ABSTRACT
Abstract Isolated left ventricular apical hypoplasia is a rare cardiomyopathy, with a broad range of clinical presentations. Since this entity was already described in association with osteomuscular diseases, mutation in the Lamin A/C gene has been regarded as a possible cause of this disease. This study describes the case of an asymptomatic teenager with isolated left ventricular apical hypoplasia and arthrogriposis but with no mutations in the entire Lamin A/C gene.
Subject(s)
Humans , Male , Adolescent , Lamin Type A/genetics , Isolated Noncompaction of the Ventricular Myocardium/physiopathology , Arthrogryposis , Lamin Type A/deficiency , Isolated Noncompaction of the Ventricular Myocardium/diagnosis , Isolated Noncompaction of the Ventricular Myocardium/etiologyABSTRACT
Abstract A rare case of congenital mitral insufficiency characterized by a fenestration in the anterior leaflet of mitral valve is reported. At operation, the mitral valve was successfully repaired by closure of unusual valvular tissue orifice with bovine pericardium and suture of the free edge between A1 and A2 without a ring annuloplasty.
Subject(s)
Humans , Infant, Newborn , Mitral Valve Annuloplasty , Heart Defects, Congenital , Mitral Valve Insufficiency/surgery , Mitral Valve Insufficiency/etiology , Mitral Valve Insufficiency/diagnostic imaging , Tricuspid Valve , Mitral Valve/surgery , Mitral Valve/diagnostic imagingABSTRACT
Relatamos o caso de um adolescente encaminhado com o diagnóstico de hipertensão pulmonar. A investigação não invasiva detectou comunicação interatrial seio venoso com sinais de hipertensão pulmonar. No estudo hemodinâmico o diagnóstico foi confirmado, sendo também notada compressão esquerda pelo tronco pulmonar. O paciente foi submetido à oclusão cirúrgica da comunicação interatrial e à plastia redutora do tronco pulmonar. Dois anos após o procedimento, o paciente encontra-se bem, com sinais clínicos e ecocardiográficos de hipertensão pulmonar discreta e sem evidências, também pelo ecocardiograma, de obstrução do tronco da artéria coronária esquerda.
We report the case of an adolescent referred with initial diagnosis of pulmonary hypertension. Non-invasive investigation disclosed a sinus venous atrial septal defect with pulmonary hypertension. The hemodynamic study confirmed diagnosis, and also showed extrinsic compression of left main coronary artery by pulmonary trunk. Surgical closure of the defect in addition to pulmonary trunk plasty were undertaken. Two years after the surgery the patient is well, with clinical signs of mild pulmonary hypertension, and showing no evidence - also on echocardiogram - of left coronary artery trunk obstruction.
Subject(s)
Humans , Male , Adolescent , Coronary Vessels/pathology , Heart Septal Defects, Atrial/diagnosis , Hypertension, Pulmonary/etiology , Pulmonary Artery/pathology , Coronary Vessels/surgery , Dilatation, Pathologic/complications , Dilatation, Pathologic/diagnosis , Dilatation, Pathologic/surgery , Echocardiography , Heart Septal Defects, Atrial/complications , Heart Septal Defects, Atrial/surgery , Hypertension, Pulmonary/diagnosis , Magnetic Resonance Angiography , Pulmonary Artery , Pulmonary Artery/surgery , Tomography, X-Ray Computed , Treatment OutcomeSubject(s)
Male , Female , Humans , Dyslipidemias , Heart Valve Diseases , Hypertension , Cardiovascular Diseases/diagnosis , Ventricular DysfunctionABSTRACT
Os acidentes escorpionicos no Brasil, assumem grande importancia nao so pela sua incidencia como pela sua potencialidade em induzir quadros clinicos graves, muitas vezes fatais, principalmente em criancas. Apresentamos neste relato os dados clinicos e laboratoriais de 4 pacientes vitimas de escorpionismo, causado pelo T. serrulatus, que evoluiram com falencia cardiaca e edema pulmonar, sendo que tres foram a obito dentro das primeiras 24 horas apos acidente...