Transposition of Great Vessels
Conditions
Keywords
transposition of the great arteries, arterial switch operation, coronary artery anomaly, outcome
Brief summary
Background: Long-term outcomes in adults with prior arterial switch operation (ASO) have not yet been well defined. The aim of this study is to elucidate incidence and predictors of adverse cardiac outcomes in a prospectively followed cohort of adults after their ASO. Methods: The comprehensive long-term follow up of adults with ASO is a project within the European collaboration for prospective outcome research in congenital heart disease (EPOCH). It is designed as a prospective, international multicenter cohort study. Consecutive patients (aged 16 years or more) with prior ASO will be included at 11 European tertiary care centers. Participants will be followed according to a standardized protocol following international recommendations, including standardized protocols for imaging and for exercise testing. Main outcome measures are all-cause and cardiac-related mortality, rate of cardiac re-intervention, neo-aortic dissection, myocardial infarction, stroke, infective endocarditis, sustained atrial and ventricular arrhythmias, new-onset or worsening pulmonary hypertension and new-onset heart failure. Secondary endpoints are frequency and progression of right ventricular outflow tract stenosis, neo-aortic root dilatation, neo-aortic valve regurgitation and ventricular dysfunction. The impact of demographic, anatomic (e.g. coronary artery anatomy) and functional variables on the above-mentioned outcomes, as well as quality of life and incidence of pregnancy related complications will also be assessed. Aim: The prospective, international, multicenter EPOCH-ASO study will provide a better understanding of adverse outcomes and their predictors in adults after ASO. The results of the EPOCH-ASO study may help to optimize future care of this novel patient cohort in adult cardiology.
Interventions
OTHERNo intervention is planed
No intervention is planed
Sponsors
University of Zurich
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
16 Years to No maximum
Healthy volunteers
Yes
Inclusion criteria
* Adults (≥16 years) * with transposition of great arteries or a Taussig-Bing anomaly * who underwent repair by an ASO, and who are actively followed at one of the participating centers will be enrolled.
Exclusion criteria
* Incapability of giving informed consent and previous heart transplant.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Incidence of all-cause mortality | during a follow up of up to 20 years | Determination of cause of death |
| Incidence of cardiac-related mortality | during a follow up of up to 20 years | Sudden cardiac death (death that ensues unexpected within one hour of onset of symptoms), death related to acute myocardial infarction, death that is primarily caused by heart failure or death within 30 days or during the hospital admission after a cardiac intervention. |
| Incidence/rate of re-intervention | during a follow up of up to 20 years | Includes all types of cardiac re-intervention with detailed analysis of the indication of re-intervention. |
| Incidence/rate of neo-aortic dissection | during a follow up of up to 20 years | Aortic dissection with entry within the neo-aortic root. |
| Incidence of myocardial infarction | during a follow up of up to 20 years | Defined according to the Fourth Universal Definition of Myocardial Infarction. Defined according to the Fourth Universal Definition of Myocardial Infarction. |
| Incidence of arrhythmias | during a follow up of up to 20 years | Atrial arrhythmias with a duration of \>30 seconds, or requiring anti-arrhythmic medication or ablation procedures and / or sustained ventricular tachycardia (heart rate \>100/min) for at least 30 seconds or requiring electrical cardioversion / defibrillation. |
| Incidence of new onset / worsening heart failure | during a follow up of up to 20 years | Hospital admission for heart failure or initiation of heart failure medication for symptoms of heart failure (excludes initiation of medication for asymptomatic deterioration of ventricular function), according to the current guidelines of the European Society of Cardiology. |
| Incidence of stroke | during a follow up of up to 20 years | Focal neurological symptoms and confirmation of cerebral ischemia or infarction by cerebral magnetic resonance imaging or computed tomography. |
| Incidence of infective endocarditis | during a follow up of up to 20 years | Defined according to the modified Duke's criteria, according to the current ESC guidelines. |
| Incidence of pulmonary hypertension | during a follow up of up to 20 years | Defined as an increase in mean pulmonary arterial pressure (PAPm) ≥20 mmHg at rest as assessed by right heart catheterization. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Incidence of worsening right ventricular function | during a follow up of up to 20 years | Echocardiography (not valid in case of worsening tricuspid regurgitation) - At least 2 of the following: * Decrease in Fac \> 10% * Decrease in TAPSE \>5mm * Decrease in TAPSE S' \>3cm/s CMR: \- Decrease in RVEF \>5% |
| Rate of right ventricular outflow tract stenosis | during a follow up of up to 20 years | At least 1 of the following criteria: * Branch pulmonary artery minimal diameter: maximal diameter at least 1:2 on CT or MRI * Echocardiographic systolic peak gradient across branch pulmonary arteries \> 16mmHg (peak velocity \> 2.0m/s) * Estimated right ventricular systolic pressure \> 40mmHg (determined by RV/RA-pressure gradient and estimated central venous pressure, see echocardiography protocol for details) |
| Incidence of pregnancy | during a follow up of up to 20 years | To study the impact of pregnancy on mortality and cardiovascular morbidity. |
| Rate of poor functional capacity | during a follow up of up to 20 years | As determined by cardiopulmonary exercise testing by measuring Lung Function (flow volume loops), oxygen consumption during exercise (VO2 max), anaerobic threshold, heart performance during exercise (O2-Heart rate) |
| Rate of the different patterns of the coronary anatomy | during a follow up of up to 20 years | To study the impact of coronary artery anatomy and type of coronary reimplantation on cardiovascular morbidity, ventricular function and functional capacity with a specific focus on presence of coronary artery obstruction, acute proximal angulation and inter-arterial or intramural course. |
| Incidence/rate of neo-aortic root dilatation | during a follow up of up to 20 years | Increase of neo-aortic root dilatation of at least 3mm, determined with the same imaging modality and appropriate side-by-side comparison of actual images. |
| Incidence of progression of neo-aortic root dilatation | during a follow up of up to 20 years | Increase of neo-aortic root dilatation of at least 3mm, determined with the same imaging modality and appropriate side-by-side comparison of actual images. |
| Incidence/rate of neo-aortic regurgitation | during a follow up of up to 20 years | Assessment by echocardiography: \- Mild, moderate, severe (50) Assessment by CMR \- Regurgitation fraction and volume by flow measurements in proximal aortic root. |
| Incidence of progression of neo-aortic regurgitation | during a follow up of up to 20 years | Increase \> 1 grade (echocardiography) Increase of regurgitant fraction \>10% on CMR. |
| Incidence/rate of left ventricular systolic dysfunction | during a follow up of up to 20 years | Echocardiography: \- Left ventricular ejection fraction (LVEF) biplane Simpson \<52% for men and \<54% for women (33) CMR: \- LVEF \< 52% |
| Incidence/rate of left ventricular diastolic dysfunction | during a follow up of up to 20 years | Echocardiography: \- Defined according to the current recommendations for the evaluation of left ventricular diastolic function by the America society of echocardiography and the European association of cardiovascular Imaging. |
| Incidence of worsening left ventricular function | during a follow up of up to 20 years | Decrease of LVEF \>5% |
| Incidence/rate of Right ventricular dysfunction | during a follow up of up to 20 years | Echocardiography: \- At least 2 of the following * FAC \<30% * TAPSE \<18mm * TAPSE S' \<10cm/s CMR: \- RVEF \< 50% |
Countries
Austria, France, Netherlands, Spain, Switzerland
Outcome results
None listed