Sindrome de Eisenmenger Online (Eisenmenger Syndrome)

¨A síndrome de Eisenmenger é um termo geral aplicado à hipertensão pulmonar e à inversão de shunt na presença de um defeito congênito, incluindo comunicações interatrial ou interventricular, defeito de canal interventricular, janela aortopulmonar. A síndrome consiste em hipertensão pulmonar com shunt reverso ou bidirecional ao nível atrioventricular ou aortopulmonar. Na gestação, essa síndrome está associada com alta taxa de mortalidade (30 a 50%). Normalmente, a hipertensão pulmonar é agravada durante a gestação, levando a um resultado desfavorável. As alterações hemodinâmicas que ocorrem durante a gestação contribuem para aumento da mortalidade materna em portadoras de síndrome de Eisenmenger. A taxa de mortalidade materna em portadoras de síndrome de Eisenmenger é alta (em torno de 30 a 50%). No eletrocardiograma e no ecocardiograma as hipertrofias atrial e ventricular direitas são evidentes. A função ventricular direita pode ser normal até os estágios tardios da doença, tempo no qual o átrio direito aumenta. Os achados hemodinâmicos patognomônicos são pressão elevada na artéria pulmonar, resistência vascular periférica aumentada e shunt da direita para esquerda. A expectativa de vida é significativamente encurtada nos pacientes com síndrome de Eisenmenger; entretanto, o tratamento clínico eficaz pode resultar em maior longevidade em adultos. A indicação de reparo cirúrgico é contra-indicado quando a hipertensão pulmonar é fixa¨.

■ What is the position of pulmonar arterial hypertension-specific drug therapy in patients with Eisenmenger syndrome?: a systematic review and meta-analysis

Qiang Li, Hong-Yu Kuang, Yu-Hao Wu, Tie-Wei Lu, Qi-Jian Yi

Medicine (Baltimore) 2019 May; 98(20): e15632

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6531184/

■ Evaluation of macitentan in patients with Eisenmenger syndrome: results from the randomized, controlled MAESTRO study

Michael A. Gatzoulis, Michael Landzberg, Maurice Beghetti, et al

Circulation. 2019 Jan 2; 139(1): 51–6

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6314514/

■ Increased microvolt T-wave alternas in children and adolescents with Eisenmenger syndrome

Derya Karpuz, Olgu Hallıoğlu, Dilek Çiçek Yılmaz

Anatol J Cardiol. 2018 May; 19(5): 303–310

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6280262/

■ Case report of an awake craniotomy in a patient with Eisenmenger syndrome

Boris D. Heifets, Erin Crawford, Ethan Jackson et al

A A Pract. 2018 May 1; 10(9): 219–22

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6309536/

■ Factors influencing outcomes in patients with Eisenmenger syndrome: a nine-year follow-up study

Mariana M. Clavé, Nair Y. Maeda, Claudia R. P. Castro et al

Pulm Circ. 2017 Sep; 7(3): 635–642

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5841908/

■ Successful pulmonar artery embolization for the management of hemoptsis in a patient with Eisenmenger syndrome caused by patent ductus arteriosus

Naoki Tamada, Kazuhiko Nakayama, Yuto Shinkura e al

Intern Med. 2017 Dec 15; 56(24): 3299–3304

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5790717/

■ An intra-cerebralabscess in a patient with Eisenmenger syndrome: an unusual case

Allan Hall, Mark A.J. White, Pasquale Gallo

Int J Surg Case Rep. 2016; 20: 138–14

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4818319/

■ Brain diffusion changes in Eisenmenger syndrome

Ferit Dogan, Dilek Sen Dokumaci, Ali Yildirim et al

Br J Radiol. December 2016; 89(1068): 20151007

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5604901/

■ Homen de 56 anos de idade, portador de comunicação interatrial e hipertensão arterial pulmonar, internado por síndrome de Eisenmenger

Santana, Carolina et al

Arq. Bras. Cardiol., Mar 2016, vol.106, no.3, p.250-258

http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0066-782X2016000300250&lng=pt&nrm=iso&tlng=pt (portuguese)

http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0066-782X2016000300250&lng=pt&nrm=iso (english)

■ Current therapy and outcome of Eisenmenger syndrome: data of the German National Register for congenital heart defects

Gerhard-Paul Diller, Marc-André Körten, Ulrike M.M. Bauer et al

Eur Heart J. 2016 May 7; 37(18): 1449–1455

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4914889/

■ Eisenmenger Syndrome in Pregnancy

Yuan, Shi-Min

Braz. J. Cardiovasc. Surg., Sept 2016, vol.31, no.4, p.325-329

http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0102-76382016000400325&lng=pt&nrm=iso

■ Eisenmenger syndrome in pregnancy

Shi-Min Yuan

Braz J Cardiovasc Surg. 2016 Jul-Sep; 31(4): 325–329

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5094422/

■ Síndrome de Eisenmenger en uma paciente embarazada de21anõs

L S Fajardo-Ruiz, R G Díaz-Novelo

Ginecol Obstet Mex2018, vol 85(3): 224-229

https://www.medigraphic.com/pdfs/ginobsmex/gom-2018/gom183i.pdf

■ Pulmonary arterial hypertension associated with congenitl heart disease and Eisenmenger syndrome: current practice in pediatrics

David B. Frank, Brian D. Hanna

Minerva Pediatr. 2015 Apr; 67(2): 169–185

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4382100/

■ Life span of patients with Eisenmenger syndrome is not superior to that of patients with other causes of pulmonar hypertension

Béatrice Bonello, Sébastien Renard, Julien Mancini, Sandrine

Cardiovasc Diagn Ther. 2014 Oct; 4(5): 341–349

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4221317/

■ Myocardial fibrosis in Eisenmenger syndrome: a descriptive cohort study exploring associations of late gadolinium enhancement with clinical status and survival

Craig S Broberg, Sanjay K Prasad, Chad Carr, Sonya V Babu-Narayan, Konstantinos Dimopoulos, Michael A Gatzoulis

J Cardiovasc Magn Reson. 2014; 16(1): 32

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4051886/

■ Juvenile severe mitral stenosis predisposing Eisenmenger syndrome in a case with ventricular septal defect, patent ductus arteriosus, coarctation of aorta & hypoplastic aorticarch: report offirst case of rare association

Soumya Patra, Basant Kumar, Kanchanahalli Siddegowda Sadananda et al

J Cardiovasc Dis Res. 2013 Sep; 4(3): 195–19

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3849316/

■ Conservative management of an elderly patient with Eisenmenger syndrome

Yasser Rodriguez, Michael Ghannam, Alexander Katz, Michael Shea

J Cardiol Cases. 2013 Apr; 7(4): e114–e116

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6275369/

■ Clubbing, cyanosis and a new diagnosis of atrial septal defect in a parturient: a straightforward diagnosis of Eisenmenger syndrome?

Charles Butcher, Carla Plyman, Marina Hughes, Fiona Walker

BMJ Case Rep. 2013; 2013: bcr201200799

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3603824/

■ Echocardiographic predictors of outcome in Eisenmenger syndrome

P Moceri, K Dimopoulos, E Liodakis et al

Circulation 2012, vol 126: 1461-1468

https://www.ahajournals.org/doi/full/10.1161/CIRCULATIONAHA.112.091421

■ Eisenmenger syndrome: not Always inoperable

Jing-Bin Huang, Jian Liang and Li-Yan Zhou

Respiratory Care September 2012, 57 (9) 1488-1495

http://rc.rcjournal.com/content/57/9/1488

■ The adult patient with Eisenmenger syndrome: a medical update after dana point Part I: epidemiology, clinical aspects and diagnostic options

H kaemmerer, S Mebus, I Schulze-Neick et al

Curr Cardiol Rev 2010, vol 6 (4): 343-355

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3083816/

■ The adult patient with Eisenmenger syndrome: a medical update after dana point Part II: medical treatment – studyresults

Siegrun Mebus, Ingram Schulze-Neick, Erwin Oechslin et al

Curr Cardiol Rev. 2010 Nov; 6(4): 356–362

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3083817/

■ The adult patient with Eisenmenger syndrome: a medical update after dana poing Part III: specific management and surgical aspects

Erwin Oechslin, Siegrun Mebus, Ingram Schulze-Neick et al

Curr Cardiol Rev. 2010 Nov; 6(4): 363–372

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3083818/

■ Extrinsic compression of the left coronary ostium by the pulmonary trunk: management in a case of Eisenmenger syndrome

Kothandam Sivakumar, Maruthanayagam Rajan, Gnanapragasam Francis et a

Tex Heart Inst J. 2010; 37(1): 95–98

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2829817/

■ Eisenmenger syndrome

M Beghetti , N Galiè

JACC 2009, vol 53 (9)

http://www.onlinejacc.org/content/53/9/733

■ Endothelin receptor antagonists improve exercise tolerance and oxygen saturations in patients with Eisenmenger syndrome and congenital heart defects

Puja K. Mehta, Leo Simpson, Eva K. Lee et al

Tex Heart Inst J. 2008; 35(3): 256–261.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2565520/

■ Síndrome de Eisenmenger na gravidez.

Borges, Vera Therezinha Medeiros et al.

Arq. Bras. Cardiol., Maio 2008, vol.90, no.5, p.e40-e41

http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0066-782X2008000500015&lng=pt&nrm=iso

■ Adult patients with Eisenmenger syndrome report flying safely on comercial airlines

Craig S Broberg, Anselm Uebing, Linda Cuomo et al

Heart. 2007 Dec; 93(12): 1599–1603

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2095753/

■ Síndrome de Eisenmenger y embarazo