Congenital Double-Orifice Mitral Valve with Mitral Regurgitation due to Flail Leaflet in an Elderly Patient

We report here on a case of double-orifice mitral valve with mitral regurgitation in a 75-year-old female who had complaints of mild dyspnea. Transthoracic and transesophageal echocardiography showed two orifices that were supplied by their own chordae from a different papillary muscle. Color Doppler echocardiography revealed moderate to severe mitral regurgitation due to the flail posterior leaflet of the anterolateral orifice. Except for the persistent left superior vena cava, no other congenital anomaly was demonstrated. The patient became asymptomatic with the administration of angiotensin-converting enzyme inhibitor and diuretics, and she has been scheduled for long term follow-up.

Structure of the human tricuspid valve leaflets and its chordae tendineae in unexpected death: a forensic autopsy study of 400 cases

Congenital variations are known to be potential candidates for mechanical trauma leading to tricuspid valve lesions. For this reason, a detailed examination of heart valves as well as chordae tendineae should carefully be performed to clarify the reason of sudden death with no apparent cause. The aim of this study is to investigate the relationship of valvachordal anatomy in tricuspid valve in sudden deaths, and mainly cardiac disease in connection with its structure as well as leading congenital changes. The 400 human hearts were collected between 2000 and 2002 from 400 autopsy cases during a medicolegal autopsy with permission from the Council of Forensic Medicine, Izmir, Turkey. Morphometric and morphological data were obtained in Ege University Faculty of Medicine, Department of Anatomy from each valve namely area, basal width, depth of leaflets, depth of commissure, number of chordae tendineae and their relation to the leaflets. These data were correlated for cardiac and noncardiac death cases. The results of this anatomical study may explain the increased incidence in wide variations of chordae tendineae in deaths of cardiac origin. In 40 hearts, we found 2 leaflets [20%], in 140 [70%] 3 leaflets and in 20 hearts there were 4 leaflets [10%] in deaths of noncardiac origin. We found 2 leaflets in 36 hearts [18%], 3 leaflets in 130 hearts [65%] and there were 4 leaflets in 34 hearts [17%] in deaths of cardiac origin. Although chordal abnormalities were extremely rare in cardiac death cases, some chordae tendineae retained a normal or near-normal appearance, while others were thickened and shortened in cardiac death cases. Higher ratio of abnormal chordae that were too short and too thick was also significant. This condition negatively affects the feeding of chordae and leaflets and acts as a culprit of cardiac deaths, since aging starts at an early stage. This situation changes the verdict in legal affairs. For this reason, the valvachordal structure should be carefully examined prospectively in autopsies

Estenose subaórtica por cordas tendíneas anômalas da valva mitral (mitral web) / Subaortic Stenosis Due To Anomalous Cordae Tendineae of Mitral Valve (Mitral Web)

Patient with subaortic stenosis due to the insertion of anomalous chordae tendineae of the mitral valve in the interventricular septum (mitral web) associated with mitral stenosis with diagnosis made by bidimensional echocardiographic study with Doppler and confirmed by hemodynamic study. The patient was submitted to surgical treatment. The anomalous cords were resected and the mitral valve with severe malformation was replaced by a bioprosthesis. The patient had a uneventful recovery and echocardiographic evaluation showed an outflow tract without obstruction, with no gradient, with remnants of the papillary muscles and normo-functioning bioprosthesis

Paciente portador de estenose subaórtica por cordas anômalas da valva mitral, inserindo no septo interventricular ("mitral Web") e estenose da valva mitral com diagnóstico feito através de estudo ecocardiográfico bidimensional com Doppler e confirmado pelo cateterismo cardíaco. O paciente foi submetido a tratamento cirúrgico, tendo sido encontrada dupla lesão mitral com predomínio de estenose, implantação de cordas anômalas da cúspide anterior da valva mitral no septo interventricular, obstruindo parcialmente a via de saída do ventrículo esquerdo. Foi realizada ressecção das cordas anômalas, aliviando a estenose subaórtica e, devido a má-formação da valva mitral, foi necessária a sua substituição por bioprótese. O paciente teve evolução pós-operatória sem intercorrências.O estudo ecocardiográfico pós-operatório mostrou resquício do aparelho subvalvar mitral e ausência de gradiente pressórico entre o ventrículo esquerdo e sua via de saída