Inversus totalis and Unique situs Ventricle Common Entrance with Pulmonary Stenosis

Situs inversus is a congenital anomaly characterized by the inverted position of the thoracic and abdominal organs with respect to the sagittal. More often than situs inversus is accompanied by dextrocardia; of these only 3-5% of cases associated with congenital heart disease. On this basis we can say that approximately 0.00025% (1: 400,000) of the general population has situs inversus with dextrocardia and associated congenital heart disease. Causal processes situs inversus totalis are still being studied, but there is already evidence that interference mechanisms and gene expression are responsible in most cases and recessive and autosomal dominant expressions X - linked.

The single ventricle is a congenital heart defect in which can not be distinguished in the ventricular mass more than a well - developed camera so this gets most of the atrioventricular connection, representing 1% of cases of congenital heart disease infants. Some authors warn in the cause of this condition is a fault in the signaling pathways that can be given in relation to the fourth to eighth week, crucial for heart development stage. It is believed that migration atrioventricular right channel, aligned to the right ventricle and the tricuspid valve facilitates the separation of the two ventricles. The alteration in this process originates type single ventricle anomalies.

 This condition almost always accompanied by abnormal relations in the position of the great arteries, dextrocardia, valvular stenosis or pulmonary subvalvular, subaortic stenosis, Total anomalous pulmonary venous connection or partial and coarctation of the aorta. The location of the large vessels sometimes remains but the blood flows in parallel into the aorta and lungs. This becomes a systemic urgency as venous and arterial saturation equals.

The dominant clinical heart failure is generally early onset often accompanied by shock, hypoplastic right heart and cyanosis is mild. Precociously they have difficulty breathing, hepatomegaly and pulmonary crackles, with marked tendency to respiratory processes.