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0 "multimedia" => array:7 [ "identificador" => "fig0015" "etiqueta" => "Figura 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 1563 "Ancho" => 2916 "Tamanyo" => 325999 ] ] "descripcion" => array:1 [ "pt" => "<p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Ecocardiograma transesofágico (plano 121°): morfologia da válvula mitral em diástole (à esquerda) e em sístole (à direita); observam‐se os folhetos posterior (seta A) e anterior (seta B) com boa abertura e destaca‐se a presença de uma estrutura ecogénica a nível da face auricular do anel mitral (seta verde).</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Inês Almeida, Francisca Caetano, Joana Trigo, Paula Mota, Maria do Carmo Cachulo, Manuel Antunes, António Leitão Marques" "autores" => array:7 [ 0 => array:2 [ "nombre" => "Inês" "apellidos" => "Almeida" ] 1 => array:2 [ "nombre" => "Francisca" 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] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "Inês Almeida, Francisca Caetano, Joana Trigo, Paula Mota, Maria do Carmo Cachulo, Manuel Antunes, António Leitão Marques" "autores" => array:7 [ 0 => array:4 [ "nombre" => "Inês" "apellidos" => "Almeida" "email" => array:1 [ 0 => "inesalm@gmail.com" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:3 [ "nombre" => "Francisca" "apellidos" => "Caetano" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 2 => array:3 [ "nombre" => "Joana" "apellidos" => "Trigo" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 3 => array:3 [ "nombre" => "Paula" "apellidos" => "Mota" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 4 => array:3 [ "nombre" => "Maria do Carmo" "apellidos" => "Cachulo" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 5 => array:3 [ "nombre" => "Manuel" "apellidos" => "Antunes" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 6 => array:3 [ "nombre" => "António Leitão" "apellidos" => "Marques" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Serviço de Cardiologia do Hospital Geral, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Serviço de Cirurgia Cardiotorácica, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "pt" => array:1 [ "titulo" => "Quando parece estenose mitral mas não é – implicações diagnósticas e terapêuticas" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 1667 "Ancho" => 3007 "Tamanyo" => 511174 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Transthoracic echocardiography: peak transmitral gradient of 41 mmHg and mean of 21 mmHg, and valve area of 1.8 cm<span class="elsevierStyleSup">2</span> estimated by the pressure half-time method (left); right ventricle/right atrium gradient of 117 mmHg (right).</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Rheumatic disease is still the most common cause of mitral stenosis, particularly in developing countries,<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> and congenital etiology is rare.</p><p id="par0010" class="elsevierStylePara elsevierViewall">Supravalvular mitral ring, also called supramitral ring or supramitral membrane,<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> is a rare form of congenital mitral stenosis. It is characterized by the presence of a ridge of connective tissue on the atrial side of the mitral valve, often attached to the valve annulus and/or leaflets,<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2,3</span></a> and may obstruct flow to the left atrium.</p><p id="par0015" class="elsevierStylePara elsevierViewall">Supravalvular mitral ring was first described by Fisher in 1902,<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> and fewer than 100 cases had been reported by 2002.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> The largest series to date included only 25 patients over a 20-year period,<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> and there are no data on its actual incidence or predisposition by gender or race.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1–3</span></a> In 90% of cases, it is associated with other congenital malformations, particularly Shone's complex, and/or mitral valve anomalies.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,2</span></a> Isolated occurrence, first described by Chung et al. in 1974,<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> is rare.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Case report</span><p id="par0020" class="elsevierStylePara elsevierViewall">A 53-year-old man had a history of bilateral vision loss due to juvenile glaucoma, intrinsic asthma, and suspected pulmonary sarcoidosis (under corticosteroid therapy). He had been previously followed in cardiology consultations for “a murmur since childhood”, and had been diagnosed with mild mitral stenosis based on a valve area of 1.8 cm<span class="elsevierStyleSup">2</span> estimated by the pressure half-time method.</p><p id="par0025" class="elsevierStylePara elsevierViewall">In 2012, he was admitted to the emergency department for worsening fatigue and dyspnea. On physical examination, he was dyspneic, with mild hypoxemia at rest (PO<span class="elsevierStyleInf">2</span> 60 mmHg), and hemodynamically stable. Cardiac auscultation revealed regular rhythm with a harsh mid-diastolic murmur, particularly audible over the apex, with presystolic accentuation, and pulmonary auscultation revealed rales in the left lung base and diffuse wheezing; there were no signs of right heart failure. Diagnostic exams included: electrocardiography showing sinus rhythm, heart rate (HR) of 80 bpm, and criteria for bi-atrial dilatation and partial right bundle branch block; laboratory tests showing elevated inflammatory markers; and chest X-ray showing increased cardiothoracic ratio and straightening of the left heart border, bilateral hilar enlargement, cephalization of the pulmonary vasculature and a reticulonodular/micronodular interstitial pattern, particularly in both lower pulmonary fields (similar to previous exams). The patient was admitted to the pneumology department with a diagnosis of respiratory infection.</p><p id="par0030" class="elsevierStylePara elsevierViewall">Antibiotic therapy was begun but the clinical setting persisted, and so transthoracic echocardiography (TTE) was performed, which, although limited by poor image quality, revealed no left ventricular (LV) dilatation and good global and segmental systolic function; mild left atrial (LA) dilatation (area in apical 4-chamber view: 22.8 cm<span class="elsevierStyleSup">2</span>); mitral valve leaflets that were difficult to visualize but with apparently normal opening (<a class="elsevierStyleCrossRef" href="#fig0005">Figure 1</a>); a convergence zone on the atrial side of the mitral annulus plane that was difficult to visualize due to enhanced echogenicity; transmitral flow with peak diastolic gradient of 41 mmHg and mean of 21 mmHg (HR 104 bpm); valve area of 1.8 cm<span class="elsevierStyleSup">2</span> estimated by the pressure half-time method, with no mitral regurgitation; right ventricle/right atrium (RV/RA) systolic gradient of 117 mmHg, excluding pulmonary stenosis (<a class="elsevierStyleCrossRef" href="#fig0010">Figure 2</a>); and right chambers of normal dimensions and contractility.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0035" class="elsevierStylePara elsevierViewall">Given the presence of severe pulmonary hypertension, thoracic computed tomography angiography was performed, which detected dilatation of the pulmonary artery trunk and its main branches and excluded pulmonary thromboembolism; calcification of the mitral annulus and aortic valve was observed.</p><p id="par0040" class="elsevierStylePara elsevierViewall">Following optimization of therapy and consequent hemodynamic improvement, repeat TTE (at a HR of 85 bpm) showed a reduction in mean LA/LV gradient to 11 mmHg and in RV/RA gradient to 56 mmHg.</p><p id="par0045" class="elsevierStylePara elsevierViewall">The discrepancy in the echocardiographic findings – a transvalvular gradient suggestive of severe obstruction of a valve with an estimated area >1.5 cm<span class="elsevierStyleSup">2</span> by the pressure half-time method and apparently normal leaflet opening – led to the hypothesis of supravalvular mitral ring or severe annular calcification.</p><p id="par0050" class="elsevierStylePara elsevierViewall">Transesophageal echocardiography (TEE), poorly tolerated by the patient and causing sinus tachycardia (HR 115 bpm), confirmed reasonable valve leaflet opening but showed an echogenic structure adjacent to the atrial side of the mitral annulus, distal to the insertion of the left atrial appendage (<a class="elsevierStyleCrossRef" href="#fig0015">Figure 3</a>), causing a high mean LA/LV diastolic gradient (39 mmHg). The effective orifice area estimated by the proximal isovelocity surface area method was 0.7 cm<span class="elsevierStyleSup">2</span>, supporting the hypothesis of supravalvular mitral ring (<a class="elsevierStyleCrossRef" href="#fig0020">Figure 4</a>). No other congenital anomalies were identified.</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><elsevierMultimedia ident="fig0020"></elsevierMultimedia><p id="par0055" class="elsevierStylePara elsevierViewall">Catheterization revealed severe pulmonary hypertension (mean pulmonary artery pressure: 46 mmHg), high pulmonary capillary wedge pressure (PCWP) (20 mmHg) and an LA/LV gradient (based on PCWP) of 18 mmHg. Coronary angiography showed no coronary artery disease.</p><p id="par0060" class="elsevierStylePara elsevierViewall">Given the presence of severe mitral valve obstruction associated with heart failure and pulmonary hypertension, the patient was referred for cardiac surgery. The surgical approach adopted was left atriotomy, which revealed a calcified structure attached to the mitral valve leaflets, consistent with supravalvular mitral ring. The ring was resected and since the valve could not be preserved, this was also excised, the posterior leaflet being preserved, and a mechanical prosthesis was implanted.</p><p id="par0065" class="elsevierStylePara elsevierViewall">A year later, the patient showed improved functional capacity and heart failure symptoms (NYHA class I); TTE revealed a normally functioning mitral prosthesis with a mean gradient of 3.5 mmHg and RV/RA gradient of 26 mmHg.</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Discussion</span><p id="par0070" class="elsevierStylePara elsevierViewall">Supravalvular mitral ring is a rare entity with few cases reported.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> It is usually associated with other congenital anomalies and is often diagnosed in childhood. The case presented is thus unusual in that it occurred in isolation and was detected in an adult patient, an even rarer combination that was first reported in 2008.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a></p><p id="par0075" class="elsevierStylePara elsevierViewall">The absence of other congenital malformations, the poor image quality, and concomitant chronic pulmonary disease (that could have explained the patient's clinical setting) may have delayed the diagnosis. Although supramitral ring is a congenital defect, turbulent flow is known to cause a progressive increase in the stiffness and/or dimensions of the membrane, resulting in more severe obstruction over the years, leading to later onset of symptoms.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p><p id="par0080" class="elsevierStylePara elsevierViewall">Diagnosis of supravalvular mitral ring is challenging. Although there is considerable variation in the composition of the ring and in its relationship to the mitral valve, it usually presents as a thin membrane <1 mm thick, attached to the anterior mitral valve leaflet,<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2,3</span></a> and is thus difficult to visualize.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a></p><p id="par0085" class="elsevierStylePara elsevierViewall">TTE leads to diagnosis in less than 50% of cases.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> In a study by Sullivan et al., the ring was visualized before surgery in only 45% of the patients; postoperative retrospective analysis of the echocardiograms yielded the diagnosis in 91%, but this required a detailed frame-by-frame examination, which is impractical in clinical practice.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> Despite this limitation, TTE is essential in raising suspicion of the diagnosis.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> In the case presented, the discrepancy between echocardiographic findings – turbulent flow causing a high transmitral gradient in a morphologically normal valve – alerted the authors to the need for further investigation.</p><p id="par0090" class="elsevierStylePara elsevierViewall">TEE is the best diagnostic exam for detection of supravalvular mitral ring,<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2,4</span></a> as it enables visualization of the ring and anchor points, assessment of native valve competence and differential diagnosis.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a></p><p id="par0095" class="elsevierStylePara elsevierViewall">Cardiac angiography, the preferred diagnostic technique when non-invasive exams were not generally available,<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a> is not now considered advantageous for diagnostic clarification.<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4,5</span></a> Cardiac magnetic resonance imaging has failed to detect supravalvular mitral ring in the cases in which it was used.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a></p><p id="par0100" class="elsevierStylePara elsevierViewall">In our patient, as in most reported cases, diagnostic confirmation was obtained only during surgery, although TEE had identified an anomalous structure adjacent to the mitral ring. Three-dimensional echocardiography may in the future be useful for assessing these patients, although there has been little mention of its use in the literature.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">10</span></a></p><p id="par0105" class="elsevierStylePara elsevierViewall">When assessing a patient with suspected supravalvular mitral ring, it is essential to consider differential diagnosis from the more common cor triatriatum, which results from a failure of embryological development of the left pulmonary vein,<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> leading to the left atrium being divided into two chambers by a membrane of fibromuscular tissue proximal to the LA appendage and well separated from the mitral valve. By contrast, supravalvular mitral ring arises from a failure of the endocardial cushions to divide completely,<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">11</span></a> and is distal to the LA appendage, this being the main distinguishing feature between the two diagnoses.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a></p><p id="par0110" class="elsevierStylePara elsevierViewall">With regard to treatment, the first surgical repair of supravalvular mitral ring was described by Lynch et al. in 1962, in a series of 14 patients.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> In 2006, Collison et al. confirmed excellent long-term results.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> Surgical repair of the mitral valve is not always possible due to the presence of adhesions and contiguity with the anterior leaflet, and so valve replacement may be necessary as well as excision of the ring,<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">10</span></a> as in the case presented. Factors determining a less favorable result are attachment of the membrane to the mitral valve, anomalies of the subvalvular apparatus, other congenital defects and pulmonary hypertension.<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">5,6</span></a></p><p id="par0115" class="elsevierStylePara elsevierViewall">Unlike other forms of mitral stenosis, in which balloon valvotomy is an acceptable alternative, surgery appears to be the only option for supravalvular stenosis.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> In a study by Spevak et al., of nine children with congenital mitral stenosis undergoing balloon angioplasty, less favorable results were obtained when the obstruction was not purely valvular, as it is with a supramitral ring.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">12</span></a></p><p id="par0120" class="elsevierStylePara elsevierViewall">Follow-up of these patients is essential, given the risk of recurrence, which was first described by Tulloh et al.<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">13</span></a> In a study of 23 children undergoing surgical resection of supravalvular mitral stenosis, four had recurrence of stenosis after 2–9 years. Although our patient has only been followed for a year, the outcome is satisfactory so far, with marked clinical improvement and significant reduction in the RV/RA gradient following removal of the obstruction.</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conclusions</span><p id="par0125" class="elsevierStylePara elsevierViewall">The case presented draws attention to a rare entity, for which the best method of diagnosis and treatment has yet to be established. A high level of clinical suspicion is necessary when performing TTE, and TEE is recommended whenever high transmitral gradients are observed. Resection of the ring, thus removing the obstruction, gives excellent results in the long term.</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Ethical disclosures</span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Protection of human and animal subjects</span><p id="par0130" class="elsevierStylePara elsevierViewall">The authors declare that no experiments were performed on humans or animals for this study.</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Confidentiality of data</span><p id="par0135" class="elsevierStylePara elsevierViewall">The authors declare that no patient data appear in this article.</p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">Right to privacy and informed consent</span><p id="par0140" class="elsevierStylePara elsevierViewall">The authors declare that no patient data appear in this article.</p></span></span><span id="sec0045" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0065">Conflicts of interest</span><p id="par0145" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:11 [ 0 => array:2 [ "identificador" => "xres381480" "titulo" => "Abstract" ] 1 => array:2 [ "identificador" => "xpalclavsec360291" "titulo" => "Keywords" ] 2 => array:2 [ "identificador" => "xres381481" "titulo" => "Resumo" ] 3 => array:2 [ "identificador" => "xpalclavsec360292" "titulo" => "Palavras-chave" ] 4 => array:2 [ "identificador" => "sec0005" "titulo" => "Introduction" ] 5 => array:2 [ "identificador" => "sec0010" "titulo" => "Case report" ] 6 => array:2 [ "identificador" => "sec0015" "titulo" => "Discussion" ] 7 => array:2 [ "identificador" => "sec0020" "titulo" => "Conclusions" ] 8 => array:3 [ "identificador" => "sec0025" "titulo" => "Ethical disclosures" "secciones" => array:3 [ 0 => array:2 [ "identificador" => "sec0030" "titulo" => "Protection of human and animal subjects" ] 1 => array:2 [ "identificador" => "sec0035" "titulo" => "Confidentiality of data" ] 2 => array:2 [ "identificador" => "sec0040" "titulo" => "Right to privacy and informed consent" ] ] ] 9 => array:2 [ "identificador" => "sec0045" "titulo" => "Conflicts of interest" ] 10 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2013-12-22" "fechaAceptado" => "2014-03-06" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec360291" "palabras" => array:5 [ 0 => "Supravalvular ring" 1 => "Supravalvular mitral ring" 2 => "Mitral stenosis" 3 => "Congenital mitral stenosis" 4 => "Supravalvular mitral stenosis" ] ] ] "pt" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palavras-chave" "identificador" => "xpalclavsec360292" "palabras" => array:5 [ 0 => "Anel supramitral" 1 => "Anel mitral supravalvular" 2 => "Estenose mitral" 3 => "Estenose mitral congénita" 4 => "Estenose mitral supravalvular" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:2 [ "titulo" => "Abstract" "resumen" => "<p id="spar0045" class="elsevierStyleSimplePara elsevierViewall">The authors report the case of a 53-year-old man, with a long-standing history of mild mitral stenosis, admitted for worsening fatigue. Transthoracic echocardiography (limited by poor image quality) showed mitral annular calcification, leaflets that were difficult to visualize and an estimated mitral valve area of 1.8 cm<span class="elsevierStyleSup">2</span> by the pressure half-time method. However, elevated mean transmitral and right ventricle/right atrium gradients were identified (39 and 117 mmHg, respectively). This puzzling discrepancy in the echocardiographic findings prompted investigation by transesophageal echocardiography, which revealed an echogenic structure adjacent to the mitral annulus, causing severe obstruction (effective orifice area 0.7 cm<span class="elsevierStyleSup">2</span>). The suspicion of supravalvular mitral ring was confirmed during surgery. Following ring resection and mitral valve replacement there was significant improvement in the patient's clinical condition and normalization of the left atrium/left ventricle gradient.</p><p id="spar0050" class="elsevierStyleSimplePara elsevierViewall">Supravalvular mitral ring is an unusual cause of congenital mitral stenosis, characterized by an abnormal ridge of connective tissue on the atrial side of the mitral valve, which often obstructs mitral valve inflow. Few cases have been reported, most of them in children with concomitant congenital abnormalities. Diagnosis of a supravalvular mitral ring is challenging, since it is very difficult to visualize in most diagnostic tests. It was the combination of clinical and various echocardiographic findings that led us to suspect this very rare condition, enabling appropriate treatment, with excellent long-term results.</p>" ] "pt" => array:2 [ "titulo" => "Resumo" "resumen" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Os autores reportam o caso de um doente de 53 anos, com diagnóstico prévio de estenose mitral ligeira, admitido por agravamento do cansaço. O ecocardiograma (Eco) transtorácico, limitado pela janela acústica, mostrava válvula mitral com ecogenicidade aumentada a nível do anel, deficiente observação dos folhetos e área valvular estimada de 1,8 cm<span class="elsevierStyleSup">2</span> por tempo de hemipressão. No entanto, a identificação de elevados gradientes transmitral médio e ventrículo direito/aurícula direita (atingindo respetivamente 39 e 117 mmHg) intrigou os autores. No Eco transesofágico foi observada estrutura hiperecogénica sobre o anel mitral a condicionar obstrução grave (área do orifício efetivo de 0,7 cm<span class="elsevierStyleSup">2</span>), o que levantou a suspeita de anel supramitral. Esta foi confirmada durante a cirurgia. Após a ressecção do anel e implantação de prótese mecânica, verificou-se uma franca melhoria clínica e normalização do gradiente aurícula esquerda (AE)/ventrículo esquerdo (VE).</p><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">O anel supramitral é uma causa invulgar de estenose mitral congénita, caracterizado pela presença de uma membrana fibrosa adjacente à face auricular da válvula mitral. Existem poucos casos reportados na literatura, sendo a maioria descritos em idade pediátrica e em associação a outras anomalias congénitas. O diagnóstico é desafiante, dado que o anel raramente é visualizado nos exames complementares. Uma elevada suspeita clínica e a integração dos vários achados ecocardiográficos são aspetos fundamentais para a sua identificação, permitindo o tratamento adequado com bons resultados a longo prazo.</p>" ] ] "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Almeida I, Caetano F, Trigo J, et al. Quando parece estenose mitral mas não é – implicações diagnósticas e terapêuticas. Rev Port Cardiol. 2014;33:471.</p>" ] ] "multimedia" => array:4 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1819 "Ancho" => 1590 "Tamanyo" => 164765 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Transthoracic echocardiography in parasternal long-axis view showing morphology of the mitral valve, with limited visualization of the leaflets due to poor image quality but with apparently normal opening.</p>" ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 1667 "Ancho" => 3007 "Tamanyo" => 511174 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Transthoracic echocardiography: peak transmitral gradient of 41 mmHg and mean of 21 mmHg, and valve area of 1.8 cm<span class="elsevierStyleSup">2</span> estimated by the pressure half-time method (left); right ventricle/right atrium gradient of 117 mmHg (right).</p>" ] ] 2 => array:7 [ "identificador" => "fig0015" "etiqueta" => "Figure 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 1569 "Ancho" => 2923 "Tamanyo" => 390495 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Transesophageal echocardiography (121°) showing morphology of the mitral valve in diastole (left) and systole (right); good opening of the posterior (arrow A) and anterior (arrow B) leaflets can be seen, together with an echogenic structure on the atrial side of the mitral annulus (green arrow).</p>" ] ] 3 => array:7 [ "identificador" => "fig0020" "etiqueta" => "Figure 4" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr4.jpeg" "Alto" => 1368 "Ancho" => 2923 "Tamanyo" => 414750 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">Transesophageal echocardiography (121°): a small convergence zone can be seen (poorly visualized on transthoracic echocardiography), suggesting supravalvular mitral stenosis; effective orifice area of 0.7 cm<span class="elsevierStyleSup">2</span> estimated by the proximal isovelocity surface area method (left); peak transmitral gradient of 54 mmHg and mean of 39 mmHg (right).</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:13 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "An analysis of 24 autopsied cases with supramitral rings" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "P. 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Year/Month | Html | Total | |
---|---|---|---|
2024 November | 7 | 3 | 10 |
2024 October | 59 | 28 | 87 |
2024 September | 65 | 27 | 92 |
2024 August | 66 | 39 | 105 |
2024 July | 55 | 34 | 89 |
2024 June | 63 | 23 | 86 |
2024 May | 68 | 20 | 88 |
2024 April | 46 | 24 | 70 |
2024 March | 76 | 20 | 96 |
2024 February | 60 | 34 | 94 |
2024 January | 58 | 28 | 86 |
2023 December | 53 | 31 | 84 |
2023 November | 72 | 24 | 96 |
2023 October | 69 | 20 | 89 |
2023 September | 51 | 20 | 71 |
2023 August | 43 | 17 | 60 |
2023 July | 102 | 9 | 111 |
2023 June | 60 | 16 | 76 |
2023 May | 86 | 27 | 113 |
2023 April | 80 | 4 | 84 |
2023 March | 102 | 26 | 128 |
2023 February | 89 | 18 | 107 |
2023 January | 69 | 20 | 89 |
2022 December | 73 | 19 | 92 |
2022 November | 107 | 34 | 141 |
2022 October | 97 | 28 | 125 |
2022 September | 82 | 31 | 113 |
2022 August | 84 | 36 | 120 |
2022 July | 91 | 44 | 135 |
2022 June | 77 | 22 | 99 |
2022 May | 70 | 34 | 104 |
2022 April | 99 | 23 | 122 |
2022 March | 75 | 32 | 107 |
2022 February | 87 | 33 | 120 |
2022 January | 101 | 32 | 133 |
2021 December | 64 | 26 | 90 |
2021 November | 87 | 36 | 123 |
2021 October | 131 | 41 | 172 |
2021 September | 79 | 35 | 114 |
2021 August | 68 | 37 | 105 |
2021 July | 52 | 35 | 87 |
2021 June | 41 | 23 | 64 |
2021 May | 56 | 33 | 89 |
2021 April | 78 | 37 | 115 |
2021 March | 73 | 19 | 92 |
2021 February | 83 | 17 | 100 |
2021 January | 80 | 14 | 94 |
2020 December | 29 | 11 | 40 |
2020 November | 88 | 12 | 100 |
2020 October | 77 | 10 | 87 |
2020 September | 79 | 20 | 99 |
2020 August | 43 | 3 | 46 |
2020 July | 79 | 9 | 88 |
2020 June | 36 | 6 | 42 |
2020 May | 64 | 2 | 66 |
2020 April | 63 | 21 | 84 |
2020 March | 61 | 7 | 68 |
2020 February | 170 | 37 | 207 |
2020 January | 38 | 5 | 43 |
2019 December | 41 | 6 | 47 |
2019 November | 41 | 4 | 45 |
2019 October | 36 | 10 | 46 |
2019 September | 93 | 7 | 100 |
2019 August | 31 | 4 | 35 |
2019 July | 60 | 18 | 78 |
2019 June | 26 | 14 | 40 |
2019 May | 72 | 9 | 81 |
2019 April | 45 | 18 | 63 |
2019 March | 111 | 9 | 120 |
2019 February | 130 | 12 | 142 |
2019 January | 111 | 7 | 118 |
2018 December | 61 | 15 | 76 |
2018 November | 121 | 6 | 127 |
2018 October | 265 | 21 | 286 |
2018 September | 97 | 15 | 112 |
2018 August | 89 | 9 | 98 |
2018 July | 55 | 6 | 61 |
2018 June | 65 | 8 | 73 |
2018 May | 78 | 10 | 88 |
2018 April | 79 | 6 | 85 |
2018 March | 97 | 12 | 109 |
2018 February | 57 | 10 | 67 |
2018 January | 87 | 14 | 101 |
2017 December | 155 | 7 | 162 |
2017 November | 111 | 16 | 127 |
2017 October | 74 | 13 | 87 |
2017 September | 96 | 13 | 109 |
2017 August | 142 | 24 | 166 |
2017 July | 84 | 14 | 98 |
2017 June | 142 | 14 | 156 |
2017 May | 249 | 13 | 262 |
2017 April | 89 | 14 | 103 |
2017 March | 215 | 22 | 237 |
2017 February | 191 | 7 | 198 |
2017 January | 81 | 7 | 88 |
2016 December | 85 | 16 | 101 |
2016 November | 116 | 5 | 121 |
2016 October | 93 | 8 | 101 |
2016 September | 208 | 8 | 216 |
2016 August | 58 | 1 | 59 |
2016 July | 42 | 2 | 44 |
2016 June | 24 | 4 | 28 |
2016 May | 24 | 3 | 27 |
2016 April | 56 | 2 | 58 |
2016 March | 132 | 17 | 149 |
2016 February | 95 | 27 | 122 |
2016 January | 65 | 22 | 87 |
2015 December | 80 | 7 | 87 |
2015 November | 87 | 11 | 98 |
2015 October | 87 | 13 | 100 |
2015 September | 86 | 20 | 106 |
2015 August | 71 | 13 | 84 |
2015 July | 65 | 9 | 74 |
2015 June | 37 | 4 | 41 |
2015 May | 58 | 12 | 70 |
2015 April | 59 | 17 | 76 |
2015 March | 46 | 10 | 56 |
2015 February | 67 | 8 | 75 |
2015 January | 66 | 16 | 82 |
2014 December | 57 | 12 | 69 |
2014 November | 56 | 14 | 70 |
2014 October | 113 | 27 | 140 |
2014 September | 74 | 30 | 104 |