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Dextrocardia with Situs Inversus is a rare heart condition characterized 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.
Electrocardiography reveals an inversion of the electrical waves from the heart and is the diagnostic measure of choice.
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.
Dextrocardia with Situs Inversus is present at birth. The condition affects males and females in equal numbers.
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.
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 counseling may be helpful for affected families.
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Nakagawa T, et al., Tranesophageal echocardiography combined with magnetic resonance imaging for detecting venous anomolies in dextrocardia. A case report. Angiology. 1995;46:531-35.
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Kronzon I; Tunick PA; Rosenzweig BP. "Quantification of left-to-right shunt in patent ductus arteriosus with the PISA method". Journal of the American Society of Echocardiography. 2002; 15: 376
Report last updated: 2007/08/08 00:00:00 GMT+0