Real World Difference After Changing Medication From Nonselective to Selective Endothelin Receptor Antagonist in Stable Eisenmenger Syndrome

NCT04732650 | Unknown

• 1 other identifier: 2020-09-212
• Study Type: observational
• Target: 40
• Locations: 0 countries
• Sites: N/A

Brief Summary

In this study, the investigator will evaluate the treatment effects and safety, patient compliance of Ambrisentan in Eisenmenger syndrome in PAH patients who have been previously treated with Bosentan.

Conditions

Eisenmenger Complex

Keywords

pulmonary arterial hypertension; endothelin receptor antagonist; Eisenmenger complex

Outcome Measures

Primary Outcomes (8)

• Change of WHO FC

  WHO functional class I, II, III, IV.

  change from baseline to 6 months

• Changes in Borg dyspnea scale

  scale 0 -10

  change from baseline to 6 months

• TAPSE (TTE measure)

  mm pericardial effusion(presence, absence), RA size(mm), RV strain(%)

  change from baseline to 6 months

• pericardial effusion (TTE measure)

  presence, or absence

  change from baseline to 6 months

• RA size (TTE measure)

  mm

  change from baseline to 6 months

• RV strain(TTE measure)

  change from baseline to 6 months

• 6-minute walk distances (6MWT)

  m

  change from baseline to 6 months

• blood pressure at rest (SBP and DBP)

  mmHg

  change from baseline to 6 months

Other Outcomes (1)

• adverse drug response

  baseline, 12 week, 24 week, till 6 months if available.

Eligibility Criteria

• Age: 19 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Probability Sample

Study Population

* Patient who was scheduled to change Ambrisentan from Bosentan (prospective arm) or who already changed to Ambrisentan from Bosentan (retrospective arm) * Presence of cyanosis with \< 95 % arterial oxygen saturation (measured by transcutaneous pulse oximetry) or documented during exercise test (6 minute walk distance test or CPT stress test)

You may qualify if:

• Age at least 18 years
• Patient who was scheduled to change Ambrisentan from Bosentan (prospective arm) or who already changed to Ambrisentan from Bosentan (retrospective arm)
• Presence of cyanosis with \< 95 % arterial oxygen saturation (measured by transcutaneous pulse oximetry) or documented during exercise test (6 minute walk distance test or CPT stress test)
• Bosentan treatment more than 3months before changing to Ambrisentan and stable medication dosage for 1 month before changing medication
• Presence of PAH as diagnosed by invasive methods with Rp:Rs \> 0.75 measured at rest or diagnosed by echocardiography with TR Vmax \> 3.5m/s and bidirectional or right to left shunt.
• One of the following diagnosis:
• i) non-corrected large congenital shunting defect at atrial, ventricular or arterial level: Partial anomalous venous return, atrial septal defect, ventricular septal defect, atrioventricular cushion defect, persistent ductus arteriosus, or a combination of these.
• ii) Surgically corrected shunting defect (diagnoses as above) with significant residual defect iii) Other diagnoses with univentricular physiology/haemodynamics.

You may not qualify if:

• pregnancy or lactation
• women of child-bearing age who are sexually active without practicing reliable methods of contraception
• any disease or impairment that, in the opinion of the investigator, excludes a subject from participation
• substance abuse (alcohol, medicines, drugs)
• acute decompensated heart failure within 7 days before the invasive procedure
• significant anemia (Hb \< 9.0 g/dl)
• decompensated symptomatic polycythaemia
• significant impairment of hepatic function (Child Pugh class C)
• Significant left ventricular diseases (LV EF \< 45%)
• significant valvular diseases other than tricuspid or pulmonary regurgitation ( mitral or aortic valvular impairment more than moderate degree)
• pericardial constriction
• history of stroke, myocardial infarction or life-threatening arrhythmia within 6 months before screening
• bronchopulmonary dysplasia or other chronic severe lung diseases
• history of significant pulmonary embolism (in situ thromboembolism with optimal anticoagulation can be enrolled)
• other relevant diseases (e.g. HIV infection)

Sponsors & Collaborators

• Samsung Medical Center: lead
• GlaxoSmithKline: collaborator

Related Publications (3)

• Bever CT Jr, Asofsky R. Augmented IgG anti-acetylcholine receptor response following chronic penicillamine administration. J Neuroimmunol. 1991 Dec;35(1-3):131-7. doi: 10.1016/0165-5728(91)90168-7.

  PMID: 1955562 BACKGROUND

• Galie N, Beghetti M, Gatzoulis MA, Granton J, Berger RM, Lauer A, Chiossi E, Landzberg M; 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. 2006 Jul 4;114(1):48-54. doi: 10.1161/CIRCULATIONAHA.106.630715. Epub 2006 Jun 26.

  PMID: 16801459 BACKGROUND

• Gatzoulis MA, Beghetti M, Galie N, Granton J, Berger RM, Lauer A, Chiossi E, Landzberg M; BREATHE-5 Investigators. Longer-term bosentan therapy improves functional capacity in Eisenmenger syndrome: results of the BREATHE-5 open-label extension study. Int J Cardiol. 2008 Jun 23;127(1):27-32. doi: 10.1016/j.ijcard.2007.04.078. Epub 2007 Jul 20.

  PMID: 17658633 BACKGROUND

MeSH Terms

Conditions

Eisenmenger Complex; Pulmonary Arterial Hypertension

Condition Hierarchy (Ancestors)

Heart Defects, Congenital; Cardiovascular Abnormalities; Cardiovascular Diseases; Heart Diseases; Congenital Abnormalities; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Hypertension, Pulmonary; Lung Diseases; Respiratory Tract Diseases

Study Design

• Study Type: observational
• Observational Model: CASE ONLY
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Associate Professor

Study Record Dates

• First Submitted: December 10, 2020
• First Posted: February 1, 2021
• Study Start: February 4, 2021
• Primary Completion: December 31, 2023
• Study Completion: December 31, 2024
• Last Updated: February 1, 2021

Record last verified: 2021-01

Data Sharing

• IPD Sharing: Will not share