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Annals of Cardiac Anaesthesia Annals of Cardiac Anaesthesia Annals of Cardiac Anaesthesia
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Table of Contents
INVITED COMMENTARY  
Year : 2014  |  Volume : 17  |  Issue : 2  |  Page : 116-117
Cor-Triatriatum: When to worry?


Department of Anaesthesiology, Sanjay Gandhi Post Graduate Institute, Lucknow, Uttar Pradesh, India

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Date of Web Publication1-Apr-2014
 

How to cite this article:
Tewari P. Cor-Triatriatum: When to worry?. Ann Card Anaesth 2014;17:116-7

How to cite this URL:
Tewari P. Cor-Triatriatum: When to worry?. Ann Card Anaesth [serial online] 2014 [cited 2020 Aug 7];17:116-7. Available from: http://www.annals.in/text.asp?2014/17/2/116/129835


Cor triatriatum sinister (CTS) consists of the presence of an abnormal thick fibromuscular septum or only a membrane dividing the left atrium (LA) in a proximal chamber receiving pulmonary veins and a distal chamber, having the mitral valve. During the development of the cardiovascular system in the fetal life failure of the common pulmonary vein to get incorporated or resorbed into the LA in a normal manner can give rise to this problem. [1] Incomplete reabsorption of the common pulmonary vein in fetal life may leave either a membrane, which causes pulmonary venous obstruction, or may leave only a hemodynamically insignificant ridge of tissue within the LA. [2] The common theories are malincorporation, malseptation or entrapment phenomenon. Reports have shown an association of the left superior vena cava with CTS, but its significance in the pathogenesis of CTS is unclear. Loeffler, in 1949, [2] classified CTS into three groups on the basis of the number and size of the openings in the anomalous membrane: Group I no opening; Group II one or more small openings; and Group III comprised of a wide opening in the membrane. Patients with Group III CTS reach late adulthood without showing many symptoms. [2] Abnormal septum on the right side, Cor triatriatum dexter (CTD), if obstructive, presents with increased jugular venous pressure, distended neck veins, hepatic congestion, ascites along with raised liver enzymes, and edema over lower extremities can be presenting features.

When significant obstruction of the pulmonary venous drainage occurs, the common pulmonary vein dilates to become a sac, protruding into the LA superiorly and posteriorly. This is then referred to as a third atrium. Variations in the anatomy of the lesion concern two general features: first, the size of any orifices which may be present in the abnormal septum; and second, the relationship between the foramen ovale and the point of insertion of the abnormal septum medially in the interatrial septum. Adult patients present with signs of pulmonary venous and arterial hypertension, and the condition must be distinguished from mitral valve disease which it closely resembles and from less common left-sided obstructive lesions. CTS in the adult has been reported in association with ostium secundum atrial septal defect, dilated coronary sinus due to persistent left superior vena cava, and bicuspid aortic valve. Cine-angiocardiography shows the distal chamber to contract vigorously during atrial systole, but the proximal accessory chamber contracts poorly; the membrane moves toward the mitral valve during ventricular diastole, but straightens after closure of the mitral valve. The value of echocardiography in the differential diagnosis of lesions causing pulmonary venous hypertension is clear. A normal mitral valve echo excludes hemodynamically significant mitral stenosis, and abnormal echoes may indicate the presence of LA myxoma, CTS, or supravalvar stenosing ring. The technique is noninvasive and therefore, safer than transseptal LA puncture or angiocardiography. The most common symptoms present in adults are dyspnea, hemoptysis, and orthopnea, but CTS as well as CTD can be asymptomatic and the diagnosis can be incidental. Cardiac murmurs are the most significant finding during physical examination. Apical systolic, diastolic, and continuous, murmurs have been reported, but typical mitral diastolic and presystolic murmurs are unusual, and there is no opening snap. [4] Murmurs of pulmonary regurgitation or tricuspid regurgitation may be present. Other features suggesting CTS are normal LA size and normal (or right atrial) P waves, though there is sometimes radiological evidence of LA enlargement and P mitrale may occur; atrial fibrillation is rare. As the age advances, the asymptomatic CTS can turn into a symptomatic one and is due to one or more of the precipitating factors viz., fibrosis and calcification of the orifice in the anomalous septum; the development of mitral regurgitation; and the development of atrial fibrillation.

In this issue of Annals of Cardiac Anaesthesia, Scavonetto et al., have reviewed the perioperative anesthetic management of patients having nonobstructed CTS or CTD undergoing different surgeries. The authors have retrospectively collected their own institutions data, Cor triatriatum (CT) being a rare disorder they could only present 12 patients, seven being CTS and five as CTD anomaly. It is noticeable in their report that the incidence of atrial fibrillation is 10% in CTS variety, whereas it was up to 60% in right sided CT. The regional anesthesia was tolerated well in few of the patients. [3] Except two patients; the remaining 10 patients had uneventful anesthesia and surgery. The two patients became hemodynamically unstable; one patient developed hypoxia and cyanosis after atrial fibrillation, right to left shunting through associated ASD was considered as the cause, the hemodynamic instability resolved once ASD was closed. The other patient remained hypotensive in spite of fluid resuscitation and underwent balloon dilation of the restrictive membrane; however, the patient succumbed to other complications.

Any patient showing signs mimicking mitral stenosis in the presence of normal mitral valve or that of tricuspid stenosis without the involvement of the tricuspid valve itself, presence of other causes such as CTS or CTD should be meticulously searched for and if present, the gradient across the proximal and distal chambers should be measured. In presence of a significant gradient, a balloon septostomy of the aperture in the membrane should be considered before resorting to noncardiac surgery. [5]

A high level of suspicion for CT is essential and hence that the perioperative anesthetic management is carved to suit problems that may manifest. The hemodynamic goals are similar to that of the management of mitral and tricuspid stenosis and are perioperative heart rate control, adequate fluid balance, and avoidance of all the factors that may increase pulmonary resistance and reduce pulmonary flow. The principles of regional anesthesia are to be guided by the degree of the aperture stenosis and also by the established guidelines, if the patient is on any kind of anticoagulant therapy. [6]

 
   References Top

1.Jordan JD, McNamara DG, Contell J, Rosenberg HS. Cor triatriatum: An anatomic and physiologic study. Cardiovasc Res Cent Bull 1963;1:79-85.  Back to cited text no. 1
[PUBMED]    
2.Loeffler E. Unusual malformation of the left atrium; pulmonary sinus. Arch Pathol (Chic) 1949;48:371-6.  Back to cited text no. 2
[PUBMED]    
3.Scavonetto F, Yeoh TY, Welch TL, Weingarten TN, Sprung J. Anesthesia and cor triatriatum. Ann Card Anaesth 2014;17:111-6.  Back to cited text no. 3
  Medknow Journal  
4.McGuire LB, Nolan TB, Reeve R, Dammann JF Jr. Cor triatriatum as a problem of adult heart disease. Circulation 1965;31:263-72.  Back to cited text no. 4
[PUBMED]    
5.Huang TC, Lee CL, Lin CC, Tseng CJ, Hsieh KS. Use of Inoue balloon dilatation method for treatment of Cor triatriatum stenosis in a child. Catheter Cardiovasc Interv 2002;57:252-6.  Back to cited text no. 5
    
6.Park KJ, Park IK, Sir JJ, Kim HT, Park YI, Tsung PC, et al. Adult cor triatriatum presenting as cardioembolic stroke. Intern Med 2009;48:1149-52.  Back to cited text no. 6
    

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Correspondence Address:
Prabhat Tewari
Department of Anaesthesiology, Sanjay Gandhi Post Graduate Institute, Lucknow, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


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