Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
| Name | Class |
|---|---|
| Hartekind | UNKNOWN |
| Hartstichting | UNKNOWN |
Not provided
Not provided
Not provided
Not provided
Not provided
Patients with the transposition of great arteries (TGA) who undergo atrial switch operation and congenitally corrected TGA (ccTGA) patients have the right ventricle as their systemic ventricle. Function of the systemic right ventricle (SRV) could deteriorate which is associated with impaired prognosis. It is of paramount importance to understand the course and fate of these patients during a long-term follow-up to identify the determinants of adverse outcomes.
In the year 2000, the prevalence of congenital heart diseases (CHD) patients with transposition of great arteries (TGA) was 0.027% in living children and 0.004% in living adults (1). Considering a complete transition to arterial switch operation in the 1990s, it is expected that the number of patients with systemic right ventricle (SRV) decrease over time (2). However, considering the existing number of patients with the TGA who have not undergone arterial switch surgery, SRV remains a challenging issue in the practice of adult congenital heart disease (ACHD) specialists.
TGA is characterized by AV concordance and ventriculo-arterial discordance and is called simple without the presence of associated congenital anomalies. However, complex TGA is when other anomalies are present including VSD (∼45%), LVOTO (∼25%), and CoA (∼5%). TGA's pathogenesis is controversial and there is rare familial occurrence. Male are two times more affected than females. The prognosis of TGA patients without surgery is poor and only exceptional cases survive to adulthood (3).
Heart failure and sudden cardiac death (SCD) are the predominant causes of mortality in TGA patients (4). Patients who undergo atrial switch operation and congenitally corrected TGA (ccTGA) patients are at risk of developing SRV failure in the future (3). The RV is a thin-walled triangular structure acting as a low pressure pump in the normal heart. Because of having only 2 layers, the RV cannot cause the torsion caused by the LV. Due to this geometry and anatomy, the right ventricular function is highly dependent on the loading conditions. The increased afterload that the RV faces in the systemic position causes compensatory RV dilation to maintain the stroke volume. Subsequently, there is increased myocardial wall stress and oxygen demand. The development of heart failure in the SRV is multifactorial. Other potential factors contributing to the SRV failure are impaired coronary reserve or ischemia, myocardial fibrosis, chronotropic incompetence, volume overload from tricuspid regurgitation and arrhythmias (4). Another contributing factor is the reduced baffle compliance in patients with atrial switch. This impairs the preload and stroke volume, especially when there is increased demand. The non-contractile atrial baffles cause impaired atrioventricular transport during tachycardia, therefore causing an inadequate RV filling (4).
Unfortunately, at the moment, the hypothesis that angiotensin-converting enzyme inhibitors (ACE inhibitors), angiotensin II receptor blockers (ARBs), aldosterone antagonists, and beta-blockers can improve the outcome of such patients alone or in combination is not supported by data and evidence. There is no solid recommendation in the 2020 guidelines for the management of ACHD (3).
Previous studies evaluating the fate and outcome of patients with an SRV are either mostly single-centered with a small number of patients or have a short follow-up period (5,6). In a study done by Richard Dobson and colleagues on a national cohort in Scotland, the investigators concluded that patients with an SRV who survive to adulthood have low mortality and good functional status up to the age of 40 (7).
It is of paramount importance to understand the course and fate of these patients during a long-term follow-up to identify the determinants of adverse outcomes. This will enable the investigators to investigate mechanistic pathways of such outcomes. By understanding the risk factors and pathophysiological basis, the investigators can also investigate new diagnostic methods and therapeutic options to improve the quality of life and reduce the mortality of patients with an SRV.
Not provided
Not provided
Not provided
Not provided
Not provided
| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Systemic right ventricle patients |
Not provided
| Measure | Description | Time Frame |
|---|---|---|
| All-cause mortality | Death | Through study completion, an average of 18 years |
| Incident heart failure | Initiation or increase in dosage (if previously prescribed for another cause, i.e. hypertension) of loop diuretic, ACE-inhibitor/ARB therapy, or evidence-based beta blocker therapy because of new heart failure signs and symptoms (shortness of breath, fatigue, reduced exercise tolerance) AND AT LEAST 1 OF THE FOLLOWING: • BNP ≥400 pg/mL OR the following NT-proBNP levels according to age: < 50 years, ≥450 ng/L; 50-75 years, ≥900 ng/L; >75 years, ≥1800 ng/L OR systolic dysfunction (Systemic ventricle EF <50%) OR • BNP 100-400 pg/mL (or NT-proBNP levels below the thresholds according to age given above) AND structural or functional heart disease | Through study completion, an average of 18 years |
| Number of patients with heart failure hospitalization | Rehospitalization, emergency ward visit, 24h observation stay AND Treatment with or increase in dosage if previously prescribed for another cause (i.e.hypertension) of loop diuretics or treatment with IV vasoactive agents. | Through study completion, an average of 18 years |
| Number of patients with arrhythmias | Events that cause emergency ward visit or hospitalization. | Through study completion, an average of 18 years |
| Number of patients with thromboembolic events | Cerebrovascular events including any of (Hemorrhagic or ischemic): 1, TIA (symptoms less than 24 hours) 2, CVA (symptoms more than 24 hours) and also pulmonary embolism. | Through study completion, an average of 18 years |
| Infective endocarditis |
| Measure | Description | Time Frame |
|---|---|---|
| Number of patients with decreased exercise capacity | Evaluated by cardiopulmonary exercise test results. | At two time points. 1: Baseline 2. Through study completion, an average of 18 years |
| Number of patients with right ventricular systolic dysfunction |
Not provided
Inclusion Criteria:
Exclusion Criteria:
* None.
Not provided
Not provided
Not provided
Patients with systemic right ventricles who are followed up in different academic centers in the Netherlands and Belgium.
Not provided
| Name | Affiliation | Role |
|---|---|---|
| Joost P van Melle, MD, PhD | University Medical Center Groningen | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Universitair Ziekenhuis Leuven | Leuven | Belgium | ||||
| Amsterdam University Medical Centre |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 17210844 | Background | Marelli AJ, Mackie AS, Ionescu-Ittu R, Rahme E, Pilote L. Congenital heart disease in the general population: changing prevalence and age distribution. Circulation. 2007 Jan 16;115(2):163-72. doi: 10.1161/CIRCULATIONAHA.106.627224. Epub 2007 Jan 8. | |
| 10784538 | Background | Bull C, Yates R, Sarkar D, Deanfield J, de Leval M. Scientific, ethical, and logistical considerations in introducing a new operation: a retrospective cohort study from paediatric cardiac surgery. BMJ. 2000 Apr 29;320(7243):1168-73. doi: 10.1136/bmj.320.7243.1168. |
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
| ID | Term |
|---|---|
| D014188 | Transposition of Great Vessels |
| D006333 | Heart Failure |
| D001145 | Arrhythmias, Cardiac |
| ID | Term |
|---|---|
| D006330 | Heart Defects, Congenital |
| D018376 | Cardiovascular Abnormalities |
| D002318 | Cardiovascular Diseases |
| D006331 | Heart Diseases |
Not provided
Not provided
Not provided
Not provided
Not provided
Definitie Infective Endocarditis |
| Through study completion, an average of 18 years |
| Number of patients with tricuspid valve surgery | Tricuspid valve surgery | Through study completion, an average of 18 years |
| Number of patients with ventricular Assist Device implantation | Implantation of ventricular assist device | Through study completion, an average of 18 years |
| Number of patients with heart transplantation | Heart transplantation | Through study completion, an average of 18 years |
| Number of patients with aortic aneurysm or dissection | Aortic root more than 40 mm or aneurysm in other parts of aorta. Aortic dissection. | Through study completion, an average of 18 years |
| Number of patients with pulmonary artery hypertension | Mean PAP of more than 25mmHg or systolic PAP of more than 40 mmHg. | Through study completion, an average of 18 years |
| Number of patients with baffle interventions | Surgical or angiographic reintervention of baffles in patients with surgically corrected transposition of the great arteries (due to stenosis or leakage). | Through study completion, an average of 18 years |
| Number of patients with coronary artery interventions | Due to ischemia. Angiographic or surgical intervention. | Through study completion, an average of 18 years |
| Number of patients with device implantation | Including pacmakers, ICDs and CRTs. | Through study completion, an average of 18 years |
Measured by echocardiography qualitatively |
| At two time points. 1: Baseline 2. Through study completion, an average of 18 years |
| Number of patients with left ventricular systolic dysfunction | Measured by echocardiography qualitatively | At two time points. 1: Baseline 2. Through study completion, an average of 18 years |
| Amsterdam |
| Netherlands |
| Medisch Spectrum Twente Enschede | Enschede | Netherlands |
| University Medical Center Groningen | Groningen | Netherlands |
| Leiden University Medical Centre | Leiden | Netherlands |
| Maastricht University Medical Centre | Maastricht | Netherlands |
| Radboud University Medical Centre | Nijmegen | Netherlands |
| Erasmus University Medical Centre | Rotterdam | Netherlands |
| University Medical Centre Utrecht | Utrecht | Netherlands |
| 32860028 | Background | Baumgartner H, De Backer J, Babu-Narayan SV, Budts W, Chessa M, Diller GP, Lung B, Kluin J, Lang IM, Meijboom F, Moons P, Mulder BJM, Oechslin E, Roos-Hesselink JW, Schwerzmann M, Sondergaard L, Zeppenfeld K; ESC Scientific Document Group. 2020 ESC Guidelines for the management of adult congenital heart disease. Eur Heart J. 2021 Feb 11;42(6):563-645. doi: 10.1093/eurheartj/ehaa554. No abstract available. |
| 31853794 | Background | Andrade L, Carazo M, Wu F, Kim Y, Wilson W. Mechanisms for heart failure in systemic right ventricle. Heart Fail Rev. 2020 Jul;25(4):599-607. doi: 10.1007/s10741-019-09902-1. |
| 29525133 | Background | Dennis M, Kotchetkova I, Cordina R, Celermajer DS. Long-Term Follow-up of Adults Following the Atrial Switch Operation for Transposition of the Great Arteries - A Contemporary Cohort. Heart Lung Circ. 2018 Aug;27(8):1011-1017. doi: 10.1016/j.hlc.2017.10.008. Epub 2017 Oct 31. |
| 30760124 | Background | Smood B, Kirklin JK, Pavnica J, Tresler M, Johnson WH Jr, Cleveland DC, Mauchley DC, Dabal RJ. Congenitally Corrected Transposition Presenting in the First Year of Life: Survival and Fate of the Systemic Right Ventricle. World J Pediatr Congenit Heart Surg. 2019 Jan;10(1):42-49. doi: 10.1177/2150135118813125. |
| 23490252 | Background | Dobson R, Danton M, Nicola W, Hamish W. The natural and unnatural history of the systemic right ventricle in adult survivors. J Thorac Cardiovasc Surg. 2013 Jun;145(6):1493-501; discussion 1501-3. doi: 10.1016/j.jtcvs.2013.02.030. Epub 2013 Mar 13. |
| D000013 | Congenital Abnormalities |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D010335 | Pathologic Processes |
| D013568 | Pathological Conditions, Signs and Symptoms |