The MIDA-Q mortality risk score: a quantitative prognostic tool for the mitral valve prolapse spectrum

Background:Mitral valve prolapse (MVP) is responsible for a considerable disease burden but is widely heterogeneous. The lack of a comprehensive prognostic instrument covering the entire MVP spectrum, encompassing the quantified consequent degenerative mitral regurgitation (DMR), hinders clinical management and therapeutic trials.Methods:The new Mitral Regurgitation International Database Quantitative (MIDA-Q) registry enrolled 8187 consecutive patients (ages 63±16 years, 47% women, follow-up 5.5±3.3 years) first diagnosed with isolated MVP, without or with DMR quantified prospectively (measuring effective regurgitant orifice [ERO] and regurgitant volume) in routine practice of 5 tertiary care centers from North America, Europe, and the Middle East. The MIDA-Q score ranges from 0 to 15 by accumulating guideline-based risk factors and DMR severity. Long-term survival under medical management was the primary outcome end point.Results:MVP was associated with DMR absent/mild (ERO 2) in 50%, moderate (ERO 20–40 mm2) in 25%, and severe or higher (ERO ≥40 mm2) in 25%, with mean ERO 24±24 mm2, regurgitant volume 37±35 mL. Median MIDA-Q score was 4 with a wide distribution (10%–90% range, 0–9). MIDA-Q score was higher in patients with EuroScore II ≥1% versus P R2 0.18). Five-year survival under medical management was strongly associated with MIDA-Q score, 97±1% with score 0, 95±1% with score 1 to 2, 82±1% with score 3 to 4, 67±1% with score 5 to 6, 60±1% with score 7 to 8, 44±1% with score 9 to 10, 35±1% with score 11 to 12, and 5±4% with MIDA-Q score ≥13, with hazard ratio 1.31 [1.29–1.33] per 1-point increment. Excess mortality with higher MIDA-Q scores persisted after adjustment for age, sex, and EuroScore II (adjusted hazard ratio, 1.13 [1.11–1.15] per 1-point increment). Subgroup analysis showed persistent association of MIDA-Q score with mortality in all possible subsets, in particular, with EuroScore IIPPConclusions:This large, international cohort of isolated MVP, with prospective DMR quantification in routine practice, demonstrates the wide range of risk factor accumulation and considerable heterogeneity of outcomes after MVP diagnosis. The MIDA-Q score is strongly, independently, and incrementally associated with long-term survival after MVP diagnosis, irrespective of presentation, and is therefore a crucial prognostic instrument for risk stratification, clinical trials, and management of patients diagnosed with all forms of MVP.Cardiolog

International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional and research purposes

This International Consensus Classification and Nomenclature for the congenital bicuspid aortic valve condition recognizes 3 types of bicuspid valves: 1. The fused type (right-left cusp fusion, right-non-coronary cusp fusion and left-non-coronary cusp fusion phenotypes); 2. The 2-sinus type (latero-lateral and antero-posterior phenotypes); and 3. The partial-fusion (forme fruste) type. The presence of raphe and the symmetry of the fused type phenotypes are critical aspects to describe. The International Consensus also recognizes 3 types of bicuspid valve-associated aortopathy: 1. The ascending phenotype; 2. The root phenotype; and 3. Extended phenotypes.Cardiolog

Sex differences in bicuspid aortic valve disease

Bicuspid aortic valve (BAV), the most frequent congenital heart disease, is characterized by an uneven distribution between sexes. BAV is three to four times more frequent in men than in women which could be associated with a reduced dosage of X chromosome genes. In addition, BAV has a multifactorial inheritance, low penetrance and variable phenotypes that may lead to different form of valve degeneration and dysfunction over time as well as different incidence of aortic valve and vascular complications between men and women.Definition of the phenotype is the first step in the evaluation of patients with BAV. Among the various phenotypes, BAV with a fusion raphe between the left and the right coronary cusp is the most frequent phenotype observed in men and women. It has been hypothesized that the valve and vascular related complications vary according to the BAV phenotype and this could explain differences in the clinical outcomes of men versus women. However, the evidence on the distribution of the various BAV phenotypes between sexes in not consistent and while some series have described differences between male and female, others have not confirmed those findings.In terms of valvular complications, women present more frequently with aortic stenosis while aortic regurgitation is more frequently diagnosed in men. Furthermore, endocarditis is more frequently reported in men as compared to women. In terms of vascular complications, men show larger diameters of the various parts of the aortic root and ascending aorta and more frequently present complications in terms of aortic aneurysm and dissection as compared to women. Although there are no survival differences between men and women with BAV, compared to the general population some large series have shown that women have worse prognosis. The present review article summarizes the differences between men and women in terms of BAV phenotype, type and incidence of aortic valve and vascular complications that will determine the differences in clinical outcomes. (c) 2020 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY license (http:// creativecommons.org/licenses/by/4.0/).Cardiolog

Speaking a common language: Introduction to a standard terminology for the bicuspid aortic valve and its aortopathy

There is a growing need to develop a common language when referring to a frequent and heterogeneous condition such as the congenital bicuspid aortic valve and its aortopathy. The following short manuscript serves as an introduction to a standard terminology for the bicuspid aortic valve and its aortopathy.(c) 2020 Elsevier Inc. All rights reserved.Cardiolog

The Bicuspid Aortic Valve Condition: The Critical Role of Echocardiography and the Case for a Standard Nomenclature Consensus

The bicuspid aortic valve (BAV) condition is characterized by prominent heterogeneity of its valvular phenotypic expression, of its associated disorders, its complications and its prognosis. Echocardiography serves as the first-line imaging modality in 5 major capacities: (1) BAV diagnosis, (2) valvular phenotyping, (3) assessment of valvular function, (4) measurement of the thoracic aorta and exclusion of aortic coarctation, and (5) assessment of uncommon but serious complications such as infective endocarditis and aortic dissection. This article presents a nosological perspective on the BAV condition, discusses the critical role of echocardiography as it relates to these 5 aforementioned capacities; and makes the case for a unified standard nomenclature for the BAV condition

A roadmap to investigate the genetic basis of bicuspid aortic valve and its complications: Insights from the international BAVCon (bicuspid aortic valve consortium)

Bicuspid aortic valve (BAV) is the most common adult congenital heart defect and is found in 0.5% to 2.0% of the general population. The term "BAV" refers to a heterogeneous group of disorders characterized by diverse aortic valve malformations with associated aortopathy, congenital heart defects, and genetic syndromes. Even after decades of investigation, the genetic determinants of BAV and its complications remain largely undefined. Just as BAV phenotypes are highly variable, the genetic etiologies of BAV are equally diverse and vary from complex inheritance in families to sporadic cases without any evidence of inheritance. In this paper, the authors discuss current concepts in BAV genetics and propose a roadmap for unraveling unanswered questions about BAV through the integrated analysis of genetic and clinical data. © 2014 by the American College of Cardiology Foundation

Echocardiographic findings and subsequent risk of native valve endocarditis

Aim: The association of echocardiographic findings and subsequent risk of left-sided native valve endocarditis (LS-NVE) is undefined. The aim of this study was to determine if transthoracic echocardiography (TTE) measurements are associated with the subsequent development of LS-NVE in patients without cardiac predisposing conditions. Methods: Institutional databases were evaluated for adults diagnosed with LS-NVE from 2008 to 2020. Patients with prosthetic valves, cardiovascular implantable electronic devices, intracardiac devices, injection drug use, and predisposing cardiac conditions were excluded. Only patients who had a TTE performed 6 months to 3 years before the development of LS-NVE were included as cases. Controls were patients within the same Mayo location with a TTE report and were matched in a 1:3 ratio according to age, gender, Charlson comorbidity index, and echocardiography date. Results: There were 148 cases and 431 matched controls. As compared to controls, infective endocarditis (IE) cases had a higher prevalence of diabetes mellitus (46.6% vs. 30.4%) and chronic kidney disease (46.6% vs. 28.1%) (P < 0.001). Left ventricular outflow tract velocity (P = 0.017), left ventricular ejection fraction (P = 0.018), and E:e’ ratio (P = 0.050) were associated with LS-NVE. Conclusions: Echocardiographic measurements were associated with subsequent LS-NVE development in this pilot study. A larger cohort of LS-NVE patients, however, is needed to validate these findings

Summary: International Consensus Statement on Nomenclature and Classification of the Congenital Bicuspid Aortic Valve and Its Aortopathy, for Clinical, Surgical, Interventional and Research Purposes

This International evidence-based nomenclature and classification consensus on the congenital bicuspid aortic valve and its aortopathy recognizes 3 types of bicuspid aortic valve: 1. Fused type, with 3 phenotypes: right-left cusp fusion, right-non cusp fusion and left-non cusp fusion; 2. 2-sinus type with 2 phenotypes: Latero-lateral and antero-posterior; and 3. Partial-fusion or forme fruste. This consensus recognizes 3 bicuspid-aortopathy types: 1. Ascending phenotype; root phenotype; and 3. extended phenotypes. (Ann Thorac Surg 2021;112:1005-22) 2021 Jointly between The Society of Thoracic Surgeons, the American Association for Thoracic Surgery, the European Association for Cardio-Thoracic Surgery, and the Radiological Society of North America. Published by Elsevier Inc.Cardiolog

International Consensus Statement on Nomenclature and Classification of the Congenital Bicuspid Aortic Valve and Its Aortopathy, for Clinical, Surgical, Interventional and Research Purposes

This International Consensus Classification and Nomenclature for the congenital bicuspid aortic valve condition recognizes 3 types of bicuspid valves: 1. The fused type (right-left cusp fusion, right-non-coronary cusp fusion and left-non coronary cusp fusion phenotypes); 2. The 2-sinus type (latero-lateral and antero-posterior phenotypes); and 3. The partial-fusion (forme fruste) type. The presence of raphe and the symmetry of the fused type phenotypes are critical aspects to describe. The International Consensus also recognizes 3 types of bicuspid valve-associated aortopathy: 1. The ascending phenotype; 2. The root phenotype; and 3. Extended phenotypes. (Ann Thorac Surg 2021;112:e203-35) 2021 Jointly between The Society of Thoracic Surgeons, the American Association for Thoracic Surgery, the European Association for Cardio-Thoracic Surgery, and the Radiological Society of North America. Published by Elsevier Inc.Cardiolog