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].

• Atrial Septal Defect

  KEYWORDS: atrial septal defect; cardiac magnetic resonance imaging; cor triatriatum dexter; echocardiography; prominent eustachian valve [ncbi.nlm.nih.gov]

  In her cardiac history we noted uncomplicated repair of an ostium secundum atrial septal defect at the age of 5 years. [bmccardiovascdisord.biomedcentral.com]

  The posterior atrial septal defect is not visible. *SVC and IVC mark the venous canulas in the superior and inferior vena cava. [jtcvs.org]

• 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]

  Conclusion The natural history of CTS most often manifests with symptoms of congestive heart failure. Patients with obstructive membrane most often have associated cardiac defects and higher risk for infections and haemorrhage. [heart.bmj.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]

• Collapse

  When that didn’t work, they had to change over to a hand pump just to help her breathe because her lungs had collapsed. After giving us this information, the doctors told us that the only way to fix this was to go in and do surgery. [themighty.com]

  Transthoracic echocardiography revealed membrane (perforated) dividing right atrium into two compartments; severe tricuspid incompetence (TI), V max = 5.2 m/s, peak gradient = 108 mmHg, inferior vena cava collapses [Figure 2] and [Figure 3]. [smjonline.org]

  CASE REPORT A 15-year old female with unknown past medical history presented to the emergency department (ED) after collapsing while walking. She was found to be acutely dyspneic by family and soon became pulseless. [westjem.com]

  While it appears that only the accessory membrane needs to be resected, the small size of the left atrium and collapse of the structure during cardiopulmonary bypass can make visualization difficult. [11] [13] (Move Mouse on Image to Enlarge) References [statpearls.com]

  While it appears that only the accessory membrane needs to be resected, the small size of the left atrium and collapse of the structure during cardiopulmonary bypass can make visualization difficult.[11][13] Review Questions Figure Figure 1 A) Transthoracic [ncbi.nlm.nih.gov]

• Trisomy 21

  Images in cardiology A 25 year old man with Down's syndrome (trisomy 21) was evaluated for dyspnoea. [heart.bmj.com]

• Cyanotic Congenital Heart Disease

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

• Exertional Dyspnea

  It is rarely first diagnosed in adulthood. [1], [2], [3], [4], [5] The usual presenting symptoms although not characteristic include exertional dyspnea, orthopnea, and hemoptysis. [nigjcardiol.org]

  On repeat evaluation 3 months later, he was still in atrial fibrillation and had debilitating exertional dyspnea. [innovationsincrm.com]

• Failure to Thrive

  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]

  Full Text We illustrate the case of a 2-year-old girl presenting with failure to thrive, dyspnea, and systolic murmur. [revespcardiol.org]

  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]

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

• Vomiting

  Endotracheal intubation was attempted twice en route without success and was complicated by copious vomiting. Upon arrival to the ED, CPR was continued and the trachea was intubated. [westjem.com]

• Hepatomegaly

  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]

  […] cava Symptoms depend on size of opening in accessory membrane between the atria Symptoms include dyspnea on exertion, orthopnea, easy fatiguability, low exercise tolerance, palpitations Increased risk of thrombus formation in LA Murmur Elevated JVD Hepatomegaly [wikem.org]

  Physical examination revealed cyanosis (systemic saturation 60%), clubbing, oedema, accentuated pulmonary component of second heart sound, and hepatomegaly. Chest x ray demonstrated cardiomegaly and pulmonary venous congestion. [heart.bmj.com]

  There was hepatomegaly of 4 cm along the mid-clavicular line and spleen was not palpable. Patient was admitted in ICU, sepsis work up was normal. Chest X-ray showed cardiomegaly (C:T ratio > 0.6) with perihilar opacity. ECG showed RVH & RAH. [file.scirp.org]

  There was tender hepatomegaly. We examined the cardiovascular system and found regular pulse, visible apex beat. Apex was situated 4 cm apart from the midsternal line in the left 4th intercostals space medial to the midclavicular line. [ibimapublishing.com]

• Heart Disease

  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]

  Adult cases of congenital heart disease (grown-up congenital heart [GUCH] disease), although rare, are mainly found through echocardiographic examinations. [oapublishinglondon.com]

  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]

• Heart Failure

  On physical examination a loud pulmonary component of the 2 nd heart sound and a diastolic murmur was heard in the mitral area. Signs of right-sided heart failure were absent. [cardiothoracicsurgery.biomedcentral.com]

  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]

• Palpitations

  The most common presenting symptoms are dyspnea, orthopnea, hemoptysis, palpitations and chest pain. [scielo.br]

  Abstract We present a 20-year-old male patient with cor triatriatum sinistrum with fenestrations and long-standing dyspnea on exertion, fatigue, and palpitation. [ncbi.nlm.nih.gov]

  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]

  A 46-year-old man consulted for palpitations and dyspnea, and atrial fibrillation was noted on the electrocardiogram. Cardiac catheterization was performed and the diagnosis of cor triatriatum was made on echocardiography findings. [revespcardiol.org]

• Continuous Murmur

  Unusual features included a continuous murmur high in the left axilla and striking enlargement of the left atrium. [pediatrics.aappublications.org]

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].

• Pericardial Effusion

  While there is a paucity of literature specifically examining the utility of FoCUS in pediatric patients, research in adults suggests it can effectively identify potentially reversible causes of PEA, such as cardiac tamponade due to pericardial effusion [westjem.com]

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]

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. [rarediseases.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]

[…] patient information click here Cor triatriatum ICD-10 Q24.2 ICD-9 746.82 DiseasesDB 31741 MeSH D003310 Cor triatriatum Microchapters Home Patient Info Overview Pathophysiology Epidemiology & Demographics Risk Factors Natural History, Complications & Prognosis [wikidoc.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]

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]

Jump to navigation Jump to search For patient information click here Cor triatriatum ICD-10 Q24.2 ICD-9 746.82 DiseasesDB 31741 MeSH D003310 Cor triatriatum Microchapters Home Patient Info Overview Pathophysiology Epidemiology & Demographics Risk Factors [wikidoc.org]

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 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]

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]

[…] progressive increase in pulmonary artery pressure.2 Syncope, heart failure, and sudden cardiac arrest are well cited.5,6 Early diagnosis by echocardiography is important to expedite surgical cure.2,7,8 References Cor Triatriatum: Background, Pathophysiology, Epidemiology [link.springer.com]

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]

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]

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]

Jump to navigation Jump to search For patient information click here Cor triatriatum ICD-10 Q24.2 ICD-9 746.82 DiseasesDB 31741 MeSH D003310 Cor triatriatum Microchapters Home Patient Info Overview Pathophysiology Epidemiology & Demographics Risk Factors [wikidoc.org]

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

| Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies References External Links Images in Clinical Medicine in NEJM, 2004 Image at Yale University Diagram at University of Hawaii Description at University of Hawaii [wikidoc.org]

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]

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]

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. [n.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.