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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 -
European Heart Journal Nov 2019
Topics: Aged; Cardiomyopathies; Echocardiography, Transesophageal; Fatal Outcome; Female; Humans; Imaging, Three-Dimensional; Mitral Valve Insufficiency; Papillary Muscles; Rupture, Spontaneous
PubMed: 31377807
DOI: 10.1093/eurheartj/ehz549 -
Annals of Cardiac Anaesthesia 2021An asymptomatic 30-year-old male was referred for a transthoracic echocardiogram because of a systolic murmur that was noted on a pre-employment physical exam....
An asymptomatic 30-year-old male was referred for a transthoracic echocardiogram because of a systolic murmur that was noted on a pre-employment physical exam. Transthoracic imaging demonstrated a single papillary muscle from which the chordae of both mitral valve leaflets were attached. The mitral valve was seen to have a parachute-like configuration. Given the benign nature of the presentation, the patient did not seek further investigation.
Topics: Adult; Diagnostic Tests, Routine; Echocardiography; Humans; Male; Mitral Valve; Mitral Valve Insufficiency; Papillary Muscles; Referral and Consultation
PubMed: 33938836
DOI: 10.4103/aca.ACA_82_19 -
Seminars in Thoracic and Cardiovascular... Mar 2018We report the techniques and long-term outcome of mitral valve (MV) repair to correct congenital mitral stenosis in children. Between 1986 and 2014, 137 children (mean... (Review)
Review
We report the techniques and long-term outcome of mitral valve (MV) repair to correct congenital mitral stenosis in children. Between 1986 and 2014, 137 children (mean age 4.1 ± 5.0, range 1 month-16.8 years) underwent repair of congenital mitral stenosis (CMS). In 48 patients, CMS is involved in Shone's anomaly. The typical congenital MS (type I) was seen in 56 patients. Hypoplastic MV (type II, n = 15) was associated with severe left ventricular outflow tract abnormalities and hypoplastic left ventricular cavity and muscle mass. Supravalvar ring (type III, n = 48) ranged from a thin membrane to a thick discrete fibrous ridge. Parachute MV (type IV, n = 10) have 2 leaflets and barely distinguishable commissures, but all chordae merged either into 1 major papillary muscle or asymmetric papillary muscles-1 dominant and the other minuscule. Hammock valve (type IV, n = 8) appeared dysplastic with shortened chordae directly inserted into the posterior left ventricular muscle mass. MV repair was performed using commissurotomy, chordal division, papillary muscle splitting and fenestration, and mitral ring resection, each applied according to the presenting morphology. During the 28-year follow-up period, 23 patients underwent repeat MV repair and 3 underwent MV replacement after failed attempts at repeat repair. At 1 and 15 years postoperatively, freedom from reoperation was 89.3 ± 5.1% and 52.8 ± 11.8%, and cumulative survival rates were 92.3 ± 4.3% and 70.3 ± 8.9, respectively. Mortality unrelated to repair accounted for 9 (20%) deaths. Long-term functional outcome of MV repair in children with CMS is satisfactory. Repeat repair or replacement may be deemed necessary during the course of follow-up.
Topics: Adolescent; Child; Child, Preschool; Female; Follow-Up Studies; Humans; Infant; Male; Mitral Valve Stenosis; Papillary Muscles; Plastic Surgery Procedures; Retrospective Studies; Time Factors; Treatment Outcome
PubMed: 29425525
DOI: 10.1053/j.pcsu.2017.11.008 -
Journal of Nuclear Cardiology :... Feb 2022
Topics: Ammonia; Coronary Circulation; Humans; Ischemia; Myocardial Ischemia; Myocardial Perfusion Imaging; Nitrogen Radioisotopes; Papillary Muscles; Positron-Emission Tomography; Tomography, Emission-Computed
PubMed: 32909237
DOI: 10.1007/s12350-020-02336-5 -
Interactive Cardiovascular and Thoracic... May 2022
Topics: Humans; Disruptive Technology; Mitral Valve Insufficiency; Papillary Muscles; Printing, Three-Dimensional
PubMed: 35137090
DOI: 10.1093/icvts/ivac015 -
Journal of Cardiovascular... Apr 2022
Topics: Catheter Ablation; Catheters; Humans; Papillary Muscles; Tachycardia, Ventricular
PubMed: 35132708
DOI: 10.1111/jce.15403 -
JACC. Clinical Electrophysiology Dec 2022
Topics: Humans; Papillary Muscles; Heart Ventricles; Mitral Valve Insufficiency
PubMed: 36543497
DOI: 10.1016/j.jacep.2022.08.030 -
Current Opinion in Cardiology Sep 2015This section reviews abnormalities of the mitral valve apparatus that are associated with hypertrophic obstructive cardiomyopathy. (Review)
Review
PURPOSE OF REVIEW
This section reviews abnormalities of the mitral valve apparatus that are associated with hypertrophic obstructive cardiomyopathy.
RECENT FINDINGS
Mitral valve abnormalities in hypertrophic obstructive cardiomyopathy include leaflet elongation and thickening and hypertrophy with anterior displacement of the papillary muscles. This combination contributes to the development of systolic anterior motion. The resultant flow forces during systolic anterior motion pulling the leaflets into the left ventricular outflow tract also result in reduced leaflet coaptation and a posteriorly directed mitral regurgitant jet. Additional mitral valve abnormalities include degenerative leaflet changes, aberrant chordal attachments and papillary muscle anomalies.
SUMMARY
Mitral valve abnormalities are common in hypertrophic obstructive cardiomyopathy and play an important role in the pathophysiology of dynamic left ventricular outflow tract obstruction.
Topics: Cardiomyopathy, Hypertrophic; Echocardiography; Hemodynamics; Humans; Mitral Valve; Mitral Valve Insufficiency; Papillary Muscles; Ventricular Outflow Obstruction
PubMed: 26192489
DOI: 10.1097/HCO.0000000000000200 -
Journal of Interventional Cardiac... Aug 2018The His bundle divides into the right bundle branch (RBB) and the more complex left bundle branch (LBB). The latter is typically represented as further dividing into... (Review)
Review
The His bundle divides into the right bundle branch (RBB) and the more complex left bundle branch (LBB). The latter is typically represented as further dividing into anterior and posterior fascicles, and possibly an additional septal fascicle. This construct raises questions as to why conduction can be preserved in patients with "trifascicular block" and why it sometimes very difficult to ablate fascicular or papillary muscle tachycardias. The answer may be that the LBB is much more complex, variable, and interconnected and that the simple familiar constructs may have obstructed our understanding of conduction and tachycardias. Remarkably, the actual explanatory histopathology has been known for nearly half a century, spearheaded by the work of Demoulin and Kulbertus. One picture is worth a thousand words.
Topics: Bundle of His; Bundle-Branch Block; Catheter Ablation; Electrocardiography; Female; Follow-Up Studies; Heart Conduction System; Humans; Male; Papillary Muscles; Risk Assessment; Tachycardia; Treatment Outcome
PubMed: 30128802
DOI: 10.1007/s10840-018-0440-1