[Uhl's anomaly. A case report with anatomoechocardiographic correlation]. / Anomalía de Uhl. Informe de un caso con correlación anátomo-ecocardiográfica.

UNLABELLED: Uhl's anomaly is an uncommon malformation of the right ventricle, which is characterized by the absence of the myocardium in the right ventricular free wall, while the tricuspid and pulmonary valves are morphologically normal. The aim of this work was to present a clinical case of a patient with Uhl's anomaly, to describe the echocardiographic findings and to perform an anatomoechocardiographic correlation with a corresponding specimen. This is a case report of a 33 year old man with suspicion of atrial septal defect, who was in functional class II of the NYHA and presented signs of right ventricular failure. The echocardiogram showed dilation of the right cavities, the free wall of the right ventricle was thin and smooth, and the tricuspid and pulmonary valves were normal, which prompted the diagnosis of Uhl's anomaly. Also, an anatomoechocardiographic correlation was done with an equivalent specimen of the Embryology Department from the Instituto Nacional de Cardiologia Ignacio Chavez's collection. CONCLUSIONS: Uhl's anomaly is an extremely rare entity. The clinical findings and echocardiography are the main tools for its diagnosis. The anatomoechocardiographic correlation was precise and allowed a better understanding of this anomaly."

[Congenital mitral valve anomalies in adults. Clinical and echocardiographic study]. / Anomalías congénitas de la válvula mitral en adultos. Estudio clínico- cocardiográfico.

INTRODUCTION: Congenital anomalies of the mitral valve (CAMV) comprise a wide range of leaflet anomalies and of the subvalvular apparatus. Its presentation in adults is not frequent. OBJECTIVE: In this work, we assessed the clinical and echocardiographic aspects as well as the treatment of five adult patients with CAMV. METHODS: A complete clinical history was made for each patient. All patients were subjected to an electrocardiogram, Chest X-rays, and an echocardiogram. RESULTS: Two patients were in functional class (FC) I, one in FC II, and two in FC III of the New York Heart Association. Diagnoses were: 1) prolapsed mitral valve with severe mitral failure, 2) parachute mitral valve associated with a subvalvular aortic fibrous ring and patent ductus arteriosus, 3) tri-leaflet mitral valve with subaortic obstruction, 4) double mitral orifice associated with bicuspid aorta and aortic coarctation, and 5) tunnel-forming mitral valve associated with ostium primum interatrial communication and pulmonary arterial hypertension and pulmonary artery hypertension. One patient was subjected to mitral valve change, one to dilation of the aortic coarctation, and another is under sitaxentan treatment, with improvement in the FC. The two remaining patients are awaiting surgery. CONCLUSIONS: Review of these cases with CAMV reveals the relevance of the echocardiogram in the evaluation of the mitral valve and of the subvalvular apparatus, because it allows for the identification of different types of malformations and their hemodynamic repercussion, to be able to propose the precise and timely treatment for these patients.