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Molecular Genetics and Metabolism... Dec 2020describe cardiovascular findings from echocardiograms and electrocardiograms in patients with Classic Homocystinuria.
OBJECTIVE
describe cardiovascular findings from echocardiograms and electrocardiograms in patients with Classic Homocystinuria.
METHODS
this retrospective exploratory study evaluated fourteen subjects with Classic Homocystinuria (median age = 27.3 years; male = 8, B6-non-responsive = 9 patients), recruited by convenience sampling from patients seen Hospital de Clínicas de Porto Alegre (Brazil), between January 1997 and July 2020. Data on clinical findings, echocardiogram and electrocardiogram were retrieved from medical records.
RESULTS
Eight patients presented some abnormalities on echocardiogram ( = 6) or electrocardiogram ( = 5). The most frequent finding was mild tricuspid regurgitation ( = 3), followed by mitral valve prolapse, mild mitral regurgitation, enlarged left atrium and aortic valve sclerosis ( = 2 patients each). Aortic root ectasia was found in one patient. Venous thrombosis was reported in six patients: deep vein thrombosis of lower limbs (n = 3), ischaemic stroke ( = 1), cerebral venous sinus thrombosis (n = 1) and pulmonary vein thrombosis (n = 1).
CONCLUSION
mild valvulopathies seen to be common in patients with Classic Homocystinuria, but more studies regarding echocardiogram and electrocardiogram in this population are needed to draw absolute conclusions.
PubMed: 33335839
DOI: 10.1016/j.ymgmr.2020.100693 -
Kardiologia Polska Nov 2019Stress echocardiography is a safe, low‑cost, widely available, radiation‑free versatile imaging modality that is becoming increasingly recognized as a valuable tool... (Review)
Review
Stress echocardiography is a safe, low‑cost, widely available, radiation‑free versatile imaging modality that is becoming increasingly recognized as a valuable tool in the assessment of coronary heart disease. In recent years, there has also been an increasing use of stress echocardiography in the assessment of nonischemic cardiac disease given its unique ability for simultaneous assessment of both functional performance and exercise‑related noninvasive hemodynamic changes, which can help guide treatment and inform about the prognosis of the patients. Today, in the echocardiography laboratory, we can not only detect wall motion abnormalities resulting from coronary artery stenosis, but also detect alterations to the coronary microvessels, left ventricular systolic and diastolic parameters, heart valves, pulmonary circulation, alveolar‑capillary barrier, and right ventricle. The role of stress echo has been well established in several pathologies, such as aortic stenosis and hypertrophic cardiomyopathy; however, other indications, namely the results of diastolic stress testing and pulmonary hypertension, need additional data and research. This paper presents the current evidence for the role of stress testing in mitral regurgitation, aortic stenosis, hypertrophic cardiomyopathy, heart failure with preserved ejection fraction, and pulmonary hypertension.
Topics: Aortic Valve Stenosis; Cardiomyopathy, Hypertrophic; Cardiovascular Diseases; Echocardiography, Stress; Heart Failure; Humans; Hypertension, Pulmonary; Mitral Valve Insufficiency
PubMed: 31647477
DOI: 10.33963/KP.15032 -
The Journal of Thoracic and... Dec 2017The rapid development of surgical and less-invasive percutaneous mitral valve repair procedures has increased interest in mitral valve anatomy. We characterize the...
OBJECTIVES
The rapid development of surgical and less-invasive percutaneous mitral valve repair procedures has increased interest in mitral valve anatomy. We characterize the morphologic variability of the mitral valve leaflets and provide the size of their particular parts.
METHODS
We studied 200 autopsied human hearts from white individuals without any valvar diseases. We measured the intercommissural and aorto-mural diameters of the mitral annulus and identified the leaflets and their scallops. We also noted the base and the height of the inferoseptal commissure, superolateral commissure, anterior mitral leaflet, and posterior mitral leaflet with their scallops.
RESULTS
Variations in posterior mitral leaflet were found in 55 specimens (27.5%), and variations in anterior mitral leaflet were found in 5 hearts (2.5%). The most common variations included valves with 1 accessory scallop between P3 and inferoseptal commissure (7%), accessory scallop between P1 and superolateral commissure (4%), connections of P2 and P3 scallops (4%), connections of P1 and P2 scallops (3%), and accessory scallop in anterior mitral leaflet (2.5%).
CONCLUSIONS
In all cases, the mitral valve is built by 2 main leaflets with possible variants in scallops (29.5%). The variations are largely associated with posterior mitral leaflet and are mostly related to the presence of accessory scallop. Anatomically, the anterior mitral leaflet is not divided into scallops, but could have an accessory scallop (2.5%). Understanding the anatomy of the mitral valve leaflets helps with the planning and performing of mitral valve repair procedures. Variations in scallops may affect repair procedures, but unfortunately cannot be predicted by any of the annular sizes.
Topics: Adult; Aged; Anatomic Landmarks; Autopsy; Female; Heart Defects, Congenital; Humans; Male; Middle Aged; Mitral Valve
PubMed: 28893395
DOI: 10.1016/j.jtcvs.2017.07.067 -
Chinese Medical Journal Sep 2019In recent years, attempting the biventricular pathway or biventricular conversions in patients with borderline ventricle has become a hot topic. However, inappropriate... (Review)
Review
OBJECTIVE
In recent years, attempting the biventricular pathway or biventricular conversions in patients with borderline ventricle has become a hot topic. However, inappropriate pursuit of biventricular repair in borderline candidates will lead to adverse clinical outcomes. Therefore, it is important to accurately assess the degree of ventricular development before operation and whether it can tolerate biventricular repair. This review evaluated ventricular development using echocardiography for a better prediction of biventricular repair in borderline ventricle.
DATA SOURCES
Articles from January 1, 1990 to April 1, 2019 on biventricular repair in borderline ventricle were accessed from PubMed, using keywords including "borderline ventricle," "congenital heart disease," "CHD," "echocardiography," and "biventricular repair."
STUDY SELECTION
Original articles and critical reviews relevant to the review's theme were selected.
RESULTS
Borderline left ventricle (LV): (1) Critical aortic stenosis: the Rhodes score, Congenital Heart Surgeons Society regression equation and another new scoring system was proposed to predict the feasibility of biventricular repair. (2) Aortic arch hypoplasia: the LV size and the diameter of aortic and mitral valve (MV) annulus should be taken into considerations for biventricular repair. (3) Right-dominant unbalanced atrioventricular septal defect (AVSD): atrioventricular valve index (AVVI), left ventricular inflow index (LVII), and right ventricle (RV)/LV inflow angle were the echocardiographic indices for biventricular repair. Borderline RV: (1) pulmonary atresia/intact ventricular septum (PA/IVS): the diameter z-score of tricuspid valve (TV) annulus, ratio of TV to MV diameter, RV inlet length z-score, RV area z-score, RV development index, and RV-TV index, etc. Less objective but more practical description is to classify the RV as tripartite, bipartite, and unipartite. The presence or absence of RV sinusoids, RV dependent coronary circulation, and the degree of tricuspid regurgitation should also be noted. (2) Left-dominant unbalanced AVSD: AVVI, LV, and RV volumes, whether apex forming ventricles were the echocardiographic indices for biventricular repair.
CONCLUSIONS
Although the evaluation of echocardiography cannot guarantee the success of biventricular repair surgery, echocardiography can still provide relatively valuable basis for surgical decision making.
Topics: Echocardiography; Heart Defects, Congenital; Heart Ventricles; Hemodynamics; Humans
PubMed: 31348032
DOI: 10.1097/CM9.0000000000000375 -
European Review For Medical and... May 2023Mitral regurgitation (MR) represents an important feature in patients with hypertrophic cardiomyopathy (HCM) due to left ventricular outflow tract (LVOT) obstruction and...
OBJECTIVE
Mitral regurgitation (MR) represents an important feature in patients with hypertrophic cardiomyopathy (HCM) due to left ventricular outflow tract (LVOT) obstruction and mitral valve systolic anterior motion (SAM). Mitral valve anatomical variants associated with HCM also contribute to the severity of MR. The aim of this study is to evaluate MR severity and its correlation with different parameters in patients with HCM using cardiac magnetic resonance imaging (cMRI).
PATIENTS AND METHODS
130 patients with HCM underwent cMRI. Parameters assessed for the quantification of MR severity were mitral regurgitation volume (MRV) and mitral regurgitation fraction (MRF). cMRI was also used to characterize LV function, left atrium volume (LAV) index, filling pressures and structural abnormalities associated with HCM, all in correlation to MR.
RESULTS
Patients with HCM had mild (26.9%), moderate (52.3%) or severe (20.7%) MR. Most relevant parameters related to MR severity were MRV and MRF; other parameters with strong correlation with MR were LAV index and E/E' ratio, both increasing with its severity. Patients with LVOT obstruction had more severe MR (70.3%), 79% of them due to SAM. LV ejection fraction (LVEF) increased proportionally with the severity of MR, while LV strain (LAS) was inversely correlated with it. Independent predictors for quantifying the severity of MR, after the adjustment for covariates, were MRV, MRF, SAM, LAV index and E/E'.
CONCLUSIONS
cMRI can accurately assess MR in patients with HCM, especially by using novel indicators, MRV and MRF respectively, along with LAV index and E/E' ratio. Severe MR, due to SAM, is more frequent in the obstructive form of HCM (HOCM). Also, the severity of MR is significantly associated with significantly associated with MRV, MRF, LAV index and E/E' ratio.
Topics: Humans; Mitral Valve Insufficiency; Cardiomyopathy, Hypertrophic; Mitral Valve; Heart Atria; Magnetic Resonance Imaging; Ventricular Outflow Obstruction
PubMed: 37203824
DOI: 10.26355/eurrev_202305_32306 -
The Journal of Thoracic and... May 2018To characterize morphologic variations in the papillary muscles and tendinous cords of the left ventricle and ventricular zones of the mitral valve leaflets.
OBJECTIVES
To characterize morphologic variations in the papillary muscles and tendinous cords of the left ventricle and ventricular zones of the mitral valve leaflets.
METHODS
A total of 100 autopsied human hearts from healthy donors with classical mitral valve type were investigated.
RESULTS
In 1 heart, only 1 group of papillary muscles was found, and in the remaining 99%, we could distinguish 2 groups of muscles: Superolateral (SLPM) and inferoseptal papillary muscle (ISPM) groups. The SLPM group had 1 papillary muscle (75.8%), 2 in 20.2%, and >3 in 4.0%. In the ISPM group, the muscle percentages were 38.4%, 36.4%, and 25.2%, respectively. The apex of at least 1 papillary muscle was situated higher than the plane of the opened anterior leaflet (AML) in 47.5% and 50.5% for the SLPM and ISPM groups, respectively. The number of strut cords arising from the SLPM group was 0 (2.0%), 1 (50.5%), 2 (33.3%), 3 (12.1%), and 4 (2.0%), and from the ISPM group was 0 (6.1%), 1 (52.5%), 2 (35.4%), or 3 (6.1%). Cords to left ventricular outflow tract were present in 14 specimens. Muscular cords were found in eight hearts. In all hearts specimens AML had rough and clear zones. The classical zones (rough, clear, and basal) in the posterior mitral leaflet were observed in 38.4%.
CONCLUSIONS
There is a high variability in the papillary muscles and tendinous cords in the mitral valve complex. Proper nomenclature, simple classification, and the most common variants for papillary muscle groups and tendinous cords were presented.
Topics: Adult; Aged; Anatomic Landmarks; Autopsy; Chordae Tendineae; Dissection; Female; Humans; Male; Middle Aged; Mitral Valve; Papillary Muscles; Terminology as Topic
PubMed: 29397976
DOI: 10.1016/j.jtcvs.2017.12.061 -
Anatolian Journal of Cardiology Feb 2020Mitral stenosis (MS) is a progressive and devastating disease and most often occurs among young women. Given its considerable prevalence in Mediterranean and Eastern... (Review)
Review
Mitral stenosis (MS) is a progressive and devastating disease and most often occurs among young women. Given its considerable prevalence in Mediterranean and Eastern European countries according to the Euro Heart Survey, new imaging modalities are warranted to improve the management of patients with this condition. A wide spectrum of abnormalities occurs involving all parts of this complex structure and causing different grades of MS and/or regurgitation as a consequence of rheumatic affection. Novel imaging modalities significantly improved the assessment of several aspects of this rheumatic destructive process including the morphological alterations of the mitral valve apparatus, left atrial (LA) function, LA appendage, right and left ventricular function, and complications, namely, atrial fibrillation and thromboembolic events. Furthermore, new imaging modalities improved the prediction of outcome of patients who underwent percutaneous balloon mitral comissurotomy and changed the paradigm of patient selection for intervention and risk stratification. The present review aimed to summarize the role of new multimodality, multiparametric imaging approaches to assess the morphological characteristics of the rheumatic MS and its associated complications, and to guide patient management.
Topics: Echocardiography, Transesophageal; Humans; Mitral Valve Stenosis; Rheumatic Heart Disease
PubMed: 32120357
DOI: 10.14744/AnatolJCardiol.2020.01575 -
JACC. Case Reports Feb 2021Unguarded mitral valve orifice is a rare disease with only 7 described cases in the literature. We describe the first known case of unguarded mitral valve orifice with...
Unguarded mitral valve orifice is a rare disease with only 7 described cases in the literature. We describe the first known case of unguarded mitral valve orifice with normal segmental cardiac anatomy, severe left ventricular dilatation and dysfunction, aortic atresia, and atrial flutter. ().
PubMed: 34041497
DOI: 10.1016/j.jaccas.2020.11.020 -
Interactive Cardiovascular and Thoracic... Jul 2021With improvements in early survival after the stage I palliation (S1P) Norwood procedure for hypoplastic left heart syndrome (HLHS) and its variants, inter-stage death...
OBJECTIVES
With improvements in early survival after the stage I palliation (S1P) Norwood procedure for hypoplastic left heart syndrome (HLHS) and its variants, inter-stage death accounts for an increasing proportion of mortality. Our aim was to identify the risk factors for inter-stage mortality.
METHODS
The records of 322 neonates with HLHS or a variant who underwent the Norwood procedure at our centre between 2001 and 2019 were retrospectively analysed.
RESULTS
The diagnoses included 271 neonates with HLHS (84%) and 51 with variants (16%). Aortic atresia was observed in 138 (43%) patients, mitral atresia in 91 (28%), extracardiac anomalies in 42 (13%) and genetic disorder in 14 (4%). The median age and weight of the patients at the S1P Norwood procedure were 9 (interquartile range: 7-12) days and 3.2 (2.9-3.5) kg, respectively. The median cardiopulmonary bypass time was 137 (107-163) min. Modified Blalock-Taussig shunts were used in 159 (49%) and unvalved right ventricle-to-pulmonary artery shunts in 163 (51%) patients. The number of inter-stage deaths was as follows: between S1P and stage II palliation (S2P), 61 including 38 early (<30 days) and 23 late (>30 days) deaths, and between S2P and stage III palliation, 32 deaths. Low birth weight (<2.5 kg) (odds ratio 4.37, P = 0.020) and restrictive atrial septum (odds ratio 2.97, P = 0.013) were identified as risks for early mortality. Low birth weight [hazard ratio (HR) 0.99/g, P = 0.002] was a risk for inter-stage mortality between S1P and S2P. Extracardiac anomalies (HR 4.75, P = 0.049) and significant pre-S1P atrioventricular valve regurgitation (HR: 7.72, P = 0.016) were risks for inter-stage mortality between S2P and stage III palliation. Other anatomical variables including aortic atresia, anatomical subtypes and the diameter of the ascending aorta nor shunt type were not identified as risk factors for mortality during any inter-stage period.
CONCLUSIONS
The risk factors for inter-stage attrition after the Norwood procedure were different between each stage. Preoperative factors, including birth weight, restrictive atrial septum and extracardiac anomalies, adversely affected the inter-stage mortality.
Topics: Humans; Hypoplastic Left Heart Syndrome; Norwood Procedures; Palliative Care; Pulmonary Artery; Retrospective Studies; Risk Factors; Treatment Outcome
PubMed: 33948647
DOI: 10.1093/icvts/ivab073 -
Journal of Thoracic Disease Jun 2017Ischemic mitral regurgitation (IMR) is a form of mitral insufficiency that is characterized by papillary muscle (PM) displacement, leaflet tethering, reduced closing... (Review)
Review
Ischemic mitral regurgitation (IMR) is a form of mitral insufficiency that is characterized by papillary muscle (PM) displacement, leaflet tethering, reduced closing forces, and different degree of annular dilatation. Treatment of this condition includes mitral valve replacement or mitral valve repair with restrictive annuloplasty. Recent evidences in mitral valve repair showed that addressing only the annulus and neglecting the subvalvular apparatus provides a suboptimal operation with poor long-term results. However, the complexity of the geometrical aberrances occurring in IMR demands for more accurate analysis also involving the biomechanics underlying the failing mitral valve and subvalvular apparatus. Finite element analysis (FEA) is a powerful tool in this context and we developed a biomechanical model of mitral valve and subvalvular unit using 3D geometry of the leaflets, annulus, chordae and PM. After the application of structural properties of materials to these elements and simulation of systemic pressure loading, FEA could be used to directly determine biomechanical changes and geometry variations. We believe this approach can provide valuable information to better address the surgical treatment of IMR and answer some of the questions still pending in IMR management.
PubMed: 28740716
DOI: 10.21037/jtd.2017.05.63