Abstract Objective: To compare the outcome of infants and children who have right atrial isomerism and normal pulmonary venous drainage with those who have anomalous drainage, and to determine factors associated with poor outcome. Design and patients: Retrospective review of management and outcome of infants and children determined to have right atrial isomerism between January and December Setting: Tertiary paediatric cardiac centre. Results: The patients presented at a median of one day range 1 day to 3. The mean SEM survival estimates for those with normal pulmonary venous drainage at 1, 5, 10, and 15 years was 81 5.

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Contact Us Online What is heterotaxy syndrome isomerism? Heterotaxy syndrome is a rare birth defect that involves the heart and other organs. All usually involve heart defects of varying types and severity. In addition, organs such as the stomach, intestines, liver and lungs may be in abnormal places in the chest and abdomen. The intestines may have malrotation, which is when the loops of bowel are lined up incorrectly. With this problem the bowel can twist on itself volvulus.

Many children with malrotation need abdominal surgery to correct it. Some children with heterotaxy syndrome can have a very serious condition of the liver called biliary atresia. This also may require surgical intervention. There may also be irregularities with the skeleton, central nervous system and urinary tract.

In some cases of heterotaxy syndrome, the spleen may not work correctly or may be missing entirely. This can cause many problems, because the spleen helps the body fight infections. In some cases, there may be a functioning spleen, but it may be divided into several smaller spleens polysplenia.

This means the heart is in the right chest instead of the left chest. Here are general descriptions of two types of heterotaxy syndrome. The morphology structure of the heart varies from child to child. Types of heterotaxy syndrome include: Right atrial isomerism: Children with this condition have multiple heart defects. They may have septal defects holes between the tissue dividing the two sides of the heart and problems with heart valves, particularly the pulmonary valve.

They may also have abnormalities of the blood returning from the lungs to the heart anomalous pulmonary venous connection. The spleen may be absent asplenia , and the liver and other organs may be on the wrong side of the body. Some children with this problem have complete heart block , which is when the upper-chamber electrical system does not communicate with the lower-chamber electrical system.

Most children require pacemakers for this problem. The spleen may be absent, or there may be several small spleens polysplenia , instead of one spleen. In nearly all cases of right atrial isomerism and some cases of left atrial isomerism, symptoms will appear at birth or a few days after because the heart defects are severe.

Testing and diagnosis of isomerism In many cases, heterotaxy syndrome is diagnosed before birth. CHOP has its own delivery unit on the same floor as cardiac operating rooms and cardiac patient care units. Newborns can be in the care of pediatric cardiologists and specially trained cardiac nurses immediately after birth.

Other diagnostic tests on other areas of the body will also be required, including renal ultrasound and abdominal ultrasound. Throughout diagnosis and treatment, your Cardiac Center team will work with specialists, such as urologists, gastroenterologists and others, to provide the best care for this complex condition. Treatment for heterotaxy syndrome Treatment for heterotaxy syndrome varies depending on the severity of the heart defects and other organ abnormalities.

Most patients with left atrial isomerism will require surgical procedures or catheterization to repair holes in the heart or other problems. Some patients will require implantable pacemakers or defibrillators to control abnormal heart rhythm.

Nearly all patients with right atrial isomerism, and some patients with left atrial isomerism, will require a series of major heart surgeries. Surgeons will reconfigure the heart and circulatory system so that the heart functions with one ventricle pumping chamber , instead of two. This is called Fontan circulation and requires three open heart surgeries, called staged reconstruction. The procedures include the Norwood procedure, hemi-Fontan or Glenn operation, and the Fontan procedure.

Follow-up care Between the Norwood and Glenn operations Though early outcomes for patients with single ventricle heart defects after staged reconstruction have improved dramatically, the period between the Norwood procedure and the Glenn operation remains a very vulnerable time for infants. CHOP created the Infant Single Ventricle Monitoring Program to focus on the care and monitoring of infants with single ventricle heart defects between the first and second reconstructive surgeries.

Through age 18 Patients with Fontan circulation are referred to as single ventricle patients. As these patients get older, doctors are recognizing that, while some do fine, many experience complications, including lung, liver and gastrointestinal diseases.

In addition, as a group, children with complex congenital heart defects who have had open heart surgery as infants are at a higher risk for neurodevelopmental issues when compared to children without congenital heart defects.

All children with heterotaxy syndrome will require lifelong care by a cardiologist. In many cases, children with this condition will have many needs and will require care from different teams at CHOP for many years. For instance, in patients who do not have a spleen, an infection can quickly become life-threatening. If the child develops a fever one sign of infection , the family must take the child to a hospital quickly, as IV antibiotics may be required.

Your team at the Cardiac Center will make sure you understand the steps you need to take to help keep your child safe. Into adulthood We will help older patients with heterotaxy syndrome transition to an adult cardiologist.


Clinical implications of atrial isomerism.

Click on the image to see a larger version. Selected References These references are in PubMed. This may not be the complete list of references from this article. Isomerism of the cardiac atria in the asplenia syndrome. Lab Invest.


Situs ambiguus

Acute symptoms can be due to both cardiac and non-cardiac defects. Cyanosis or blue skin coloration, primarily affecting the lips and fingernails, can indicate a systemic or circulatory issue. Poor feeding, failure to thrive , and rapid shallow breathing may also be observed due to poor circulation. Upon examination, arrhythmia and heart murmur may raise further suspicion of a cardiac abnormality.


Heterotaxy Syndrome (Isomerism)


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