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F1000Research 2019Tetralogy of Fallot (ToF) is the most common type of cyanotic congenital heart disease. Since the first surgical repair in 1954, treatment has continuously improved. The... (Review)
Review
Tetralogy of Fallot (ToF) is the most common type of cyanotic congenital heart disease. Since the first surgical repair in 1954, treatment has continuously improved. The treatment strategies currently used in the treatment of ToF result in excellent long-term survival (30 year survival ranges from 68.5% to 90.5%). However, residual problems such as right ventricular outflow tract obstruction, pulmonary regurgitation, and (ventricular) arrhythmia are common and often require re-interventions. Right ventricular dysfunction can be seen following longstanding pulmonary regurgitation and/or stenosis. Performing pulmonary valve replacement or relief of pulmonary stenosis before irreversible right ventricular dysfunction occurs is important, but determining the optimal timing of pulmonary valve replacement is challenging for several reasons. The biological mechanisms underlying dysfunction of the right ventricle as seen in longstanding pulmonary regurgitation are poorly understood. Different methods of assessing the right ventricle are used to predict impending dysfunction. The atrioventricular, ventriculo-arterial and interventricular interactions of the right ventricle play an important role in right ventricle performance, but are not fully elucidated. In this review we present a brief overview of the history of ToF, describe the treatment strategies currently used, and outline the long-term survival, residual lesions, and re-interventions following repair. We discuss important remaining challenges and present the current state of the art regarding these challenges.
Topics: Cardiac Surgical Procedures; Humans; Pulmonary Valve Insufficiency; Survival Rate; Tetralogy of Fallot; Ventricular Dysfunction, Right
PubMed: 31508203
DOI: 10.12688/f1000research.17174.1 -
Lancet (London, England) Oct 2009Tetralogy of Fallot is the most common form of cyanotic congenital heart disease, and one of the first to be successfully repaired by congenital heart surgeons. Since... (Review)
Review
Tetralogy of Fallot is the most common form of cyanotic congenital heart disease, and one of the first to be successfully repaired by congenital heart surgeons. Since the first procedures in the 1950s, advances in the diagnosis, perioperative and surgical treatment, and postoperative care have been such that almost all those born with tetralogy of Fallot can now expect to survive to adulthood. The startling improvement in outcomes for babies born with congenital heart disease in general-and for those with tetralogy of Fallot in particular-is one of the success stories of modern medicine. Indeed, in many countries adults with tetralogy of Fallot outnumber children. Consequently, new issues have emerged, ranging from hitherto unpredicted medical complications to issues with training for caregivers and resource allocation for this population of survivors. Therefore, evolution of treatment, recognition of late complications, research on disease mechanisms and therapies-with feedback to changes in care of affected children born nowadays-are templates on which the timely discussion of organisation of care of those affected by congenital heart diseases from the fetus to the elderly can be based. Here, we focus on new developments in the understanding of the causes, diagnosis, early treatment, and late outcomes of tetralogy of Fallot, emphasising the continuum of multidisciplinary care that is necessary for best possible lifelong treatment of the 1% of the population born with congenital heart diseases.
Topics: Adult; Aftercare; Arrhythmias, Cardiac; Cardiac Catheterization; Causality; Child; Echocardiography; Heart Valve Prosthesis Implantation; Humans; Infant, Newborn; Patient Care Team; Perioperative Care; Prenatal Diagnosis; Preoperative Care; Prognosis; Pulmonary Valve Insufficiency; Survival Rate; Tetralogy of Fallot; Treatment Outcome
PubMed: 19683809
DOI: 10.1016/S0140-6736(09)60657-7 -
Cardiology 2021Carcinoid heart disease (CHD) is a rare and potentially lethal manifestation of an advanced carcinoid (neuroendocrine) tumor. The pathophysiology of CHD is related to... (Review)
Review
Carcinoid heart disease (CHD) is a rare and potentially lethal manifestation of an advanced carcinoid (neuroendocrine) tumor. The pathophysiology of CHD is related to vasoactive substances secreted by the tumor, of which serotonin is most prominent in the pathophysiology of CHD. Serotonin stimulates fibroblast growth and fibrogenesis, which can lead to cardiac valvular fibrosis. CHD primarily affects right heart valves, causing tricuspid and pulmonic regurgitation and less frequently stenosis of these valves. Left heart valves are usually spared because vasoactive substances such as serotonin are enzymatically inactivated in the lung vasculature. The pathology of CHD is characterized by plaque-like deposition of fibrous tissue on valvular cusps, leaflets, papillary muscles, chordae, and ventricular walls. Symptomatic CHD usually presents between 50 and 70 years of age, initially as dyspnea and fatigue. Echocardiography is the mainstay of imaging and demonstrates thickened right heart valves with limited mobility and regurgitation. Treatment focuses on control of the underlying carcinoid syndrome, targeting subsequent valvular heart disease and managing consequent heart failure. Surgical valve replacement and catheter-directed valve procedures may be effective for selected patients with CHD.
Topics: Carcinoid Heart Disease; Heart Valve Diseases; Heart Valves; Humans; Pathology, Clinical; Pulmonary Valve Insufficiency
PubMed: 33070143
DOI: 10.1159/000507847 -
JACC. Cardiovascular Interventions Dec 2020
Topics: Humans; Pulmonary Valve; Pulmonary Valve Insufficiency; Stents; Treatment Outcome
PubMed: 33303114
DOI: 10.1016/j.jcin.2020.09.015 -
JACC. Clinical Electrophysiology Oct 2021
Topics: Cardiac Surgical Procedures; Defibrillators, Implantable; Humans; Pulmonary Valve; Pulmonary Valve Insufficiency; Tetralogy of Fallot
PubMed: 34674838
DOI: 10.1016/j.jacep.2021.03.014 -
JACC. Cardiovascular Interventions Sep 2022
Topics: Cardiac Catheterization; Heart Valve Prosthesis; Heart Valve Prosthesis Implantation; Humans; Pulmonary Valve; Pulmonary Valve Insufficiency; Tetralogy of Fallot; Treatment Outcome
PubMed: 36137693
DOI: 10.1016/j.jcin.2022.05.021 -
The Journal of Thoracic and... Dec 2014
Topics: Cardiac Surgical Procedures; Female; Heart Ventricles; Humans; Magnetic Resonance Imaging; Male; Pulmonary Valve Insufficiency; Tetralogy of Fallot; Ventricular Dysfunction, Right; Ventricular Function, Right
PubMed: 25173119
DOI: 10.1016/j.jtcvs.2014.07.056 -
JACC. Cardiovascular Interventions Mar 2018
Topics: Heart Valve Prosthesis; Heart Valve Prosthesis Implantation; Humans; Pulmonary Artery; Pulmonary Valve; Pulmonary Valve Insufficiency
PubMed: 29566800
DOI: 10.1016/j.jcin.2018.02.017 -
Hellenic Journal of Cardiology : HJC =... 2022The majority of patients with congenital heart disease (CHD) who have undergone open heart surgery during childhood are possible candidates for additional transcatheter... (Review)
Review
The majority of patients with congenital heart disease (CHD) who have undergone open heart surgery during childhood are possible candidates for additional transcatheter or surgical interventions. One fifth of these conditions usually involve the right ventricular outflow tract (RVOT). Percutaneous pulmonary valve replacement (PPVR) has been widely established as an alternative, less invasive option to surgical pulmonary valve replacement (SPVR). The variability of RVOT anatomy and size, the relative course of the coronary arteries, and the anatomy of the pulmonary artery branches are factors that determine the success of the intervention and the complication rates. Careful and reliable pre-interventional imaging warrants the selection of suitable candidates and minimizes the risk of complications. 2D and 3D fluoroscopy have been extensively used during pre- and peri-interventional assessment. Established imaging techniques such as cardiovascular magnetic resonance (CMR) and computed tomography (CT) and newer techniques such as fusion imaging have proved to be efficient and reliable tools during pre-procedural planning in patients assessed for PPVR.
Topics: Cardiac Catheterization; Cardiac Surgical Procedures; Heart Defects, Congenital; Heart Valve Prosthesis; Heart Valve Prosthesis Implantation; Humans; Pulmonary Valve; Pulmonary Valve Insufficiency; Retrospective Studies; Treatment Outcome
PubMed: 35863726
DOI: 10.1016/j.hjc.2022.06.004 -
Texas Heart Institute Journal Feb 2019Carcinoid heart disease is the collective term for all cardiac manifestations in patients who have carcinoid syndrome. Carcinoid heart disease has a multifactorial... (Review)
Review
Carcinoid heart disease is the collective term for all cardiac manifestations in patients who have carcinoid syndrome. Carcinoid heart disease has a multifactorial pathophysiology, and the right side of the heart is usually involved. Symptoms and signs vary depending upon the affected cardiac components; most typical is right-sided heart failure secondary to diseased tricuspid and pulmonary valves. Despite no single ideal diagnostic test, strong suspicion, coupled with serologic and imaging results, usually enables diagnosis. Advances in imaging, such as speckle-tracking echocardiography and cardiac magnetic resonance, have improved the diagnostic yield. Treatment is challenging, warrants a multidisciplinary approach, and can be medical or surgical depending on the cardiac manifestations. Investigators are exploring the therapeutic use of monoclonal antibodies and new somatostatin analogues. In this review, we cover current knowledge about the pathophysiology, diagnosis, and treatment of carcinoid heart disease.
Topics: Carcinoid Heart Disease; Combined Modality Therapy; Diagnostic Imaging; Disease Management; Global Health; Humans; Morbidity
PubMed: 30833833
DOI: 10.14503/THIJ-17-6562