Cor triatriatum may exist as a stand alone abnormality [1], or may be associated with other congenital heart malformations, some caused by absent or abnormal conotruncal inversion, some by persistence of embryonic structures, some by lack of atrial, ventricular or atrioventricular septum development [2]. Some associations, such as between cor triatriatum and a bicuspid aortic valve, may be harder to explain as a consequence of a single event in embryonic development and may occur as separate entities [3]. Other left heart obstructive lesions may also exist [4].

The patient presents for various reasons, depending on heart anatomy and function. If the abnormality is left- sided, symptoms are similar to those induced by mitral stenosis, appear sooner and are more dramatic if the opening in the accessory membrane separating the true left atrium from the supranumerary chamber is smaller. If this communication is large, the patient may be asymptomatic and diagnosis is made on an echocardiography examination performed for other reasons. If complaints do exist, they consist of hemoptysis, orthopnea and signs of left heart failure: dyspnea on exercise and asthenia. Children with a restrictive membrane defect fail to thrive or may present with severe neonatal respiratory distress [5] due to the pressure gradient between the true and accessory atrial chambers. In cor triatriatum dexter, right heart failure signs appear first and consist of edema, ascites, painful hepatomegaly and jaundice. Rhythm abnormalities like atrial fibrillation or tachycardia may coexist in both forms. Physical examination of a cor triatriatum sinister patient may reveal further abnormalities: signs of systemic embolism, pulmonary rales or pleural effusion, heart murmurs, tachypnea and respiratory distress. In a right sided abnormality, the physician may find elevated jugular venous pressure, tricuspid regurgitation and right ventricular heave. Pulmonary hypertension may also be present. Adults become symptomatic if the membrane within the left atrium becomes calcified [6] or during sustained effort, such as labor [7] [8].

• Mediastinal Lymphadenopathy

  Author information 1 Department of Cardiology, Nationwide Children's Hospital, Columbus, Ohio, USA. [email protected] Abstract This report describes a patient with a superior mediastinal mass and extensive intrathoracic lymphadenopathy [ncbi.nlm.nih.gov]

• Congestive Heart Failure

  We present here a 6-year old boy who was diagnosed with cor triatriatum sinister, initially showing symptoms similar to mitral valve stenosis and congestive heart failure, and who underwent subsequent surgical correction using a left atrial approach. [ncbi.nlm.nih.gov]

  It is imperative to address AF in these patients, irrespective of their CHA₂DS₂-VASc scores (congestive heart failure, hypertension, age, diabetes mellitus, stroke, vascular disease, age, sex category) due to their higher risks of thromboembolism. [cureus.com]

  Case presentation A 33 - year old woman presented to our cardiology service with signs and symptoms of congestive heart failure. [cardiothoracicsurgery.biomedcentral.com]

  We recommend anticoagulation as secondary prevention therapy in adult patients with ischemic stroke associated with cor triatriatum sinistrum and no congestive heart failure syndrome. [healthcare-bulletin.com]

• Hypoxemia

  This is clinically important as these patients may develop paradoxical emboli, [18] or hypoxemia due to the right-to left shunting. [annals.in]

  Hypoxemia may be noticed in a newborn with severe respiratory distress caused by cor triatriatum. [symptoma.com]

  Other symptoms may include palpitation, effort intolerance, and features suggestive of systemic thromboembolism. [20] Physical findings include murmur, pulmonary congestion, tachypnea, respiratory distress, pulmonary rales, pleural effusion, hypoxemia [nigjcardiol.org]

• Lymphadenopathy

  Author information 1 Department of Cardiology, Nationwide Children's Hospital, Columbus, Ohio, USA. [email protected] Abstract This report describes a patient with a superior mediastinal mass and extensive intrathoracic lymphadenopathy [ncbi.nlm.nih.gov]

  On examination, he was pale, afebrile, anicteric, no significant lymphadenopathy, no pedal edema. Pulse was 84 beat/min, large volume, regular, and collapsing. His jugular venous pressure was not elevated. [nigjcardiol.org]

• Respiratory Distress

  Hypoxemia may be noticed in a newborn with severe respiratory distress caused by cor triatriatum. [symptoma.com]

  We report a case of a toddler who presented with respiratory distress and cardiomegaly and was found to have cor triatriatum sinister with a restrictive communication, decompressing vertical vein, pulmonary hypertension, severe tricuspid regurgitation [ncbi.nlm.nih.gov]

  Image 3 Post-Intubation chest radiograph demonstrating worsening edema consistent with acute respiratory distress syndrome. [westjem.com]

• Failure to Thrive

  We present a rare case of cor triatriatum sinistrum in a 6-month-old female infant who was presented with cyanosis and failure to thrive. [jctm.mums.ac.ir]

  Every patient who has palpitation and failure to thrive should have thorough cardiologic evaluation to find out the rare cardiac abnormalities and managed accordingly. [ibimapublishing.com]

  Typical presentation includes dyspnea, heart failure, and failure to thrive 5. [radiopaedia.org]

• Jaundice

  In cor triatriatum dexter, right heart failure signs appear first and consist of edema, ascites, painful hepatomegaly and jaundice. Rhythm abnormalities like atrial fibrillation or tachycardia may coexist in both forms. [symptoma.com]

• Heart Disease

  Cyanotic congenital heart diseases. Radiopaedia. http://radiopaedia.org/articles/cyanotic-congenital-heart-disease [wikem.org]

  disease (CCHD) screening utilizes pulse oximetry to detect heart disease in the neonatal period. [jcnonweb.com]

  Cor triatriatum is a rare congenital anomaly known to be associated with other inherited heart diseases. [ncbi.nlm.nih.gov]

  Session Title: FIT Clinical Decision Making: Pulmonary Hypertension, VTE, Congenital Heart Disease and Prevention Abstract Category: Congenital Heart Disease Presentation Number: 1241-203 2018 American College of Cardiology Foundation [onlinejacc.org]

  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. [rarediseases.org]

• Heart Failure

  Second, it may present as right heart failure. [jiaecho.org]

  However, features of systemic embolization, heart failure, atrial fibrillation, cyanosis, cardiac asthma, syncope, and sudden cardiac arrest have also been reported in the literature. [ncbi.nlm.nih.gov]

  If complaints do exist, they consist of hemoptysis, orthopnea and signs of left heart failure: dyspnea on exercise and asthenia. [symptoma.com]

• Suggestibility

  A computed tomography of the chest showed a left atrial mass suggestive of atrial myxoma confirmed with transesophageal echocardiogram. [ncbi.nlm.nih.gov]

  Features suggestive of the correct diagnosis would include the absence of an opening snap, the absence of a typical murmur of mitral stenosis, regular rhythm, and lesser evidence of left atrial enlargement in the presence of obvious pulmonary hypertension [ahajournals.org]

• Chorea

  Cor triatriatum sinister, not mitral stenosis, in an adult with previous Sydenham’s chorea: diagnosis and preoperative assessment by cross sectional echocardiography. Br Heart J. 1992;68:9-11. Google Scholar | Crossref | Medline33. [journals.sagepub.com]

  Cor triatriatum sinister, not mitral stenosis, in an adult with previous Sydenham's chorea: diagnosis and preoperative assessment by cross sectional echocardiography. Br Heart J. 1992 Jul. 68(1):9-11. [Medline]. [Full Text]. [emedicine.medscape.com]

  Cor triatriatum sinister, not mitral stenosis, in an adult with previous Sydenham's chorea: Diagnosis and preoperative assessment by cross sectional echocardiography. Br Heart J 1992;68:9-11. 7. Bartel T, Müller S, Erbel R. [annals.in]

• Asthenia

  If complaints do exist, they consist of hemoptysis, orthopnea and signs of left heart failure: dyspnea on exercise and asthenia. [symptoma.com]

Hypoxemia may be noticed in a newborn with severe respiratory distress caused by cor triatriatum. The electrocardiogram may be normal or it may show signs of right ventricular hypertrophy and strain, right axis deviation, left, right or biatrial enlargement and supraventricular arrhythmia. Thoracic radiography may reveal pulmonary congestion and pleural effusion and cardiomegaly due to enlarged atria and dilated pulmonary arteries. Heart catheterization shows increased left atrial, right atrial, ventricular and pulmonary artery pressures, that become worse during exercise [9], with possible systemic hypotension and a gradient across the accessory membrane. Cardiac computer tomography and magnetic resonance are used when diagnosis is uncertain [10], but echocardiography usually elucidates the diagnosis [11], the morphology of the atria and venous return and the hemodynamic consequences of the abnormality. If a restrictive opening in the accessory membrane exists, the flow across it will be found both during the systole and diastole [12]. Three dimensional fully-sampled matrix array transoesophageal echocardiography (TEE) offers further information, if needed [13].

• Mediastinal Mass

  Author information 1 Department of Cardiology, Nationwide Children's Hospital, Columbus, Ohio, USA. [email protected] Abstract This report describes a patient with a superior mediastinal mass and extensive intrathoracic lymphadenopathy [ncbi.nlm.nih.gov]

• Superior Mediastinal Mass

  Author information 1 Department of Cardiology, Nationwide Children's Hospital, Columbus, Ohio, USA. [email protected] Abstract This report describes a patient with a superior mediastinal mass and extensive intrathoracic lymphadenopathy [ncbi.nlm.nih.gov]

Surgical treatment subsequently proved to be successful. In patients with an incomplete membrane and a septal defect with well-defined rims, percutaneous treatment can be the first choice. [ncbi.nlm.nih.gov]

Our Nemours Cardiac Center team has the experience and advanced pediatric imaging needed to provide diagnosis, treatment and surgical repair of rare pediatric heart defects like cor triatriatum. [nemours.org]

Surgery is the first line of treatment in patients with CTS. However, when the patient presented at our clinic, she had two or more chronic diseases or conditions (comorbidities) and surgery was ruled out as a treatment option. [atlasofscience.org]

Treatment and prognosis A cor triatriatum is usually fatal within the first two years of life. The membrane can be surgically resected. [radiopaedia.org]

Moreover, there cannot be generalized prediction of prognosis as the communicating orifice is the sole determinant of the progression to sequelae. [4] Hence, course and prognosis will vary from case to case. [jiaecho.org]

If the communication between the proximal and distal chambers is not restrictive or if an atrial septal defect allows decompression of the hypertensive left atrium, the prognosis is significantly improved. [chdbabies.com]

In contrast to mitral stenosis where there is only a diastolic flow across the valve, in CTT there is typically a trans-membrane gradient throughout the cardiac cycle resulting in flow both during systole and diastole. 1 The natural history and prognosis [academic.oup.com]

The initial evaluation of these patients typically focuses on searching for a pulmonary etiology for arterial desaturation, which often leads to a negative work up. [ncbi.nlm.nih.gov]

We present the case of a young ischemic stroke patient, in whom after detailed etiological investigation, a rare congenital cardiac anomaly was found. [healthcare-bulletin.com]

It can be divided into primary and secondary types in terms of its etiology. [springerplus.springeropen.com]

The workup for alternative etiologies had been negative, and no additional cardiac congenital abnormalities were discovered. [hindawi.com]

Key Concepts Epidemiology Cor triatriatum is an uncommon congenital cardiac anomaly in which either the left (cor triatriatum sinister) or right (cor triatriatum dexter) atrium is divided into two chambers by a membrane. [accesssurgery.mhmedical.com]

Epidemiology Represents 0.1-0.4% of all congenital abnormalities. Associated cardiac lesions include atrial septal defect, anomalous pulmonary venous return, bicuspid aortic valve and PDA. [pedecho.org]

Therefore, active search for cor-triatriatum dexter should be encouraged during echocardiography because this may change the epidemiology of this disease. [jcecho.org]

Epidemiology Frequency United States US epidemiologic statistics regarding cor triatriatum include the following: The incidence of cor triatriatum has been variously reported as 0.1-0.4%. [emedicine.medscape.com]

The entitiy can mimick the pathophysiology of mitral stenosis, necessitating surgical resection. Occasionally, percutaneous balloon dilatation of the membrane has been successfully performed. [ncbi.nlm.nih.gov]

Pathophysiology In the absence of associated cardiac lesions, the pathophysiology of subdivided left atrium depends on the resulting obstruction to pulmonary blood flow. [accesssurgery.mhmedical.com]

Very few adult patients with cor triatriatum sinistrum and ischemic stroke are reported in the literature, and we discuss the importance of transesophageal echocardiogram and the underlying pathophysiologic mechanisms. [healthcare-bulletin.com]

Given the proposed left atrial pathophysiologic similarities with mitral stenosis, the decision was made to treat with a vitamin K antagonist. [hindawi.com]

Pathophysiology A malincorporation theory [3] presented by Dr. Clifford G. Parsons explains how cor triatriatum sinister occurs. During normal fetal development, the pulmonary vein incorporates into the left atrium. [statpearls.com]

At Nemours, we’re actively researching new approaches for childhood disease prevention, diagnosis and treatment. Learn more about your child’s medical condition from the experts at Nemours’ KidsHealth. [nemours.org]

Reduced preload because of CTD with spastic quadriplegia that prevented physical exertion is believed to have facilitated this patient's unusually long survival. © 2012 Wiley Periodicals, Inc. [ncbi.nlm.nih.gov]

The need for further investigations regarding optimal stroke prevention in this subset of patients is emphasized. Disclosure: Dr. Diestro has nothing to disclose. Dr. Espiritu has nothing to disclose. Dr. Dorotan has nothing to disclose. Dr. [neurology.org]

1. Peleato A, Portero M, Ruiz J. [Cor triatriatum in adults: intracardiac echocardiography]. Rev Esp Cardiol. 2006;59(1):67.
2. Tuccillo B, Stumper O, Hess J, et al. Transoesophageal echocardiographic evaluation of atrial morphology in children with congenital heart disease. Eur Heart J. 1992;13(2):223-231.
3. Hamdan R, Mirochnik N, Celermajer D, et al. Cor Triatriatum Sinister diagnosed in adult life with three dimensional transesophageal echocardiography. BMC Cardiovasc Disord. 2010;10:54.
4. Goyal S, Punnam S, Verma G, et al. Persistent left superior vena cava: a case report and review of literature. Cardiovasc Ultrasound. 2008 Oct 10;6:50.
5. Humpl T, Reineker K, Manlhiot C, et al. Cor triatriatum sinistrum in childhood. A single institution’s experience. Can J Cardiol. 2010;26(7):371–376.
6. Slight R, Nzewi O, Buell R, et al. Cor-triatriatum sinister presenting in the adult as mitral stenosis: an analysis of factors which may be relevant in late presentation. Heart Lung Circ. 2005;14(1):8–12.
7. Bai W, Kaushal S, Malviya S, et al. Anesthetic management for resection of cor triatriatum during the second trimester of pregnancy. Int J Obstet Anesth. 2010;19(1):103–106.
8. Davlouros P, Koutsogiannis N, Karatza A, et al. An unusual case of cor triatriatum sinister presenting as pulmonary oedema during labor. Int J Cardiol. 2009;150(3):e92-e93.
9. Thakrar A, Shapiro MD, Jassal DS, et al. Cor triatriatum: the utility of cardiovascular imaging. Can J Cardiol. 2007;23(2):143-145.
10. Chen K, Thng CH. Multislice computed tomography and two-dimensional echocardiographic images of cor triatriatum in a 46-year-old man. Circulation. 2001;104(17):2117.
11. Modi K, Annamali S, Ernest K, et al. Diagnosis and surgical correction of cor triatriatum in an adult: combined use of transesophageal and contrast echocardiography, and a review of literature. Echocardiography. 2006;23(6):506-509.
12. Einav E, Perk G, Kronzon I. Three-dimensional transthoracic echocardiographic evaluation of cor triatriatum. Eur J Echocardiogr. 2008;9(1):110-112.
13. Sugeng L, Shernan S, Salgo I, et al. Live 3-dimensional transesophageal echocardiography initial experience using the fully-sampled matrix array probe. J Am Coll Cardiol. 2008;52(6):446–449.