Dextrocardia with Situs Inversus Dextrocardia with Situs Inversus
"I am shocked not so much by the fact that the Lord prefers the left hand as by the fact that he still appears to be left-right symmetric when he expresses himself strongly." - Wolfgang Pauli, 1957
Introduction This page has been compiled from information collected from various sources on the Internet about the extremely rare disorder Dextrocardia with Situs Inversus . My Sister recently gave birth to a beautiful baby girl named Bethany. Bethany was diagnosed with DSI 18 weeks into the pregnancy during a routine ultrasound.
While chatting with my sister's husband about the Internet and what a great research tool (amongst other things) it is, he asked if I could find out more detailed information about the condition as what they'd been told from the doctors had been very scant to say the least.
The information I have compiled so far isn't very much at all, but my aim in creating this page is to collect as much information as possible from others who may have this condition, and be able to provide more information to other people in the community who may also have a child diagnosed with this DSI.
I have recently added a forum section so visitors to this site may discuss personal experiences they may have on such things as how they, or others have been effected by the condition or information they've been given by their doctor which could be of interest to others. A link to the forum can be found at the bottom of this page.
Michael Wilson
Diagnosis
Dextrocardia with Situs Inversus is a rare heart condition characterised by abnormal positioning of the heart. In this condition, the tip of the heart (apex) is positioned on the right side of the chest. Additionally, the position of the heart chambers as well as the visceral organs such as the liver and spleen is reversed (situs inversus). However, most affected individuals can live a normal life without associated symptoms or disability.
Dextrocardia with Situs Inversus is characterised by the reversal of the normal position of the heart chambers and abdominal organs such as the liver and spleen. The electrocardiogram shows an inversion of the electrical waves from the heart. The apex of the heart is positioned on the right side of the chest whereas it is normally located on the left.
Symptoms
Dextrocardia is an anomaly in which the primitive heart tube folds to the left in a mirror image of a normal bulboventricular loop. This usually occurs when all the organ systems are reversed, a condition called situs inversus.
Dextrocardia with a normal abdominal situs has a high incident of association with congenital heart disease although dextrocardia with reversed abdominal situs, i.e. situs inversus, has a much lower incidence.
There is complete situs inversus. The liver is only mildly cirrhotic in configuration with slight prominence of the caudate and left hepatic lobes. No focal hepatic lesions are seen. The spleen is moderately enlarged. There is extensive diverticulosis of the colon.
The patient's anatomical arrangement is reversed resulting in a mirror image of normal chest and abdominal anatomy. The cardiac apex points to the right (dextrocarida). The aortic arch and descending throacic aorta are on the right. The stomach is right-sided and the liver is left-sided. There is a single spleen on the right.
Normal anatomical positioning is termed situs solitus. The cardiac apex, aortic arch, and stomach are all normally on the left. In addition, the anatomic left atrium is on the same side as the hyparterial bronchus and bilobed lung; the anatomic right atrium is on the same side as the eparterial bronchus and trilobed lung. With normal situs and levocardia, the incidence of congenital heart disease is less than 1%; with dextrocardia, the incidence rises to 95%.
As shown above, situs inversus is a mirror image of normal anatomy. In situs inversus totalis, the cardiac apex, stomach and aortic arch are all on the right. Situs inversus totalis is associated with a 5-10% incidence of congenital heart disease. Situs inversus with levocardia (right-sided stomach and left-sided heart) is associated with congenital heart disease in 95% of cases, but is very rare.
Causes
Dextrocardia with Situs Inversus, a rare condition that is present at birth, is transmitted by autosomal recessive genes. The primitive loop in the embryo moves into the reverse direction of its normal position during fetal development, causing displacement of organs. Human traits including the classic genetic disorders are the product of the interaction of two genes for that condition, one received from the father and one from the mother. In recessive disorders, the condition does not appear unless a person inherits the same defective gene for the same trait from each parent. If an individual receives one normal gene and one gene for the disease, the person will be a carrier for the disease, but usually will not show symptoms.
The risk of transmitting the disease to the children of a couple, both of whom are carriers for a recessive disorder, is 25 percent. Fifty percent of their children risk being carriers of the disease, but generally will not show symptoms of the disorder. Twenty-five percent of their children may receive both normal genes, one from each parent, and will be genetically normal (for that particular trait). The risk is the same for each pregnancy.
Affected Population
Dextrocardia with Situs Inversus is present at birth. The condition affects males and females in equal numbers. Approximately %.01 of infants are born with this disorder.
Related Disorders
Dextroversion of the heart means that the location of the heart is abnormally positioned in the right half of the chest. The left ventricle remains on the left, but lies in front of the right ventricle. The heart is rotated to the right. The waves representing the heart beat on the electrocardiogram will indicate an abnormality.
Dextroposition of the heart is a displacement of the heart to the right. It is usually caused by acquired disease of the lungs, the membrane around the lungs (pleura), or diaphragm. The electrocardiogram is usually normal.
Kartagener Syndrome is a combination of Dextrocardia with chronic dilatation of the bronchi, difficulty breathing, recurrent respiratory infection (bronchiectasis) and infection of the sinuses (sinusitis). Clubbed fingers and bluish discoloration of the skin (cyanosis) may also be present.
Kartagener's syndrome may also be seen in connection with situs inversus. Kartagener's syndrome represents a triad of situs inversus (seen in 50% of cases), sinusitis, and bronchiectasis. The latter two parts of the triad are due to immotile or dysmotile cilia affecting the respiratory epithelium, auditory epithelium, and the sperm in males; therefore, hearing difficulties and infertility may also be found in this syndrome.Two other situs abnormalities are worth noting. Situs ambiguous with bilateral "right-sidedness" is known as Asplenia syndrome. In this syndrome, the spleen is absent, both lungs have three lobes, and the left and right lobes of the liver are similar. The cardiac apex may be in either hemithorax. Complex cardiac anomalies are usually found in this syndrome.
Situs ambiguous with bilateral "left-sidedness" is called Polysplenia syndrome. The lungs each have two lobes, multiple small spleens are present, and the cardiac apex may be in either hemithorax. The cardiac anomalies associated with polysplenia syndrome are less complex giving this syndrome a better prognosis than asplenia syndrome.
Standard Therapies
Treatment of Dextrocardia with Situs Inversus is symptomatic and supportive when needed. In most cases, affected individuals can live a normal life without any symptoms or discomfort. If the condition is associated with other more serious heart malformations, the prognosis and treatment will vary. Genetic counselling may be helpful for affected families.
Forum
Would you like to chat with other people about SID?? I have created a forum so you can ask questions, and generally discuss issues, experiences you may have with others. To enter the forum, just click here or below
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The Author of this page is not medically certified, and the information in these pages has been gathered from various Internet medical libraries. Michael Wilson Consulting will not be held responsible for the accuracy of this content, and the information in this article should be not be used in the diagnosis or treatment of any personnel, but used for information purposed only.
