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Polish Journal of Radiology 2021The left ventricular outflow is an anatomically complex region situated between the anterior leaflet of the mitral valve and the left ventricular aspect of the muscular... (Review)
Review
The left ventricular outflow is an anatomically complex region situated between the anterior leaflet of the mitral valve and the left ventricular aspect of the muscular and membranous interventricular septum. It gives rise to the aorta, provides support to the aortic valvular cusps, and houses important components of the conduction system. The left ventricular outflow handles high pressures and pressure variations and is subsequently affected by a variety of aetio-pathological conditions. Diseases involving the left ventricular outflow can be intraluminal, mural, or extramural, and the consequent complications of the lesions can be local, loco-regional, or even systemic. Appropriate evaluation requires comprehensive multimodality imaging with each modality contributing to assessment of different aspects of diagnosis, lesion characterization, local extension, prognostication for systemic complications and mortality, and the decision for the approach and type of intervention and aggressive follow-up in case non-interventional management is decided. In this review, we briefly describe the relevant anatomy and the gamut of structural abnormalities pertaining to the left ventricular outflow on multidetector computed tomography angiography.
PubMed: 34093915
DOI: 10.5114/pjr.2021.105588 -
Pediatric Cardiology Oct 2022Subclinical alterations in left ventricular structure and function are detectable in adolescents with hypertension or obesity. However, data on early echocardiographic...
Subclinical alterations in left ventricular structure and function are detectable in adolescents with hypertension or obesity. However, data on early echocardiographic abnormalities in seemingly healthy children are lacking. Sex differences in cardiac structure and function have been previously reported, but sex-specific reference values are not available. Specifically, the potential interaction of sex and overweight has not been addressed at all. Anthropometric data, blood pressure and exercise tests were obtained in 356 healthy children. Echocardiographic parameters comprised peak early (E) and late (A) mitral inflow Doppler velocities, E/A ratio, tissue Doppler peak velocities of early (e') and late diastolic (a') excursion of mitral/septal annulus and isovolumetric relaxation time (IVRT). Left ventricular mass index (LVMI) and LVMI z-score were calculated. Interaction terms between BMI and sex and stratification by sex were used for analysis. We provide values for echocardiographic parameters for children of two age groups separated by BMI. Overweight/obese children had a significant higher LVMI, lower E/A ratio, higher E/e' ratios and a longer IVRT. For a given BMI in the upper ranges we demonstrated a higher LVMI in girls than in boys, the IVRT extended significantly more in girls than in boys with increasing BMI. There are sex differences in structural and functional echocardiographic parameters in children and adolescents. Our data not only confirms the importance of overweight and obesity, but demonstrates important interactions between sex and overweight. The greater susceptibility of overweight girls toward echocardiographic changes associated with potential long-term functional impairment needs further exploration and follow-up.Trial registration number DRKS00012371; Date 18.08.2017.
Topics: Adolescent; Child; Diastole; Echocardiography; Female; Humans; Male; Mitral Valve; Overweight; Pediatric Obesity; Ventricular Dysfunction, Left; Ventricular Function, Left
PubMed: 35394150
DOI: 10.1007/s00246-022-02876-2 -
Indian Journal of Thoracic and... Jan 2021Anomalous origin of coronary artery from pulmonary artery (AOCAPA), as is evident from the name, is defined as abnormal origin of either coronary artery from the...
BACKGROUND
Anomalous origin of coronary artery from pulmonary artery (AOCAPA), as is evident from the name, is defined as abnormal origin of either coronary artery from the pulmonary artery. The consequences vary in most cases and these anomalies lead to severe coronary hypo-perfusion and ventricular dysfunction. The common variants of this cardiac malformation are an anomalous origin of a left coronary artery from a pulmonary artery (ALCAPA) and anomalous origin of the right coronary artery from a pulmonary artery (ARCAPA). Another rare variant is left main coronary artery atresia that resembles ALCAPA in its mode of presentation. This article presents a single surgeon experience of managing this complex subset of the coronary anomaly from April 2006 to July 2019.
MATERIAL AND METHODS
The 105 patients, who underwent surgery for AOCAPA from April 2006 to July 2019, have been included in the study. The patients have been analysed by follow-up echocardiography and electrocardiography (ECG) at our hospital by paediatric cardiologists. Out of 105 patients of AOCAPA, 98 (93.3%) patients underwent ALCAPA repair, of which 59 (60.2%) were males and 39 (39.7%) were females. Four out of five patients, who had an anomalous origin of the left coronary artery from the right pulmonary artery (ALCARPA), had an intramural aortic course. Three patients (3%) had left main coronary artery atresia and four patients (4%) had ARCAPA. It may be mentioned that seven infants (7.14%) and one adult patient (1%) underwent concomitant mitral valve repair. All the patients with ALCAPA, left main coronary artery atresia and ARCAPA, and 1 of the patients with ALCARPA, underwent coronary relocation. In four out of five patients with ALCARPA, unroofing of intra-aortic intramural course was performed.
RESULTS
Out of 105 patients of AOCAPA, 9 (8.5%) patients had in-hospital mortality. Five infants (5.0%) with ALCAPA and one patient (1%) with ALCARPA died in the post-operative period due to severe left ventricular dysfunction, mitral regurgitation (MR) and sepsis. One adult patient (1%) with ALCAPA, who underwent coronary relocation using in situ trap door technique and mitral valve (MV) repair, died due to massive intracranial bleeding. Two patients out of three (66.6%) with left main coronary atresia died in intensive care unit (ICU) after 3rd and 4th postoperative day, due to low cardiac output, severe ventricular dysfunction and severe MR. Patients were followed up for a median 5.9 years. Seven patients were lost to follow-up, including the sole survivor of left main coronary atresia, after a median follow-up of 4 years after surgery. Three patients underwent mitral valve replacement for progressive residual MR. There has been no late mortality.
CONCLUSION
AOCAPA is a rare congenital cardiac anomaly, which usually presents in infancy with left ventricular dysfunction and mitral valve regurgitation. Early diagnosis and surgical re-establishment of the dual coronary system has given gratifying results, with improvement in left ventricular function in survivors. Mitral valve intervention for MR was required, in both early and late phases.
PubMed: 33526963
DOI: 10.1007/s12055-021-01147-8 -
Journal of Cell Science Sep 2022Mitral and tricuspid valves are essential for unidirectional blood flow in the heart. They are derived from similar cell sources, and yet congenital dysplasia affecting...
Mitral and tricuspid valves are essential for unidirectional blood flow in the heart. They are derived from similar cell sources, and yet congenital dysplasia affecting both valves is clinically rare, suggesting the presence of differential regulatory mechanisms underlying their development. Here, we specifically inactivated Dicer1 in the endocardium during cardiogenesis and found that Dicer1 deletion caused congenital mitral valve stenosis and regurgitation, whereas it had no impact on other valves. We showed that hyperplastic mitral valves were caused by abnormal condensation and extracellular matrix (ECM) remodeling. Our single-cell RNA sequencing analysis revealed impaired maturation of mesenchymal cells and abnormal expression of ECM genes in mutant mitral valves. Furthermore, expression of a set of miRNAs that target ECM genes was significantly lower in tricuspid valves compared to mitral valves, consistent with the idea that the miRNAs are differentially required for mitral and tricuspid valve development. We thus reveal miRNA-mediated gene regulation as a novel molecular mechanism that differentially regulates mitral and tricuspid valve development, thereby enhancing our understanding of the non-association of inborn mitral and tricuspid dysplasia observed clinically.
Topics: Extracellular Matrix; MicroRNAs; Mitral Valve; Tricuspid Valve
PubMed: 35946425
DOI: 10.1242/jcs.259783 -
World Journal For Pediatric &... Nov 2023The recent special issue of the devoted to hypoplastic left heart syndrome, and its related anomalies, contained significant information of great clinical relevance....
The recent special issue of the devoted to hypoplastic left heart syndrome, and its related anomalies, contained significant information of great clinical relevance. Very little attention, however, was devoted to the integrity of ventricular septum as providing a criterion to distinguish between the phenotypes to be included within the syndrome, as opposed to the related anomalies. In this commentary, we summarize the evidence in support of the notion that the phenotypes to be included within the syndrome can be interpreted on the basis of an acquired disease of fetal life. We suggest that it is the integrity of the ventricular septum that provided the major criterion for the distinction between the lesions making up the syndrome and the related anomalies. The subsets of lesions to be included within the syndrome can then be recognized in terms of the time, subsequent to the closure of the embryonic interventricular communication, at which the left ventricle ceased its growth relative to the remainder of the cardiac components. On this basis, it is possible to recognize the combinations of aortic and mitral atresia, mitral stenosis with aortic atresia, combined mitral and aortic stenosis, and hypoplasia of the left ventricle with commensurate hypoplasia of the aortic and mitral valves; the latter combination now recognized as the hypoplastic left heart complex.
Topics: Humans; Child; Hypoplastic Left Heart Syndrome; Heart Defects, Congenital; Mitral Valve; Mitral Valve Stenosis; Heart Ventricles; Phenotype
PubMed: 37738500
DOI: 10.1177/21501351231181313 -
Journal of Clinical Medicine Dec 2023Left ventricular (LV) non-compaction (LVNC) is a rare genetic cardiomyopathy due to abnormal intra-uterine arrest of compaction of the myocardial fibers during... (Review)
Review
Left ventricular (LV) non-compaction (LVNC) is a rare genetic cardiomyopathy due to abnormal intra-uterine arrest of compaction of the myocardial fibers during endomyocardial embryogenesis. Due to the partial or complete absence of LV compaction, the structure of the LV wall shows characteristic abnormalities, including a thin compacted epicardium and a thick non-compacted endocardium with prominent trabeculations and deep intertrabecular recesses. LVNC is frequently associated with chronic heart failure, life-threatening ventricular arrhythmias, and systemic embolic events. According to recent findings, in the presence of LVNC, dysfunctional LV proved to be associated with left atrial volumetric and functional abnormalities and consequential dilated and functionally impaired mitral annulus, partly explaining the higher prevalence of regurgitation. Although the non-compaction process morphologically affects only the LV, signs of remodeling of the right heart were also detected. Moreover, dilation and stiffening of the aorta were present. The aim of the present detailed review was to summarize findings regarding changes in cardiac mechanics, valvular abnormalities, and vascular remodeling detected in patients with LVNC.
PubMed: 38202085
DOI: 10.3390/jcm13010078 -
Northern Clinics of Istanbul 2023The aim of present study is to compare ventricular and supraventricular arrhythmia incidences in subjects with and without mitral valve prolapse (MVP) syndrome and to...
OBJECTIVE
The aim of present study is to compare ventricular and supraventricular arrhythmia incidences in subjects with and without mitral valve prolapse (MVP) syndrome and to examine if an association exists between ventricular arrhythmias and repolarization parameters in patients with MVP syndrome.
METHODS
This cross-sectional study involved 41 subjects with MVP Syndrome and 41 subjects with palpitation but without MVP (control group). All subjects were subjected to lead-electrocardiogram, transthoracic echocardiography, and 24-h Holter monitoring to identify repolarization abnormalities, structural abnormalities, and supraventricular and ventricular arrhythmias. The QRS width, QTC interval, and Tpeak-Tend intervals were measured for each participant.
RESULTS
The number of subjects who had premature ventricular contractions (PVCs), couplets, and non-sustained ventricular tachycardia (NSVTs) was significantly higher in the MVP group compared to the control group. Left ventricular end-systolic diameter (LVESD) and left ventricular end-diastolic diameter (LVEDD) and left atrial diameter were also significantly higher in the MVP group than the control group. QRS width and Tpeak-Tend interval were also significantly higher in subjects with MVP than the controls. Correlation analysis showed a positive correlation between the severity of mitral regurgitation (MR) and the number of PVCs and couplets, while there was a significant correlation between left atrium (LA) diameter and the number of the PVCs and NSVTs.
CONCLUSION
Subjects with MVP experience ventricular arrhythmias more often including PVCs, couplets, and NSVTs compared to subjects without MVP. LVESD, LVEDD, LA diameter, QRS width, and Tpeak-Tend interval were increased in MVP subjects than those without MVP. There is an association between the severity of the MR and the frequency of the PVCs, couplets, or NSVTs.
PubMed: 37181056
DOI: 10.14744/nci.2021.12058 -
Interactive Cardiovascular and Thoracic... Apr 2021Barlow's disease (BD) is characterized by thick, redundant mitral valve (MV) leaflets, which can lead to prolapse and significant mitral regurgitation (MR). MV annular...
OBJECTIVES
Barlow's disease (BD) is characterized by thick, redundant mitral valve (MV) leaflets, which can lead to prolapse and significant mitral regurgitation (MR). MV annular abnormalities are also commonly observed and increasingly recognized as possible primary pathology, with leaflet thickening being secondary to increased stress on the MV apparatus. To provide more insights into this hypothesis, the evolution of MV abnormalities over time in patients with BD was assessed.
METHODS
A total of 64 patients (54 ± 12 years, 72% male) with BD who underwent MV surgery and had multiple transthoracic echocardiograms (TTE) before surgery were included. In total, 186 TTE were analysed (median time interval 4.2, interquartile range 2.2-6.5 years) including specific MV characteristics.
RESULTS
At baseline, MV leaflet length, thickness, billowing height and annular diameter were larger in patients with BD compared to 59 healthy subjects. Systolic outward motion (curling) of the annulus was observed in 77% and severe mitral annular disjunction (≥5 mm) in 38% of patients with BD. Forty (63%) patients had MR grade I-II and 24 (37%) MR grade III-IV; at baseline, the 2 groups only differed in left atrial volume and in thickness and billowing height of the posterior leaflet, showing comparable MV annular abnormalities and dilatation despite different grades of MR. Over time, MV annulus diameter, leaflet length and billowing height increased significantly along with MR grade.
CONCLUSIONS
In patients with BD, MV annulus abnormalities are present at an early stage and precede the development of significant MR, suggesting their substantial role in the pathophysiology of this disease and as an important target for surgical treatment.
Topics: Adult; Aged; Cardiac Surgical Procedures; Echocardiography; Female; Humans; Male; Middle Aged; Mitral Valve; Mitral Valve Insufficiency; Mitral Valve Prolapse
PubMed: 33367628
DOI: 10.1093/icvts/ivaa304 -
The Journal of Thoracic and... Aug 2018
Topics: Aortic Valve; Bicuspid Aortic Valve Disease; Heart Valve Diseases; Humans; Mitral Valve; Tricuspid Valve
PubMed: 29656816
DOI: 10.1016/j.jtcvs.2018.03.025