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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 -
Vascular Health and Risk Management 2023Pulmonary stenosis (PS) is mainly a congenital defect that accounts for 7-12% of congenital heart diseases (CHD). It can be isolated or, more frequently, associated with... (Review)
Review
Pulmonary stenosis (PS) is mainly a congenital defect that accounts for 7-12% of congenital heart diseases (CHD). It can be isolated or, more frequently, associated with other congenital defects (25-30%) involving anomalies of the pulmonary vascular tree. For the diagnosis of PS an integrated approach with echocardiography, cardiac computed tomography and cardiac magnetic resonance (CMR) is of paramount importance for the planning of the interventional treatment. In recent years, transcatheter approaches for the treatment of PS have increased however, meaning surgery is a possible option for complicated cases with anatomy not suitable for percutaneous treatment. The present review aims to summarize current knowledge regarding diagnosis and treatment of PS.
Topics: Humans; Pulmonary Valve Stenosis; Echocardiography; Magnetic Resonance Imaging
PubMed: 37416511
DOI: 10.2147/VHRM.S380240 -
Journal of the American College of... Oct 2022
Topics: Humans; Hypertension, Pulmonary; Aortic Valve Stenosis; Transcatheter Aortic Valve Replacement; Aortic Valve; Treatment Outcome; Risk Factors; Heart Valve Prosthesis
PubMed: 36265956
DOI: 10.1016/j.jacc.2022.09.005 -
Texas Heart Institute Journal Jun 2015Patients with congenital heart disease and pulmonary valve disease need multiple procedures over their lifetimes to replace their pulmonary valves. Chronic pulmonary... (Review)
Review
Patients with congenital heart disease and pulmonary valve disease need multiple procedures over their lifetimes to replace their pulmonary valves. Chronic pulmonary stenosis, regurgitation, or both have untoward effects on ventricular function and on the clinical status of these patients. To date, all right ventricle-pulmonary artery conduits have had relatively short lifespans. Percutaneous pulmonary valve implantation, although relatively new, will probably reduce the number of operative procedures that these patients will have to undergo over a lifetime. Refinement and further development of this procedure holds promise for the extension of this technology to other patient populations.
Topics: Cardiac Catheterization; Heart Valve Prosthesis Implantation; Humans; Pulmonary Valve
PubMed: 26175629
DOI: 10.14503/THIJ-14-4276 -
Trends in Cardiovascular Medicine Feb 2022In patients with severe aortic stenosis (AS), pulmonary hypertension (PH) typically is indicative of a decompensated disease state with exhausted compensatory mechanisms... (Review)
Review
In patients with severe aortic stenosis (AS), pulmonary hypertension (PH) typically is indicative of a decompensated disease state with exhausted compensatory mechanisms of the left ventricle, meaning a heart failure state resulting from AS-related "cardiac injury". In the present review article, we discuss new insights into the pathophysiology of AS-induced PH, the prognostic impact, and potential options to prevent and treat PH in this setting. We emphasize recent data from studies focused on invasive hemodynamics in patients with severe AS that are being evaluated for aortic valve replacement, particularly the key relevance of combined pre- and post-capillary PH. This latter represents an advanced form of cardiac injury that is often associated with right ventricular dysfunction and poor prognosis. Given this context, we highlight the relevance of performing right heart catheterization in combination with non-invasive imaging for the comprehensive assessment of AS patients that are being evaluated for aortic valve replacement. Such comprehensive assessment plays a key role not only to precisely define the extent of AS-related cardiac injury but also to distinguish those PH forms that are unrelated to AS.
Topics: Aortic Valve; Aortic Valve Stenosis; Cardiac Catheterization; Heart Valve Prosthesis Implantation; Hemodynamics; Humans; Hypertension, Pulmonary
PubMed: 33346089
DOI: 10.1016/j.tcm.2020.12.005 -
JACC. Cardiovascular Imaging Apr 2019
Topics: Aortic Valve; Aortic Valve Stenosis; Heart Valve Prosthesis; Humans; Hypertension, Pulmonary; Prognosis; Transcatheter Aortic Valve Replacement
PubMed: 30553680
DOI: 10.1016/j.jcmg.2018.04.003 -
BMJ Case Reports Nov 2017A 32-year-old woman suffering from systemic lupus erythematosus presented with a 6-week history of progressive dyspnoea and pleuritic chest pain. Examination was normal...
A 32-year-old woman suffering from systemic lupus erythematosus presented with a 6-week history of progressive dyspnoea and pleuritic chest pain. Examination was normal apart from reduced air entry at the lung bases.Arterial blood gases showed hypoxaemia and chest X-ray revealed raised hemidiaphragms without any pleural effusions. Lung function showed a restrictive pathology while high-resolution chest CT and CT pulmonary angiogram were negative. Echocardiography showed normal ventricular diameters and no pericardial effusion. Reduced lung volumes and a positive fluoroscopic sniff test lead to a diagnosis of shrinking lung syndrome. Symptoms improved following treatment with glucocorticoids and non-invasive ventilation, but there was no change in lung function.A year later, our patient presented again with worsening dyspnoea. This time echocardiography revealed severe mitral stenosis with pulmonary hypertension. Mitral valve replacement was performed and dyspnoea resolved. Histology showed Libman-Sachs endocarditis.
Topics: Adult; Diagnosis, Differential; Dyspnea; Echocardiography; Familial Primary Pulmonary Hypertension; Female; Humans; Lung; Lupus Erythematosus, Systemic; Mitral Valve Stenosis; Rare Diseases; Respiratory Function Tests; Treatment Outcome
PubMed: 29122894
DOI: 10.1136/bcr-2017-220162