Assessment of bicuspid aortic valve phenotypes and associated pathologies: A transesophageal echocardiographic study.

OBJECTIVE: We investigated the frequency of different bicuspid aortic valve disease (BAV) phenotypes,the associated valvular pathologies, and the aortopathy phenotypes, using 2-dimensional (2D) transthoracic, 2D transesophageal echocardiography (TEE) and 3-dimensional (3D) TEE. METHODS: A total of 154 patients with BAV were included. Five BAV phenotypes were detected. To better define valvular pathologies, binary classifications of BAV were used: BAV with antero-posterior commisural line (BAV-AP) and right-left commissural line (BAV-RL). Aortopathy phenotype was classified according to the involved tract(s). RESULTS: Of the patients, 53.2% had type 1, 16.2% type 2, 15.6% type 3, 1.3% type 4, and 13.6% had type 5 BAV. The prevalence of BAV-AP and BAV-RL was 68.2% and 31.8%, respectively. No difference was detected with respect to aortic regurgitation between BAV-AP and BAV-RL (p=0.9), but the BAVRL group had an increased propensity to have a stenotic aortic valve (p=0.003). The indexed aortic diameter was larger in BAV-AP cases than BAV-RL at the sinus of Valsalva (p=0.008). In patients with dilatation of the root and tubular portion, a predominance of BAV-AP versus BAV-RL was observed (85% vs 15%). A markedly low prevalence of the root phenotype (3.2%) was observed. In 90.1% of the patients, 2D TEE was sufficient to classify BAV phenotypes; further 3D imaging was needed in 9.9% of the cases. CONCLUSION: There may be racial differences in the frequency of valvular and aortopathy phenotypes in patients with BAV. BAV phenotypes differ with respect to aortic stenosis and aortopathy phenotypes. TEE may have good diagnostic utility in differentiating BAV phenotypes.

Assessment of Lung Ultrasound B-Lines in Dogs with Different Stages of Chronic Valvular Heart Disease.

BACKGROUND: In dogs with chronic valvular heart disease (CVHD), early recognition of pulmonary edema (PE) is of paramount importance. Recent studies in dogs showed that lung ultrasound examination (LUS) is a useful technique to diagnose cardiogenic PE. OBJECTIVES: To describe LUS features in dogs with different stages of CVHD, and to determine its diagnostic accuracy in detecting PE using thoracic radiography as the reference standard. ANIMALS: Sixty-three dogs with CVHD. METHODS: Prospective, multicenter, cross-sectional study. Each dog underwent physical examination, echocardiography, thoracic radiography, and LUS. The LUS findings were classified as absent, rare, numerous, or confluent B-lines. Sensitivity, specificity, and positive and negative predictive values of LUS B-lines to identify PE were calculated using thoracic radiography as the reference standard. RESULTS: Dogs in stage B1 had absent or rare B-lines in 14 of 15 cases (93.3%). Dogs in stage B2 had absent or rare B-lines in 16 of 18 cases (88.9%). All dogs in stage C, without radiographic signs of PE, had absent or rare B-lines. Dogs in stage C, with radiographic signs of PE, had numerous or confluent B-lines in 18 of 20 cases (90%). Lung ultrasound examination detected PE with a sensitivity of 90%, specificity of 93%, and with positive and negative predictive values of 85.7 and 95.2%, respectively. CONCLUSIONS AND CLINICAL IMPORTANCE: Lung ultrasound examination showed good diagnostic accuracy to identify cardiogenic PE and might be helpful in staging dogs with CVHD. Lung ultrasound examination should be considered as a new, noninvasive diagnostic tool for clinicians managing CVHD in dogs.

Functional assessment of thoracic aortic aneurysms - the future of risk prediction?

Introduction: Treatment guidelines for the thoracic aorta concentrate on size, yet acute aortic dissection or rupture can occur when aortic size is below intervention criteria. Functional imaging and computational techniques are a means of assessing haemodynamic parameters involved in aortic pathology. Sources of data: Original articles, reviews, international guidelines. Areas of agreement: Computational fluid dynamics and 4D flow MRI allow non-invasive assessment of blood flow parameters and aortic wall biomechanics. Areas of controversy: Aortic valve morphology (particularly bicuspid aortic valve) is associated with aneurysm of the ascending aorta, although the exact mechanism of aneurysm formation is not yet established. Growing points: Haemodynamic assessment of the thoracic aorta has highlighted parameters which are linked with both clinical outcome and protein changes in the aortic wall. Wall shear stress, flow displacement and helicity are elevated in patients with bicuspid aortic valve, particularly at locations of aneurysm formation. Areas timely for developing research: With further validation, functional assessment of the aorta may help identify patients at risk of aortic complications, and introduce new haemodynamic indices into management guidelines.

The natural history of guidelines: the case of aortopathy related to bicuspid aortic valves.

Clinical guidelines represent statements that seek to synthesize the best evidence to guide a course of action to improve health outcomes and more cost-effective use of resources. However, even when the evidence is not definitive, comprehensive documents are still needed to guide clinical decision-making. In these circumstances, guidelines are inevitably affected by perceptions of the problem and their possible solutions. From 1998 to 2014, 10 different international guidelines have focused on the aortopathy related to bicuspid aortic valves. Recommended thresholds for intervention started at a cutoff level of 5.5cm in 1998, reached a nadir of 4 to 4.5cm in 2010, and returned to a 5.5cm cutoff level in 2014. During this time, no conclusive objective proof was published to support either an aggressive or conservative strategy. The consequence was that an undefined number of patients underwent surgery (and potential complications thereof) at an earlier disease stage than might have been necessary. This experience might provide a clue as to how guidelines evolve, and provide insight as to how to avoid a similar process in the future.

Rationale and design of GISSI OUTLIERS VAR Study in bicuspid aortic valve patients: prospective longitudinal, multicenter study to investigate correlation between surgical, echo distinctive features, histologic and genetic findings in phenotypically homogeneous outlier cases.

BACKGROUND/OBJECTIVES: Bicuspid aortic valve (BAV) is the most common congenital heart disorder, affecting up to 2% of the population. Involvement of aortic root and ascending aorta (aneurysm or, eventually, dissection) is frequent in patients with pathologic or normal functioning BAV. Unfortunately, there are no well-known correlations between valvular and vascular diseases. In VAR protocol, with a new strategy of research, we analysemultiple aspects of BAV disease through correlation between surgical, echo, histologic and genetic findings in phenotypically homogeneous outlier cases. METHODS: VAR protocol is a prospective, longitudinal, multicenter study. It observes 4 homogeneous small groups of BAV surgical patients (15 patients each): isolated aortic regurgitation, isolated ascending aortic aneurysm, aortic regurgitation associated with aortic aneurysm, isolated aortic stenosis in older patients (>60years). Echo analysis is extended to first-degree relatives and, in case of BAV, genetic test is performed. Patients and relatives are enrolled in 10 cardiac surgery/cardiologic centers throughout Italy. CONCLUSIONS: The aim of the study is to identify predictors of favorable or unfavorable evolution of BAV in terms of valvular dysfunction and/or aortic aneurysm. Correlations between different features could help in identification of various BAV risk groups, rationalizing follow-up and treatment.

Bicuspid valve-related aortic disease: flow assessment with conventional phase-contrast MRI.

RATIONALE AND OBJECTIVES: Abnormal blood flow with bicuspid aortic valve (BAV) has been characterized with four-dimensional flow magnetic resonance imaging (MRI), but this approach is time consuming and requires technical expertise. We assess the relationship between different leaflets fusion patterns with BAV, eccentric systolic flow, and dilation patterns of the ascending aorta using two-dimensional (2D) phase-contrast (PC) MRI. MATERIALS AND METHODS: Fifty-nine patients with BAV who underwent cardiac MRI were identified; 47 had right-left (RL) aortic leaflet fusion and 12 had right-noncoronary (RN) fusion. Flow displacement was calculated, and patients with abnormal displacement (>0.1) were classified as either rightward or leftward. Patterns of aortopathy were determined (0-3), and correlation between leaflet fusion, flow direction, aortopathy type, and other clinical parameters was performed with Pearson correlation, the Fisher exact test and chi-square analysis. RESULTS: Normal systolic flow was seen in 24% of cases and was significantly correlated with normal aortas (P = .011). Abnormal flow displacement with RL fusion was strongly associated with rightward deviation (36 of 37 cases), whereas RN fusion skewed leftward (seven of eight cases; P < .01). In patients with aortopathy, RL fusion was strongly associated with type 2 aortopathy and RN with type 3 aortopathy (P < .01). CONCLUSIONS: Conventional PC MRI can identify abnormal systolic flow and differences in jet orientation with BAV. RL leaflet fusion is associated with rightward flow deviation and type 2 aortopathy, whereas RN fusion is linked to leftward deviation and type 3 aortopathy. The presence and direction of eccentric flow jets may help risk stratify these patients for valve-related aortic disease.

A novel low-fidelity simulator for both mitral valve and tricuspid valve surgery: the surgical skills trainer for classic open and minimally invasive techniques.

OBJECTIVES: Simulators have been proven to equip trainee surgeons with better skills than the traditional, standard approach to skill development. The purpose of this study was to develop a low-fidelity, low-cost, reusable and portable simulation device, which could provide training in nearly the full range of mitral valve surgery techniques, in both the classic, open approach as well as the minimally invasive approach. METHODS: This novel simulator is made up of commonly available components. The basic elements are a classic baby bottle, with the associated feeding teat and screw ring, in combination with a sheet of dental dam. The detailed process for making this simulator is outlined in this article. Maximum suture tensile strength on the different components was tested with a digital force gauge. Reusability and the rate of wear as a result of suturing were documented. Total cost was calculated in euros (€). RESULTS: This study resulted in a simulation model very similar in size to the actual anatomical dimensions of the mitral valve. Various pathological conditions, according to Carpentier's Functional Classification, could be simulated. This led to the possibility of providing training in several mitral valve surgical techniques. As the model developed, it became clear that it could also be used to practice tricuspid valve surgery techniques. Maximum mean suture tensions on the silicone teat and dental dam were 42.11 and 11.15 N/m(2), respectively. The feeding teat started wearing after approximately 45 suture placements. Total cost of the study model was €5.14. CONCLUSIONS: This relatively simple, low-cost, low-fidelity model can provide simulation training in nearly the full range of mitral valve and tricuspid valve surgical techniques, in both the classic open approach and the minimally invasive approach-and do so almost anywhere. Especially when used by young cardiothoracic surgeons in training, this model may contribute to the development of technical skills and procedural knowledge required for adequate performance in the operating room.

Cost-effectiveness analysis of TAVR.

Transcather aortic valve replacement (TAVR) has rapidly gained worldwide acceptance for treating very high-risk patients with symptomatic severe aortic stenosis. Two valve systems are currently in common use worldwide and under trial in the United States. The Edwards SAPIEN valve has completed its PARTNER trial and has been approved for use in nonoperative patients. The Medtronic CoreValve is currently completing its US pivotal trial. Both plan studies of intermediate-risk patients. The use of TAVR in Europe has grown rapidly and is now about 23% of the total aortic valve replacements done in which a tissue valve is chosen (generally patients over 60 to 65 years of age). This technology is used in a patient population that was either not receiving any surgical therapy due to extreme risk or was considered very high risk for conventional surgery. The procedure requires a highly trained TAVR team, advanced imaging, and the devices themselves, which are expensive. Medical device trials are generally designed to establish if the device works as planned. For TAVR in today's world of rising health care costs, the additional question of cost effectiveness is important to address. Fortunately, the PARTNER trial addressed this and the CoreValve trial has built this into the trial design as well. This article examines what is currently known about the cost-effectiveness of TAVR.

Computational assessment of bicuspid aortic valve wall-shear stress: implications for calcific aortic valve disease.

The bicuspid aortic valve (BAV) is associated with a high prevalence of calcific aortic valve disease (CAVD). Although abnormal hemodynamics has been proposed as a potential pathogenic contributor, the native BAV hemodynamic stresses remain largely unknown. Fluid-structure interaction models were designed to quantify the regional BAV leaflet wall-shear stress over the course of CAVD. Systolic flow and leaflet dynamics were computed in two-dimensional tricuspid aortic valve (TAV) and type-1 BAV geometries with different degree of asymmetry (10 and 16% eccentricity) using an arbitrary Lagrangian­Eulerian approach. Valvular performance and regional leaflet wallshear stress were quantified in terms of valve effective orifice area (EOA), oscillatory shear index (OSI) and temporal shear magnitude (TSM). The dependence of those characteristics on the degree of leaflet calcification was also investigated. The models predicted an average reduction of 49% in BAV peak-systolic EOA relative to the TAV. Regardless of the anatomy, the leaflet wall-shear stress was side-specific and characterized by high magnitude and pulsatility on the ventricularis and low magnitude and oscillations on the fibrosa. While the TAV and non-coronary BAV leaflets shared similar shear stress characteristics, the base of the fused BAV leaflet fibrosa exhibited strong abnormalities, which were modulated by the degree of calcification (6-fold, 10-fold and 16-fold TSM increase in the normal, mildly and severely calcified BAV, respectively, relative to the normal TAV). This study reveals the existence of major differences in wall-shear stress pulsatility and magnitude on TAV and BAV leaflets. Given the ability of abnormal fluid shear stress to trigger valvular inflammation, the results support the existence of a mechano-etiology of CAVD in the BAV.

Liquefaction necrosis of mitral annular calcification (LNMAC): review of pathology, prevalence, imaging and management: proposed diagnostic imaging criteria with detailed multi-modality and MRI image characterization.

Liquefactive necrosis within a large spheroid zone of mitral annular calcification (LNMAC) is an atypical but increasingly recognized variant of mitral annular calcification (MAC). Proposed MRI, echo, and CT imaging criteria for diagnosis of this unusual disease entity are discussed along with a review of the prognosis, histopathology, and management implications. A comprehensive ECHO, CT, and MRI imaging approach to diagnostic differentiation from other cardiac masses, allowing characterization of the differing components of this unusual lesion is emphasized. Differentiation from surrounding myocardium, and demonstration of peripheral ring type hyperenhancement, or hyperintense signal in the wall of this lesion, seen with specific inversion recovery MRI sequences is presented as a major diagnostic criterion. The relationship of these MRI image findings to underlying pathology is also discussed. An illustrative case vignette is provided for clinical reference.

Sex differences in procedure selection and outcomes of patients undergoing mitral valve surgery.

BACKGROUND: There is a paucity of data on sex differences in procedure selection and outcomes of patients undergoing mitral valve surgery. METHODS AND RESULTS: The National Inpatient Sample database from 2005 to 2008 was searched to identify patients ≥30 years of age who underwent mitral valve repair or replacement (ICD-9-CM codes 35.12, 35.23, and 35.24). Women constituted 51.6% of the patients, and they were older, were less affluent, had higher values for the Charlson comorbidity index, and more often presented on an urgent/emergent basis. Women underwent repair less often than men (37.9% versus 55.9%, P < .001) and more often underwent concomitant tricuspid surgery or a Maze procedure. After adjustment for propensity scores, women were more likely to undergo replacement (odds ratio, 1.78; 95% confidence interval, 1.64-1.93; P = .0001), they had longer lengths of stay, and less favorable disposition. Among the patients who underwent mitral valve repair, women had a higher hospital mortality (2.06% versus 1.36%, P = .0328). After adjustment for propensity scores and concomitant procedures, this relationship was no longer statistically significant. CONCLUSIONS: Women are less likely than men to receive mitral valve repair. Although the higher hospital mortality of women presenting for mitral valve surgery was accounted for by their worse preoperative profiles, this sex disparity reflects the current reality in surgical practice and identifies an important area for future improvement in the care of patients with valvular heart disease.

Assessment of global and regional left ventricular volume and shape by real-time 3-dimensional echocardiography in dogs with myxomatous mitral valve disease.

BACKGROUND: Left ventricular (LV) remodeling occurs in response to chronic volume overload. Real-time 3-dimensional (RT3D) echocardiography offers new modalities for LV assessment. OBJECTIVE: To investigate LV changes in shape and volume in response to different severities of naturally acquired myxomatous mitral valve disease (MMVD) in dogs by RT3D echocardiography. ANIMALS: Sixty-five client-owned dogs. METHODS: Prospectively recruited dogs were classified by standard echocardiography into healthy, mild, moderate, and severe MMVD groups. Endocardial border tracking of LV RT3D dataset was performed, from which global and regional (automatically acquired basal, mid, and apical segments based on LV long-axis length) end-diastolic (EDV) and end-systolic volumes (ESV), LV long-axis length, and sphericity index were obtained. RESULTS: Global and regional EDV and ESV (indexed to body weight) were most prominently increased in dogs with severe MMVD. All 3 regional LV segments contributed to increased global EDV and ESV with increasing MMVD severity, but mid-EDV contributed the most to the global EDV increase. Furthermore, LV long-axis length and LV sphericity index increased with increasing MMVD severity. Basal and apical EDV segments displayed the strongest association with sphericity index (P < .0001). CONCLUSIONS AND CLINICAL IMPORTANCE: The most prominent LV volume expansion was found in dogs with severe MMVD. Increased EDV, primarily in the mid-segment, leads to rounding of LV apical and basal segments in response to increasing MMVD severity. Assessment of LV volume and shape potentially could allow early detection of dogs at risk for rapid progression into congestive heart failure.

[Magnetic resonance imaging in the assessment of valvular heart disease]. / Papel de la resonancia magnética en la evaluación de las valvulopatías.

Although Doppler echocardiography remains the most frequently used imaging modality for assessing valvular heart disease, the technique has a number of limitations that could affect the quality of imaging studies and make the results difficult to interpret. Cardiac magnetic resonance (CMR) imaging could be superior to echocardiography in a number of ways: for example, for assessing ventricular dimensions, volumes, function and mass, for quantifying valvular regurgitation, and for investigating areas of myocardial fibrosis and extracardiac structures. In carrying out these tasks, CMR uses a variety of pulse sequences that are specially created to obtain information on specific tissue characteristics or on particular aspects of blood flow through heart valves. This general review article focuses on the usefulness of CMR in the clinical diagnosis of valvular heart disease and reviews how the data acquired using the technique can be incorporated into algorithms for the clinical management of patients with significant valvular heart lesions.

Assessment of valvular heart disease by cardiovascular magnetic resonance imaging: a review.

CMR is a comprehensive non-invasive tool capable of evaluating all aspects of valvular heart disease. It has advantages over echo including direct quantification of regurgitant lesions, highly accurate assessment of ventricular size and function, visualisation myocardial scar, and interrogation of extracardiac abnormalities. Although these gains can be realised with current scanning techniques, CMR's full potential has yet to be realised, and further studies of clinical outcomes are needed before CMR data can be integrated into the management algorithms for patients with significant valvular lesions.

Utility of 3D transoesophageal echocardiography for the assessment of tricuspid and pulmonary valves in carcinoid heart disease.

We report on the case of a 45-year-old lady with metastatic carcinoid tumour and carcinoid syndrome who develops severe valvulopathy involving the tricuspid and pulmonary valve. The use of three-dimensional transoesophageal echocardiography allowed more detailed morphological assessment of tricuspid and pulmonary valve pathology, sub-valvular apparatus and improved delineation of the relationship between these structures and surrounding cardiac chambers.

Stress Doppler echocardiography in valvular heart diseases: utility and assessment.

This article is a review on the role of stress echocardiography in valvular heart diseases, describing what the validated indications are, how to perform the test and the utility of performing this examination. Most valve diseases are characteristically dynamic and this dynamic component is best appreciated by exercise Doppler echocardiography. Dobutamine stress echocardiography is also useful in patients with severe aortic stenosis and left ventricular dysfunction. The main advantage of stress echocardiography is to concomitantly allow the evaluation of symptoms, exercise capacity and the hemodynamic consequences of valve diseases, especially in patients with severe valve diseases who deny symptoms or present equivocal symptoms. It also provides important prognostic information and may help to optimize surgical timing in difficult cases. Whether these data should be integrated in the management of patients needs further validation.

Echocardiographic and histological assessment of age-related valvular changes in normal rats.

Aging is associated with morphologic and functional alterations of the rat's left ventricle. However, the time-course of valvular function and morphology in normal aging rats has not yet been studied. For this purpose, 30 male Wistar rats (318 +/- 5g, 10 weeks old) underwent serial echocardiograms for 58 weeks under sodium pentobarbital 50 mg/kg IP anesthetization followed by necropsy. Histopathology was also performed in two additional groups of 10 rats at 10 and 30 weeks of age. Regurgitations were considered as any retrograde flow on 2-D or M-mode color Doppler echocardiography. Tricuspid regurgitation was already found at 10 weeks of age and became more frequent with age. Pulmonary, mitral and aortic regurgitation was seldom observed at 10 weeks but became more frequent after 30 weeks. For the mitral and aortic valve, this was also associated with an increase in valvular thickness because of nodular or segmental myxoid leaflet changes. The severity of valvular regurgitations did not increase with age. In conclusion, aging leads to morphologic and functional valvular changes in normal rats. This is important when investigating models of valvular heart disease in small animals.

Assessment of cardiac structure and left atrial appendage functions in primary antiphospholipid syndrome: a transesophageal echocardiographic study.

BACKGROUND AND PURPOSE: Although thromboembolic events are the major complication of primary antiphospholipid syndrome (PAPS), cardiac involvement is commonly present. Left atrial appendage (LAA) is recognized as an important source for thrombus formation and thromboembolism. The purpose of the study was to assess the structure and function of LAA with transesophageal echocardiography (TEE) in PAPS patients. METHODS: Thirty-one PAPS patients (22 women, mean age 36+/-9 years) in sinus rhythm and 31 (17 women, mean age 37+/-7 years) controls with normal TEE examination were investigated. RESULTS: Eighty-four percent of the PAPS patients had functional and structural valvular defect predominantly in the mitral valve. Valvular lesions were especially frequent in PAPS patients with a history of cerebrovascular events, patients with history of arterial thrombosis (91.6%), and patients with high titers of IgG anticardiolipin antibodies (100%). Intracardiac thrombus was present in 5 patients and in 1 of them it was located in LAA. The structure of LAA was similar between groups. Left atrial appendix ejection fraction (51.8+/-4 versus 48.6+/-5.5%; P<0.05) and LAA peak outflow velocity (87+/-10.9 versus 80.6+/-10.3 cm/s; P=0.02) was significantly higher in PAPS group compared with controls. In PAPS patients with mitral regurgitation (MR), LAA outflow peak velocity (84.3+/-10 versus 98.6+/-6.5 cm/s; P=0.002) and LAA inflow peak velocity (67.8+/-10.5 versus 80.8+/-8.6 cm/s; P=0.009) were significantly lower compared with PAPS patients without MR. CONCLUSIONS: It was concluded that disease process in PAPS frequently involved cardiac valves especially mitral valve but spared LAA function. LAA function was normal, but intracardiac thrombus was present in 5 patients and 1 of them was located in LAA. MR in PAPS patients seems to impair LAA function.

Cardiac valvular tumors: cardiac papillary fibroelastoma.

Case histories of proposed life insurance are presented to introduce the topic of cardiac valvular tumors. Using fibroelastoma as the prototypical cardiac tumor, pathology, diagnosis, echocardiographic findings and clinical course are reviewed, based on available clinical literature. Although the natural history of benign cardiac tumors is uncertain, because of the risk of adverse outcomes, cases must be underwritten on an individual basis until long-term studies become available.