tetralogy of fallot adult

BACKGROUND: Historically, Dr William Glenn performed the first classic superior cavopulmonary anastomosis in a seven-year-old child at Yale in 1958. By 1990, this operation was performed consecutively in over 90 patients. With over 60 years of follow-up, this is the longest survival record of early Glenn patients from the first 30 years. METHODS: We performed a single center, retrospective evaluation of patients undergoing a Glenn operation. A collected list of surviving patients, previously updated in 1988, included demographics, age at procedure, and underlying diagnosis...

OBJECTIVE: Residual sequelae after surgical repair of tetralogy of Fallot (rTOF) affect clinical outcome. We investigated the prognostic impact of right ventricular (RV) dyssynchrony in adults with rTOF years after the surgical repair. METHODS: Patients from the Swiss Adult Congenital HEart disease Registry were included. NT-proBNP levels, echocardiography, exercise testing and MRI data were collected. An offline strain analysis to quantify RV-ventricular and interventricular dyssynchrony was performed...

OBJECTIVE: To assess risks and benefits of cardiac intervention in adults with Down syndrome (DS). PATIENTS AND METHODS: A retrospective review was conducted using data from a study we published in 2010. Patients aged 18 years or older with DS who underwent cardiac operation or percutaneous intervention from February 2009 through April 2022 (new cohort) were compared with patients in the previous study (January 1969 through November 2007; remote cohort) at Mayo Clinic...

An adult with unrepaired tetralogy of Fallot presented with frequent tet spells. Her course was complicated by severe cyanotic spells and tachycardia-bradycardia syndrome that limited beta blocker use to stabilize her spells. She markedly improved after disopyramide initiation and underwent successful tetralogy of Fallot repair with excellent functional outcome.

This manuscript describes the feasibility and approach to the assessment and performance of transcatheter pulmonary valve replacement (tPVR) in patients with surgically-created "double-barrel" right ventricular outflow tracts (RVOT). Patients with tetralogy of Fallot may have coronary anomalies which prohibit the performance of traditional tetralogy of Fallot repair. In certain cases, this may necessitate the placement of a right ventricle to pulmonary artery conduit in addition to the native RVOT, which is left in situ, creating so-called "double-barrel" RVOTs...

The increasing prevalence of atrial fibrillation (AF) in adults with congenital heart disease raises significant questions regarding its management. The unique underlying anatomic and physiological background further adds to the difficulty in eliminating the AF burden in these patients. Herein, we provide an overview of the current knowledge on the pathophysiology and risk factors for AF in adult congenital heart disease, with a special focus on the existing challenges in AF ablation. Emerging imaging modalities and ablation techniques might have a role to play...

A 33-year-old man, who had previously undergone repair for Tetralogy of Fallot, presented with extensive infective endocarditis. Following thorough preoperative preparation and evaluation, we performed a simultaneous quadruple valve replacement alongside the repatching of the remaining defect. We posit that this comprehensive one-stage surgical intervention not only enhanced the patient's quality of life but also reduced the necessity for future reoperations. Our approach offers valuable insights for managing adult patients with repaired congenital heart diseases and multiple valve pathologies...

Tetralogy of Fallot (TOF) is a congenital heart defect characterized by four interlinked cardiac anomalies, leading to cyanosis and significant health risks if left untreated. We present the management and outcome of an adult patient with unrepaired TOF at 34 weeks of pregnancy at a central Johannesburg hospital. Uncorrected TOF in adulthood, especially in the late stages of pregnancy, is rare, presenting challenges for both the mother and baby. This case report outlines our successful management strategy, highlighting the importance of careful hemodynamic stability...

In the general population, the most cited barriers to physical activity (PA) are time, energy, and motivation. Consequently, despite the significant contribution of PA to health and well-being, many individuals are insufficiently active. Physical inactivity and sedentary lifestyles increase the risk of acquired cardiometabolic disease, a risk that may complicate and is compounded by the anatomic and physiologic features inherent in the patient with repaired tetralogy of Fallot (ToF). Individuals with ToF commonly present with reduced exercise capacity and PA levels...

Neonatal repair has completely changed the clinical history of patients with tetralogy of Fallot (ToF); however, these patients carry a significant risk of severe arrhythmias and sudden cardiac death in the long term. The exact mechanism for late sudden cardiac death is multifactorial and still not well defined, and the risk stratification for primary prophylaxis in these patients remains challenging. Cardiopulmonary exercise testing (CPET) is a well-established and safe method to assess cardiopulmonary function in children and adults with congenital heart disease...

There has been significant progress in the prevention of sudden cardiac death in repaired tetralogy of Fallot. Contemporary cohorts report greater survival attributable to improved surgical techniques, heart failure management, and proactive strategies for risk stratification and management of ventricular arrhythmias including defibrillator implantation and ablation technology. Over the last 25 years, our understanding of predictive risk factors has also improved from invasive and more limited measures to individualized risk prediction scores based on extensive demographic, imaging, electrophysiological, and functional data...

The majority of women with repaired tetralogy of Fallot are able to tolerate pregnancy with a low risk of cardiovascular events. However, proactive contraceptive advice, prepregnancy counselling, and care by a pregnancy heart team with expertise in congenital heart disease are key to ensuring a good outcome for mother and baby. Maternal and fetal risks are increased in the presence of severe valvular stenosis, poorly tolerated arrhythmia, significant ventricular dysfunction, and cyanosis. It is unusual to see cyanotic adults with tetralogy of Fallot, whether unoperated or shunt palliated; pregnancy risks are greatly reduced by completing their repair before pregnancy is undertaken...

Individuals living with tetralogy of Fallot require lifelong specialized congenital heart disease care to monitor for and manage potential late complications. However, access to cardiology care remains a challenge for many patients, as does access to mental health services, dental care, obstetrical care, and other specialties required by this population. Inequities in health care access were highlighted by the COVID-19 pandemic and continue to exist. Paradoxically, many social factors influence an individual's need for care, yet inadvertently restrict access to it...

The risk of premature death in adult patients with repaired tetralogy of Fallot is real and not inconsiderable. From the third decade of life, the incidence of malignant ventricular arrhythmia (VA) is known to exponentially rise. Progressive adverse mechanoelectrical modelling because of years of volume and/or pressure overload from residual pulmonary valve dysfunction and ventricular scar creates the perfect catalyst for VA. Although potentially lifesaving, implantable cardiac defibrillators are associated with substantial psychological and physical morbidity...

Background: Despite surgical advances, children with tetralogy of Fallot/pulmonary atresia/major aortopulmonary collaterals (TOF/PA/MAPCAs) are subject to chronic right ventricular (RV) pressure and volume overload. Current diagnostic tools do not identify adverse myocardial remodeling and cannot predict progression to RV failure. We sought to identify a noninvasive, circulating signature of the systemic response to right heart stress to follow disease progression. Methods: Longitudinal data were collected from patients with TOF/PA/MAPCAs (N = 5) at the time of (1) early RV pressure overload and (2) late RV pressure and volume overload...

To demonstrate prognostic utility of left atrial strain (LAS) in adult patients with repaired Tetralogy of Fallot (rTOF). Adults patients with rTOF were prospectively enrolled in this study between years 2011 and 2015. Left atrium (LA) phasic functions were assessed using 2D speckle tracking echocardiography. Association of LA strain (LAS) with primary (any cardiovascular event) and secondary (death, heart failure or arrhythmia) composite endpoints was assessed using Cox regression analysis. Hundred-and-twelve rTOF patients, in whom LAS was feasible and were in sinus rhythm, were included in the final analysis (age 33 ± 10 years, 68[61%] male)...

BACKGROUND: Tetralogy of Fallot (TOF) is a complex cyanotic congenital heart disease. As most patients with TOF undergo palliative or radical surgical repair during childhood, cardiac surgery under cardiopulmonary bypass (CPB) for adult survivors with unrepaired TOF is exceedingly rare. CASE PRESENTATION: A 41-year-old woman with unrepaired TOF, pulmonary atresia (PA), and major aortopulmonary collateral arteries (MAPCAs) developed acute infectious endocarditis (IE)...

INTRODUCTION AND OBJECTIVES: Our aim was to assess the impact of prosthetic pulmonary valve replacement (PVR) in patients with repaired tetralogy of Fallot (rTOF) on changes in biventricular volumes and function and on adverse cardiac events. METHODS: Adults with rTOF were identified from the SACHER-registry. Data from serial cardiac magnetic resonance imaging, echocardiography, exercise capacity and n-terminal pro b-type natriuretic peptide (NT-proBNP) were collected...

Pediatric heart disease is a large and diverse field with an overall prevalence estimated at 6 to 13 per 1,000 live births. This document discusses appropriateness of advanced imaging for a broad range of variants. Diseases covered include tetralogy of Fallot, transposition of great arteries, congenital or acquired pediatric coronary artery abnormality, single ventricle, aortopathy, anomalous pulmonary venous return, aortopathy and aortic coarctation, with indications for advanced imaging spanning the entire natural history of the disease in children and adults, including initial diagnosis, treatment planning, treatment monitoring, and early detection of complications...

OBJECTIVE: Machine learning (ML) can facilitate prediction of major adverse cardiovascular events (MACEs) in repaired tetralogy of Fallot (rTOF). We sought to determine the incremental value of ML above expert clinical judgement for risk prediction in rTOF. METHODS: Adult congenital heart disease (ACHD) clinicians (≥10 years of experience) participated (one cardiac surgeon and four cardiologists (two paediatric and two adult cardiology trained) with expertise in heart failure (HF), electrophysiology, imaging and intervention)...