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BACKGROUND In cardiac amyloidosis (CA), misfolded proteins deposit in the extracellular space of cardiac tissue. These deposits classically cause restrictive cardiomyopathy with diastolic dysfunction. Although there are at least 30 proteins known to cause amyloid aggregates, 2 main types make up most diagnosed cases: light chain amyloidosis (AL) and transthyretin amyloidosis (ATTR). Since CA is considered a rare condition, it is often underdiagnosed or recognized in the advanced stages. Once amyloid deposits involve the heart tissue, they are associated with a worse outcome and higher mortality rates, especially in patients presenting symptoms of heart failure. CASE REPORT We report a case of a 22-year-old man presenting with acute severe mitral regurgitation, secondary to posterior mitral leaflet chordae tendineae rupture (CTR). Surgical mitral valve replacement with a mechanical prosthesis was performed, and cardiac tissue biopsy samples were obtained. After surgery, the patient improved significantly but suddenly presented with hemodynamic deterioration, until he died due to severe hemodynamic compromise and multiorgan failure. Although the etiology of the CTR was not established before surgical intervention, the histopathological analysis suggested CA. CONCLUSIONS CA diagnosis can be complex, especially in a 22-year-old-man with atypical clinical and imaging manifestations. In this patient, other differential diagnoses were considered, since CA presenting in a young patient is a rare phenomenon and acute mitral regurgitation secondary to CTR presents more frequently in other heart conditions. Furthermore, rapid postoperative deterioration resulted in the patient's death before biopsy samples were available because suspicion of amyloidosis had not been raised until that point.

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El síndrome bronquial obstructivo recurrente o sibilante recurrente constituye uno de los motivos de consulta más frecuentes en pediatría. Entre los diagnósticos etiológicos diferenciales, se encuentran las cardiopatías congénitas. En esta presentación, se describe el caso de un niño de 4 años derivado a nuestra Institución para valoración cardiológica por presentar sibilancias recurrentes. Se trató de un caso de estenosis mitral congénita grave secundaria a arcada mitral. La arcada mitral es una cardiopatía congénita infrecuente. Constituye una variante de obstrucción al tracto de entrada del ventrículo izquierdo, que afecta el aparato subvalvular mitral y genera hipertensión pulmonar retrograda poscapilar y edema intersticial. El diagnóstico se realizó mediante la evaluación clínica, electrocardiográfica, radiológica y, fundamentalmente, ecocardiográfica. Se efectuó una corrección quirúrgica exitosa con remoción completa de la obstrucción mitral.

Recurrent wheezing is a very common clinical ailment throughout infancy and childhood. The most common diagnosis in children with wheezing is asthma. However, some other causes should be considered in the differential diagnosis such as a congenital cardiac defect. We present a case of a four year old boy presenting with recurrent wheezing who was referred to our institution for cardiac evaluation. Severe mitral stenosis secondary to an anomalous mitral arcade was diagnosed by physical examination, chest X-ray, electrocardiogram and mainly transthoracic and transesophageal echocardiography. Anomalous mitral arcade is a rare congenital malformation of the mitral tensor apparatus which comprises the chordae tendineae and papillary muscles. This abnormal anatomy leads to increased filling pressure of the left ventricle, a retrograde post capillary pulmonary hypertension and interstitial aedema. The patient was referred to cardiac surgery and underwent a successful procedure with complete removal of the obstructive mitral lesion.

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Recurrent wheezing is a very common clinical ailment throughout infancy and childhood. The most common diagnosis in children with wheezing is asthma. However, some other causes should be considered in the differential diagnosis such as a congenital cardiac defect. We present a case of a four year old boy presenting with recurrent wheezing who was referred to our institution for cardiac evaluation. Severe mitral stenosis secondary to an anomalous mitral arcade was diagnosed by physical examination, chest X-ray, electrocardiogram and mainly transthoracic and transesophageal echocardiography. Anomalous mitral arcade is a rare congenital malformation of the mitral tensor apparatus which comprises the chordae tendineae and papillary muscles. This abnormal anatomy leads to increased filling pressure of the left ventricle, a retrograde post capillary pulmonary hypertension and interstitial aedema. The patient was referred to cardiac surgery and underwent a successful procedure with complete removal of the obstructive mitral lesion.

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OBJECTIVE: Echocardiography is a valuable noninvasive technique for identifying the site and type of obstruction in the left ventricular outflow tract. Knowledge of the morphological details of each type of obstruction is the basis for correct interpretation of the diagnostic images and clinical decisions. METHODS: This study was undertaken to provide improved understanding of the anatomy of left ventricular outflow obstruction with corresponding side-by-side echocardiographic images. Specimens were part of the collection of the Department of Embryology of the Instituto Nacional de Cardiología "Ignacio Chávez," Mexico City, Mexico. Twenty-eight patients were studied by echocardiography, and 22 specimens with different types of left ventricular outflow tract obstruction were analyzed. RESULTS: Echocardiographic characteristics: Eighteen (64.3%) patients were women and 10 (35.7%) men. Three had fibrous rings, 19 with obstructive asymmetric septal hypertrophic cardiomyopathy, 1 with posterior displacement of the infundibular septum, 2 with accessory mitral valve tissue, 2 with anomalous insertion of mitral valve chordae tendineae, and 1 with malposition of the mitral valve and papillary muscles. Anatomic characteristics: Twelve had fibrous ring, 2 asymmetric septal hypertrophic cardiomyopathy, 2 posterior displacement of the infundibular septum, 1 ectopic mitral tissue, 1 anomalous insertion of mitral valve chordae tendineae in the ventricular septum, 3 malposition of the mitral valve and papillary muscles, and 1 subaortic infundibulum. CONCLUSION: The anatomoechocardiographic correlation clearly demonstrates the mechanism of subaortic obstruction. The comparison with corresponding echocardiographic images of different subaortic obstruction types is potentially quite valuable in enhancing the echocardiographer's understanding especially of complex subaortic obstruction.

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A new technique is proposed for mitral incompetence due to elongated chordae tendineae of the anterior leaflet. It consists in shortening the chordae tendineae of the leaflet level. First, we make a longitudinal orifice in the anterior leaflet just near its free edge and the elongated chordae tendineae. Through the orifice and with the help of a micro nerve hook we pull the elongated chordae the necessary to keep the anterior leaflet in the same level of the posterior leaflet. The orifice is then closed with a polipropilene 5-0 suture. The mitral annuloplasty was performed with a Gregori IMC prosthetic ring. The excellent clinical and laboratorial data suggest that mitral insufficiency due to elongated chordae tendineae can be corrected by this technique.

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Different anatomo-clinic aspects from three mitral valve prolapse cases are compared to those commonly presented in the literature and are also utilized as a basis for a new classification of this disease. The patients are more than 77 years old, what is in contrast with the current concept of MVP as a disease of young-middle aged women. The first case shows marked ostial dilation and many ruptured chordae: as a consequence, this patient showed severe cardiac dysfunction. The anterior, rather than the posterior leaflet, presented intense myxoid degeneration. In the second case, no ruptured chordae were detected and, consequently, the degree of heart failure was lesser than the first one, in spite of the same degree of ostial dilation. Both leaflets showed the same degree of myxoid degeneration. The third patient, who does not have heart failure, showed myxomatous degeneration of both cusps, but no ostial dilation or chordal rupture were present. These aspects reinforce the impression that isolated mixomatous degeneration of the cusps is not so deleterious when compared to those cases where the mitral valvar ring is dilated or its chordae are also involved by that degenerative process. Therefore ostial dimension (normal or enlarged) and the state of the chordae (with or without rupture) appears to be important points to be considered in MVP.

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Säo apresentadas as características anátomo-clínicas de três casos de Prolapso da Valva Mitral (PVM) detectados em pacientes idosos e do sexo masculino, o que contrasta em termos da sua conhecida prevalência entre mulheres jovens. Do ponto de vista anátomo-patológico os três casos ilustram duas importantes características anatômicas que o PVM pode apresentar: dimensäo do óstio e estado da cordoalha. O 1§ caso exibe grande dilataçäo ostial, preferencial acometimento da cúspide anterior e acentuada ruptura de cordas, de que resultou grave quadro congestivo, o 2§ caso mostra grande dilataçäo ostial, sem lesäo significativa de cordoalha, sendo menor, o grau do defeito valvar; no 3§ caso, o processo degenerativo mixomatoso, tal como nos casos anteriores, acomete ambas as cúspides, mais acentuado na posteror, sendo normal o diâmetro do óstio e íntegras as cordas, embora nitidamente alongadas, observando-se adequada competência valvar. Conclui-se que a dilataçäo do anel fibroso valvar e a ruptura da cordoalha säo mais deletérios que o processo degenerativo isolado

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