Structural abnormalities in hypertrophic cardiomyopathy beyond left ventricular hypertrophy by multimodality imaging evaluation.

BACKGROUND: Echocardiography- and cardiovascular magnetic resonance (CMR)-based studies have revealed a wide range of phenotypic manifestations in hypertrophic cardiomyopathy (HCM) apart from hypertrophy. This study sought to comprehensively describe a number of structural abnormalities in HCM beyond hypertrophy, by multimodality imaging. METHODS: A total of 100 HCM patients were prospectively enrolled, undergoing standard and contrast echocardiography, and CMR. Morphological characteristics involving mitral valve leaflets (MVL), subvalvular apparatus, and left ventricular cavity and wall were investigated. Seventy healthy volunteers served as control population. RESULTS: As assessed by echo, MVLs were longer in HCM patients than in controls (anterior method 1: 24[22,28] vs 19[18,20] mm, P < 0.01; anterior method 2: 27[24, 29] vs 21[19, 23] mm, P < 0.01; posterior: 15[12,19] vs 14[13,15] mm, P < 0.01). Abnormal chordal attachment to anterior MVL, anterior papillary muscle displacement, and accessory apical-basal muscle bundle were present in 42 (42%), 61 (61%), and 35 (35%) patients, respectively (P values vs controls <0.01); direct papillary muscle insertion into MVL and hypertrabeculation were found in two and five patients, respectively. Contrast echocardiography (n = 94) detected myocardial crypts in 15 patients (16%). Overall, 83% of HCM subjects had at least one of these phenotypic manifestations. Echocardiography and CMR agreement for MVL length was poor, while for structural characteristics was moderate to substantial (Cohen's Kappa: 0.53-1.00). Except for posterior MVL length and hypertrabeculation, the phenotypic characteristics studied had acceptable reproducibility by echocardiography and CMR. CONCLUSIONS: Structural abnormalities in HCM beyond hypertrophy are significantly common. Multimodality imaging approach to these HCM facets by echocardiography and CMR is feasible and desirable.

Emergency endovascular stent grafting in acute complicated type B dissection.

OBJECTIVE: The objective of this study was to assess midterm results of emergency endovascular stent grafting for patients with life-threatening complications of acute type B aortic dissection. METHODS: Between March 1999 and November 2011, 110 patients (86 men, 24 women) with complications of acute type B aortic dissection (mean age, 61 years; range, 19-87 years) were treated with thoracic endovascular aortic repair for malperfusion (55.5%) or aortic rupture (53.6%) in five major European referral centers and one U.S. referral center. Additional comorbidities included hypertension in 90 patients (82%), diabetes in 14 patients (13%), and chronic obstructive pulmonary disease in six patients (6%). Eleven patients (10%) had undergone previous aortic surgery. RESULTS: Overall hospital mortality was 12% (n = 13), with 14 late deaths after hospital discharge. In-hospital complications occurred in 32 patients (36%); 10 patients developed postoperative renal failure, five patients experienced new permanent neurologic symptoms, and six patients (5.4%) experienced retrograde type A aortic dissection. Furthermore, nine patients (8%) developed an early type I endoleak. Actuarial survival at 1 and 5 years was 85% and 73%, respectively. Postprocedural computed tomography angiography showed complete or partial thrombosis of the false lumen at the stent graft level in 61% and 23% of all patients, respectively. Freedom from treatment failure according to the Stanford classification was 82%, 75%, and 59% at 1, 3, and 5 years. CONCLUSIONS: Endovascular repair of complicated acute type B aortic dissection is proven to be a technically feasible and effective treatment modality in this relatively difficult patient cohort. Short-term and midterm results are persuasive; however, the long-term efficacy needs to be further evaluated.

Unraveling Bicuspid Aortic Valve Enigmas by Multimodality Imaging: Clinical Implications.

Multimodality imaging is the basis of the diagnosis, follow-up, and surgical management of bicuspid aortic valve (BAV) patients. Transthoracic echocardiography (TTE) is used in our clinical routine practice as a first line imaging for BAV diagnosis, valvular phenotyping and function, measurement of thoracic aorta, exclusion of other aortic malformations, and for the assessment of complications such are infective endocarditis and aortic. Nevertheless, TTE is less useful if we want to assess accurately other aortic segments such as mid-distal ascending aorta, where computed tomography (CT) and magnetic resonance (CMR) could improve the precision of aorta size measurement by multiplanar reconstructions. A major advantage of CT is its superior spatial resolution, which affords a better definition of valve morphology and calcification, accuracy, and reproducibility of ascending aorta size, and allows for coronary artery assessment. Moreover, CMR offers the opportunity of being able to evaluate aortic functional properties and blood flow patterns. In this setting, new developed sequences such as 4D-flow may provide new parameters to predict events during follow up. The integration of all multimodality information facilitates a comprehensive evaluation of morphologic and dynamic features, stratification of the risk, and therapy guidance of this cohort of patients.

Contrast-Enhanced Echocardiographic Measurement of Left Ventricular Wall Thickness in Hypertrophic Cardiomyopathy: Comparison with Standard Echocardiography and Cardiac Magnetic Resonance.

BACKGROUND: Left ventricular wall thickness (LVWT) measurement is key in the diagnostic and prognostic assessment of hypertrophic cardiomyopathy (HCM). Recent investigations have highlighted discrepancies in LVWT by cardiac magnetic resonance (CMR) and standard echocardiography (S-Echo) in this condition. The aim of this study was to elucidate the role of contrast-enhanced echocardiography (C-Echo) to optimize LVWT measurement in patients with HCM. METHODS: Fifty patients with HCM were prospectively enrolled, undergoing S-Echo, C-Echo, and CMR. Blinded LVWT measurements were performed according to a 16-segment left ventricular model using all three imaging techniques. Agreement between both echocardiographic modalities and CMR (as the reference technique) at the segmental level was tested using Bland-Altman analyses. Reproducibility on segmental measurements by S-Echo and C-Echo was also investigated. RESULTS: Patients' mean age was 47 ± 21 years, and 35 (70%) were men. Maximal mean LVWT by S-Echo (20.1 ± 3.8 mm) was greater than the values derived using C-Echo (17.6 ± 4.0 mm, P < .01) and CMR (17.7 ± 4.5 mm, P < .01), with no statistically significant difference between the latter two. Segmental Bland-Altman models demonstrated globally smaller bias and narrower 95% limits of agreement for C-Echo compared with S-Echo. Across all left ventricular segments, LVWT by C-Echo was 2.4 mm lower (range, 1.0-2.5 mm) than that derived by S-Echo, which accounted for a 25% intertechnique difference. Regarding maximal LVWT, the mean absolute difference between C-Echo and S-Echo was 3.0 ± 1.9 mm (range, 0.0-7.9 mm), which represented a 15% intertechnique change. Data analyses demonstrated globally less intra- and interobserver variability in segmental LVWT derived from C-Echo compared with S-Echo. CONCLUSIONS: C-Echo rendered LVWT measurements closer to those derived by the reference technique (CMR) and improved reproducibility compared with S-Echo. C-Echo represents a suitable tool for LVWT measurement in patients with HCM as an alternative to CMR whenever this is not available or possible.

Cardiologists and the Cardiology of the Future. Vision and proposals of the Spanish Society of Cardiology for the Cardiology of the 21st Century.

The Cardiology of the Future is a project of the Spanish Society of Cardiology (SEC) whose objectives are as follows: to define the action policies of the SEC; to analyze the trends and changes in the environment that will influence the practice of cardiology in Spain; to define the profile of the cardiologists needed in the future; to propose policies to achieve the objectives resulting from the identified needs; and to identify the role of the SEC in the development and implementation of these policies. This article describes the methodology and the most relevant findings of the final report of this project and the strategic lines to be developed by the SEC in the immediate future, resulting from the analysis performed.

[Natural history and therapeutic management of acute aortic syndrome]. / Historia natural y tratamiento del síndrome aórtico agudo.

Acute aortic syndrome, an acute lesion of the aortic wall involving the media, comprises aortic dissection, intramural hematoma and penetrating ulcer. Recent advances in imaging techniques have helped us to understand the natural history and dynamics of this medical condition. Despite considerable advances in surgical treatment, the current high mortality rate in the acute phase can still be reduced by early clinical suspicion and improved surgical expertise. The advent and incorporation of endovascular treatment has opened up new perspectives in the management of this disease, and may improve the long-term prognosis. This article aims to review our current understanding of the natural history and therapeutic management of acute aortic syndrome.

[Predictive factors of abnormal dynamic intraventricular gradient after valve replacement in severe aortic stenosis]. / Aparición de gradiente dinámico intraventricular después de la sustitución valvular aórtica en pacientes con estenosis aórtica severa.

INTRODUCTION: Dynamic intraventricular gradients (DIG) after valve replacement in severe aortic stenosis have been reported, although the incidence of DIG and clinical signs are still poorly understood.Aim. To evaluate the incidence of DIG)and determine risk factors and associated morbimortality. PATIENTS AND METHOD: One hundred nine consecutive patients with severe aortic valve stenosis undergoing valve replacement were studied prospectively by echocardiography to detect the postoperative appearance of DIG, defined as a maximum flow velocity >/= 2.5 m/s. RESULTS: Sixteen patients (14.9%) developed postoperative DIG. Significant differences between the patients with or without DIG were found for ventricular diameter (left end-diastolic ventricular diameter (LEDVD) 43.2 vs. 47.7 mm, respectively, p < 0.001; left end-systolic ventricular diameter (LESVD) 21 vs. 29 mm, p < 0.001); left ventricular mass index (165 vs. 193 g/m(2), p < 0.05); mean aortic valve gradient (68 vs. 59 mmHg, p < 0.01),; ejection fraction (73 vs. 61%, p < 0.001). No significant differences were found with respect to ventricular wall thicknesses (septal 16.3 vs. 15.7; posterior 14.37 vs. 14.62), the presence of aortic insufficiency, or other postoperative factors (anemia, inotropic agents, etc.). CONCLUSIONS: DIG after aortic valve replacement to treat severe stenosis is not unusual (15%). DIG is usually found at a midventricular location, close to the septum. In patients with postoperative DIG the most common associated factors were small LEDVD, high ejection fractions and ratios of intraventricular septal to posterior wall ratios, high valve gradients and small left ventricular masses. Preoperative echocardiography can identify patients with a higher risk of developing DIG after aortic valve replacement.

[Progress in the acute aortic syndrome]. / Avances en el síndrome aórtico agudo.

Acute aortic syndrome is an acute lesion of the aortic wall involving the aortic media. The term covers aortic dissection, intramural hematoma, and penetrating ulcer. In the last few years, imaging techniques have increased our understanding of the natural history of these disease entities and of the dynamics of the disease processes. Despite significant advances in diagnosis and surgical treatment, the mortality rate in the acute phase remains high. Early clinical suspicion and greater surgical expertise appear to be the only factors that are able reduce mortality. Once the acute phase is past, the descending aorta continues to be involved in most patients, 30% of whom develop complications within 3-5 years. During this later phase, it is essential to optimize medical treatment and to use imaging techniques to follow-up the patient closely. The availability of endovascular treatment has provided new approaches to the management of the condition and could improve long-term prognosis. The aim of this article was to review recent progress in the diagnosis and therapeutic management of this syndrome.