Canadian Adult Congenital Heart Disease Intervention Registry
Atrial Septal DefectPatent Foramen Ovale5 moreThe ACHDi Registry study will create a foundational database for adult congenital heart disease interventions. This Pan-Canadian Registry will collect clinical and patient-reported information that will enable the evaluation of care processes and outcomes in five most common ACHDi interventions by enabling prospective and retrospective registry-based studies to answer important clinical practice and policy-relevant questions.
Clinical Impact of Rapid Prototyping 3D Models for Surgical Management
Double Outlet Right VentricleTransposition of the Great Arteries2 morePatient-specific, 3D printed models have been utilized in preoperative planning for many years. Among researchers and clinicians, there is a perception that preoperative exposure to 3D printed models, derived from patient images (CT or MRI), aid in procedural planning. 3D printed models for heart surgery have the potential to improve a clinician's preparedness and therefore may reduce surgically-related morbidity and mortality. This randomized clinical trial aims to evaluate whether pre-procedural planning of surgeons exposed to a patient-specific 3D printed heart model will decrease cardiopulmonary bypass time, morbidity, and mortality.
Effect of Phosphodiesterase-5 Inhibition With Tadalafil on SystEmic Right VEntricular Size and Function...
Heart DefectsCongenital1 moreThis study assesses in a double-blind, randomized, placebo-controlled multi-center pilot trial the effect of PDE-5 inhibition with Tadalafil on right ventricle size and function, exercise capacity and neurohumoral activation in adults with congenital heart disease and a right ventricle in subaortic position over a 3-year follow-up period.
Oxygen Consumption-based Assessments of Hemodynamics in Neonates Following Congenital Heart Surgery...
Transposition of the Great VesselsHypoplastic Left Heart Syndrome1 moreThe purpose of the Oxy-CAHN study is to improve the monitoring capabilities of newborn infants recovering from congenital heart surgery. Currently, we utilize important but unsophisticated measures, such as vital signs and lactate measurements, to monitor these patients. Although they are useful in categorizing patients as well or unwell, these signs currently lack the power quantify a patient's risk for cardiac arrest. More to the point, they are mostly indirect measures of what we really are assessing, which is tissue oxygen delivery. Our group has significant expertise with devices which quantify the amount of oxygen that a baby consumes every minute. Historically, these values are more commonly used in combination with other measures to assess nutritional and metabolism status. In critically ill patients, however, the volume of oxygen consumed by a patient may be limited by the amount of oxygen their circulation delivers. This may represent a critical relationship, which has been previously described, but not exploited for the purpose of identifying patients with critically low oxygen delivery. The aims of this study are therefore (1) to demonstrate that oxygen consumption can be safely and precisely measured continuously in newborns undergoing one of two common congenital heart surgeries, (2) to determine whether postoperative circulatory failure is associated with a precedent change in oxygen consumption, and (3) to determine whether the addition of the oxygen-based measurements (including oxygen consumption and venous oxygen saturations) to standardly measured parameters will add power in predicting which patients will experience postoperative circulatory failure. If successful, this study may improve our capacity to non-invasively and continuously monitor patients following the highest risk congenital heart surgeries, and in the future,to create an algorithm which quantifies a patients risk for having a cardiac arrest. This may permit providers to intervene on these patients earlier, improving the morbidity and mortality associated with congenital heart disease.
The Boston Circulatory Arrest Study: Antecedents and Correlates of Well-Being in Adults With Congenital...
Transposition Great ArteriesExecutive DysfunctionIn the landmark Boston Circulatory Arrest Study, neurologic and developmental status was measured following infant heart surgery and then prospectively at ages 1, 2.5, 4, 8, and 16 years, with findings of significant neurocognitive deficits and brain MRI abnormalities regardless of operative management. To date, no study has evaluated the neuropsychological and neuroimaging antecedents and correlates of well-being in adults with congenital heart disease, a population now >1 million and projected to grow at 5% per year. The investigators propose to study the Boston cohort at ages 24-29 years to assess the associations of adult well-being with childhood and adolescent executive function, other measures of mental health and cognitive function, adolescent brain MRI findings, and clinical variables; findings will guide the design of interventions in childhood to optimize outcomes in adults with congenital heart disease.
Effects of Beta-adrenergic in Adults w/Transposition of Great Arteries on Systemic Ventricular Function...
Congenital DisordersThe purpose of this chart review study is to examine the effects of beta -adrenergic blocking agents on systemic ventricular dimensions, systemic atrioventricular valve function and exercise tolerance in patients with transposition of the great arteries (TGA) and systemic ventricular dysfunction.
Acute Maternal Hyperoxygenation for Fetal Transposition of the Great Arteries (TGA)
TGA - Transposition of Great ArteriesThis prospective study will examine whether transient maternal hyperoxygenation is useful as a diagnostic test to more accurately detect TGA patients with poor vs. good neonatal intra-cardiac mixing of blood, based on the in-utero response to oxygen exposure.
Reproduction and Survival After Cardiac Defect Repair
Cardiovascular DiseasesHeart Diseases14 moreTo create a registry of all Oregon children undergoing surgical repair of congenital heart disease since 1958 in order to determine mortality, morbidity, and disability after surgery and to assess the safety of pregnancy in women with corrected congenital heart disease and the risk of prematurity and occurrence of congenital heart defects in offspring.
Outcomes of Transposition of the Great Arteries After Arterial Switch Operation
Transposition of Great VesselsOutcome1 moreTransposition of the great arteries (TGA) is a complex cyanotic congenital heart disease and patients suffer from a high mortality rate within one year of age without appropriate management. The therapeutic effect of arterial switch operation (ASO) is satisfactory with low surgery mortality of 2-5%, and thus, has become the treatment of choice for surgical correction of d-TGA. Outcomes of ASO in TGA in china are rare. This is a retrospective study reporting the outcomes of ASO in TGA.
Sudden Cardiac Death in Systemic Right Ventricle
Sudden Cardiac DeathCongenital Heart Disease1 moreIn complete transposition of the great arteries (TGA) with previous atrial switch repair, and congenitally corrected transposition of the great arteries (ccTGA), the morphological right ventricle and its tricuspid valve continue to support the systemic circulation. This results in late complications including including sudden death. This retrospective multicentric study aims to evaluate the prevalence of SCD in a contemporary population of patients with a systemic RV and identify specific risk factors for SCD and hemodynamically significant ventricular arrhythmia This registry records demographics, clinical, imaging data, electrophysiological and laboratory of patients with a sRV and a transposition of the great arteries Primary end points are defined by sudden cardiac death, near-miss sudden death, as well as sustained VT requiring defibrillation.