Background: Although sildenafil has been shown to be safe and effective in idiopathic pulmonary arterial hypertension (PAH) and PAH related to connective tissue disease, its effects in Eisenmenger syndrome are less clear.
Objective: To investigate whether long-term treatment (12 months) with the phosphodiesterase type 5 inhibitor sildenafil improves clinical and haemodynamic parameters in patients with Eisenmenger syndrome.
Design: Prospective, open-label, multicentre study.
Setting: Four pulmonary hypertension centres in China.
Patients: 84 Eisenmenger syndrome functional class II-IV patients.
Interventions: Oral sildenafil 20 mg orally three times a day.
Outcome Measures: 6-min walk distance (6MWD) test, resting systemic arterial blood oxygen saturation (SaO(2)) in room air, haemodynamic parameters assessed by right heart catheterisation, safety and tolerability.
Results: The overall treatment effects at 12 months versus baseline (mean changes with 95% CIs) were 56 m increase (42 to 69, p<0.0001) in 6MWD, and 2.4% increase (1.8% to 2.9%, p<0.0001) in resting room air SaO(2). Improvements were also seen in mean pulmonary arterial pressure and pulmonary vascular resistance index (-4.7 mm Hg (-7.5 to -1.9), p=0.001; and -474 dyn×s×cm(-5)×m(2) (-634 to -314), p<0.0001, respectively). Sildenafil was well tolerated. Most adverse events were mild and transient, and occurred in the first 2 weeks of treatment.
Conclusions: Twelve months of oral sildenafil treatment was well tolerated and appeared to improve exercise capacity, systemic arterial oxygen saturation and haemodynamic parameters in patients with Eisenmenger syndrome.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1136/heartjnl-2011-300344 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Safety, tolerability, and efficacy of an in-class combination therapy switch from bosentan plus sildenafil to ambrisentan plus tadalafil in children with pulmonary arterial hypertension.
Pulm Circ
October 2024
UK Service for Pulmonary Hypertension in Children, Great Ormond Street Hospital for Children London UK.
The aim of this single-centre retrospective observational study was to evaluate the safety, tolerability, and efficacy of an in-class combination therapy switch from bosentan plus sildenafil to ambrisentan plus tadalafil in children with pulmonary arterial hypertension. Children aged over 5 years who were established on sildenafil plus bosentan were offered to undergo a therapy switch from May 2014 to May 2021 and, if remaining in the service, followed up to May 2024. Children with Eisenmenger syndrome, open intra or extra-cardiac shunt, or with pulmonary hypertension-associated lung disease were excluded.
View Article and Find Full Text PDFEisenmenger syndrome during pregnancy: A true anesthetic challenge.
Med J Armed Forces India
December 2024
Professor & Senior Advisor, (Anaesthesia & Critical Care), Command Hospital (Eastern Command), Kolkata, West Bengal, India.
Eisenmenger syndrome (ES) is a rare congenital heart disease (CHD) that occurs in persons with large intracardiac anatomical defects which may go undetected for many years, consequently results in reduced life expectancies and high mortality in the third and fourth decade of life. Pregnancy in patients with CHD and pulmonary arterial hypertension (PAH) is presaged with challenges, and perioperative management of such cases remains a topic of debate. Our case had both, atypical presentation, not diagnosed until her third trimester of third pregnancy and a quick progression to a severe stage with PAH and CorPulmonale.
View Article and Find Full Text PDFIntraoperative Hemodynamic Instability in a Patient With Ebstein's Anomaly Complicated With Eisenmenger Syndrome.
Case Rep Cardiol
December 2024
Department of Anesthesiology and Pain Management, Cleveland Clinic, Cleveland, Ohio, USA.
Ebstein's anomaly is a rare congenital displacement of the tricuspid valve resulting in atrialization of the right ventricle. About half of the patients with Ebstein's anomaly also have atrial septal defects, which may lead to chronic shunting and development of Eisenmenger syndrome. We describe a case of a sexagenarian male patient with a history of Ebstein's anomaly complicated with Eisenmenger syndrome undergoing robotic laparoscopic adrenalectomy who presented hemodynamic instability, hypoxemia, and likely right-to-left shunting intraoperatively, as well as the actions taken to correct it and have a successful outcome.
View Article and Find Full Text PDFPulmonary arterial hypertension with left to right shunts: When to treat and/or close?
Int J Cardiol Congenit Heart Dis
September 2024
Royal Brompton Hospital, Part of Guys St Thomas NHS Trust, and National Heart and Lung Institute, Imperial College London, London, United Kingdom.
Pulmonary arterial hypertension (PAH) is defined as increase in mean pulmonary arterial pressure and pulmonary vascular resistance (PVR). It can be associated with congenital heart disease (CHD) with the following subtypes: 1) uncorrected left-to-right (L-R) intracardiac shunt leading to overload of the pulmonary circulation and a progressive increase of PVR; 2) Eisenmenger syndrome, appearing when a large post-tricuspid shunt is left uncorrected and pulmonary vascular disease (PVD) is severe, so the shunt becomes bidirectional or right-to-left, causing cyanosis; 3) PAH after shunt closure, when PVR arises after a defect correction; and 4) PAH associated with small or coincidental defects. While the treatment of patients with Eisenmenger syndrome is well established, the treatment of patients with PAH in whom there is a L-R shunt (with no cyanosis) remains unclear and requires expertise.
View Article and Find Full Text PDFUpdate on Eisenmenger syndrome - Review of pathophysiology and recent progress in risk assessment and management.
Int J Cardiol Congenit Heart Dis
September 2024
The Cincinnati Adult Congenital Heart Disease Program, Heart Institute, Cincinnati Children's Hospital, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA.
Longstanding left-to-right shunting associated with congenital heart disease (CHD) can ultimately lead to pulmonary vascular remodeling, pulmonary arterial hypertension, and shunt reversal, the hallmark feature of Eisenmenger Syndrome (ES). ES is a multisystem disease, with hematologic, cardiovascular, renal, neurologic, immune, and other manifestations, each of which inform its management. Many of the most distinct and clinically important consequences relate to chronic hypoxemia.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!