Artur Evangelista

Bicuspid Aortic Valve Identifying Knowledge Gaps and Rising to the Challenge From the International Bicuspid Aortic Valve Consortium (BAVCon)

> Everything should be kept as simple as possible, but no simpler. > > —Albert Einstein1 Since its estimated first description >500 years ago by Leonardo da Vinci,2 the bicuspid aortic valve (BAV) has progressively built a reputation; initially, as a curious valvular phenotype with a tendency to develop obstruction and insufficiency. In more contemporary times, however, the BAV is recognized as underlying almost 50% of isolated severe aortic stenosis cases requiring surgery,3 and has been extensively associated with ominous outcomes such as bacterial endocarditis and aortic dissection.4 These associations, coupled with the high prevalence of BAV in humans,5 have prompted investigative efforts into the condition, which although insightful, have generated more questions than answers. This review describes our current knowledge of BAV, but, more importantly, it highlights knowledge gaps and areas where basic and clinical research is warranted. Our review has 2 sections. The first section outlines the multifaceted challenge of BAV, our current understanding of the condition, and barriers that may hamper the advancement of the science. The second section proposes a roadmap to discovery based on current imaging, molecular biology, and genetic tools, recognizing their advantages and limitations. ### A Condition Characterized by Variable Clinical Presentation The clinical presentation and consequences of BAV in humans are exceedingly heterogeneous, with few clinical or molecular markers to predict associated complications.4,6 BAV can be diagnosed at any stage during a lifetime, from newborns7 to the elderly,8 and in the setting of variable clinical circumstances. Some are benign circumstances such as auscultatory abnormalities or incidental echocardiographic findings in otherwise healthy patients8; other circumstances are morbid, such as early severe aortic valve dysfunction, premature congestive heart failure, and thoracic aortic aneurysms (TAAs).8,9 Life-threatening circumstances include bacterial endocarditis and acute aortic dissection.8–11 These complications may present …

Long-Term Outcome of Aortic Dissection With Patent False Lumen Predictive Role of Entry Tear Size and Location

Background— Patent false lumen in aortic dissection has been associated with poor prognosis. We aimed to assess the natural evolution of this condition and predictive factors. Methods and Results— One hundred eighty-four consecutive patients, 108 surgically treated type A and 76 medically treated type B, were discharged after an acute aortic dissection with patent false lumen. Transesophageal echocardiography was performed before discharge, and computed tomography was performed at 3 months and yearly thereafter. Median follow-up was 6.42 years (quartile 1 to quartile 3: 3.31–10.49). Forty-nine patients died during follow-up (22 type A, 27 type B), 31 suddenly. Surgical or endovascular treatment was indicated in 10 type A and 25 type B cases. Survival free from sudden death and surgical-endovascular treatment was 0.90, 0.81, and 0.46 (95% CI, 0.36–0.55) at 3, 5, and 10 years, respectively. Multivariate analysis identified baseline maximum descending aorta diameter (hazard ratio [HR]: 1.32 [1.10–1.59]; P =0.003), proximal location (HR: 1.84 [1.06–3.19]; P =0.03), and entry tear size (HR: 1.13 [1.08–1.2]; P <0.001) as predictors of dissection-related adverse events, whereas mortality was predicted by baseline maximum descending aorta diameter (HR: 1.36 [1.08–1.70]; P =0.008), entry tear size (HR: 1.1 [1.04–1.16]; P =0.001), and Marfan syndrome (HR: 3.66 [1.65–8.13]; P =0.001). Conclusions— Aortic dissection with persistent patent false lumen carries a high risk of complications. In addition to Marfan syndrome and aorta diameter, a large entry tear located in the proximal part of the dissection identifies a high-risk subgroup of patients who may benefit from earlier and more aggressive therapy. # Clinical Perspective {#article-title-39}Background— Patent false lumen in aortic dissection has been associated with poor prognosis. We aimed to assess the natural evolution of this condition and predictive factors. Methods and Results— One hundred eighty-four consecutive patients, 108 surgically treated type A and 76 medically treated type B, were discharged after an acute aortic dissection with patent false lumen. Transesophageal echocardiography was performed before discharge, and computed tomography was performed at 3 months and yearly thereafter. Median follow-up was 6.42 years (quartile 1 to quartile 3: 3.31–10.49). Forty-nine patients died during follow-up (22 type A, 27 type B), 31 suddenly. Surgical or endovascular treatment was indicated in 10 type A and 25 type B cases. Survival free from sudden death and surgical-endovascular treatment was 0.90, 0.81, and 0.46 (95% CI, 0.36–0.55) at 3, 5, and 10 years, respectively. Multivariate analysis identified baseline maximum descending aorta diameter (hazard ratio [HR]: 1.32 [1.10–1.59]; P=0.003), proximal location (HR: 1.84 [1.06–3.19]; P=0.03), and entry tear size (HR: 1.13 [1.08–1.2]; P<0.001) as predictors of dissection-related adverse events, whereas mortality was predicted by baseline maximum descending aorta diameter (HR: 1.36 [1.08–1.70]; P=0.008), entry tear size (HR: 1.1 [1.04–1.16]; P=0.001), and Marfan syndrome (HR: 3.66 [1.65–8.13]; P=0.001). Conclusions— Aortic dissection with persistent patent false lumen carries a high risk of complications. In addition to Marfan syndrome and aorta diameter, a large entry tear located in the proximal part of the dissection identifies a high-risk subgroup of patients who may benefit from earlier and more aggressive therapy.

Impact of contrast-enhanced echocardiography on the diagnostic algorithm of acute aortic dissection

AIMS To determine the usefulness of contrast echocardiography in the diagnosis of aortic dissection (AD) and in the assessment of findings necessary for adequate patient management. METHODS AND RESULTS Conventional and contrast-enhanced transthoracic echocardiography (TTE) and transoesophageal echocardiography (TOE) were performed in 128 consecutive patients with clinically suspected acute AD. Results were validated independently against intraoperative findings in 45 patients and computed tomography information in 83. Sensitivity and specificity of conventional TTE increased after contrast enhancement from 73.7 to 86.8% (P< 0.005) and 71.2 to 90.4% (P < 0.05), respectively. Sensitivity and specificity of enhanced TTE were similar to conventional TOE in ascending aorta (93.3 vs. 95.6% and 97.6 vs. 96.4%, respectively) and in the arch (88.4 vs. 93.0% and 95.3 vs. 98.82%, respectively). Contrast-enhanced TOE permitted the location of non-visualized entry tear in seven cases (10.6%), helped to correctly identify the true lumen in six (9.1%), and diagnosed retrograde dissection in nine (13.6%). CONCLUSION Contrast enhancement substantially improves TTE in the diagnosis of AD and should be considered as the initial imaging modality in the emergency setting. Contrast enhancement also has significant value for obtaining critical morphological and haemokinetic information by TOE useful for adequate patient management.

Bicuspid aortic valve aortopathy in adults: Incidence, etiology, and clinical significance

Bicuspid aortic valve is the most common congenital heart defect and is associated with an aortopathy manifested by dilatation of the ascending thoracic aorta. The clinical consequences of this aortopathy are the need for periodic monitoring of aortic diameters, elective prophylactic surgical aortic repair, and the occurrence of aortic dissection or rupture. This review describes the current knowledge of BAV aortopathy in adults, including incidence, pathophysiologic insights into its etiology, contemporary hypothesis-generating observations into its complications, and recommendations for monitoring and intervention.

Prognostic value of preoperative indexed end-systolic left ventricle diameter in the outcome after surgery in patients with chronic aortic regurgitation

BACKGROUND End-systolic diameter (ESD) is an important parameter in the prognosis and indication for surgery in chronic aortic regurgitation (AR). It has been suggested that ESD values noncorrected for body surface area (BSA) could be inappropriate in the management of patients with extreme BSA. The aim of the study was to assess the usefulness of indexed ESD (IESD) of the left ventricle in the management of patients with severe isolated chronic AR. METHODS One hundred forty-seven patients underwent surgery for chronic AR and were followed up for a mean of 8 +/- 6 years (1-22 years). A post hoc assessment was made of the prognostic value of preoperative ESD and IESD in different BSA percentiles: group 1, <or= 25th percentile (BSA 1.43-1.68 m(2), n = 40); group 2, >25th percentile and <or=75th percentile (BSA 1.69-1.91 m(2), n = 68); and group 3, >75th percentile (BSA 1.92-2.24 m(2), n = 39). RESULTS Age-adjusted preoperative ESD and IESD were independent predictors of mortality or heart failure in the entire population. Magnitude of the relative risk was slightly greater using preoperative IESD than ESD (HR 1.07, 95% CI 1.01-1.29, P = .017; HR 1.04, 95% CI 1.01-1.08, P = .016). In group 1, the age-adjusted expected mortality rate would drop if IESD of 25 mm/m(2) was used as a surgical criterion instead of ESD 50 mm, from 37.94% to 24.27% at 10 years (P = .002). CONCLUSIONS The use of IESD improves the prediction of unfavorable outcomes after surgery in patients with low BSA but not in those with high BSA. In patients with low BSA, IESD >or=25 mm/m(2) should be used as a cutoff point for surgery rather than ESD >50 mm.

Diagnóstico y cuantificación del foramen oval permeable. ¿Cuál es la técnica de referencia? Estudio simultáneo con Doppler transcraneal, ecocardiografía transtorácica y transesofágica

INTRODUCTION AND OBJECTIVES Patent foramen ovale (PFO) is the most common cause of cryptogenic stroke in patients younger than 55. Transesophageal echocardiography (TEE) has been accepted as the reference diagnostic technique. The purpose of this study was to compare the accuracy of transthoracic echocardiography (TTE), TEE and transcranial Doppler (TCD) in the diagnosis and quantification of patent foramen ovale. METHODS We studied 134 patients prospectively. Simultaneous TTE with TCD and TEE with TCD were performed, using agitated saline solution to detect right to left shunt. RESULTS In 93 patients diagnosed with PFO, the shunt was visualized at baseline by TCD in 69% of cases, by TTE in 74% and by TEE in 58%. The Valsalva maneuver produced a similar improvement in shunt diagnosis with all 3 techniques (26%-28%). TTE and TCD showed higher sensitivity (100% vs 97%; non significant difference) than TEE in the diagnosis of PFO (86%; P<.001). TCD performed during TEE did not diagnose 12 (13%) shunts previously diagnosed during TTE. Similarly, TEE underestimated shunt severity. CONCLUSIONS TTE enables adequate diagnosis and quantification of PFO. TEE is less sensitive and tends to underestimate the severity of the shunt.

Aortic biomechanics by magnetic resonance: Early markers of aortic disease in Marfan syndrome regardless of aortic dilatation?

BACKGROUND Previous studies demonstrated the usefulness of MRI in the evaluation of aortic biomechanics in Marfan patients with aortic dilatation. However, these parameters have not been well studied in earlier stages of aortic disease. The present work aimed to study aortic biomechanics: aortic distensibility (AD) and pulse wave velocity (PWV), by MRI in Marfan patients without advanced aortic disease. METHODS Eighty consecutive Marfan patients were compared with 36 age- and sex-matched controls. MRI images at the level of ascending, descending and abdominal aorta were used to determine AD and PWV. RESULTS Marfan patients (27 men; age: 32.0 ± 10.5 years; mean aortic root diameter: 37.2 ± 4.6mm) had lower AD at all levels (ascending 2.6 ± 2.1 vs. 6.2 ± 3.7 mm Hg(-1)·10(-3), p<0.001; descending 3.1 ± 2.0 vs. 8.3 ± 4.2, p<0.001; and abdominal 4.5 ± 2.2 vs. 14.0 ± 5.2, p<0.001), higher aortic arch PWV (8.1 ± 6.5 vs. 4.3 ± 1.8m/s, p<0.01) and ascending-to-abdominal PWV (6.1 ± 3.0 vs. 4.7 ± 1.5m/s, p<0.01) compared with controls. Thirty-five Marfan patients had a non-dilated aortic root (mean aortic root diameter: 34.5 ± 3.8 mm). In multivariable analyses, after adjustment for age, pulse pressure and aortic dimensions, AD remained lower and PWV higher in Marfan patients; even Marfan patients with non-dilated aortic root showed impaired aortic biomechanics compared with controls. Z-score for ascending AD<-3.5 distinguished Marfan patients from controls with 82.5% sensitivity and 86.1% specificity. CONCLUSIONS Aortic biomechanics by MRI were abnormal in the entire aorta in Marfan patients. Moreover, Marfan patients without dilated aortic root showed clear impairment of aortic biomechanics, which suggests that they may be used as early markers of aortic involvement in these patients.

Usefulness of real-time three-dimensional transoesophageal echocardiography in the assessment of chronic aortic dissection

AIMS To assess the usefulness of three-dimensional transoesophageal echocardiography (3D-TOE) vs. two-dimensional (2D)-TOE in the evaluation of morphological and dynamic findings of aortic dissection, and compare the results with those obtained by multi-slice computed tomography (CT). METHODS AND RESULTS Twenty-six patients (21 men and 5 women, median age: 67 years, range: 28-74 years) diagnosed of chronic aortic dissection with patent false lumen were studied. A comprehensive 2D-TOE and a real-time 3D-TOE study targeted at assessing dissection variables were performed and compared with CT within 3 months. Both 3D-TOE and 2D-TOE visualized the intimal flap extension and presence of flow in aortic dissection lumina in the same aortic segments. Three-dimensional TOE correctly identified true lumen in all cases, being superior to 2D-TOE in three cases with a spiroidal course of the dissection in descending aorta. Maximum entry tear diameter measured by 3D-TOE showed a better correlation with CT than 2D-TOE (0.96 and 0.87, P< 0.001, respectively). Compared with CT, 2D-TOE underestimated maximum entry tear diameter (-1.75 ± 3.28 mm, P< 0.01) but 3D-TOE did not (-0.20 ± 1.92 mm, P: n.s.). However, entry tear area measured by 3D-TOE and CT showed the best correlation (r: 0.97) and agreement (0.05 ± 0.20 cm(2), P: n.s.). CONCLUSION Three-dimensional TOE provides additional information to 2D-TOE in aortic dissection assessment, particularly in entry tear size quantification. Agreement between entry tear area defined by 3D-TOE and CT was excellent. Three-dimensional TOE permits better morphological and dynamic understanding of aortic dissection when the flap is spiroidal.

Can simulation help to answer the demand for echocardiography education

There has been a recent explosion of education and training in echocardiography in the specialties of anesthesiology and critical care. These devices, by their impact on clinical management, are changing the way surgery is performed and critical care is delivered. A number of international bodies have made recommendations for training and developed examinations and accreditations.The challenge to medical educators in this area is to deliver the training needed to achieve competence into already overstretched curricula.The authors found an apparent increase in the use of simulators, with proven efficacy in improving technical skills and knowledge. There is still an absence of evidence on how it should be included in training programs and in the accreditation of certain levels.There is a conviction that this form of simulation can enhance and accelerate the understanding and practice of echocardiography by the anesthesiologist and intensivists, particularly at the beginning of the learning curve.

Imaging modalities for the early diagnosis of acute aortic syndrome.

The term acute aortic syndrome (AAS) incorporates aortic dissection, intramural haematoma, and penetrating atherosclerotic ulcer. The common feature of these entities is disruption of the medial layer of the aortic wall. Owing to the life-threatening nature of these conditions, prompt and accurate diagnosis is of paramount importance—misdiagnosis can be fatal. The noninvasive imaging techniques that have a fundamental role in the diagnosis and management of patients with AAS include CT, MRI, transoesophageal echocardiography (TEE), and transthoracic echocardiography (TTE). CT is the most-commonly used imaging modality owing to its wide availability, accuracy, and large field of view. CT plus TTE is the best combination for diagnosing AAS and its complications, and allows important morphological and dynamic aspects of AAS to be assessed and appropriately managed. Ideally, TEE should be performed immediately before surgery or endovascular treatment, in the operating theatre and under general anaesthesia. In stable patients with an uncertain diagnosis of intramural haematoma despite high clinical suspicion, MRI is the technique of choice to make a definitive diagnosis. Imaging techniques have an important role in the primary diagnosis, treatment strategy, and risk stratification of patients with AAS.