Cor triatriatum is an extremely rare congenital (present at birth) heart defect. Normally, the human heart has four chambers of which two are the atria. These two are separated from each other by a partition (septum) called the atrial septum. The other two chambers, known as ventricles, are also separated by a septum. In cor triatriatum there is a small extra chamber above the left atrium of the heart. The pulmonary veins, returning blood from the lungs, drain into this extra “third atrium.” The passage of blood from the lungs into the heart (left atrium and ventricle) is slowed by this extra chamber. Cor triatriatum may eventually lead to features of congestive heart failure and obstruction over time.
The symptoms of cor triatriatum vary greatly, depending on the size of the opening between the extra chamber and the left atrium. If the opening is small, symptoms usually develop early in infancy and probably will include abnormally rapid breathing (tachypnea), wheezing, coughing, and/or abnormal accumulation of fluid in the lungs (pulmonary congestion). Progressive enlargement of the heart (cardiomegaly) occurs and often results in congestive heart failure along with abnormally high pressure within the artery that leads to the heart from the lungs (pulmonary artery). Some newborns with cor triatriatum may also have abnormal heart sounds or heart murmurs.
In older people (after childhood), the symptoms of cor triatriatum may include abnormal swelling of areas of the body (generalized edema), pain and discomfort while breathing (dyspnea), an abnormally rapid heartbeat (tachycardia), and the excessive accumulation of fluid in the lungs (pulmonary congestion). Acute inflammation of the lungs (pneumonia) and bronchial tubes (bronchitis) may occur frequently and may lead to congestive heart failure. Individuals with cor triatriatum are also at increased risk for bacterial infection of the delicate membranes surrounding the heart (endocarditis).
Cor triatriatum is an extremely rare congenital heart condition that affects males and females in equal numbers. In the USA, this disorder accounts for an extremely small percentage (0.1% to 0.4%) of all infants with congenital heart disease.
The diagnosis of cor triatriatum is usually made by using imaging techniques such as magnetic resonance imaging (MRI) and echocardiography (EC). In another procedure known as a cardiac catheterization, a long fine tube (catheter) is inserted into a large vein and then channeled directly into the heart. This allows the physician to further identify heart defects and to determine the rates of blood flow through the heart. Angiography is also a very useful diagnostic procedure and allows the physician to view an enhanced x-ray of the heart. Children with cor triatriatum also have abnormal EKG patterns.
Infants with cor triatriatum should be referred to a hospital that can perform sophisticated diagnostic procedures and cardiovascular surgery. Most patients with cor triatriatum will require surgery at a young age, usually before the age of one year.
Prior to surgery, congestive heart failure associated with cor triatriatum may be managed by reducing fluid volume with diuretic drugs and, if necessary, the dietary restriction of fluids and salt. The drug digitalis may also be administered to decrease the heart rate and increase the strength of the heart's contractions. Oxygen therapy may also prove beneficial.
Because people with cor triatriatum are susceptible to bacterial infection of the membranes that surround the heart (endocarditis), any respiratory infection should be treated vigorously and early. Affected individuals should also be given antibiotics before invasive dental procedures (i.e., root canal or extractions) or other surgical procedures to help prevent potentially life-threatening infections.
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:
Schlant RC. Congenital Heart Disease. In: Stein JH, Hutton JJ, Kohler PO, et al. Eds. Internal Medicine. 4th ed. Mosby-Yearbook, Inc., St. Louis, MO. 1994:275, 284.
Friedman WF. Congenital Heart Disease in Infancy and Childhood. In: Braunwald E. Ed. Heart Disease: A Textbook of Cardiovascular Medicine. 3rd ed. W.B. Saunders Company, Philadelphia, PA; 1988:940,985.
Tueche S. Cor triatriatum dextrum. Surgical treatment in a neonate. Acta Cardiol. 2003;58:39-40.
Huang TC, Lee CL, Lin CC, et al. Use of Inoue balloon dilatation method for treatment of Cor triatriatum stenosis in a child. Catheter Cardiovasc Interv. 2002;57:252-56.
Bezante GP, Deferrari L, Molinari G, et al. Cor triatriatum sinistrum and persistant left superior vena cava: an original association. Eur J Echocradiog. 2002;3:162-65.
Buchholz S, Jenni R. Doppler echocardiographic findings in 2 identical variants of a rare cardiac anomaly, “subtotal” cor triatriatum: a critical review of the literature. J Am Soc Echocardiogr. 2001;14:846-49.
Roldan FJ, Vargas-Barron J, Espinola-Zavaleta N, et al. Cor triatriatum dexter: transesophageal echocardiographic diagnosis and 3-dimensional reconstruction. J Am Soc Echocardiog. 2001;14:634-36.
Ito M, Kikuchi S, Hachiro Y, et al. Congenital pulmonary vein stenosis associated with cor triatriatum. Ann Thorac Surg. 2001;71:722-23.
Bauer M, Alexi-Meskishvilli V, Nakic Z, et al. The correction of congenital heart defects with less invasive approaches. Thorac Cardiovasc Surg. 2000;48:67-41.
Pisanti A, Vitiello R. Wheezing as the sole clinical manifestation of cor triatriatum. Pediatr Pulmonol. 2000;30:346-49.
Rorie M, Xie GY, Miles H, et al. Diagnosis and surgical correction of cor triatriatum in an adult: copmbined use of transesophageal echocardiography and catheterization. Catheter Cardiovasc Interv. 2000;51:83-86.
Chen Q, Guhathakurta S, Vadalapali G, et al. Cor triatriatum in adults: three new cases and a brief review. Tex Heart Inst J. 1999;26:206-10.
FROM THE INTERNET
Myers JL, Jaggers J. Cor Triatrium. EMedicine. Last Updated: January 15, 2003. 14pp.
Myers JL, Jaggers J. Cor Triatrium. EMedicine. Last Updated: August 24, 2001. 11pp.
Gpnotebook. cor triatriatum. nd. 1p.
Walton B. Cor Triatriatum. Texas Heart Institute Cardiac Society case studies. nd. 2pp.
Tahernia AC, Ashcraft KW, Tutuska PJ. The Diagnosis of Cor Triatriatum Sinistrum in Children: A Continuing Dilemma. Southern Medical Journal. 1999;92:218-222.
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