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.
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.
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.
For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: [email protected]
For information about clinical trials sponsored by private sources, contact:
Friedman, WF. Congenital heart disease in infancy and childhood. In: Braunwald E, ed. Heart Disease. 3rd ed. Philadelphia, Pa., W. B. Saunders Co; 1988:963.
McAleer E; Kort S; Rosenzweig BP; Katz ES; Tunick PA; Phoon CK; Kronzon I. “Unusual echocardiographic views of bicuspid and tricuspid pulmonic valves”. Journal of the American Society of Echocardiography. 2001; 14: 1036
Friedman, WF, Child JS. Congenital heart disease in the adult. In: Fauci AS et al; eds. Harrison’s Principles of Internal Medicine. 14th ed. New York, NY; McGraw-Hill Companies, Inc., 1998:1308
Martines-Lopez, JI., ECG of the month. Turned about. Dextrocardia. J La State Med Soc. 1999;151:347-49.
Dania PG, et al., Is this right? (…or is it left?). Circulation.1999;100:209-10.
Shah RP, et al., Coronary arteriography in the presence of dextrocardia and situs inversus. Ann Acad Med Singapore. 1996;25:759-60.
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.
Ghalchi M; Rosenzweig BP; Colvin SB; Tunick PA; Kronzon I. “Rare flow pattern in a patient with cor triatriatum”. Echocardiography. 2005; 22: 705
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
The information in NORD’s Rare Disease Database is for educational purposes only and is not intended to replace the advice of a physician or other qualified medical professional.
The content of the website and databases of the National Organization for Rare Disorders (NORD) is copyrighted and may not be reproduced, copied, downloaded or disseminated, in any way, for any commercial or public purpose, without prior written authorization and approval from NORD. Individuals may print one hard copy of an individual disease for personal use, provided that content is unmodified and includes NORD’s copyright.
National Organization for Rare Disorders (NORD)
55 Kenosia Ave., Danbury CT 06810 • (203)744-0100