: JACC State-of-the-Art Review. College of Cardiology, v. 79, n. 12, p. 1183-1198, 2022.

BARAONA, F. et al. Hospitalizations and mortality in the United States for adults with Down syndrome and congenital heart disease. The American Journal of Cardiology, v. 111, n. 7, p. 1046-1051, 2013.

BÉDARD, E.; DIMOPOULOS, K.; GATZOULIS, M. A. Has there been any progress made on pregnancy outcomes among women with pulmonary arterial hypertension? European Journal of Pediatrics, v. 30, n. 3, p. 256-265, 2009.

BLANCHE, C. et al. Use of intravenous iron in cyanotic patients with congenital heart disease and/or pulmonary hypertension. International Journal of Cardiology, v. 267, p. 79-83, 2018.

CHAIX, M. A. et al. Eisenmenger Syndrome: A Multisystem Disorder - Do not destabilize balanced but fragile physiology. Canadian Journal of Cardiology, v. 35, p. 1664-1674, 2019.

CHRISTIE, J. D. et al. The Registry of the International Society for Heart and Lung Transplantation: twenty-seventh official adult lung and heart-lung transplant report--2010. Journal Heart Lung Transplant, v. 29, n. 10, p. 1104-1118, 2010.

CUA, C. L. et al. Down syndrome patients with pulmonary hypertension have elevated plasma levels of asymmetric dimethylarginine. European Journal of Pediatrics, v. 170, n. 7, p. 859-863, 2011.

DILLER, G. P. et al. German Competence Network for Congenital Heart Defects Investigators. Current therapy and outcome of Eisenmenger syndrome: data of the German National Register for congenital heart defects. European Heart Journal, v. 37, n. 18, p. 1449-1455, 2016.

DILLER, G. P.; LAMMERS, A. E.; OECHSLIN, E. Treatment of adults with Eisenmenger syndrome-state of the art in the 21st century: a short overview. Cardiovascular Diagnosis Therapy, v. 11, n. 4, p. 1190-1199, 2021.

GALIÈ, N. et al. Bosentan Randomized Trial of Endothelin Antagonist Therapy-5 (BREATHE-5) Investigators. Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, double-blind, randomized, placebo-controlled study. Circulation, v. 114, n. 1, p. 48-54, 2006.

GATZOULIS, M. A. et al. Evaluation of Macitentan in Patients With Eisenmenger Syndrome. Circulation, v. 139, n. 1, p. 51-63, 2019.

GRÜNIG, E. et al. Plasma Drug Concentrations in Patients with Pulmonary Arterial Hypertension on Combination Treatment. Respiration, v. 94, n. 1, p. 26-37, 2017.

HJORTSHØJ, C. M. S. et al. Past and current cause-specific mortality in Eisenmenger syndrome. European Heart Journal, v. 38, n.26, p. 2060-2067, 2017.

KEMPNY, A. et al. Reference values for exercise limitations among adults with congenital heart disease. Relation to activities of daily life--single centre experience and review of published data. European Heart Journal, v. 33, n. 11, 2012.

MARELLI, A. J. et al. Congenital heart disease in the general population: changing prevalence and age distribution. Circulation, v. 115, n. 2, p. 163-172, 2007.

NASHAT, H. et al. Eisenmenger syndrome: current perspectives. Dovepress, v. 8, p. 1-12, 2016.

RIDA, M.; GATZOULIS, M. A. Pulmonary arterial hypertension in adult congenital heart disease. Heart, v. 104, p. 1568-1574, 2018.

SILVERSIDES, C. K. et al. Canadian Cardiovascular Society 2009 Consensus Conference on the management of adults with congenital heart disease: shunt lesions. Canadian Journal of Cardiology, v. 26, n. 3, p. 70-79, 2010.

SKORO-SAJER, N. et al. Subcutaneous treprostinil in pulmonary arterial hypertension related to congenital heart disease. Heart, v. 104, p. 1195-1999, 2018.

VAN DE BRUAENE, A. et al. Iron deficiency is associated with adverse outcome in Eisenmenger patients. European Heart Journal, v. 32, n. 22, p. 2790-2799, 2011.

Published

08/08/2024

How to Cite

RECKEL, L. de O.; MACHADO, L. de B.; ALVES, C. V.; VASCONCELOS, D. S.; HELMER, B. V.; NASCIMENTO, F. M. do; MASSARIOL, L. M. G.; CARRETTA, A. L. R.; FIENI, D.; FUNDEHELLER, N. S. L. B. Abordagens atuais na Síndrome de Eisenmenger: fisiopatologia, manifestações clínicas e estratégias terapêuticas. Brazilian Journal of Health Review, [S. l.], v. 7, n. 4, p. e71784, 2024. DOI: 10.34119/bjhrv7n4-227. Disponível em: https://ojs.brazilianjournals.com.br/ojs/index.php/BJHR/article/view/71784. Acesso em: 18 oct. 2026.

Issue

Section

Original Papers