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World Journal For Pediatric &... Sep 2022Left ventricular hypoplasia is a common finding in various forms of congenital heart disease. Echocardiography in the setting of left ventricular hypoplasia must...
Left ventricular hypoplasia is a common finding in various forms of congenital heart disease. Echocardiography in the setting of left ventricular hypoplasia must comprehensively assess the size and function of all left-sided structures including the mitral valve, left ventricular outflow tract, aortic valve and aortic arch. Of most importance in any variation of left ventricular hypoplasia is the left ventricular inlet. In neonates, the left ventricular inlet often determines the adequacy of the left ventricle and is the most difficult component to treat surgically.
Topics: Echocardiography; Heart Defects, Congenital; Heart Ventricles; Humans; Hypoplastic Left Heart Syndrome; Infant, Newborn; Mitral Valve
PubMed: 36053101
DOI: 10.1177/21501351221114767 -
Seminars in Thoracic and Cardiovascular... 2023Mitral valve replacement (MVR) in the very young is an imposing clinical challenge. Early and late mortality risk is substantial, severe adverse events are common, and... (Review)
Review
Mitral valve replacement (MVR) in the very young is an imposing clinical challenge. Early and late mortality risk is substantial, severe adverse events are common, and redo mitral valve replacement is inevitable. Therapeutic options are limited. In the older infant with an annulus of 17mm or larger, mechanical MVR is associated with low risk of mortality and predictable durability. For the very young with annular hypoplasia, bovine jugular vein conduit MVR appears to offer equivalent or better early outcomes with the possibility of subsequent valve expansion, potentially prolonging the interval to redo MVR. Experience with cylinder MVR and other forms of surgeon-manufactured MVR is quite limited, and there is currently no information on late outcomes or durability.
Topics: Humans; Infant; Animals; Cattle; Mitral Valve; Heart Valve Prosthesis Implantation; Heart Valve Prosthesis; Reoperation; Catheters; Treatment Outcome; Retrospective Studies
PubMed: 36842801
DOI: 10.1053/j.pcsu.2023.01.001 -
Journal of the College of Physicians... Oct 2020Parachute mitral valve is a rare congenital heart defect characterised by a distorted mitral geometry with a single papillary muscle for all mitral chords to attach. It...
Parachute mitral valve is a rare congenital heart defect characterised by a distorted mitral geometry with a single papillary muscle for all mitral chords to attach. It may develop in isolation or as a constillation of Shone syndrome. By comprehensive retrieval of the pertinent articles published since 2000, 22 articles with 149 cases of parachute mitral valve were recruited into this study. The present article revealed that most patients with a parachute mitral valve had a stenotic or regurgitant mitral valve, which is often associated with left heart obstructions, thereby leading to haemodynamic compromise. Therefore, multiple surgical maneuvers are warranted for such patients. Mitral valve repair is preferred over mitral valve replacement for the mitral valve abnormalities so as to avoid the associated complications of valve replacement procedure. The patients' outcomes are satisfactory with a total event-free survival of surgical patients of 84.8%. Key Words: Cardiac surgical procedures, Congenital heart defects, Mitral valve.
Topics: Cardiac Surgical Procedures; Heart Defects, Congenital; Humans; Mitral Valve; Mitral Valve Stenosis; Papillary Muscles
PubMed: 33143830
DOI: 10.29271/jcpsp.2020.10.1069 -
Journal of Cardiovascular Development... Jan 2021The morphological changes that occur in myxomatous mitral valve disease (MMVD) involve various components, ultimately leading to the impairment of mitral valve (MV)... (Review)
Review
The morphological changes that occur in myxomatous mitral valve disease (MMVD) involve various components, ultimately leading to the impairment of mitral valve (MV) function. In this context, intrinsic mitral annular abnormalities are increasingly recognized, such as a mitral annular disjunction (MAD), a specific anatomical abnormality whereby there is a distinct separation between the mitral annulus and the left atrial wall and the basal portion of the posterolateral left ventricular myocardium. In recent years, several studies have suggested that MAD contributes to myxomatous degeneration of the mitral leaflets, and there is growing evidence that MAD is associated with ventricular arrhythmias and sudden cardiac death. In this review, the morphological characteristics of MAD and imaging tools for diagnosis will be described, and the clinical and functional aspects of the coincidence of MAD and myxomatous MVP will be discussed.
PubMed: 33498935
DOI: 10.3390/jcdd8020009 -
Cardiology in the Young Sep 2023Congenital mitral stenosis is a broad-spectrum pathology in which blood flow to the left ventricle is obstructed both functionally and anatomically. Hammock mitral...
Congenital mitral stenosis is a broad-spectrum pathology in which blood flow to the left ventricle is obstructed both functionally and anatomically. Hammock mitral valve, also known as anomalous mitral arcade, is a rare congenital anomaly particularly in infants and children. Hammock mitral valve may not be suitable for repair regarding the advanced dysplastic mitral valve structure. Aortopulmonary window is an unusual cardiac anomaly which is defined as a communication between the main pulmonary artery and the ascending aorta. As a result of the excessive left-to-right shunt, early intervention and surgical closure deemed mandatory to avoid development of severe pulmonary hypertension and its consequences. All patients with an aortopulmonary window necessitates prompt repair immediately. In this brief report, mitral valve replacement with a mechanical valve and repair of aortopulmonary window with a Dacron patch were performed simultaneously in a 5-month-old patient with a hammock mitral valve and accompanying aortopulmonary window.
Topics: Infant; Child; Humans; Mitral Valve; Aortopulmonary Septal Defect; Mitral Valve Stenosis; Pulmonary Artery; Mitral Valve Insufficiency
PubMed: 37092647
DOI: 10.1017/S1047951123000914 -
The American Journal of Cardiology Sep 2020Patients with Shone complex (SC) have multiple left-sided obstructive lesions and thus are at risk for left ventricular (LV) remodeling, LV diastolic dysfunction and...
Patients with Shone complex (SC) have multiple left-sided obstructive lesions and thus are at risk for left ventricular (LV) remodeling, LV diastolic dysfunction and pulmonary hypertension. Yet, to date, there has been no description of hemodynamics in adults with SC. Retrospective chart review of 25 patients with SC who underwent cardiac catheterization at Mayo Clinic, MN between 2002 and 2019 was performed. SC was defined as multiple left-sided obstructive lesions in the presence of an anatomically abnormal mitral valve. Median age was 32 years (22.5, 42) and 15 patients (60%) were female. The majority of patients (84%) had history of coarctation of the aorta, 10 (40%) had subaortic stenosis, 11 (44%) had prior aortic valve replacement, and 10 (40%) had prior mitral valve replacement. Structural disease at the time of catheterization which warranted intervention within the next year was present in 13 patients (52%). The mean LV end-diastolic pressure was 21.3 ± 9.0 mm Hg (>15 mm Hg in 71%), pulmonary artery peak systolic pressure was 55.4 ± 13.4 mm Hg, and the pulmonary artery mean pressure was 37.0 ± 9.4 mm Hg (>20 mm Hg in 96%). During a mean follow-up of 8.3 ± 4.4 years, there were 7 deaths (28%) and 3 additional patients (12%) underwent cardiac transplantation. In conclusion, adults with SC who underwent catheterization showed significant left-sided heart and pulmonary vascular remodeling. Elevated LV end-diastolic pressure and pulmonary artery pressures were highly prevalent. There were high mortality and cardiac transplant rates in our cohort.
Topics: Adult; Aortic Coarctation; Aortic Valve Stenosis; Female; Hemodynamics; Humans; Male; Mitral Valve; Retrospective Studies; Young Adult
PubMed: 32703525
DOI: 10.1016/j.amjcard.2020.06.024 -
Current Treatment Options in... May 2022The purpose of this review is to explore the prevalence and risk factors for a malignant phenotype in mitral valve prolapse (MVP) characterized by life-threatening...
PURPOSE OF REVIEW
The purpose of this review is to explore the prevalence and risk factors for a malignant phenotype in mitral valve prolapse (MVP) characterized by life-threatening ventricular arrhythmias and sudden cardiac arrest and death (SCD), including mechanistic and pathophysiologic findings and mechanism-based potential therapies.
RECENT FINDINGS
A malignant phenotype in MVP characterized by life-threatening arrhythmias has long been recognized, although MVP is often benign. Efforts to identify this malignant phenotype have revealed potential risk factors for SCD that include elongated, myxomatous leaflets, ECG changes and complex ventricular ectopy. More recently, malignant MVP has been associated with myocardial fibrosis in the papillary muscles and inferobasal left ventricular wall. This localization suggests a central role of prolapse-induced mechanical forces on the myocardium in creating an arrhythmogenic substrate and triggering life-threatening arrhythmias. This mechanism for fibrosis is also consistent with imaging evidence of prolapse-induced mechanical changes in the papillary muscles and inferobasal left ventricular wall. Currently, no therapy to prevent SCD in malignant MVP has been established and limited clinical data are available. Mechanistic information and prospective study have the potential to identify patients at risk of SCD and preventive strategies.
SUMMARY
Malignant MVP relates to unique properties and mechanical abnormalities in the mitral valve apparatus and adjacent myocardium. Increased understanding of disease mechanisms and determinants of arrhythmias is needed to establish effective therapies.
PubMed: 35784809
DOI: 10.1007/s11936-022-00956-3 -
Current Cardiology Reviews 2023Mitral valve prolapse (MVP) is the most frequent valvulopathy in the general population, with usually a favourable prognosis. Although it can be associated with some...
Mitral valve prolapse (MVP) is the most frequent valvulopathy in the general population, with usually a favourable prognosis. Although it can be associated with some complications, ventricular arrhythmias (VA) and sudden cardiac death (SCD) are the most worrying. The estimated risk of SCD in MVP is between 0.2% to 1.9% per year, including MVP patients with and without severe mitral regurgitation (MR). The association between SCD and MVP is expressed by a phenotype called "malignant MVP" characterized by transthoracic echocardiography (TTE) findings such as bileaflet myxomatous prolapse and mitral annulus disjunction (MAD), ECG findings such as repolarization abnormalities, complex ventricular arrhythmias (c-VAs) and LV fibrosis of papillary muscles (PMs) and inferobasal wall visualized by late gadolinium enhancement cardiac magnetic resonance (LGE-CMR). Therefore, attention is raised for patients with "arrhythmic MVP" characterized from an ECG point of view by frequent premature ventricular contractions (PVCs) arising from one or both PMs as well as by T-wave inversion in the inferolateral leads. In athletes, SCD is the most frequent medical cause of death and in young subjects (< 35 years) usually is due to electrical mechanism affecting who has a silent cardiovascular disease and are not considered per se a cause of increased mortality. In MVP, SCD was reported to happen during sports activity or immediately after and valve prolapse was the only pathological aspect detected. The aim of the present paper is to explore the association between SCD and MVP in athletes, focusing attention on ECG, TTE in particular, and CMR findings that could help to identify subjects at high risk for complex arrhythmias and eventually SCD. In addition, it is also examined if sports activity might predispose patients with MVP to develop major arrhythmias.
Topics: Humans; Mitral Valve Prolapse; Contrast Media; Gadolinium; Death, Sudden, Cardiac; Mitral Valve; Ventricular Premature Complexes; Prolapse; Athletes
PubMed: 36545732
DOI: 10.2174/1573403X19666221220163431 -
BioRxiv : the Preprint Server For... Aug 2023Mitral valve (MV) disease including myxomatous degeneration is the most common form of valvular heart disease with an age-dependent frequency. Genetic evidence indicates...
BACKGROUND
Mitral valve (MV) disease including myxomatous degeneration is the most common form of valvular heart disease with an age-dependent frequency. Genetic evidence indicates mutations of the transcription factor are associated with MV defects, including mitral valve regurgitation. In this study, we sought to determine whether murine and its closely related factor, , are required in valvular endothelial cells (VECs) for the maintenance of MV leaflets, including VEC junctions and the stratified trilaminar extracellular matrix (ECM).
METHODS
Adult mice carrying tamoxifen-inducible, endothelial cell (EC)-specific, compound mutations (i.e., EC--DKO mice) were used to study the function of and in the maintenance of mitral valves. The EC-mutations of were induced at 7 - 8 weeks of age by tamoxifen treatment, and abnormalities in the MVs of EC--DKO mice were assessed via whole-mount immunostaining, immunohistochemistry, and Movat pentachrome/Masson's Trichrome staining.
RESULTS
EC-deletions of and in mice resulted in abnormally extended and thicker mitral valves by causing defects in regulation of ECM organization with increased proteoglycan and decreased collagen. Notably, reticular adherens junctions were found in VECs of control MV leaflets, and these reticular structures were severely disrupted in EC- mutant mice. PROX1, a key regulator in a subset of VECs on the fibrosa side of MVs, was downregulated in EC- mutant VECs. Furthermore, we determined the precise location of lymphatic vessels in murine MVs, and these lymphatic vessels were aberrantly expanded in EC- mutant mitral valves.
CONCLUSIONS
Our results indicate that and are required for maintaining the integrity of the MV, including VEC junctions, ECM organization, and lymphatic vessels to prevent myxomatous mitral valve degeneration.
PubMed: 37693499
DOI: 10.1101/2023.08.30.555455 -
Journal of Cardiac Surgery Jul 2022The present study sought to study the imaging associations of left atrioventricular valve (mitral) atresia and patent aortic root in patients with isomerism of atrial...
AIM
The present study sought to study the imaging associations of left atrioventricular valve (mitral) atresia and patent aortic root in patients with isomerism of atrial appendages as detected on multidetector computed tomography (CT) angiography MATERIALS AND METHODS: The CT angiography studies performed at a tertiary medical center from January 2014 to December 2021 were retrospectively evaluated to identify patients with left atrioventricular valve atresia and patent aortic root with isometric atrial chambers. The morphology of the atrial appendage and extent of pectinate muscles was used to identify the type of isomeric atrial chambers. A comprehensive assessment of discordant arrangement of organs and the variations in venoatrial connections was done.
RESULTS
Left atrioventricular valve atresia in the presence of patent aortic root and isomeric atrial appendages was identified in eight patients (four males). The mean age at diagnosis was 7 years. Right isomerism of the atrial appendages was seen in five patients while three patients had left isomerism. Disharmonious patterns of abdominal visceral arrangement were seen in two patients. Atrial and ventricular septal defects were seen in all patients. The most common pattern of ventricular arterial connection was DORV (double outlet right ventricle) (n = 4). While five patients had normally related great arteries, three had aorta anterior to the pulmonary trunk. Right ventricular outflow obstruction was seen in seven patients. Right aortic arch with mirror image branching was seen in four patients. Coronary artery anomalies were seen in four patients.
CONCLUSIONS
The presence of left atrioventricular valve atresia and patent aortic root with isomeric atrial appendages is a rare congenital anomaly frequently associated with anomalous systemic and pulmonary drainage, conotruncal anomalies, and right ventricular outflow obstruction. Hence, comprehensive CT-based evaluation is indispensable in providing detailed anatomy and aids in presurgical planning.
Topics: Aorta; Atrial Appendage; Double Outlet Right Ventricle; Heart Defects, Congenital; Humans; Male; Multidetector Computed Tomography; Retrospective Studies; Ventricular Outflow Obstruction
PubMed: 35396763
DOI: 10.1111/jocs.16468