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ORIGINAL ARTICLE  
Year : 2014  |  Volume : 17  |  Issue : 2  |  Page : 111-116
Anesthesia and cor triatriatum


Department of Anesthesiology, Mayo Clinic, Rochester, Minnesota, USA

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Date of Submission20-Jul-2013
Date of Acceptance28-Jan-2014
Date of Web Publication1-Apr-2014
 

   Abstract 

Aims and Objectives: Cor triatriatum sinistrum (CTS) and cor triatriatum dextrum (CTD) are rare congenital anomalies characterized by the presence of a perforated septum which divides the respective atrium into a proximal and distal chamber. This report reviews the perioperative course of patients with uncorrected cor triatriatum (CT) undergoing procedures requiring anesthesia. In addition, we performed a literature search that examines the experience of others regarding the peri-operative course of patients with CT. Materials and Methods: A computerized search of a medical record database was conducted to identify patients with a clinical diagnosis of uncorrected CTD and CTS undergoing surgical procedures. Descriptive statistics were used. Results: We identified 12 adult patients with asymptomatic CTS (n = 7) and CTD (n = 5) who underwent 23 anesthetics. There were no perioperative complications which could be attributed directly to the anatomy of CT. Conclusions: Our observation and review of the literature suggest that patients with asymptomatic CT typically tolerate anesthesia and surgical procedures well.

Keywords: Anesthesia, Cor triatriatum dextrum, Cor triatriatum sinistrum, Surgery

How to cite this article:
Scavonetto F, Yeoh TY, Welch TL, Weingarten TN, Sprung J. Anesthesia and cor triatriatum. Ann Card Anaesth 2014;17:111-6

How to cite this URL:
Scavonetto F, Yeoh TY, Welch TL, Weingarten TN, Sprung J. Anesthesia and cor triatriatum. Ann Card Anaesth [serial online] 2014 [cited 2021 Nov 28];17:111-6. Available from: https://www.annals.in/text.asp?2014/17/2/111/129833

This article is accompanied by an invited commentary by Dr. Prabhat Tewari



   Introduction Top


Cor triatriatum (CT) is a rare congenital heart malformation that results from the division of the atrium into two chambers by a perforated fibromuscular septum [Figure 1]. A division in a form of fenestrated accessory atrial membrane in the left atrium is known as CT sinistrum (CTS) and accounts for approximately 0.1% of all congenital heart malformations [Figure 1]a. [1] In CTS the pulmonary veins generally enter the proximal chamber and blood flows from the proximal to the distal chamber through a single opening or multiple openings in the accessory membrane. CT dextrum (CTD) occurs when the right atrium is divided by persistence of the right valve of the embryonic sinus venosus [Figure 1]b. [2],[3],[4] Blood flow from the proximal to distal chamber is dependent upon the size of the perforation on intra-atrial membrane in both CTS and CTD. If the aperture is adequate, blood flow is unimpeded and CT is asymptomatic. However, if the size of the aperture restricts blood flow between the proximal and distal atrial chambers, patients develop signs and symptoms resembling tricuspid or mitral valve stenosis (depending on the side of heart affected). [5],[6] This heart defect rarely exists in isolation and the most common coexistent anomalies include atrial septal defect, mitral regurgitation, tricuspid valve abnormalities and pulmonary artery stenosis or atresia. [7],[8] The diagnosis and surgical correction of symptomatic CTS usually occurs in childhood because the morbidity and mortality in untreated patients are very high. [9] When diagnosed in an adulthood, CT is mostly an incidental finding. [10] There is a paucity of literature on the perioperative course of patients with unrepaired CT undergoing surgery. The aim of this review is to provide educational material for anesthesia providers regarding this extremely rare cardiac anomaly. We report on the perioperative course of our patients with unrepaired CT who underwent anesthesia, and review the current literature pertaining anesthetic management of these patients.
Figure 1: Cor triatriatum sinistrum (panel a) and cor triatriatum dextrum (panel b). IAM: Intra-atrial membrane, IVC: Inferior vena cava orifice, PFO: Patent foramen ovale, PV: Pulmonary vein orifice, SVC: Superior vena cava orifice

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   Materials and Methods Top


This retrospective chart review study received approval from our Institutional Review Board. To be consistent with Minnesota State Statute 144.335 Subd 3a (d), we included only patients who have provided authorization for research use of their medical records (historically >95% of patients at our institution). A computerized search of our institutional medical record databases was conducted to identify all patients with a clinical diagnosis of uncorrected CTS and CTD. The medical records of these patients were reviewed to select those who had operations or procedures under anesthesia care prior to the repair of CT. Medical records were reviewed for demographics, comorbidities, type and severity of CT and clinical presentation (signs and symptoms) of CT before diagnosis was established. Medical records were examined for both the type of surgery or procedure, anesthetic technique, duration of anesthesia, and perioperative outcomes. Descriptive summaries of demographic, epidemiologic, perioperative complications, and other data were used and continuous variables were described as medians and range or interquartile range (25 th , 75 th percentiles). In order to review the current knowledge regarding perioperative outcomes of patients with CT, we performed a literature search of: MEDLINE (January 1, 1946-April 2, 2013) and EMBASE (January 1, 1988-April 2, 2013) databases using the following text words: Anesthesia, anesthetics, CT. Searches were not limited by language or publications type (articles or letters). Manuscripts and selected bibliographies were reviewed for relevancy.


   Results Top


We identified 12 patients (CTS, five male, two female patients; CTD, one male, and four female patients) with uncorrected CT who underwent anesthesia for various surgical procedures. All patients were diagnosed with CT between January 1, 1990 and December 31, 2012. In all patients, the diagnosis of CT was confirmed with echocardiography [Table 1] either during teenage years or in adulthood during work-up of a heart murmur, new onset atrial fibrillation, chest pain, onset of transient ischemic attacks, or acute development of shortness of breath. Six patients (four CTD, two CTS) had a coexisting atrial septal defect (ASD). In two of these adult patients (one CTD, one CTS), the acute development of hypoxemia due to right-to-left shunting through ASD was a presenting sign. First was a 79-year-old female, with previously undiagnosed CTD and a small ASD in proximal atrial chamber, developed atrial fibrillation and became acutely hypoxemic due to right-to-left atrial shunting. Shunting was caused by increased right atrial pressure secondary to atrial fibrillation as well as presence of right atrial metastatic melanoma restricting the blood flow across intra-atrial accessory membrane [Figure 2]. Her hypoxemia resolved after ASD repair. Second was a 66-year-old female with CTS and ASD who developed new-onset atrial fibrillation associated with dyspnea and cyanosis related to right-to-left shunting. On pre-operative echocardiogram, she had right atrial enlargement and the estimated right ventricular systolic pressure was 41 mmHg. Her hypoxemia and dyspnea resolved after ASD repair.
Table 1: Demographics, clinical characteristics and procedures in 12 patients with CTS and CTD

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Figure 2: Cor triatriatum dextrum with atrial septal defect (ASD). Scheme depicts atrial fibrillation (AF) which may result in increased atrial pressure due to inadequate atrial emptying which may facilitate right to left shunting across ASD (blue arrow). (see text for details) IAM: Intra-atrial membrane, IVC: Inferior vena cava orifice, SVC: Superior vena cava orifice

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A total of 23 procedures were performed in 12 patients with CT (CTS n = 12 patients; CTD n = 11 patients), 16 procedures were with general anesthesia, two were under spinal anesthesia, and five with monitored anesthesia care. The median (25 th , 75 th percentile) duration of anesthetic exposure was 115 (76, 212) min for patients with CTS, and 105 (63, 133) min for patients with CTD. Patient demographics, clinical characteristics, echocardiography reports and types of procedures are summarized in [Table 1]. The median (range) age at diagnosis of CT was 43 (21, 65) years for patients with CTS and 70 (68, 79) years for CTD. The median hospital stay was 1.5 days. There were no intraoperative complications and there was one post-operative complication primarily attributed to perioperative blood loss. Specifically, a 16-year-old male with Goldenhar's syndrome with spinal stenosis underwent extensive thoracic spine surgery. During infancy, a cardiac murmur was noted and echocardiogram at that time suggested diagnosis of CTS, but the patient did not have further follow-up. A pre-operative echocardiogram confirmed CTS with unrestricted blood flow across the intra-atrial membrane. His 11-h long surgery was characterized with estimated 5 l blood loss and he received 11 l of crystalloids, 2.6 l of salvaged blood, 1 unit of platelets, and 2 units of fresh frozen plasma. On the first post-operative day, he developed acute respiratory distress due to acute pulmonary edema. Symptoms improved with biphasic positive airway pressure ventilation and he fully recovered.


   Discussion Top


We report a series of patients with uncorrected CT undergoing anesthesia for procedures and surgeries. The main observation is that our patients with non-obstructive CT had no perioperative complications that could be attributed to their CT defect. We identified only six publications that describe patients with uncorrected CT undergoing anesthesia. [11],[12],[13],[14],[15],[16] Two reports described two adult patients with asymptomatic CTS who had uneventful perioperative course. [14],[15] In four isolated reports patients had CTD. [11],[12],[13],[16] One patient, a 67-year-old man with non-obstructive CTD, underwent hydrocelectomy uneventfully. [13] Second patient, a 30-year-old woman on hemodialysis had non-obstructive CTD, underwent uneventful pulmonary artery exploration to remove a migrated vascular stent, but she died later from pancreatitis. [11] Third patient, a 30-year-old man required escharotomy after he sustained burns over 85% of his body, and developed hypotension recalcitrant to volume resuscitation and vasoconstrictors. [12] In this patient a transesophageal echocardiogram identified the presence of CTD with a 15 mmHg gradient between the proximal and distal right atrial chambers. He underwent right heart catheterization with percutaneous balloon dilatation of accessory membrane opening which reduced the gradient to 3 mmHg and the hypotension was corrected. He subsequently developed sepsis and died from multisystem organ failure. Finally, forth patient was a 48-year-old man with congenital spastic quadriplegia, who had a combination of uncorrected tetralogy of Fallot and CTD. [16] He underwent an emergent appendectomy, which was followed by a prolonged stay in the intensive care unit and ultimately died from heart failure.

When CT is diagnosed in adulthood as was in all our patients, it is usually an incidental finding during the evaluation of symptomatology such as a heart murmur, atrial fibrillation, dyspnea, transient ischemic attacks or unexplained hypoxemia. [17] In our series, the most common (50%) malformation associated with CT was ASD (CTD n = 4, CTS n = 2). This is clinically important as these patients may develop paradoxical emboli, [18] or hypoxemia due to the right-to left shunting. For example, our 79-year-old patient [Table 1] with a lifetime asymptomatic CTD developed acute hypoxemia in a setting of metastatic right atrial melanoma, which obstructed the blood flow across the atrial accessory membrane. Since this patient had an ASD in the proximal atrial chamber, the increase in right atrial pressure resulted in a right-to-left shunting, which was resolved with removal of the obstructive tumor and closure of the ASD.

When adult patients with CT become symptomatic, clinical presentation may resemble mitral (CTS) [6] or tricuspid valve stenosis (CTD). [5],[17] Both lesions can lead to an increased strain on the respective atria leading to atrial dilation with atrial fibrillation. [19] As with other stenotic valvular lesions, the CT patients with restrictive membrane poorly tolerate dysrhythmias that diminish diastolic filling time (tachycardia, atrial fibrillation). [20],[21],[22],[23] Other conditions can contribute to cardiac decompensation in patients with CT, specifically hyperdynamic circulatory states which may overburden the blood flow capacity across accessory atrial membrane. In their study Bisinov et al. [12] have reported a man with severe burns who remained hypotensive despite massive fluid resuscitation which did not improve until the aperture of his right intra-atrial membrane was percutaneously dilated to allow for unrestricted blood flow. Furthermore, the obstetric literature provides examples of women with CTS who have developed pulmonary edema and heart failure during late pregnancy and labor. [20],[21],[22],[23],[24],[25],[26],[27] In these women the aperture across the accessory atrial membrane, adequate for a pre-pregnancy hemodynamic state, may become inadequate to accommodate large physiologic increases in hemodynamics associate with uterine autotransfusion after delivery. [28]

Pre-operative treatments

Dependent on presenting symptomatology of CT, a corrective surgery or rarely percutaneous balloon dilatation of intra-atrial aperture [29] may be indicated before major elective surgery. Pre-operatively, all symptomatic patients with CT should be medically treated and special emphasis should be placed on controlling of the fast ventricular rate to promote adequate diastolic filling. Chronic anticoagulant therapy may be needed in those with atrial fibrillation. [30]

Anesthetic management

Optimal heart rate for this type of cardiac lesion is a normal sinus rhythm, which maximizes blood flow across the intra-atrial membrane and facilitates ventricular filling. Conversely, severe bradycardia can also exert adverse effects. In order to maintain optimal blood flow across stenotic lesions (in both the CTD and CTS) it is important to maintain left and right ventricular preloads. However, the margin between fluid under loading and overloading is narrow, as excessive fluids in light of a restricted stenotic valvular lesion can result in cardiac decompensation. Finally, cardiac output is dependent on preserving ventricular contractility and normal pulmonary vascular resistance. Intraoperative assessment of cardiac performance by transesophageal echocardiography would be the most appropriate monitoring modality, with special attention to assessment of myocardial contractility and volume status (via transgastric short axis view of the left ventricle and midesophageal four-chamber view to evaluate the performance of the right ventricle). [12] It has been shown that insertion of a pulmonary artery catheter may be difficult, if not impossible, task in patients with CTD. [12] In patients with CTD and ASD anesthetic management needs to include strategies that prevent increases in right atrial pressures (Trendelenburg positioning, excessive positive end-expiratory pressure, Valsalva maneuver) or avoid development of pulmonary hypertension (hypoxemia, hypercapnia) in order to avoid right-to-left shunting. Finally, in order to prevent paradoxical embolism a bubble-free technique should always be employed when administering intravenous medications or fluids. Rarely, the developement of acute heart failure and severe pulmonary hypertension during increased cardiovascular stress (e.g., peripartum) in patients with CTS may mandate emergent operation. [22] The anesthesiologist must follow the hemodynamic principles used for management of mitral (for CTS)/tricuspid (for CTD) stenosis (avoid tachycardia, avoid hypotension primarily by maintaining euvolemia, and avoid increases in pulmonary vascular resistance from hypoxia, hypercapnia). [31] When neuraxial anesthesia is contemplated, adequate fluid preloading may be needed to minimize the risk of hypotension. In few of the published cases of women with CTS undergoing Cesarean delivery, the anesthetic course was uneventful under epidural, [23],[24] spinal, [25],[32] and general anesthesia. [23],[33]


   Conclusion Top


Asymptomatic CTS and CTD are well tolerated during surgical anesthesia. However, if the patient develops a high cardiac output state (hyperdynamic circulatory state), asymptomatic CT may become symptomatic resembling the clinical picture of either mitral or tricuspid valvular stenosis, therefore the principles of anesthetic management of these cardiac lesions should be followed. Patients with CTD frequently have ASD; therefore usual precautions to prevent the development of paradoxic embolization or shunting should be undertaken.

 
   References Top

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2.Gussenhoven WJ, Essed CE, Bos E. Persistent right sinus venosus valve. Br Heart J 1982;47:183-5.  Back to cited text no. 2
[PUBMED]    
3.Muñoz Castellanos L, Kari Nivon M, García Arenal F, Salinas CH. The pathological remnants of the right sinus venosus valve. A presentation of 6 hearts of the cor tritriatum dexter type. Arch Inst Cardiol Mex 1991;61:293-301.  Back to cited text no. 3
    
4.Trento A, Zuberbuhler JR, Anderson RH, Park SC, Siewers RD. Divided right atrium (prominence of the eustachian and thebesian valves). J Thorac Cardiovasc Surg 1988;96:457-63.  Back to cited text no. 4
    
5.Choudhary D, Sivasankaran S, Venkateshwaran S, Sasidharan B. Cor triatiratum dexter: A rare cause of isolated right atrial enlargement. Pediatr Cardiol 2013;34:198-9.  Back to cited text no. 5
    
6.De Belder MA, Argano V, Burrell CJ. 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.  Back to cited text no. 6
    
7.Bartel T, Müller S, Erbel R. Dynamic three-dimensional echocardiography using parallel slicing: A promising diagnostic procedure in adults with congenital heart disease. Cardiology 1998;89:140-7.  Back to cited text no. 7
    
8.Maroun LL, Graem N, Skibsted L. Fetal cor triatriatum dexter: A report of two cases associated with nuchal edema in early second trimester. Pediatr Dev Pathol 2008;11:59-62.  Back to cited text no. 8
    
9.Alphonso N, Nørgaard MA, Newcomb A, d'Udekem Y, Brizard CP, Cochrane A. Cor triatriatum: Presentation, diagnosis and long-term surgical results. Ann Thorac Surg 2005;80:1666-71.  Back to cited text no. 9
    
10.Jeong JW, Tei C, Chang KS, Tanaka N, Lee SK, Toda H. A case of cor triatriatum in an eighty-year-old man: Transesophageal echocardiographic observation of multiple defects. J Am Soc Echocardiogr 1997;10:185-8.  Back to cited text no. 10
    
11.Aldawoodi NN, Arora H, Kumar PA. Incidental discovery of an unusual right atrial membrane in an adult patient. Ann Card Anaesth 2012;15:309-11.  Back to cited text no. 11
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12.Bisinov EA, Dieter RS, Ballantyne F 3 rd , Wolff MR, Stein JH. Echocardiographic diagnosis and catheter treatment of hypotension caused by cor triatriatum dexter. J Am Soc Echocardiogr 2003;16:897-8.  Back to cited text no. 12
    
13.Eroglu ST, Yildirir A, Simsek V, Bozbas H, Bilgi M, Ozin B, et al. Cor triatriatum dexter, atrial septal defect, and Ebstein's anomaly in an adult given a diagnosis by transthoracic and transesophageal echocardiography: A case report. J Am Soc Echocardiogr 2004;17:780-2.  Back to cited text no. 13
    
14.Lee HM, Sung HS, Kim SY. Anesthetic management of non-cardiac surgery with adult onset type of cor triatriatum sinister: A case report. Korean J Anesthesiol 2011;60:444-8.  Back to cited text no. 14
    
15.Tanaka F, Itoh M, Esaki H, Isobe J, Inoue R. Asymptomatic cor triatriatum incidentally revealed by computed tomography. Chest 1991;100:272-4.  Back to cited text no. 15
    
16.Udovièiæ M, Bioèiæ S, Vincelj J, Crnogorac M, Sakiæ I, Starèeviæ B. Tetralogy of Fallot with cor triatriatum dexter in an adult patient: A case report. Congenit Heart Dis 2013;8:E77-80.  Back to cited text no. 16
    
17.Hoye DJ, Wilson EC, Fyfe DA, Guzzetta NA. Cor triatriatum dexter: A rare cause of neonatal cyanosis. Anesth Analg 2010;110:716-8.  Back to cited text no. 17
    
18.Vicol C, Danov V, Struck E. Paradoxical embolism in the presence of right-to-left shunt due to tricuspid occlusion. Ann Thorac Surg 1995;60:1111-2.  Back to cited text no. 18
    
19.Raggi P, Vasavada BC, Parente T, Prasada S, Sacchi TJ. Uncommon etiologies of atrial fibrillation. Clin Cardiol 1996;19:513-6.  Back to cited text no. 19
    
20.Sentilhes L, Verspyck E, Bauer F, Marpeau L. Management of maternal cor triatriatum during pregnancy. Obstet Gynecol 2004;104:1212-5.  Back to cited text no. 20
    
21.Davlouros PA, Koutsogiannis N, Karatza A, Alexopoulos D. An unusual case of cor triatriatum sinister presenting as pulmonary oedema during labor. Int J Cardiol 2011;150:e92-3.  Back to cited text no. 21
[PUBMED]    
22.Bai W, Kaushal S, Malviya S, Griffith K, Ohye RG. Anesthetic management for resection of cor triatriatum during the second trimester of pregnancy. Int J Obstet Anesth 2010;19:103-6.  Back to cited text no. 22
    
23.Gavand Y, Krausz-Grignard M, Barrucand B, Courtois L, Samain E. Anaesthesia for caesarean section in a pregnant woman with cor triatriatum. Ann Fr Anesth Reanim 2011;30:688-91.  Back to cited text no. 23
    
24.Thorin D, Aebischer N, Landolt J, DeQuay N, Ravussin P, De Grandi P. Acute pulmonary edema in the post partum and cor triatriatum sinistrum. Int J Obstet Anesth 1995;4:113-6.  Back to cited text no. 24
    
25.LeClair SJ, Funk KJ, Goff DR. Cor triatriatum presenting as postcesarean section pulmonary edema. J Cardiothorac Vasc Anesth 1996;10:638-9.  Back to cited text no. 25
    
26.Kaneko M, Haruyama Y, Higo T, Fujisaki S. A case of cor triatriatum diagnosed in pregnancy. Nihon Sanka Fujinka Gakkai Zasshi 1993;45:387-90.  Back to cited text no. 26
    
27.Kokotsakis J, Anagnostakou V, Almpanis G, Paralikas I, Nenekidis I, Kratimenos T, et al. Cor triatriatum presenting as heart failure with reduced ejection fraction: A case report. J Cardiothorac Surg 2011;6:83.  Back to cited text no. 27
    
28.Bojaniæ K, Bursaæ D, Zmijanac J, Duiæ Z, Scavonetto F, Weingarten TN, et al. Isolated cor triatriatum sinistrum and pregnancy: Case report and review of the literature. Can J Anaesth 2013;60:577-83.  Back to cited text no. 28
    
29.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. 29
    
30.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. 30
    
31.Wallace A. Cardiovascular disease. In: Stoelting RK, Miller RD, editors. Basics of Anesthesia. 5 th ed. Philadelphia, PA: Churchill Livingstone; 2007. p. 365-92.  Back to cited text no. 31
    
32.Kanbara T, Miyake T, Murao K, Jomura S, Yamada M, Sato N, et al. Anesthesia for cesarean section in a patient with cor triatriatum. Masui 2005;54:160-2.  Back to cited text no. 32
    
33.Mathew PJ, Subramaniam R, Rawat RS, Kulkarni A. A case of cor triatriatum with pregnancy: An anaesthetic challenge. J Postgrad Med 2004;50:79-80.  Back to cited text no. 33
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Correspondence Address:
Juraj Sprung
Department of Anesthesiology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905
USA
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Source of Support: The Department of Anesthesiology, Mayo Clinic, Rochester, Minnesota., Conflict of Interest: None


DOI: 10.4103/0971-9784.129833

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