In vivo mitral valve morphology and motion in mitral valve prolapse

doi:10.1016/0002-9149(94)90287-9

The American Journal of Cardiology

Volume 73, Issue 15, 1 June 1994, Pages 1080-1088

https://doi.org/10.1016/0002-9149(94)90287-9 Get rights and content

Abstract

Mitral leaflet morphology in mitral valve prolapse (MVP) has been suggested to be prognostically important, but in vivo valvular morphology is incompletely described in patients with MVP or in normal subjects. Accordingly, the length of both mitral leaflets and their zone of apposition, the thickness of their rough and clear zones, diastolic and systolic mitral annular diameters, and indexes of abnormal leaflet motion were measured in the parasternal long-axis echocardiographic view in 100 patients with MVP and 100 age- and sex-matched normal subjects. In both groups posterior leaflet thickness was related to age, as were anterior leaflet thickness and posterior leaflet length in patients with MVP. Compared with normal subjects, MVP patients without mitral regurgitation had thickened leaflets, elongated anterior leaflets, and large annular diameters (p < 0.0001). Patients with severe regurgitation had thicker leaflets, longer posterior leaflet and annular dimensions, and more abnormal leaflet motion than MVP patients without regurgitation. Leaflet thicknesses of different zones were supranormal in 60% to 67% and in 49% to 59% of MVP patients with and without severe regurgitation, respectively. MVP patients with regurgitation also had higher prevalences of abnormal mitral annular diameter than those without regurgitation (67% vs 29%) and > 3 mm posterior leaflet billowing into the left atrium (60% vs 34%). Thus, mitral valve size and leaflet thickness are increased in MVP patients without as well as with clinically important mitral regurgitation. The usefulness of leaflet thickening as a marker of severe MVP is limited by its high prevalence in patients with clinically mild MVP.

References (29)

SW MacMahon et al.
Mitral valve prolapse and infective endocarditis
Am Heart J
(1987)
P Kligfield et al.
Arrhythmias and sudden death in mitral valve prolapse
Am Heart J
(1987)
AJ Kolibash et al.
Evidence for progression from mild to severe mitral regurgitation in mitral valve prolapse
Am J Cardiol
(1986)
SW MacMahon et al.
Risk of infective endocarditis in mitral valve prolapse with and without precordial systolic murmurs
Am J Cardiol
(1987)
RA Levine et al.
Reconsideration of echocardiographic standards for mitral valve prolapse: lack of association between leaflet displacement isolated to the apical four chamber view and independent echocardiographic evidence of abnormality
J Am Coll Cardiol
(1988)
R Pini et al.
Comparison of mitral valve dimensions and motion in mitral valve prolapse with severe mitral regurgitation to uncomplicated mitral prolapse and severe mitral regurgitation without mitral valve prolapse
Am J Cardiol
(1988)
R Pini et al.
Mitral valve dimensions and motion and familial transmission of mitral valve prolapse with and without mitral leaflet billowing
J Am Coll Cardiol
(1988)
RB Devereux et al.
Relation between clinical features of the mitral valve prolapse syndrome and echocardiographically documented mitral valve prolapse
J Am Coll Cardiol
(1986)
MJ Roman et al.
Comparison of cardiovascular and skeletal features of primary mitral valve prolapse and Marfan syndrome
Am J Cardiol
(1989)
RB Devereux et al.
Diagnosis and classification of severity of mitral valve prolapse: methodologic, biologic and prognostic considerations
Am Heart J
(1987)

R Pini et al.

Time-motion reconstruction of mitral leaflet motion from two-dimensional echocardiography in mitral valve prolapse

Am J Cardiol

(1991)

Y Sahasakul et al.

Age-related changes in aortic and mitral valve thickness: implications for two-dimensional echocardiography based on an autopsy study of 200 normal human hearts

Am J Cardiol

(1988)

G de Simone et al.

Left ventricular mass and body size in normotensive children and adults: assessment of allometric relations and of the impact of overweight

J Am Coll Cardiol

(1992)

D Levy et al.

Prevalence and clinical features of mitral valve prolapse

Am Heart J

(1987)

Cited by (67)

Predictive Factors for Progression of Mitral Regurgitation in Asymptomatic Patients With Mitral Valve Prolapse
2019, American Journal of Cardiology
Risk factors predicting progression from low grade to severe mitral regurgitation (MR), which is a guideline criterion for surgical intervention, remain unknown. We hypothesized that abnormalities of cardiac structure and function may predict progression in MR severity. We followed 82 asymptomatic mitral valve prolapse (MVP) patients (65 ± 12 years, 51% men) with mild or moderate MR (36 mild, 46 moderate, mean LVEF: 62%), without significant co-morbidities. We examined clinical findings and 13 echo measurements. The primary end point was progression to severe MR. In a mean follow-up period of 4.5 ± 2.7 years, mortality and heart failure development were similar for mild and moderate MR. No mild MR patient progressed to severe, but 23 moderate MR patients (50.0%) progressed to severe with 9 patients (39.1%) who underwent surgery. No clinical variables were predictive for progression. Only mean mitral annulus diameter (apical 4 and 2 chamber) was predictive for progression to severe MR (hazards ratio 1.14, 95% confidence interval 1.03 to 1.26, p = 0.01). A cut-off annulus diameter of 39.6 mm had a good accuracy (area under the curve 0.78, sensitivity 100%, and specificity 63.8%) for progression to severe. In conclusion, over a 4.5-year period, 50% of asymptomatic MVP patients with moderate MR, but none with mild, progressed to severe MR. Only mitral annular dimension predicted progression of moderate to severe MR, and values >39.6 mm predicted progression accurately. Mitral annulus diameter may be of value in identifying asymptomatic MVP patients at risk of developing severe MR.
Mitral valve prolapse syndrome and MASS phenotype: Stability of aortic dilatation but progression of mitral valve prolapse
2016, IJC Heart and Vasculature
Citation Excerpt :
We verified the diagnosis of MVPS and MASS clinically in all individuals at the age of > 20 years [8], and we did not find a causative FBN1 mutation in all these individuals. As Ghent-2 recommends, we diagnosed MVP with the presence of ≥ 1 of the echocardiographic standard criteria as specified below [8,25]. We extracted DNA from EDTA blood samples using standard procedures, and amplified the coding region and flanking intronic sequences including 20 nucleotides of the introns at each acceptor (positions − 1 to − 20) and donor splice site (positions + 1 to + 20) of the FBN1, (NM 000138.4), TGFBR1 (NM 004612.2) and TGFBR2 (NM 001024847.2) genes by PCR in all patients.
Mitral valve prolapse syndrome (MVPS) and MASS phenotype (MASS) are Marfan-like syndromes that exhibit aortic dilatation and mitral valve prolapse. Unlike in Marfan syndrome (MFS), the presence of ectopia lentis and aortic aneurysm preclude diagnosis of MVPS and MASS. However, it is unclear whether aortic dilatation and mitral valve prolapse remain stable in MVPS or MASS or whether they progress like in MFS.
This retrospective longitudinal observational study examines clinical characteristics and long-term prognosis of 44 adults with MVPS or MASS (18 men, 26 women aged 38 ± 17 years) as compared with 81 adults with Marfan syndrome (MFS) with similar age and sex distribution. The age at final contact was 42 ± 15 years with mean follow-up of 66 ± 49 months.
At baseline, ectopia lentis and aortic sinus aneurysm were absent in MVPS and MASS, and systemic scores defined by the revised Ghent nosology were lower than in MFS (all P < .001). Unlike in MFS, no individual with MVPS and MASS developed aortic complications (P < .001). In contrast, the incidence of endocarditis (P = .292), heart failure (P = .644), and mitral valve surgery (P = .140) was similar in all syndromes. Cox regression analysis identified increased LV end-diastolic (P = .013), moderate MVR (P = .019) and flail MV leaflet (P = .017) as independent predictors of mitral valve surgery.
The study provides evidence that MVPS and MASS are Marfan-like syndromes with stability of aortic dilatation but with progression of mitral valve prolapse. Echocardiographic characteristics of mitral valve disease rather than the type of syndrome, predict clinical progression of mitral valve prolapse.
Mitral valve enlargement in chronic aortic regurgitation as a compensatory mechanism to prevent functional mitral regurgitation in the dilated left ventricle
2013, Journal of the American College of Cardiology
The aim of this study was to test the hypothesis that mitral valve (MV) enlargement occurring in chronic aortic regurgitation (AR) prevents functional mitral regurgitation (FMR).
Chronic AR causes left ventricular (LV) dilation, creating the potential for FMR. However, FMR is typically absent during compensated AR despite substantial LV enlargement. Increased mitral leaflet area has been identified in AR, but it is unknown whether increased MV size can represent a compensatory mechanism capable of preventing FMR.
Database review of 816 patients with at least moderate AR evaluated the prevalence of FMR. A total of 90 patients were enrolled prospectively for 3-dimensional echocardiography (30 AR, 30 FMR, and 30 controls) to assess MV geometry including total leaflet area.
FMR was present in 5.6% of AR patients by database review. Prospectively, only 1 AR patient had more than mild FMR despite increased LV end-diastolic volume (82 ± 22, 86 ± 23, and 51 ± 12 cm³/m², respectively, for AR, FMR vs. control patients; p < 0.01) and similar sphericity index, annular area, and tethering distances compared with FMR. Total MV area was largest in AR (31.3% greater than normal), increasing significantly more than in FMR. The ratio of valve size to closure area was maintained in AR, whereas decreases in this ratio and LV ejection fraction independently predicted FMR.
FMR prevalence is low in chronic AR. MV leaflet area is significantly increased compared with control and FMR patients, preserving a normal relationship to the area needed for closure in the dilated LV. Understanding the mechanisms underlying this adaptation could lead to new therapeutic interventions to prevent FMR.
CMR Predictors of Mitral Regurgitation in Mitral Valve Prolapse
2010, JACC: Cardiovascular Imaging
We sought to assess the correlation between mitral valve characteristics and severity of mitral regurgitation (MR) in subjects with mitral valve prolapse (MVP) undergoing cardiac magnetic resonance (CMR) imaging.
Compared with extensive echocardiographic studies, CMR predictors of MVP-related MR are unknown. The severity of MR at the time of diagnosis has prognostic implication for patients; therefore, the identification of determinants of MR and its progression may be important for risk stratification, follow-up recommendations, and surgical decision making.
Seventy-one MVP patients (age 54 ± 11 years, 58% males, left ventricular [LV] ejection fraction 65 ± 5%) underwent cine CMR to assess annular dimensions, maximum systolic anterior and posterior leaflet displacement, papillary muscle (PM) distance to coaptation point and prolapsed leaflets, as well as diastolic anterior and posterior leaflet thickness and length, and LV volumes and mass. Velocity-encoded CMR was used to obtain aortic outflow and to quantify MR volume.
Using multiple linear regression analysis including all variables, LV mass (p < 0.001), anterior leaflet length (p = 0.006), and posterior displacement (p = 0.01) were the best determinants of MR volume with a model-adjusted R² = 0.6. When the analysis was restricted to valvular characteristics, MR volume correlated with anterior mitral leaflet length (p < 0.001), posterior mitral leaflet displacement (p = 0.003), posterior leaflet thickness (p = 0.008), and the presence of flail (p = 0.005) with a model-adjusted R² = 0.5. We also demonstrated acceptable intraobserver and interobserver variability in these measurements.
Anterior leaflet length, posterior leaflet displacement, posterior leaflet thickness, and the presence of flail are the best CMR valvular determinants of MVP-related MR. The acceptable intraobserver and interobserver variability of our measurements confirms the role of CMR as an imaging modality for assessment of MVP patients with significant MR.
Myxomatous Mitral Valve Disease
2009, Valvular Heart Disease: A Companion to Braunwalds Heart Disease Expert Consult - Online and Print
Annular-to-Leaflet Mismatch and the Need for Reductive Annuloplasty in Patients Undergoing Mitral Repair for Chronic Mitral Regurgitation Due to Mitral Valve Prolapse
2007, American Journal of Cardiology
Annular dilation is a common feature of chronic degenerative mitral regurgitation caused by leaflet prolapse. Accordingly, patients undergoing surgical repair usually undergo concomitant reductive annuloplasty to restore a normal annular-to-leaflet relation. With the evolution of transcatheter valve repair technologies, patient selection criteria for those who do not require annuloplasty are needed. A series of patients undergoing mitral repair was analyzed, and the role of annular-to-leaflet mismatch in identifying patients requiring reductive annuloplasty was explored. Preoperative data for 82 patients undergoing mitral repair with annuloplasty for degenerative mitral regurgitation were prospectively collected, including annular intercommissural (IC) and septolateral (SL) dimensions and heights of anterior (ALH) and posterior leaflets. An SL/ALH ratio >1.4 was used to define annular-to-leaflet mismatch. After mitral repair, the ratio between preoperative IC distance and the size of the implanted annular prosthesis (Seguin ring [SR], IC/SR <1.2) was used to identify patients for whom annuloplasty was nonreductive. All patients underwent successful mitral repair. Mean preoperative IC was 46 ± 6 mm, SL was 42 ± 5 mm, and ALH was 31 ± 5 mm. Mean SR was 34 ± 3 mm, with a mean IC/SR ratio of 1.34 ± 0.14. Sixteen patients (19%) had an IC/SR ratio <1.2. IC/SR ratio <1.2 was predicted by a SL/ALH ratio ≤1.4 (p = 0.009). In conclusion, annular dilation is negligible in <20% of surgical candidates. In this subgroup, an isolated leaflet repair may be indicated. SL/ALH ratio is a good indicator of annular-to-leaflet mismatch and could be used as an adjunct to other methods of annular function assessment to select patients for ringless mitral repair.

View all citing articles on Scopus

^☆: This study was supported in part by Grant HL-22006 from the National Heart, Lung, and Blood Institute, and by Grant M01-RR00047 (CRC) from the National Institutes of Health, Bethesda, Maryland.

^∗: Died June 15, 1993.

View full text

In vivo mitral valve morphology and motion in mitral valve prolapse☆

Abstract

Am Heart J

Am Heart J

Am J Cardiol

Am J Cardiol

J Am Coll Cardiol

Am J Cardiol

J Am Coll Cardiol

J Am Coll Cardiol

Am J Cardiol

Am Heart J

Am J Cardiol

Am J Cardiol

J Am Coll Cardiol

Prevalence and clinical features of mitral valve prolapse

Am Heart J