Eisenmenger syndrome: a clinical perspective in a new therapeutic era of pulmonary arterial hypertension

M Beghetti, N Galiè - Journal of the American College of Cardiology, 2009 - jacc.org
Eisenmenger syndrome (ES), the most advanced form of pulmonary arterial hypertension
(PAH) associated with congenital heart disease, is a devastating condition that has a …

Combination therapy with bosentan and sildenafil in Eisenmenger syndrome: a randomized, placebo-controlled, double-blinded trial

K Iversen, AS Jensen, TV Jensen… - European heart …, 2010 - academic.oup.com
Aims To evaluate the efficacy of combining the dual endothelin receptor antagonist,
bosentan, and the phosfodiesterase-5-inhibitor, sildenafil, in patients with Eisenmenger …

Bosentan–sildenafil association in patients with congenital heart disease-related pulmonary arterial hypertension and Eisenmenger physiology

M D'Alto, E Romeo, P Argiento, B Sarubbi… - International journal of …, 2012 - Elsevier
OBJECTIVES: The aim of the present study was to evaluate the safety, tolerability, clinical
and haemodynamic impact of add-on sildenafil in patients with congenital heart disease …

Ambrisentan for pulmonary arterial hypertension due to congenital heart disease

WA Zuckerman, D Leaderer, CA Rowan… - The American journal of …, 2011 - Elsevier
Eisenmenger syndrome (ES) is a known complication of congenital heart disease
associated with unrepaired systemic to pulmonary shunts. Evidence for use of targeted …

Long-term effect of bosentan in adults versus children with pulmonary arterial hypertension associated with systemic-to-pulmonary shunt: does the beneficial effect …

RLE van Loon, ES Hoendermis, MGJ Duffels… - American heart …, 2007 - Elsevier
BACKGROUND: Data on long-term response to bosentan in adults and especially children
with pulmonary arterial hypertension (PAH) associated with systemic-to-pulmonary shunt …

Disease targeting therapies in patients with Eisenmenger syndrome: response to treatment and long-term efficiency

GP Diller, R Alonso-Gonzalez, K Dimopoulos… - International journal of …, 2013 - Elsevier
Objectives To examine long-term efficacy of disease targeting therapies (DTT) in patients
with Eisenmenger syndrome. Methods All adult patients with Eisenmenger syndrome treated …

Eisenmenger syndrome: a multisystem disorder—do not destabilize the balanced but fragile physiology

MA Chaix, MA Gatzoulis, GP Diller, P Khairy… - Canadian Journal of …, 2019 - Elsevier
Eisenmenger syndrome is the most severe and extreme phenotype of pulmonary arterial
hypertension associated with congenital heart disease. A large nonrestrictive systemic left-to …

Effect of bosentan on exercise capacity and quality of life in adults with pulmonary arterial hypertension associated with congenital heart disease with and without …

MGJ Duffels, JC Vis, RLE van Loon… - The American journal of …, 2009 - Elsevier
Pulmonary arterial hypertension associated with congenital heart disease caused by
systemic-to-pulmonary shunting was associated with a high risk of morbidity and mortality. In …

[HTML][HTML] Prolonged beneficial effect of bosentan treatment and 4-year survival rates in adult patients with pulmonary arterial hypertension associated with congenital …

JC Vis, MG Duffels, P Mulder, RH de Bruin-Bon… - International journal of …, 2013 - Elsevier
Pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD) due
to systemic to pulmonary shunting is associated with a high risk of morbidity and mortality. In …

Pulmonary arterial hypertension in adults with congenital heart disease: distinct differences from other causes of pulmonary arterial hypertension and management …

K Dimopoulos, G Giannakoulas, SJ Wort… - Current opinion in …, 2008 - journals.lww.com
Although new advanced therapies hold promise in PAH secondary to CHD, long-term data
are clearly needed. Advanced therapies should be considered when other causes of …