Dextrocardia with Situs Inversus

Dextrocardia with Situs Inversus

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Dextrocardia with Situs Inversus is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • Mirror-Image Dextrocardia

Disorder Subdivisions

  • None

General Discussion

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.

Symptoms

Electrocardiography reveals an inversion of the electrical waves from the heart and is the diagnostic measure of choice.

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 Populations

Dextrocardia with Situs Inversus is present at birth. The condition affects males and females in equal numbers.

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 counseling may be helpful for affected families.

Investigational Therapies

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: prpl@cc.nih.gov



For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com

References

TEXTBOOKS

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



JOURNAL ARTICLES

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

Resources

March of Dimes Birth Defects Foundation

1275 Mamaroneck Avenue

White Plains, NY 10605

Tel: (914)997-4488

Fax: (914)997-4763

Tel: (888)663-4637

Email: Askus@marchofdimes.com

Internet: http://www.marchofdimes.com



American Heart Association

7272 Greenville Avenue

Dallas, TX 75231

Tel: (214)784-7212

Fax: (214)784-1307

Tel: (800)242-8721

Email: Review.personal.info@heart.org

Internet: http://www.heart.org



NIH/National Heart, Lung and Blood Institute

P.O. Box 30105

Bethesda, MD 20892-0105

Tel: (301)592-8573

Fax: (301)251-1223

Email: nhlbiinfo@rover.nhlbi.nih.gov

Internet: http://www.nhlbi.nih.gov/



Adult Congenital Heart Association

6757 Greene Street, Suite 335

Philadelphia, PA 19119-3508

USA

Tel: (215)849-1260

Fax: (215)849-1261

Tel: (888)921-2242

Email: info@achaheart.org, advocacy@achaheart.org

Internet: http://www.achaheart.org/



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.

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