Cardiac Imaging in Adults With Congenital Aortic Stenosis

Active, not recruiting

• Conditions: Congenital Aortic Stenosis

• Registration Number: NCT05252351
• Lead Sponsor: Erasmus Medical Center

Brief Summary

The CAS study is a prospective observational cohort study investigating the effects of congenital aortic stenosis (ConAoS) on the left ventricular function and the prevalence, pattern and expanse of left ventricular hypertrophy (LVH), myocardial stiffness and myocardial fibrosis.

Detailed Description

Congenital aortic stenosis (ConAoS) accounts for 4-8% of all congenital cardiac diagnoses. It is often caused by a bicuspid aortic valve (BAV), which has an estimated prevalence of 0.5-2% in the general population. Patients with ConAoS may remain asymptomatic, but gradual deterioration of the stenosis and the strong association of BAV with aortic dilatation contributes to important morbidity and mortality. The prevalent nature of this heart defect implies an important health problem resulting in hospitalization and (re-) interventions. As it is still largely unknown which markers predict adverse outcome, the aim of this study is to evaluate trends in imaging and biomarkers in this patient population and their relation with clinical outcome.

It is increasingly acknowledged that aortic stenosis is not only a disease of the valve, but also of the left ventricle (LV) and the aorta. In the course of disease progression, pressure overload and ventricular wall stress lead to remodeling of the LV, which eventually leads to left ventricular hypertrophy (LVH) and myocardial fibrosis. Although these processes have been described in patients with aortic stenosis, little is known about the prevalence and prognostic relevance of LVH and myocardial fibrosis in patients with ConAoS, who are often relatively young. Applying upcoming innovative imaging modalities such as high frame rate echocardiography and T1-mapping in patients with ConAoS will increase our knowledge on tissue characterization, which in turn will facilitate identifying patients at high risk for complications and rapid disease progression.

The CAS study is a clinical observational study investigating the effects of ConAoS on the left ventricular function and the prevalence, pattern and expanse of LVH, myocardial stiffness and myocardial fibrosis. Moreover, the prognostic capacity of the presence of these pathological processes will be assessed, correlating findings at baseline to clinical outcome by assessing the occurrence of cardiovascular events and all-cause mortality during 3-year clinical follow-up. The investigators will unravel biomarker and imaging predictors for myocardial dysfunction (systolic and diastolic) with specific attention for male-female differences. This newly gained knowledge will enable the investigators to improve and individualize current treatment protocols and derive novel therapeutic strategies for adult patients with ConAoS.

Study Timeline

• Study Start: 2022-01-13
• Study First Submitted: 2022-01-17
• Study First Submitted QC: 2022-02-11
• Study First Posted: 2022-02-23
• Status Verified: 2024-03
• Last Update Submitted: 2024-03-08
• Last Update Posted: 2024-03-12
• Primary Completion: 2026-11-01
• Study Completion: 2026-11-01

Recruitment & Eligibility

• Status: ACTIVE_NOT_RECRUITING
• Sex: All
• Target Recruitment: 75

Inclusion Criteria

Not provided

Exclusion Criteria

• Patients with severe aortic regurgitation

• Presence of any of the following contra-indications for MRI

  • Contra-indication to gadolinium based contrast media (eGFR <30 ml/min or contrast allergy)
  • Other contra-indications such as presence of pacemaker/implantable cardioverter defibrillator, severe claustrophobia or pregnancy

• Patients known with or previously treated because of aortic coarctation.

• Patients known with genetic syndromes or connective tissue disorders

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
Prevalence of myocardial fibrosis	Baseline	Assessed at baseline using cardiovascular magnetic resonance imaging (late gadolinium enhancement (LGE) and T1 mapping pre- and post-contrast)

Secondary Outcome Measures

Name	Time	Method
All-cause mortality	3 years
Aortic flow patterns	Baseline	This outcome is measured using 4D flow CMR
Quality of life (SF-36)	Baseline	This outcome is assessed by using the SF-36 questionnaire
Cardiovascular events	3 years	Incidents of heart failure, arrhythmias, endocarditis, aortopathy (aortic dissection and/or aortic aneurysm), (re-) interventions and (re-) operations of the aortic valve and/or ascending aorta and hospitalizations for other cardiac reasons
Blood biomarkers	Baseline
Conduction abnormalities on ECG	Baseline	For this outcome the conduction times on ECG will be measured
Occurence of ventricular arrhythmias	Baseline	This outcome is assessed by the occurence of ventricular ectopies and non-sustained ventricular tachycardia (VT) on Holter ECG for 48 hours.
Left ventricular function	Baseline	This outcome is measured by strain measurements using echocardiography and cardiovascular magnetic resonance imaging (CMR)
Shear wave velocity	Baseline	Shear wave velocity is measured using high frame rate echocardiography
Physical activity in daily life	Baseline	This outcome is measured by the following questionnaire: Short Questionnaire to Assess Health-enhancing physical activity (SQUASH)
Fear of movement	Baseline	This outcome is measured using the following questionnaire: Tampa Scale for Kinesiophobia (TSK-NL Heart)

Trial Locations

Locations (1)

Erasmus Medical Center; 🇳🇱 Rotterdam, Zuid-Holland, Netherlands