Isolated Left Ventricular Apical Hypoplasia without Lamin A/C Gene Mutation

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.

Mitral Valve Fenestration as a Rare Cause of Congenital Mitral Insufficiency Successfully Repaired

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.

Mitral Valve Fenestration as a Rare Cause of Congenital Mitral Insufficiency Successfully Repaired.

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.

Outcomes of Patients After Arterial Switch Operation: 18 Years of Experience in a Single Medium-Volume Center.

The objective of this paper was to describe the outcomes in patients submitted to arterial switch operation and to analyze the predictors of in-hospital mortality and further need of re-operation at a single-center institution. Between September 1995 and January 2014, 128 consecutive arterial switch operations were performed. Surgical mortality during this period was analyzed retrospectively, and a follow-up analysis of the survivors was conducted. Surgical era, cardiopulmonary bypass time (p = 0.001), and diagnosis category (p = 0.025) influenced in-hospital mortality. The estimated overall survival for the 91 hospital survivors was 96.8, 96.4, and 96.2 % at 5, 10, and 15 years, respectively. The median follow-up time was 67 months (range 0.71-222 months). Three patients (5 %) presented severe aortic regurgitation. Right ventricle outflow tract systolic gradient by echocardiography was above 60 mmHg in 2 %. Late re-interventions occurred in 12 (13 %) patients with mean time of 64 ± 34 months after the initial procedure. Actuarial freedom from re-interventions at 5, 10, and 15 years was 96.4, 69.7, and 61.9 %, respectively. Arterial switch operation remains the procedure of choice in patients with transposition of great arteries. It can be performed even in middle-volume institutions, leading to the same middle- and long-term outcomes of high-volume institutions. Early high mortality rate may occur due not only to learning curve, but also to cardiopulmonary bypass time and ventricular septal defect closure.

Extrinsic compression of left main coronary artery from aneurysmal dilation of pulmonary trunk in a adolescent: involution after surgery occlusion of sinus venosus atrial septal defect and pulmonary trunk plasty for reduction.

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.

Compressão extrínseca da artéria coronária esquerda por dilatação aneurismática do tronco pulmonar em adolescente: involução após oclusão cirúrgica de comunicação interatrial seio venoso e plastia redutora do tronco pulmonar / Extrinsic compression of left main coronary artery from aneurysmal dilation of pulmonary trunk in a adolescent: involution after surgery occlusion of sinus venosus atrial septal defect and pulmonary trunk plasty for reduction

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.

Double-chambered right ventricle in an adult patient diagnosed by transthoracic echocardiography.

BACKGROUND: Double-chambered right ventricle is a rare congenital disease frequently misdiagnosed in the adult patient. An anomalous muscle band divides the right ventricle in two cavities causing variable degree of obstruction. Although echocardiography is considered a useful method for the diagnosis of this pathology in children, it has been recognized the transthoracic scanning limitation in adults. CASE PRESENTATION: A 29 year-old patient with double-chambered right ventricle presenting mild exercise intolerance referred for follow up of a known ventricular septal defect in whom a complete diagnosis was obtained based only on transthoracic two dimensional echocardiography without the needing of cardiac catheterization. CONCLUSION: Based on non invasive echocardiographic diagnosis, patient was referred to surgical correction, which was completely successful.

Severe scorpion envenomation in Brazil: clinical laboratory and anatomopathological aspects

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...