Mitral valve (MV) prolapse (MVP) is the most common cause of surgical mitral regurgitation (MR) in developed countries. The normal mitral annulus is saddle in shape, connecting the left atrium (LA) and left ventricle (LV) into a functional entity, and is susceptible to distortion by LA and LV remodeling. Annular nonplanarity is important for alleviating mechanical stress on mitral leaflets and chordae tendinae. Recently, loss of annular saddle shape has been demonstrated as a novel mechanism in the pathogenesis of degenerative MR. Hitherto, the prevalence and features of annular flattening have not been well assessed in MVP patients, as well as potential factors related to annular flattening. / Current guidelines recommend MV repair as the preferred treatment for clinically significant MR. Annuloplasty are routinely used to restore the annular structure and function. Recent long-term studies showed that anterior leaflet or bileaflet prolpase was associated with suboptimal outcomes. However, the relation between mitral leaflets and annular geometry in MVP is not completely understood. / The application of two-dimensional (2D) echocardiography (ECHO) has been limited since mitral annulus has a nonplanar configuration. Three-dimensional (3D) ECHO was shown to be superior to 2D in defining the anatomy of MVP. In this thesis, MVP was identified as systolic displacement (≥2 mm) of any segment of mitral leaflets into the LA passing the mitral annular plane, as indicated in a long axis view (parasternal or apical three-chamber). Finally, 117 MVP patients consecutively referred to our echo laboratory for transesophageal echocardiography (TEE) were retrospectively studied. Twenty-nine patients referred for TEE for suboptimal transthoracic images, exclusion of endocarditis, or evaluation of cardiac source of embolic event and found to have no underlying structural cardiac disease or arrhythmia were included as the normal reference. Standard 2D transthoracic ECHO and 2D/3D TEE were performed in all the subjects. Custom software was used for quantification of 3D MV morphology and key geometric parameters, including mitral annulus, leaflets and chordae, were automatically measured. / The main findings were as follows: / 1. Annular saddle shape flattening (annular height to commisural width ratio <15%) was a common abnormality in MVP patients. More than 50% MVP patients had annular flattening. / 2. Atrial fibrillation (AF) and mitral annular disjunction (MAD) were found to be independent factors associated with annular flattening. MAD was associated with more severe myxomatous MV changes with excessive traction on papillary muscle. The potential roles of MAD in the development of myxomatous MV disease may be through excessive mobility of the mitral apparatus or disturbing 3D annular configuration with reduced annular height and loss of the saddle shape. / 3. Annular flattening was associated with advanced myxomatous MV changes and a higher prevalence of complex prolapse (anterior leaflet and/or bileaflet prolapse). Complex prolapse was associated with more severe annular flattening and dilation, leaflets elongation, increased billow volume and impaired mitral-aortic coupling. / In summary, this thesis provided new data of the 3D MV geometry in MVP patients with new insights into the mechanisms of disease progression and strategies to improve surgical outcomes. Using new techniques in 3D TEE, the thesis demonstrated that annular flattening is a common abnormality, associated with AF, MAD, and more advanced, complex lesions, with implication in the complexity and timing of MV repair. The results suggested that annuloplasty with saddle-shaped rings may be important to restore the structure and function of MV and to improve the durability of repair, particularly important for complex lesions. These data also implied that maintenance of sinus rhythm may help to prevent annular flattening and progression of MVP-related MR. Finally, MAD may be an echocardiographic marker of annular flattening and may require specific surgical correction. / 在發達國家，需要外科治療二尖瓣反流(MR)最常見的原因是二尖瓣脫垂（MVP）。正常二尖瓣瓣環是一個非平面的馬鞍型結構，連接左心房和左心室使之成為一個功能性整體，其結構和功能易受到心房和心室重構的影響。馬鞍型瓣環有助於降低瓣葉和腱索的機械性壓力。最新研究表明瓣環馬鞍型結構缺失是MVP患者出現退行性MR的發病機制。但沒有研究評估瓣環扁平在MVP患者中的患病率，相關特征性改變及引起瓣環扁平的因素。 / 目前，指南推薦二尖瓣修复術作為伴有嚴重MR的脫垂病人的首選治療方法。二尖瓣修复術中常規使用瓣環成形術有助於恢復瓣環的結構和功能。臨床研究顯示脱垂累及前葉或雙葉的手术预后不理想。目前尚不清楚瓣葉和瓣環幾何結構之間存在的相互關係。 / 由於瓣環的非平面結構，三維超聲評估二尖瓣脫垂的價值優於二維超聲。在本研究中，二尖瓣脫垂被定義為長軸水平（胸骨旁長軸或者心尖三腔切面）二尖瓣瓣葉的任何部分發生收縮期向左房面移位，并超過瓣環水平2毫米以上。最後，我們回顧性地研究了117名被轉診到我們心臟超聲實驗室進行經食道超聲檢查的MVP病人。本研究同時入組了29名无其他潛在結構性心臟病或心律不齊的正常對照。他們由於欠佳的經胸超聲圖質量，或者為了排除心內膜炎，或者為了評價血栓是否來自心臟接受了经食道超聲檢查。所有研究對象均接受了標準的二維經胸超聲及二維/三維經食道超聲檢查。研究採用專業軟件對受試者二尖瓣的三維形態進行定量分析。該軟件可自動測量二尖瓣的重要幾何學參數，包括瓣環，瓣葉和鍵索。 / 研究的主要發現包括： / 1.瓣環扁平（瓣環高度與連合線長度之比不超過15%）是一種常見的解剖學異常，超過50%MVP的患者罹患該種畸形。 / 2. 房顫和二尖瓣瓣環分離是引起瓣環扁平的獨立影響因素。瓣環分離與較嚴重的黏液樣改變以及過多的乳頭肌牽拉相關，其在黏液樣二尖瓣疾病中的作用可能是通過引起二尖瓣裝置的過度移動，或通過降低瓣環的高度導致馬鞍形結構缺失從而破壞二尖瓣裝置正常三維幾何學結構。 / 3. 瓣環扁平與嚴重的黏液樣改變有關，同時合併高比例的複雜脫垂（前葉和/或雙葉脫垂）。複雜脫垂與嚴重瓣環扁平和擴大，瓣葉冗長及隆起容積增加相關，同時破壞了二尖瓣-主動脈瓣耦合。 / 總之，本文對MVP疾病進展的機制提出了新的見解，并為手術治療提供有效的策略。本文採用三維經食道超聲領域中最新的成像和圖像分析技術并發現在MVP患者中瓣環扁平是一種常見解剖學發現，其發病與房顫，瓣環分離及嚴重和複雜病變有明確的相關性。這些發現有助於病變複雜程度分級和確定手術時間。採用馬鞍形瓣環成形術既可以修復二尖瓣的結構和功能又能提高修复的持久性。其重要性在複雜MVP患者中尤為明顯。盡早復律可預防瓣環馬鞍形結構缺失和防止反流的加重。最後瓣環分離可作為检测瓣環扁平的超聲學指標，可能需要特殊的外科矯正。 / Jin, Chunna. / Thesis Ph.D. Chinese University of Hong Kong 2015. / Includes bibliographical references (leaves 152-165). / Abstracts also in Chinese. / Title from PDF title page (viewed on 12, September, 2016). / Detailed summary in vernacular field only. / Detailed summary in vernacular field only. / Detailed summary in vernacular field only. / Detailed summary in vernacular field only. / Detailed summary in vernacular field only. / Detailed summary in vernacular field only. / Detailed summary in vernacular field only. / Detailed summary in vernacular field only.

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Mitral valve prolapse (MVP) is thought to be one of the causes of unexplained cardiac arrest (UCA). However, previous studies are limited by the lack of a standardized evaluation of UCA and the absence of a control group to identify predictors of cardiac arrest. We performed a systematic review of studies that examined the yield UCA evaluation. We then reported the prevalence and characteristics of MVP patients from a multi-centre registry of patients with UCA. Lastly, we completed a protocol of a matched case-control study aiming at comparing echocardiographic features of MVP patients with and without cardiac arrest. As a result of these studies, we proposed a standardized algorithm for UCA evaluation and a definition for idiopathic ventricular fibrillation. Also, we reported the prevalence of MVP in patients with UCA and described few features that could potentially help distinguish patients with MVP at risk for cardiac arrest.

Cardiac arrest has been reported in patients with mitral valve prolapse; however, clinical characteristics and survival information are limited since most of the cases reported include autopsy data. Nine patients (2 male, 7 female) with mitral valve prolapse were identified who had cardiac arrest; ventricular fibrillation was documented in 8 patients; resuscitation was unsuccessful in 2. Eight had a history of palpitations (months to 15 years duration) and ventricular arrhythmias, 3 had a history (5-15 years) of recurrent syncope, and 1 was totally asymptomatic. Cardiac catheterization-angiographic studies in 8 patients demonstrated normal coronary artery anatomy and mitral valve prolapse. All 9 patients had auscultatory and echocardiographic evidence of mitral valve prolapse. Seven survivors (6 still alive) were followed from 3 to 14 years after cardiac arrest. A subset of patients with mitral valve prolapse and cardiac arrest is described in whom past medical history is compatible with cardiac arrhythmias or syncope, and whose long-term prognosis appears better than patients with other causes of cardiac arrest.

Objective: To determine whether echocardiography affects the decisions to use beta blockers or to recommend bacterial endocarditis prophylaxis in patients suspected of having mitral valve prolapse (MVP). Design: Retrospective review of echocardiograms and clinical records. Setting: Military tertiary care hospital. Patients: 127 patients with clinically suspected MVP (105) or incidentally discovered MVP (22). Main results: Beta blockers were used more often in patients with suspected MVP and positive echocardiograms (45%) than in patients with normal echocardiograms (13%, p<0.001). Bacterial endocarditis prophylaxis was recommended more often in patients with suspected MVP and positive echocardiograms (65%) than in patients with normal echocardiograms (11%, p<0.001). Presence or absence of a murmur did not influence the decision to recommend bacterial endocarditis prophylaxis. Patients in whom MVP was incidentally discovered were unlikely to receive either beta blockers or the recommendation for bacterial endocarditis prophylaxis. Conclusions: The results of echocardiography affect the decisions to use beta blockers or to recommend bacterial endocarditis prophylaxis in patients with suspected MVP.

Cardiovascular involvement is commonly reported in various muco- polysaccharidoses. We report a first case of Sanfilippo syndrome type A in a 12-year-old white female who has developed combined progressive mitral valvar regurgitation due to prolapse and aortic regurgitation.

Os cães da raça Dachshund apresentam acondroplasia/hipocondroplasia, considerada uma condição fisiológica da raça, resultando num corpo longo com os membros curtos, arqueados e musculosos, esterno largo e proeminente, caixa torácica oval e ampla. Essas condições podem dificultar a realização e interpretação de alguns exames. A hipótese desse trabalho é que os cães da raça Dachshund tenham valores ecocardiográficos, eletrocardiográficos, radiográficos e pressóricos diferenciados. Para isso, foram estudados 69 cães adultos e sadios da raça Dachshund, sendo 28 (40,6%) machos e 41 (59,4%) fêmeas; com idade variando de 18 meses a 10 anos de idade; e peso médio de 8,4&#177;2,3 kg. Foram realizados exames ecocardiográfico, eletrocardiográfico, radiográfico, bem como determinação de pressão arterial sistêmica em todos os animais. Para a análise dos resultados, os animais foram categorizados, segundo, gênero, presença ou ausência de castração, faixa etária (A: &gt;1 a &le; 3 anos, B: &gt;3 a &le; 6 anos e C: &gt;6 a &le; 10 anos), peso (&lt; 8 kg e &ge; 8 kg) e circunferência torácica (&gt; 45 cm e &ge; 45 cm). Foram estabelecidos, em cães adultos e sadios da raça Dachshund, os valores de ecocardiografia convencional, eletrocardiografia, radiografia e de pressão arterial sistêmica por meio do método Doppler Os resultados ecocardiográficos principais, com a média e intervalo de confiança de 95% foram: septo interventricular em diástole SIVd (6,5 [6,3-6,7] cm), parede livre de ventrículo esquerdo em diástole PLVEd (6,3 [6,1-6,4] cm), diâmetro interno de ventrículo esquerdo na diástole DIVEd (2,5 [2,5-2,6] cm), diâmetro interno de ventrículo esquerdo na sístole DIVEs (1,2 [1,2-1,3] cm), fração de encurtamento - FE -(51,32 [49,79-52,84]%), fração de ejeção do ventrículo esquerdo Fej (0,84 [0,82-0,85]). Não houve diferença estatística para gênero ou castração. Mas quando os cães foram separados pela faixa etária, houve diferença estatística significativa entre os grupos A e B (p = 0,0073) para SIVd e PLVEd; sendo que os valores para o grupo A foram de: SIVd (6,2 [5,9-6,5] cm) e PLVEd (6,0 [5,7-6,2] cm). Os valores para o grupo B foram: SIVd (6,9 [6,5-7,3] cm) and PLVEd (6,5 [6,3-6,8] cm). Não houve diferença entre o grupo C e os grupos A e B; os valores para o grupo C foram: SIVd (6,4 [5,9-6,8] cm) e PLVEd (6,4 [6,1-6,7] cm). Portanto, cães adultos da raça Dachshund possuem espessura maiores de SIVd e PLVEd quando comparados com valores padronizados para outras raças. Quando os animais foram divididos pelo peso (&gt;8 kg e &ge; 8 Kg), houve diferenças significativas para DVEd (2,44&#177;0,26 cm; 2,64&#177;0,27 cm; p&gt;0,001), como também para circunferência torácica (&gt; 45 cm e &ge; 45 cm), com valores de 2,43&#177;0,25 cm e 2,63&#177;0,27 cm (p&gt;0,001). Também foram estabelecidos valores ecocardiográficos para tamanho do átrio esquerdo, nos eixos látero-lateral e ápico-basilar, respectivamente, iguais a 2,24&#177;0,31 cm e 2,17&#177;0,31 cm; bem como os valores de Doppler tecidual do anel valvar mitral lateral: onda E`=0,11&#177;0,02 m/s, onda A´=0,10&#177;0,02 m/s e razões E´/A´=1,12&#177;0,33 m/s e E/E´=5,91&#177;1,21 m/s. Cães adultos e sadios da raça Dachshund podem apresentar prolapso valvar mitral (40,58%). O valor de pressão arterial sistêmica por meio do método Doppler na cauda de cães Dachshund adultos e sadios foi de 134&#177;20 mmHg diferenciando-se estatisticamente (p &gt; 0,0001) dos valores obtidos no membro torácico (155&#177;28 mmHg). / Dachshund dogs present achondroplasia/hypochondroplasia, considered a breed physiological condition, that results in a long body with short, arched and muscular limbs, large sternum, oval and wide ribcage and developed heart and lungs. All this factors can difficult exams realization and interpretation. The hypothesis is that Dachshund dogs have differentiated echocardiographic, electrocardiographic, radiographic, and systolic blood pressure parameters. Therefore, 69 adult and healthy Dachshund dogs were studied (28 [40.6%] males and 41 [59.4%] females); from 18 months to 10 years-old, weighting 8.4&#177;2.3 kg. Echocardiographic, electrocardiographic, radiographic and systemic arterial pressure evaluations were performed. Animals were categorized according to gender, neutered or not, age group (A: &gt;1 a &le; 3 years, B: &gt;3 a 6 years e C: &gt;6 a &le; 10 years), weight (&lt; 8 kg e &ge; 8 kg) e thoracic circumference (&lt; 45 cm e &ge; 45 cm). The values of conventional echocardiography, electrocardiography, radiography and systemic arterial pressure were established in Dachshund dogs using the Doppler method. The main echocardiographic results, with a mean and 95% confidence interval were: interventricular septum in diastole IVSd (6.5 [6.3-6.7] cm), left ventricular free wall in diástole - LVFWd (6.3 [6.1-6.4] cm), left ventricular internal diameter in diastole LVIDd (2.5 [2.5-2.6] cm), left ventricular internal diameter LVIDs (1.2 [1.2-1.3] cm), shortening fraction SF (51.32 [49.79-52.84]%), left ventricular ejection fraction LVEF (0.84 [0.82-0.85]). There was no statistical difference for gender or castration. But when dogs were categorized by age, there was a statistically significant difference between groups A and B (p = 0.0073) for IVSd and LVFWd.; and the values for group A were: IVSd (6.2 [5.9-6.5] cm) and LVFWd (6.0 [5.7-6.2] cm). The values for group B were: IVSd (6.9 [6.5-7.3] cm) and LVFWd (6.5 [6.3-6.8] cm). There was no difference between group C and groups A and B; The values for group C were: IVSd (6.4 [5.9-6.8] cm) and LVFWd (6.4 [6.1-6.7] cm). Therefore, adult dogs of the breed Dachshund have thickness of IVSd and LVFWd when compared with values standardized for other breeds. When animals were divided by weight (&lt; 8 kg and &ge; 8 kg), there were significant differences for LVIDd (2.44 &#177; 0.26 cm, 2.64 &#177; 0.27 cm, p (&lt; 0.001), as well as for thoracic circumference (&lt; 45 cm and &ge; 45 cm), with values of 2.43 &#177; 0.25 cm and 2.63 &#177; 0.27 cm (p &lt; 0.001). Echocardiographic values were also established for left atrial size, on the latero-lateral and apical-basilar axes at 2.24 &#177; 0.31 cm and 2.17 &#177; 0.31 cm, respectively. In addition, the tissue Doppler values of the lateral mitral valve ring: E\' wave = 0.11&#177;0.02 m/s, A\' wave = 0.10&#177;0.02 m/s and E\'/A\' ratios = 1,12&#177;0.33 m/s and E/E \'= 5.91&#177;1.21 m/s. Adult and healthy Dachshund dogs may present with mitral valve prolapse (40.58%). The Doppler method in the tail of healthy Dachshund dogs was 134 &#177; 20 mmHg, statistically different (p &lt; 0.0001) from values obtained in left forelimb (155 &#177; 28 mmHg).

Introdução: O transtorno de ansiedade social (TAS) é uma condição que pode ser muito incapacitante, com considerável sofrimento subjetivo, alta prevalência de comorbidades psiquiátricas e impacto negativo no funcionamento psicossocial. Entretanto, existem poucos dados na literatura sobre a possível extensão deste comprometimento nos indivíduos com sinais e sintomas subclínicos do TAS. Além disso, a discussão sobre a associação entre o prolapso de valva mitral (PVM) e os transtornos de ansiedade, particularmente com o transtorno de pânico e o TAS, existe já há cerca de três décadas, mas os resultados publicados não são suficientes para definitivamente estabelecer ou excluir a associação entre essas condições, com prevalências variando de 0 a 57%. Método: O delineamento metodológico envolveu duas etapas. Na primeira, as comorbidades psiquiátricas e o comprometimento no funcionamento psicossocial foram avaliados em 355 estudantes universitários que haviam sido diagnosticados previamente como TAS (n=141), TAS subclínico (n=92) ou controles (n=122). Na segunda etapa, um total de 232 voluntários diagnosticados como transtorno de pânico (n=41), TAS (n=89) ou controles (n=102) foram avaliados em ecocardiografia quanto ao PVM. Os exames foram realizados por dois cardiologistas que estavam cegos em relação ao diagnóstico psiquiátrico dos participantes. Foram obtidas medidas utilizando os critérios atuais e antigos para o diagnóstico de PVM, para permitir a comparação e generalização dos resultados. Resultados: A taxa de comorbidade com outros transtornos psiquiátricos foi de 71,6% no grupo TAS e de 50% nos sujeitos com TAS subclínico, ambos significativamente maiores que os controles (28,7%). A presença de comorbidades foi progressivamente maior de acordo com o subtipo e a gravidade do TAS. Quanto ao funcionamento psicossocial o grupo TAS apresentou maior comprometimento que os outros dois grupos em todos os domínios avaliados, e os sujeitos com TAS subclínico apresentaram valores intermediários. Na segunda etapa, os resultados demonstraram que não há diferenças estatísticas entre os grupos quanto à prevalência de PVM, seja pelos critérios ecocardiográficos atuais para o diagnóstico de PVM (visão longitudinal paraesternal: pânico=2,4%, TAS=4,5%, controles=1,0%) ou pelos critérios antigos (visão apical de 4-câmaras: pânico=2,4%, TAS=4,5%, controles=10,8%; modo-M: pânico=2,4%, TAS=6,7%, controles=4,9%). Também não houve diferenças significativas em relação a outras características morfológicas, como presença de regurgitação mitral, espessamento valvar ou presença de alongamento de cordoalhas. Conclusões: A prevalência de comorbidades psiquiátricas e o comprometimento no funcionamento psicossocial aumentam progressivamente ao longo do espectro de ansiedade social. O fato de o TAS subclínico apresentar considerável incapacidade e sofrimento em comparação com sujeitos controles justifica uma revisão na validade desses critérios diagnósticos. Por outro lado, não houve associação entre o transtorno de pânico ou o TAS com o PVM em nossos resultados, independente dos critérios diagnósticos utilizados, com prevalências compatíveis com a esperada na população geral. Dessa forma, é preciso desmistificar a relação entre essa alteração cardíaca e o transtorno de pânico e o TAS, pelas repercussões que pode ter para o paciente e em seu tratamento psiquiátrico. / Background: Social anxiety disorder (SAD) is a highly disabling condition that causes considerable subjective suffering. It has a high prevalence rate of psychiatric comorbidities and a negative impact on psychosocial functioning. However, few data are available in the literature about the possible extent of this impairment in individuals with subthreshold signs and symptoms of SAD. In addition, the discussion about the association between mitral valve prolapse (MVP) and anxiety disorders, especially panic disorder and SAD, has been going on for over three decades, but the published results are insufficient to establish or to exclude an association between these conditions, with reported prevalence rates ranging from 0% to 57%. Method: The methodological design involved two stages. In the first, psychiatric comorbidities and psychosocial functioning impairment were evaluated in 355 college students diagnosed with SAD (n=141), subthreshold SAD (n=92) or as healthy controls (n=122) in a previous study. In the second stage, a total of 232 volunteers previously diagnosed with panic disorder (n=41), SAD (n=89) or as healthy controls (n=102) underwent echocardiographic evaluation for MVP. The exams were performed by two cardiologists who were blind to the psychiatric diagnosis of the participants. Measurements based on current and earlier MVP diagnostic criteria were taken in order to permit the comparison and generalization of the results. Results: The rate of comorbidity with other psychiatric disorders was 71.6% in the SAD group and 50% in subjects with subthreshold SAD, both significantly greater than controls (28.7%). The presence of comorbidities increased progressively according to SAD subtype and severity. Concerning psychosocial functioning, the SAD group had greater impairment than the other two groups in all domains evaluated, and subjects with subthreshold SAD presented intermediate values. In the second stage, the results demonstrated that there were no statistically significant differences among the groups in terms of MVP prevalence, whether using current diagnostic criteria (long-axis view: panic=2.4%, SAD=4.5%, control=1.0%) or earlier criteria (apical four-chamber view: panic=2.4%, SAD=4.5%, control=10.8%; M-mode: panic=2.4%, SAD=6.7%, control=4.9%). Also, there were no significant differences regarding other morphological characteristics, such as presence of mitral regurgitation, mean valve thickness or elongation of chordae. Conclusions: The rates of psychiatric comorbidities and the psychosocial functioning impairment increase progressively along the spectrum of social anxiety. The fact that subthreshold SAD causes considerable disability and suffering in comparison with control subjects justifies a review of the validity of current diagnostic criteria. On the other hand, in this investigation no association between panic disorder or SAD and MVP was documented, regardless of the diagnostic criteria used, with prevalence rates similar to those reported for the general population. Thus, it seems necessary to demystify the relationship between this cardiac alteration and panic disorder and SAD in order to avoid unwanted influences for the patient and his psychiatric treatment.