-
Journal of the American College of... Oct 2018Mitral annulus disjunction (MAD) is an abnormal atrial displacement of the mitral valve leaflet hinge point. MAD has been associated with mitral valve prolapse (MVP) and...
BACKGROUND
Mitral annulus disjunction (MAD) is an abnormal atrial displacement of the mitral valve leaflet hinge point. MAD has been associated with mitral valve prolapse (MVP) and sudden cardiac death.
OBJECTIVES
The purpose of this study was to describe the clinical presentation, MAD morphology, association with MVP, and ventricular arrhythmias in patients with MAD.
METHODS
The authors clinically examined patients with MAD. By echocardiography, the authors assessed the presence of MVP and measured MAD distance in parasternal long axis. Using cardiac magnetic resonance (CMR), the authors assessed circumferential MAD in the annular plane, longitudinal MAD distance, and myocardial fibrosis. Aborted cardiac arrest and sustained ventricular tachycardia were defined as severe arrhythmic events.
RESULTS
The authors included 116 patients with MAD (age 49 ± 15 years; 60% female). Palpitations were the most common symptom (71%). Severe arrhythmic events occurred in 14 (12%) patients. Longitudinal MAD distance measured by CMR was 3.0 mm (interquartile range [IQR]: 0 to 7.0 mm) and circumferential MAD was 150° (IQR: 90° to 210°). Patients with severe arrhythmic events were younger (age 37 ± 13 years vs. 51 ± 14 years; p = 0.001), had lower ejection fraction (51 ± 5% vs. 57 ± 7%; p = 0.002) and had more frequently papillary muscle fibrosis (4 [36%] vs. 6 [9%]; p = 0.03). MVP was evident in 90 (78%) patients and was not associated with ventricular arrhythmia.
CONCLUSIONS
Ventricular arrhythmias were frequent in patients with MAD. A total of 26 (22%) patients with MAD did not have MVP, and MVP was not associated with arrhythmic events, indicating MAD itself as an arrhythmogenic entity. MAD was detected around a large part of the mitral annulus circumference and was interspersed with normal tissue.
Topics: Adult; Arrhythmias, Cardiac; Cross-Sectional Studies; Death, Sudden, Cardiac; Echocardiography; Female; Fibrosis; Heart Arrest; Humans; Magnetic Resonance Imaging, Cine; Male; Middle Aged; Mitral Valve; Mitral Valve Insufficiency; Mitral Valve Prolapse; Papillary Muscles; Syndrome; Ventricular Premature Complexes
PubMed: 30261961
DOI: 10.1016/j.jacc.2018.07.070 -
Children (Basel, Switzerland) Jul 2022Mitral atresia with normal aortic root is a rare complex congenital heart defect (CHD) and constitute less than 1% of all CHDs. In this anomaly, the mitral valve is... (Review)
Review
Mitral atresia with normal aortic root is a rare complex congenital heart defect (CHD) and constitute less than 1% of all CHDs. In this anomaly, the mitral valve is atretic, a patent foramen ovale provides egress of the left atrial blood, either a single ventricle or two ventricles with left ventricular hypoplasia are present, and the aortic valve/root are normal by definition. Clinical, roentgenographic and electrocardiographic features are non-distinctive, but echo-Doppler studies are useful in defining the anatomic and pathophysiologic components of this anomaly with rare need for other imaging studies. Treatment consists of addressing the pathophysiology resulting from defect and associated cardiac anomalies at the time of initial presentation, usually in the early infancy. These children eventually require staged total cavo-pulmonary connection (Fontan) in three stages. Discussion of each of these stages were presented. Complications are observed in-between the stages of Fontan surgery and following completion of Fontan procedure. Attempts to monitor for early detection of these complications and promptly addressing the complications are recommended.
PubMed: 36010040
DOI: 10.3390/children9081148 -
Children (Basel, Switzerland) May 2021In this paper, the author enumerates cardiac defects with a functionally single ventricle, summarizes single ventricle physiology, presents a summary of management... (Review)
Review
In this paper, the author enumerates cardiac defects with a functionally single ventricle, summarizes single ventricle physiology, presents a summary of management strategies to address the single ventricle defects, goes over the steps of staged total cavo-pulmonary connection, cites the prevalence of inter-stage mortality, names the causes of inter-stage mortality, discusses strategies to address the inter-stage mortality, reviews post-Fontan issues, and introduces alternative approaches to Fontan circulation.
PubMed: 34073809
DOI: 10.3390/children8060441 -
Journal of Cardiovascular Development... Mar 2023As yet, there is no agreed definition for the so-called "hypoplastic left heart syndrome". Even its origin remains contentious. Noonan and Nadas, who as far as we can... (Review)
Review
As yet, there is no agreed definition for the so-called "hypoplastic left heart syndrome". Even its origin remains contentious. Noonan and Nadas, who as far as we can establish first grouped together patients as belonging to a "syndrome" in 1958, suggested that Lev had named the entity. Lev, however, when writing in 1952, had described "hypoplasia of the aortic outflow tract complex". In his initial description, as with Noonan and Nadas, he included cases with ventricular septal defects. In a subsequent account, he suggested that only those with an intact ventricular septum be included within the syndrome. There is much to commend this later approach. When assessed on the basis of the integrity of the ventricular septum, the hearts to be included can be interpreted as showing an acquired disease of fetal life. Recognition of this fact is important to those seeking to establish the genetic background of left ventricular hypoplasia. Flow is also of importance, with septal integrity then influencing its effect on the structure of the hypoplastic ventricle. In our review, we summarise the evidence supporting the notion that an intact ventricular septum should now be part of the definition of the hypoplastic left heart syndrome.
PubMed: 37103012
DOI: 10.3390/jcdd10040133 -
The Journal of Thoracic and... Aug 2018
Topics: Aortic Valve; Bicuspid Aortic Valve Disease; Heart Valve Diseases; Humans; Mitral Valve; Tricuspid Valve
PubMed: 29666012
DOI: 10.1016/j.jtcvs.2018.03.069 -
Indian Journal of Thoracic and... Sep 2021Yasui operation combines Norwood arch reconstruction with Rastelli operation for interrupted or hypoplastic aorta with aortic valvar atresia or hypoplasia with... (Review)
Review
UNLABELLED
Yasui operation combines Norwood arch reconstruction with Rastelli operation for interrupted or hypoplastic aorta with aortic valvar atresia or hypoplasia with ventricular septal and two adequately sized ventricles, establishing biventricular repair. We present a case of aortic atresia, mitral hypoplasia, and ventricular septal defect (VSD) treated by Yasui procedure, and its long-term (108 months) follow-up and brief review of literature. Review of literature was done using keywords to search on "PubMed" and "Google Scholar."
SUPPLEMENTARY INFORMATION
The online version contains supplementary material available at 10.1007/s12055-021-01174-5.
PubMed: 34511760
DOI: 10.1007/s12055-021-01174-5 -
Journal of Cardiovascular Development... Aug 2022Hypoplastic left heart syndrome (HLHS) is a collective term applied to severe congenital cardiac malformations, characterised by a combination of abnormalities mainly... (Review)
Review
Hypoplastic left heart syndrome (HLHS) is a collective term applied to severe congenital cardiac malformations, characterised by a combination of abnormalities mainly affecting the left ventricle, associated valves, and ascending aorta. Although in clinical practice HLHS is usually sub-categorised based on the patency of the mitral and aortic (left-sided) valves, it is also possible to comprehensively categorise HLHS into defined sub-groups based on the left ventricular morphology. Here, we discuss the published human-based studies of the ventricular myocardium in HLHS, evaluating whether the available evidence is in keeping with this ventricular morphology concept. Specifically, we highlight results from histological studies, indicating that the appearance of cardiomyocytes can be different based on the sub-group of HLHS. In addition, we discuss the histological appearances of endocardial fibroelastosis (EFE), which is a common feature of one specific sub-group of HLHS. Lastly, we suggest investigations that should ideally be undertaken using HLHS myocardial tissues at early stages of HLHS development to identify biological pathways and aid the understanding of HLHS aetiology.
PubMed: 36005443
DOI: 10.3390/jcdd9080279 -
Swiss Medical Weekly Jan 2010Mitral regurgitation (MR) involves systolic retrograde flow from the left ventricle into the left atrium. While trivial MR is frequent in healthy subjects, moderate to... (Review)
Review
Mitral regurgitation (MR) involves systolic retrograde flow from the left ventricle into the left atrium. While trivial MR is frequent in healthy subjects, moderate to severe MR constitutes the second most prevalent valve disease after aortic valve stenosis. Major causes of severe MR in Western countries include degenerative valve disease (myxomatous disease, flail leaflet, annular calcification) and ischaemic heart disease, while rheumatic disease remains a major cause of MR in developing countries. Chronic MR typically progresses insidiously over many years. Once established, however, severe MR portends a poor prognosis. The severity of MR can be assessed by various techniques, Doppler echocardiography being the most widely used. Mitral valve surgery is the only treatment of proven efficacy. It alleviates clinical symptoms and prevents ventricular dilatation and heart failure (or, at least, it attenuates further progression of these abnormalities). Valve repair significantly improves clinical outcomes compared with valve replacement, reducing mortality by approximately 70%. Reverse LV remodelling after valve repair occurs in half of patients with functional MR. Percutaneous, catheter-based to mitral valve repair is a novel approach currently under clinical scrutiny, with encouraging preliminary results. This modality may provide a valuable alternative to mitral valve surgery, especially in critically ill patients.
Topics: Aged; Female; Humans; Male; Mitral Valve; Mitral Valve Insufficiency
PubMed: 19950042
DOI: 10.4414/smw.2010.12893 -
Frontiers in Pediatrics 2014The concept of univentricular heart moved from hearts with only one ventricle connected with atria [double inlet ventricle or absent atrioventricular (AV) connection] to... (Review)
Review
The concept of univentricular heart moved from hearts with only one ventricle connected with atria [double inlet ventricle or absent atrioventricular (AV) connection] to hearts not amenable to biventricular repair, namely hearts with two ventricles unable to sustain separately pulmonary and systemic circulations in sequence. In the latter definition, even hearts with one hypoplastic ventricle are considered "functional" univentricular hearts. They include pulmonary/aortic atresia or severe stenosis with hypoplastic ventricle, and rare conditions like huge intramural cardiac tumors and Ebstein anomaly with extreme atrialization of right ventricular cavity. In this setting, the surgical repair is univentricular with "Fontan" operation, bypassing the ventricular mass. In other words, functionally univentricular heart is a condition in which, after surgery, only one ventricle sustain systemic circulation. Univentricular hearts (double inlet or absent AV connection) almost invariably show two ventricular chambers, one main and one accessory, which lacks an inlet portion. The latter is located posteriorly when morphologically left and anteriorly when morphologically right. As far as double inlet left ventricle, this is usually associated with discordant ventriculo-arterial (VA) connection (transposition of the great arteries) and all the blood flow to the aorta, which takes origin from the hypoplastic anterior right ventricle, is ventricular septal defect (bulbo-ventricular foramen) dependent. If restrictive, an aortic arch obstruction may be present. Double inlet left ventricle may be rarely associated with VA concordance (Holmes heart). As far as double inlet right ventricle with posterior hypoplastic left ventricular cavity, ventriculo-arterial connection is usually of double outlet type; thus the term double inlet-outlet right ventricle may be coined. Absent right or left AV connection may develop in the setting of both d- or l-loop, whatever the situs. In this condition, the contra-lateral patent AV valve may be either mitral or tricuspid in terms of morphology and the underlying ventricle (main chamber) either morphologically left or right. Establishing the loop, whatever right or left (also called right or left ventricular topology), is a fundamental step in the segmental-sequential analysis of congenital heart disease.
PubMed: 25072035
DOI: 10.3389/fped.2014.00062