Eisenmenger Syndrome: Closing the Hole (RCD code: IV-2B.1)

Hetain Patel, Pradip Jamnadas

Full Text:

PDF

Abstract


Eisenmenger syndrome represents the extreme manifestation of pulmonary arterial hypertension associated with congenital heart defects (CHD). Adults with CHD represent an increasing population which will require management in the long term. We present a case of a 38 year old female who presented with progressive dyspnea along with cyanosis at rest. Echocardiography showed a dilated right ventricle and an ostium secundum ASD with bidirectional shunting. Catheterization revealed severe pulmonary hypertension and evidence of partial anomalous pulmonary venous return. Warden procedure for PAPVR repair was performed along with a fenestrated pericardial ASD patch to close the shunt. A Maze Cox III procedure was also performed to treat the atrial fibrillation. Both procedures were performed successfully with significant improvement in patient’s clinical status. This article provides details regarding etiology, pathogenesis, clinical features, and diagnostic evaluation of Eisenmenger syndrome. It covers recent guidelines for screening and therapy as well as information regarding innovations in social and healthcare. JRCD 2014; 1 (6): 33–35


Keywords


Eisenmenger; atrial septal defect; congenital heart disease; pulmonary hypertension

References


Beghetti M, Galic N. Eisenmenger syndrome. A clinical perspective in a new therapeutic era of pulmonary arterial hypertension. J Amer Coll. Cardiol 2009; 53: 733–740.

Wood P. The Eisenmenger syndrome or pulmonary hypertension with reversed central shunt. Br Med J 1958;2701–9, 755–62.

Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol. 2002;39(12):1890–1900.

Adatia I, Kothari SS, Feinstein JA. Pulmonary hypertension associated with congenital heart disease: pulmonary vascular disease: the global perspective. Chest 2010; 137: Suppl. 6, 52S–61S.

Vongpatanasin W, Brickner ME, Hillis LD, et al. The Eisenmenger syndrome in adults. Ann Intern Med 1998; 128: 745–755.

Perloff JK. Congenital heart disease in adults: a new cardiovascular subspecialty. Circulation 1991; 84: 1881–1890).

Weiss BM, Zemp L, Seifert B, Hess OM. Outcome of pulmonary vascular disease in pregnancy: a systematic overview from 1978 through 1996. JACC 1998;31: 1650–7

Haworth SG. Pulmonary hypertension in the young. Heart. 2002;88:658–664.

Steele PM, Fuster V, Cohen M, et al. Isolated atrial septal defect with pulmonary vascular obstructive disease—long‑term followup and prediction of outcome after surgical correction. Circulation. 1987;76:1037–1042.

Sitbon O, Humbert M, Jais X, et al. Long‑term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation. 2005;111:3105–3111.

Dimopoulos K, Inuzuka R, Goletto S,et al.Improved survival among patients with Eisenmenger syndrome receiving advanced therapy for pulmonary arterial hypertension. Circulation 2010; 121: 20–25.

Galiè N, Hoeper MM, Humbert M, Torbicki A, Vachiery JL, Barbera JA, et al. Guidelines for the diagnosis and treatment of pulmonary hypertension: The Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT). Eur Heart J. Oct 2009;30(20):2493–537.

Baumgartner H, Bonhoeffer P, De Groot N, et. all. ESC Guidelines for

the management of grown‑up congenital heart disease (new version 2010) The Task Force on the Management of Grown‑up Congenital Heart Disease of the European Society of Cardiology (ESC). Eur Heart J. Aug 2010; 31(23): 2915–2957.




DOI: http://dx.doi.org/10.20418%2Fjrcd.vol1no6.125

Refbacks

  • There are currently no refbacks.
Journal of Rare Cardiovascular Diseases (JRCD)
John Paul II Hospital in Kraków, 80 Prądnicka Str., 31-202 Kraków, Poland
Phone: +48 (12) 614 33 99, +48 (12) 614 34 88 Fax: +48 (12) 614 34 88
e-mail: rarediseases@szpitaljp2.krakow.pl
Published by SoftQ sp. z o.o.
ul. Oleandry 2, 30-063 Kraków, Poland
Phone: +48 (12) 444 1650 Fax: +48 (12) 444 1659
e-mail: softq@softq.pl