TY - JOUR
AU - Lüscher, Thomas F
AB - For the podcast associated with this article, please visit https://academic.oup.com/eurheartj/pages/Podcasts Mitral valve disease is the new frontier in valvular heart disease. Although surgical mitral valve replacement was introduced in the late 1950s and mitral valve reconstruction later on, stimulated by the success of transarterial aortic valve implantation,1–3 percutaneous techniques have been developed.4,5 Whatever the technique, paravalvular leaks may occur and impact on outcome, an issue that is thoroughly discussed in an expert statement ‘Clinical trial principles and endpoint definitions for paravalvular leaks in surgical prosthesis’ by Carlos E. Ruiz and colleagues of the Paravalvular Leak Academic Research Consortium.6 They seek to provide core principles, clinical endpoints, and their definitions to be used in clinical trials of paravalvular leak closure. A five-class grading scheme to evaluate paravalvular leaks was developed in concordance with VARC recommendations,7 and unresolved issues in the field are outlined. Overall, the consortium provides recommendations for assessment of disease severity, data collection, and endpoint definitions. For planning of structural percutaneous and surgical procedures, 3D printing holds promise.8,9 Thomas Bartel from the Cleveland Clinic Abu Dhabi in the United Arab Emirates discusses this issue in his review entitled ‘Medical three-dimensional printing opens up new opportunities in cardiology and cardiac surgery’.10 Structural interventions in valvular and congenital heart disease require precise pre-procedural planning. Although current imaging modalities and post-processing software assist with peri-procedural guidance, their capabilities for spatial conceptualization remain limited in two- and three-dimensional representations. In contrast, 3D printing offers improved visualization for procedural planning and provides incremental information for surgical reconstruction and device implantation. A variety of clinical applications are available showing how accurate 3D computer reformatting and physical 3D printouts of native anatomy, embedded pathology, and implants are and how they may assist in the development of innovative therapies. Accurate imaging of pathology, including the target region for intervention, its anatomical features, and spatial relationship to the surrounding structures, is critical for selecting the optimal approach and for evaluation of procedural results. Pulmonary hypertension is a common consequence of valvular heart disease and usually, but not always, improves after correction of the lesion.11 In a FAST TRACK entitled ‘Sildenafil for improving outcomes in patients with corrected valvular heart disease and persistent pulmonary hypertension: a multicentre, double-blind, randomized clinical trial’, Javier Bermejo et al. from the Hospital General Universitario Gregorio Marañon Madrid in Spain tested in the SIOVAC study the effects of 40 mg t.i.d. sildenafil compared with placebo on outcomes of 200 patients with persistent pulmonary hypertension ≥30 mmHg systolic after correction of valvular heart disease.12 Among patients receiving sildenafil, 33% worsened their composite score, as compared with 15% of patients receiving placebo. Worse composite scores in the sildenafil group were confirmed in the per protocol analysis, and after adjustment for co-variables. Thus, as in patients with persistent pulmonary hypertension after corrected valvular heart disease sildenafil is surprisingly associated with worse clinical outcomes, its use should be avoided in such patients. These results are further discussed in an Editorial by Nazzareno Galiè from the University of Bologna in Italy.13 Certain genetic mutations are known to affect the morphology of the mitral valve and be associated with regurgitation. For instance, filamin-A was first identified as a cause of non-syndromic mitral valve dystrophy.14 Thierry Le Tourneau and colleagues from the Institut du Thorax, INSERM UMR 1087 in Nantes, France investigated its impact on outcomes in their paper entitled ‘New insights into mitral valve dystrophy: a filamin-A genotype–phenotype and outcome study’.15 They analysed 246 subjects (72 mutated) from four filamin-A mitral dystrophy families harbouring three different filamin-A mutations with echocardiography in comparison with control relatives. In this X-linked disease, valve lesions were severe in men and moderate in women. Most men had classical features of mitral valve prolapse, but without chordal rupture. In contrast to regular mitral valve prolapse, mitral leaflet motion was restricted in diastole, and the position of papillary muscles was closer to the mitral annulus (Figure 1). Abnormalities were similar in the four families, in adults and from early childhood, suggestive of a developmental disease. Also, lesions worsened over time as in degenerative prolapse. Polyvalvular involvement was frequent in males, and non-diagnostic forms were frequent in females. Overall survival was moderately impaired in men. Cardiac surgery rate was markedly increased owing mainly to a lifetime increased risk in men. Thus, filamin-A mitral dystrophy is a developmental and degenerative disease with complex phenotypic expression and unique features, with both mitral valve prolapse and paradoxical restricted diastolic motion, subvalvular mitral apparatus impairment, and polyvalvular lesions in males. These novel findings are put into context in a thought-provoking Editorial by Gaetano Thiene from the University of Padua Medical School in Italy.16 Figure 1 View largeDownload slide Mitral valve apparatus morphology averaged in systole (top) and diastole (bottom) (A) in adult controls, (B) in FLNA-MVD (FLNA +) men, and (C) in FLNA-MVD women. In men (B), the mitral annulus is 25% larger, and mitral leaflets were thickened, redundant, elongated, and prolapsed in systole (top). In addition, the distance between the tip of papillary muscles and the mitral annulus line (symbolized by the double black arrow) was reduced by 20%. In diastole (bottom), mitral leaflet motion is limited, with a doming aspect. In women (C), changes in the mitral valve apparatus were moderate (from Le Tourneau T, Le Scouarnec S, Cueff C, Bernstein D, Aalberts JJJ, Lecointe S, Mérot J, Bernstein JA, Oomen T, Dina C, Karakachoff M, DesalH, Al Habash O, Delling FN, Capoulade R, Suurmeijer AJH, Milan D, Norris RA, Markwald R, Aikawa E, Slaugenhaupt SA, Jeunemaitre X, Hagège A, Roussel J-C, Trochu J-N, Levine RA, Kyndt F, Probst V, Le Marec H, Schott J-J. New insights into mitral valve dystrophy: a Filamin-A genotype–phenotype and outcome study. See pages 1269--1277). Figure 1 View largeDownload slide Mitral valve apparatus morphology averaged in systole (top) and diastole (bottom) (A) in adult controls, (B) in FLNA-MVD (FLNA +) men, and (C) in FLNA-MVD women. In men (B), the mitral annulus is 25% larger, and mitral leaflets were thickened, redundant, elongated, and prolapsed in systole (top). In addition, the distance between the tip of papillary muscles and the mitral annulus line (symbolized by the double black arrow) was reduced by 20%. In diastole (bottom), mitral leaflet motion is limited, with a doming aspect. In women (C), changes in the mitral valve apparatus were moderate (from Le Tourneau T, Le Scouarnec S, Cueff C, Bernstein D, Aalberts JJJ, Lecointe S, Mérot J, Bernstein JA, Oomen T, Dina C, Karakachoff M, DesalH, Al Habash O, Delling FN, Capoulade R, Suurmeijer AJH, Milan D, Norris RA, Markwald R, Aikawa E, Slaugenhaupt SA, Jeunemaitre X, Hagège A, Roussel J-C, Trochu J-N, Levine RA, Kyndt F, Probst V, Le Marec H, Schott J-J. New insights into mitral valve dystrophy: a Filamin-A genotype–phenotype and outcome study. See pages 1269--1277). As indicated in the above paper, outcome is an important consideration in mitral valve disease, particularly in degenerative mitral regurgitation. However, the lack of mortality scores predicting death favours misperception of an individual patient’s risk and inappropriate decision-making, an issue addressed in a manuscript by Francesco Grigioni and the MIDA Investigators entitled ‘The MIDA Mortality Risk Score: development and external validation of a prognostic model for early and late death in degenerative mitral regurgitation’.17 The MIDA Registries include 3666 patients with isolated, degenerative mitral regurgitation diagnosed by echocardiography. The MIDA-Score was derived from 2472 patients of the MIDA-Flail-Registry and externally validated in 1194 patients of the MIDA-BNP-Registry. In predicting total mortality post-diagnosis, the MIDA-Score showed excellent concordance in both the derivation and validation cohort. In the entire MIDA population of 3666 patients, 5-year survival with scores of 0, 7–8, and 11–12 was 98 ± 1, 57 ± 4, and 21 ± 10% under medical management, and 99 ± 1, 82 ± 2, and 57 ± 9% after surgery. The MIDA-Score provided incremental prognostic information over guideline-provided prognostic markers and the EuroScoreII. Thus, the MIDA-Score represents an innovative tool for management of degenerative mitral regurgitation. Its clinical utility is critically discussed in an Editorial by Alec Vahanian from the Hôpital Bichat in Paris, France.18 Guidelines, and in this context the 2017 ESC Guidelines on Valvular Heart Disease,19 are a central part of today’s clinical practice. Whether and to what degree they are implemented is reported on in an article by Bernard Iung and colleagues from the Bichat Hospital in Paris, France entitled ‘Educational needs and application of guidelines in the management of patients with mitral regurgitation. A European mixed-methods study’.20 Following a qualitative phase (interviews), an online survey was undertaken using three case scenarios describing asymptomatic severe primary mitral regurgitation, symptomatic severe primary mitral regurgitation in the elderly, and severe secondary mitral regurgitation in 115 primary care physicians and 439 cardiologists or cardiac surgeons from seven European countries. Systematic cardiac auscultation was performed by only 54% of clinicians in asymptomatic patients. Three-quarters of cardiologists appropriately interpreted echocardiographic findings of the mechanism and quantification of primary mitral regurgitation, but only 44% recognized secondary mitral regurgitation as severe. In asymptomatic severe primary mitral regurgitation with an indication for surgery, 27% of primary care physicians did not refer the patient to a cardiologist, and medical therapy was overused by 19% of cardiologists. In the elderly patient with severe symptomatic primary mitral regurgitation, 72% of cardiologists considered mitral intervention, i.e. transcatheter edge-to edge valve repair. In severe symptomatic secondary mitral regurgitation, optimization of medical therapy was advised by only 51% of primary care physicians and 33% of cardiologists, and surgery was considered in only 30% of cases, but transcatheter edge-to-edge repair in 64%. Thus, systematic auscultation is underused, and medical therapy overused in primary mitral regurgitation and underused in secondary mitral regurgitation. Indications for interventions are appropriate in most patients with primary mitral regurgitation, but are unexpectedly frequent for secondary mitral regurgitation. These gaps identify important targets for future educational programmes. Transcatheter aortic valve replacement or TAVI is an established treatment option in high-risk patients with tricuspid, but now also bicuspid aortic stenosis.3 Evaluation of inter-ethnic differences in valve morphology and function, and aortic root dimensions is important for the worldwide spread of this therapy in this subgroup. Jeroen J. Bax and colleagues from the Leiden University Medical Center in the Netherlands evaluated differences of European and Asian patients with bicuspid aortic valve in their article entitled ‘Inter-ethnic differences in valve morphology, valvular dysfunction, and aortopathy between Asian and European patients with bicuspid aortic valve’.21 Of 1427 patients with bicuspid aortic valve, 56% were Europeans and 44% Asians. Asians had higher prevalence of type 1 bicuspid aortic valve with raphe between the right and non-coronary cusps than Europeans, whereas the Europeans had a higher prevalence of type 0 bicuspid aortic valve with two commissures and no raphe than Asians (Figure 2). The prevalence of moderate and severe aortic regurgitation was higher in Europeans than in Asians, whereas there were no differences in bicuspid aortic valve with normal function or aortic stenosis. After adjustments, the dimensions of the aortic annulus, sinus of Valsava, sinotubular junction, and ascending aorta were significantly larger among Asians compared with Europeans. These findings will have important implications for prosthesis type and size selection for transcatheter aortic valve replacement in the two ethnicities. Figure 2 View largeDownload slide Schematic presentation and echocardiographic images of the different bicuspid aortic valve (BAV) morphologies. Red bands and arrows represent the raphe (commissural fusion). Type 0 denotes BAV without raphe, and type 1 and 2 denote the presence of one or two raphes, respectively. The aortic valve is depicted in a cross-sectional short-axis view. The ostium of the right coronary artery (RCA) is depicted on the left and the left main (LM) is on the right (from Kong WKF, Regeer MV, Poh KK, Yip JW, van Rosendael PJ, Yeo TC, Tay E, Kamperidis V, van der Velde ET, Mertens B, Ajmone Marsan N, Delgado V, Bax JJ. Inter-ethnic differences in valve morphology, valvular dysfunction, and aortopathy between Asian and European patients with bicuspid aortic valve. See pages 1308--1313). Figure 2 View largeDownload slide Schematic presentation and echocardiographic images of the different bicuspid aortic valve (BAV) morphologies. Red bands and arrows represent the raphe (commissural fusion). Type 0 denotes BAV without raphe, and type 1 and 2 denote the presence of one or two raphes, respectively. The aortic valve is depicted in a cross-sectional short-axis view. The ostium of the right coronary artery (RCA) is depicted on the left and the left main (LM) is on the right (from Kong WKF, Regeer MV, Poh KK, Yip JW, van Rosendael PJ, Yeo TC, Tay E, Kamperidis V, van der Velde ET, Mertens B, Ajmone Marsan N, Delgado V, Bax JJ. Inter-ethnic differences in valve morphology, valvular dysfunction, and aortopathy between Asian and European patients with bicuspid aortic valve. See pages 1308--1313). The editors hope that this issue of the European Heart Journal will be of interest to its readers. References 1 Figulla HR, Webb JG, Lauten A, Feldman T. The transcatheter valve technology pipeline for treatment of adult valvular heart disease. Eur Heart J  2016; 37: 2226– 2239. Google Scholar CrossRef Search ADS PubMed  2 Durko AP, Osnabrugge RL, Van Mieghem NM, Milojevic M, Mylotte D, Nkomo VT, Pieter Kappetein A. 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Inter-ethnic differences in valve morphology, valvular dysfunction, and aortopathy between Asian and European patients with bicuspid aortic valve. Eur Heart J  2018; 39: 1308– 1313. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2018. For permissions please email: journals.permissions@oup.com. This article is published and distributed under the terms of the Oxford University Press, Standard Journals Publication Model (https://academic.oup.com/journals/pages/about_us/legal/notices)
TI - Mitral valve disease: news from the frontier in valvular heart disease
JO - European Heart Journal
DO - 10.1093/eurheartj/ehy215
DA - 2018-04-14
UR - https://www.deepdyve.com/lp/oxford-university-press/mitral-valve-disease-news-from-the-frontier-in-valvular-heart-disease-0mn10kRdZC
SP - 1211
EP - 1214
VL - 39
IS - 15
DP - DeepDyve
ER -