What is heterotaxes?

Heterotaxes is a term used to describe two groups of congenital heart defects that generally include the loop of defects, dextrocardia . In addition to several anomalies in the heart, the liver of a person with heterotaxia is generally medium line, instead of placing on one side of the body. The most important indicator of heterotaxia is the presence of a number of small spleen, polysplenia or the overall absence of spleen, asplenia . Heterotaxes are divided into two types, left isomerism of the left atrium and true atrial isomerism , sometimes called Icemark's syndrome.

Left atrial isomerism is generally associated with polysplelia. This form of heterotaxia is less serious than ivemark, because these multiple small spleen tend to perform part of the work of regular spleen. There are often defects of ventricular septum and pulmonary stenosis, but these two conditions, if they are not serious, may usually be repaired in one surgery.

Isomerism is usually not necessary immediately after birth. Surgeons generally decide to repair heart defects during the first five years of life. Diagnosis of dextrocardy is usually made during routine sonograms, but before birth it is difficult to determine whether there are spleen or more spleen because they cannot be easily visualized. After birth, the blood test can determine the function of splenic to confirm the diagnosis of polysplenia associated with heterotaxes.

isterism of true atrial is one of the more difficult anomalies of the heart to treatment. This form of heterotaxia is characterized by more defects except asplenia. The most important defect is the complete defect of the atrioventricular channel . The septum between the chambers usually has a large hole, allowing to mix blood. In addition, the septum between the atrium has never created and the atriums are generally undersized.

Extreme lung stenosis and arterial transposition may also be present. Also lung veins candrain on the wrong side and prepare the heart of blood rich in oxygen. These multiple defects usually require surgery soon after birth and generally surgery must be performed in the stages, as with Fontan's procedure.

Surgery for repairing the right atrial isomerism is complicated asplenia. The lack of spleen means a much greater risk of postoperative infection. A child with this form of heterotaxia will require daily antibiotics for life, which can cause problems later.

If the isomerism of the right atrium requires staged operations that can alleviate but not to mix heart defects, then the child will probably have to undergo heart transplantation. The key to successful transplantation is the ability to fight infections. However, long -term use of antibiotics creates resistance, so available types of antibiotics after heart transplantation can be slim for a child with heterotaxes.

Despite the risks of surgery and the need for daily prophylactic andNtibiotics, contemporary surgical techniques improve the rate of survival of these children. Fortunately, heterotaxes are also one of the most precious forms of congenital heart defects, and the child is much more likely to have dextrocardy with an abnormal heart than to the right or left isomerism. Research suggests that, unlike most heart defects, heterotaxes may have a genetic cause. Scientists have found a gene in mice they believe that heterotaxia can be responsible. However, the same gene has not yet been found in humans.