Congenital Heart CorTriatriatum in Adults Disease Three New Cases and a Brief Review

Qiang Chen, MD We report 3 cases of cor triatriatum that were diagnosed late, in the 4th and 5th decades Soma Guhathakurta, MCh of life. The presentations of these 2 men and 1 woman varied substantially, both in Ganesh Vadalapali, MCh Zubin Nalladaru, MCh anatomic and symptomatic aspects. The woman had an associated complex congenital Ronald Newman Easthope, anomaly, which is not uncommon in cases of cor triatriatum. We present our cases, MB ChB, FRACP along with a review of this rare congenital cardiac anomaly. (Tex Heart Inst J 1999; Ashok K. Sharma, FRACS 26:206-10)

C lassical cor triatriatum or cor triatriatum sinister is a rare congenital car- diac anomaly in which a common pulmonary venous chamber (proximal chamber) is separated from the left atrium (distal chamber) by a fibro- muscular septum. In most cases, the 2 chambers communicate through 1 or more openings. This malformation is usually isolated, but it may be associated with other cyanotic or acyanotic congenital heart diseases. The clinical manifestations of cor triatriatum depend upon the size of the open- ing in the septum. In most patients, the opening is severely restrictive and about 75% of those born with classical cor triatriatum die in infancy.' In rare cases, the onset of clinical manifestations may be delayed if the opening is large. We report 3 cases of cor triatriatum in adults who came to us with variable physical presenta- tions and symptoms, along with complex cardiac morphology. In each case, surgi- cal management differed due to varied anatomic abnormalities. Case Reports Patient 1 In March 1992, a 48-year-old man with a history ofprogressive dyspnea and palpi- tation was referred from an outlying hospital for further investigation. Since 1975, he had been admitted 3 times to the same hospital with congestive cardiac failure. In each instance, atrial fibrillation had preceded the development ofcardiac failure, and the restoration of sinus rhythm had been sufficient to relieve his symptoms and return him to apparent good health. Surprisingly, the patient had a good qual- ity of life between attacks, and had been active. During these long periods of sinus rhythm, his enlarged heart would come down considerably in size. At the time of presentation in 1992, surgery did not appear to be indicated. Key words: Adult; cor The patient's condition was reevaluated in 1994. Physical examination revealed a triatriatum/diagnosis; cor rapid, irregular pulse and elevated jugular venous pressure (JVP) with prominent v triatriatum/surgery; heart atrium/abnormalities; heart waves. On auscultation, there was a loud P2 and a systolic murmur at the left defects, congenital sternal edge. Electrocardiography showed atrial fibrillation and right axis deviation. Chest radiography revealed massive cardiomegaly and a prominent pulmonary vas- From: The Department of culature. Transthoracic echocardiography showed large atria with a clear mem- Cardiothoracic Surgery, brane across the left atrium; the left ventricle was enlarged and global hypokinesis Wellington Hospital, was present. Transesophageal echocardiography confirmed the diagnosis of cor tri- Wellington, New Zealand atriatum (Figs. 1 and 2). Subsequently, cardiac catheterization showed markedly depressed left-ventricular Address for reprints: contraction, membrane across the left atrium, a small patent foramen ovale (PFO), Ashok Kumar Sharma, FRACS, Department of Cardiothoracic normal pulmonary venous drainage, and mild mitral regurgitation. The biopsy of Surgery, Wellington Hospital, ventricular muscle showed nonspecific cardiomyopathy. Wellington, New Zealand The patient was prepared for surgical correction. Intraoperatively, we found marked cardiomegaly, a dilated right ventricle, a mildly tense pulmonary artery, C 1999 by the Texas Hearts and a small PFO of about 3 mm in diameter, above the 1st membrane just prox- Institute, Houston imal to the mitral valve. This membrane had an opening of 2 mm, which was

206 CorTriatriatum in Adults Volume 26, Number 3, 1999 Fig. 1 Patient 1: transesophageal echocardiographic view of Fig. 3 Patient 1: perioperative transesophageal echocardio- discrete membrane (arrows) in left atrium. gram confirming absence of membrane in left atrium after excision. LA = left atrium; LV = left ventricle; M = membrane; RA = right atrium LA = left atrium; LV = left ventricle; RA = right atrium; RV = right ventricle

heart, together with atrial fibrillation. Following this, his condition necessitated 1 admission in 1990, with symptoms and signs of pulmonary edema and systemic venous congestion. In January 1994, this patient, now 40 years of age, was admitted to a peripheral center for evaluation of severe pulmonary hypertension and suspected progressive pulmonary disease. Within the 1st week of hospitalization, he demonstrated increased dyspnea, hemoptysis, and pulmonary edema, so he was placed on an antifail- ure regimen and was transferred to our care in late Fig. 2 Patient 1: color- flow Doppler transesophageal echo- February 1994. cardiographic view of the membrane (M) in relation to the Examination of this middle-aged man in respira- mitral valve and to the patent foramen ovale. tory distress showed an irregular pulse rate of 100 beats/minute, blood pressure of 105/80 mmHg, and JVP of 8 cm from the sternal angle. Auscultation of the heart revealed a normal 1st heart sound, a loud situated close to the posteromedial commissure. 2nd heart sound at the pulmonary area, and a grade Another membrane, above the PFO, separated the 2/4 systolic murmur along the left sternal edge. pulmonary veins from the lower chamber, which Upon auscultation ofthe chest, there were bronchial was fenestrated and calcified. After enlarging the breath sounds over the left mid zone, a pleural rub PFO, we excised both membranes completely, under over the left axilla, and bilateral basal rales. Cor tria- cardiopulmonary bypass; we then closed the PFO triatum was suspected when an extra septum was directly. Perioperative transesophageal echocardiog- seen in the left atrium during transthoracic echocar- raphy showed normal anatomy of the left atrium diography (Fig. 4); subsequently, transesophageal after this excision (Fig. 3). Postoperatively, there was echocardiography confirmed this diagnosis. marked improvement ofleft-ventricular function, as The findings of preoperative angiography in early indicated by echocardiography and by the patient's March were as follows: pulmonary arterial pressure, tolerance of exercise. There was no evidence of 92/52 mmHg (mean, 60 mmHg); aortic pressure, mitral regurgitation. 87/52 mmHg (mean, 73 mmHg); and a marked gradient across the membrane. The patient was Patient 2 scheduled for surgical correction. Intraoperatively, A man (then age 35) was 1st evaluated at another we observed massive cardiomegaly with dilatation of center in June of 1989 for exertional dyspnea of 2 the pulmonary artery, right ventricle, and right atri- weeks' duration, at which time the diagnosis was um. There was also a thin-walled membrane in the dilated cardiomyopathy affecting primarily the right left atrium that almost completely separated the left

Texas Heart Institutejournal CorTriatriatum in Adults 207 I Fig. 5 Patient 3: this transthoracic echocardiogram indicates partial atrioventricular canal defect. Fig. 4 Patient 2: this transthoracic echocardiogram was the 1st indication of an extra septum in the left atrium, a finding Avc = atrioventricular canal; LA = left atrium; LV = left that was confirmed by transesophageal echocardiography. ventricle M = membrane

catheterization revealed normal pulmonary venous drainage, a partial AV canal defect, mitral and tri- atrial cavity from the pulmonary veins, except for a cuspid regurgitation, and left SVC. There was a 2-mm-diameter orifice, calcified at its margins, large left-to-right shunt. which provided a small communication. Periop- On 19 January, the patient was transported for erative transesophageal echocardiography was per- surgery. Intraoperatively, we found marked cardio- formed to confirm the findings. The membrane megaly: dilatation of the pulmonary artery, right was excised. The pulmonary arterial pressure ventricle, and right atrium. There was a large left dropped significantly and the patient had a good SVC, the end of which ballooned towards the left postoperative recovery. atrial appendage. There was no communication be- tween the left SVC and the right SVC. Upon open- Patient 3 ing the heart, we noted that the atrial septum was In 1994, a 36-year-old woman presented for follow- completely absent and that a partial AV canal defect up evaluation of a long-standing asymptomatic was further complicated by the presence of a cleft cardiac condition of uncertain nature. She had a anterior mitral leaflet. There was no ventricular sep- cardiac murmur that had 1st been noted at the age tal defect. Pulmonary veins could not be seen in the of 18 months. In 1964, when she was 6 years ofage, left atrium: instead, they were draining into a sepa- cardiac catheterization for evaluation of this mur- rate chamber that was connected to the left atrium mur had revealed atrioventricular (AV) canal defect, by means of a 1-cm hole at its posterosuperior as- mitral regurgitation, and left superior vena cava pect. This was a case of cor triatriatum in which (SVC). Her physical growth and development had there was a common atrium with a partial AV canal been normal. Apart from an attack of rheumatic defect, along with a left SVC that drained into the fever at the age of 14, she had remained well and left atrium behind the left atrial appendage, due to was lost to follow-up for a number ofyears (during an unroofed coronary sinus. which time she had an uneventful pregnancy). We sutured in place an intraatrial baffle in order Upon her examination in January 1994, there was to direct flow from the left SVC into the right atri- neither cyanosis nor clubbing, and her arterial pulse um (after the membrane between the pulmonary and JVP were normal. Auscultation revealed fixed veins and left atrium had been excised). The patient splitting of the 2nd heart sound without accentua- did well postoperatively, except for a brief period of tion of P2 and a pansystolic murmur at the apex. conduction abnormality. Postoperative echocardiog- Electrocardiography showed sinus rhythm, left- raphy showed no membrane in the left atrium and axis deviation, and a prolonged PR interval. Chest no residual atrial septal defect (Fig. 6). At the time radiography had shown mild cardiomegaly, with of discharge, the patient's electrocardiogram showed slight prominence of the pulmonary arteries. A a prolonged PR interval with right bundle branch transthoracic echocardiographic study confirmed block and normal sinus rhythm with left-axis devia- partial AV canal defect (Fig. 5), mitral regurgita- tion. She had some residual mitral regurgitation. tion, and tricuspid regurgitation, and was sugges- She was discharged on a regimen of warfarin be- tive ofa degree ofpulmonary hypertension. Cardiac cause of the large pericardial patch.

208 CorTriatriatum in Adults Volume 26, Number 3, 1999 ber: there are diaphragmatic, hour-glass, and tubu- lar types. Cor triatriatum most commonly manifests itself in infancy or childhood, but in some cases it does not appear until later. The most common symptoms pre- sent in adults are dyspnea, hemoptysis, and orthop- nea,10 but cor triatriatum can be asymptomatic and the diagnosis can be incidental." Cardiac murmurs are the most significant finding during physical examination.12-16 The proposed reasons for the late conversion of asymptomatic cor triatriatum to a symptomatic state are thought to be 1 or more of l the following precipitating factors: fibrosis and cal- Fig. 6 Patient 3: postoperative transthoracic echocardiogram cification of the orifice in the anomalous sep- shows no membrane in the left atrium and no residual atrial tum3,7'10'12; the development ofmitral regurgitation, septal defect. or ofa condition caused by mitral regurgitationIi4,17; LA = left atrium; LV = left ventricle; RA = right atrium; and the development of atrial fibrillation.14,15,18 RV = right ventricle All 3 ofour patients had 1 or more ofthese complica- tions, yet Patient 3 never developed symptoms. Cor triatriatum is frequently associated with other Discussion cardiac abnormalities.' In adults, the most common abnormalities associated with cor triatriatum are Cor triatriatum is a rare congenital cardiac anomaly. mitral regurgitation,"11"4'17 atrial secundum septal The incidence has been variously reported as O.1%2 defect, and the presence of left superior vena cava to 0.4%.3 The embryologic basis of this anomaly with unroofed coronary sinus.' Less common asso- remains controversial. The 3 main theories involve ciated abnormalities are aortic regurgitation with malseptation, malincorporation, and entrapment. dissecting aneurysm,'4 and an anomalous partial The malseptation hypothesis was put forward by pulmonary venous connection.'9 Our Patient 3 had Fowler4 in 1881. He proposed that the septum sub- a complex congenital cardiac anomaly (left superior dividing the left atrium was an abnormal growth of vena cava with unroofed coronary sinus), Patient 2 septum primum. The malincorporation hypothesis had an anomalous pulmonary venous communica- suggests that cor triatriatum is the result of incom- tion, and Patient 1 had a small PFO between the 2 plete incorporation of the embryonic common anomalous membranes. pulmonary vein into the left atrium.5 This theory, It has been observed that most cases of cor tria- however, fails to explain the presence of fossa ovalis triatum are at 1st misdiagnosed.""2,17'8 The most in the proximal chamber and the presence of atrial common misdiagnosis is mitral valve disease-espe- muscle fibers in the proximal chamber wall. Van cially mitral stenosis, because cor triatriatum mimics Praagh and Corsini6 proposed the entrapment the clinical, radiologic, and electrocardiographic hypothesis, which holds that the left horn of the manifestations of mitral stenosis. The other com- sinus venosus entraps the common pulmonary vein mon misdiagnoses are primary pulmonary hyper- and thereby prevents its incorporation into the left tension and the progressive pulmonary conditions atrium. As a variation on the entrapment theory, that cause pulmonary hypertension.7,20-23 Gharagozloo and colleagues7 suggest that cor tria- It is possible that diagnostic accuracy has in- triatum arises through the impingement ofa promi- creased recently, in step with the development of nent or persistent left superior vena cava on the left such investigative procedures as transthoracic and atrium, which induces the production of an abnor- transesophageal echocardiography and cardiac cath- mal left atrial membrane. eterization. This would account for a recent increase Loeffler,8 in 1949, classified cor triatriatum into 3 in the reported frequency ofcor triatriatum.'2,2'-23 groups on the basis of the number and size of the An important aspect of surgical procedure in cor- openings in the anomalous membrane into the left recting cor triatriatum and its associated anomalies atrium: Group I = no opening; Group II = 1 or more is to delineate (intraoperatively) the complex anato- small openings; and Group III = a wide opening. my at the outset, before proceeding to correction. In Patients with Group III cor triatriatum, he main- order to do that, one should approach the largest tained, may reach late adulthood. Marin-Garcia and atrial chamber on the right side 1st. If it is not the colleagues9 classified cor triatriatum on the basis of right atrium, then the original right atrium should the anatomic shape ofthe accessory left atrial cham- be opened to ensure a thorough examination of the

Texas Heart Institutejournal CorTriatriatum in Adults 209 patient's morphology. The left atrial chambers and 12. de Belder MA, Argano V, Burrell CJ. Cor triatriatum sinis- their pathologic membranes must ultimately be ap- ter, not mitral stenosis, in an adult with previous Sydenham's chorea: diagnosis and preoperative assessment proached through the interatrial septum and excised. by cross sectional echocardiography. Br Heart J 1992;68:9- Subsequently, the interatrial septum is closed with a 11. pericardial baffle or patch, in accordance with the size 13. Ikegaki J, Kimura Y, Mikawa K, Obara H, Oka Y. Cor tria- and nature of the defect, as we have done in these 3 triatum in an adult patient: implications of intraoperative cases with results. TEE for anesthetic management. J Cardiothorac Vasc gratifying Anesth 1992;6:215-7. 14. Beller B, Childers R, Eckner F, Duchelle R, Ranniger K, References Rabinowitz M. Cor triatriatum in the adult. Complicated by mitral insufficiency and aortic dissection. Am J Cardiol 1. Kirklin JW, Barratt-Boyes BG, editors. Cardiac surgery: 1967;19:749-54. morphology, diagnostic criteria, natural history, techniques, 15. Leung WH, Wong CK, Lau CP, Cheng CH. Cor triatria- results, and indications. 2nd ed. New York: Churchill tum masked by coexisting COPD in an adult. Chest 1989; Livingstone 1993:678. 96:676-8. 2. Niwayama G. Cor triatriatum. Am Heart J 1960;59:291- 16. Lengyel M, Arvay A, Biro V. Two-dimensional echocardio- 317. graphic diagnosis of cor triatriatum. Am J Cardiol 1987; 3. Jorgensen CR, Ferlic RM, Varco RL, Lillehei CW, Eliot RS. 59:484-5. Cor triatriatum. Review ofthe surgical aspects with a follow- 17. Hogue CW Jr, Barzilai B, Forstot R, Shuman TA, Cox JL. up report on the first patient successfully treated with sur- Intraoperative echocardiographic diagnosis of previously gery. Circulation 1967;36:101-7. unrecognized cor triatriatum. Ann Thorac Surg 1992;54: 4. Fowler JK. Membranous band in the left auricle. Trans 562-3. Pathol Sco London 1881;33:77-94. 18. Schluter M, Langenstein BA, Thier W, Schmiegel WH, 5. Parsons CG. Cor triatriatum: concerning the nature of an Krebber HJ, Kalmar P, et al. Transesophageal two-dimen- anomalous septum in the left auricle. Br Heart J 1950;12: sional echocardiography in the diagnosis of cor triatriatum 327-38. in the adult. J Am Coll Cardiol 1983;2:1011-5. 6. Van Praagh R, Corsini I. Cor triatriatum: pathologic anato- 19. Papagiannis J, Harrison JK, Hermiller JB, Harding MB, my and a consideration of morphogenesis based on 13 post- Armstrong BE, Ungerleider RM, et al. Use ofballoon occlu- mortem cases and a study of normal development of the sion to improve visualization of anomalous pulmonary pulmonary vein and atrial septum in 83 human embryos. venous return in an adult with cor triatriatum. Cathet Am Heart J 1969;78:379-405. Cardiovasc Diagn 1992;25:323-6. 7. Gharagozloo F, Bulkley BH, Hutchins GM. A proposed 20. Jacobstein MD, Hirschfeld SS. Concealed left atrial mem- pathogenesis of cor triatriatum: impingement of the left brane: pitfalls in the diagnosis of cor triatriatum and supra- superior vena cava on the developing left atrium. Am Heart valve mitral ring. Am J Cardiol 1982;49:780-6. J 1977;94:618-26. 21. Oglietti J, Cooley DA, Izquierdo JP, Ventemiglia R, 8. Loeffler E. Unusual malformation of the left atrium: pul- Muasher I, Hallman GL, et al. Cor triatriatum: operative monary sinus. Arch Pathol 1949;48:371-6. results in 25 patients. Ann Thorac Surg 1983;35:415-20. 9. Marin-Garcia J, Tandon R, Lucan RV Jr, Edwards JE. Cor 22. Ostman-Smith I, Silverman NH, Oldershaw P, Lincoln C, triatriatum: study of 20 cases. Am J Cardiol 1975;35:59-66. Shinebourne EA. Cor triatriatum sinistrum. Diagnostic fea- 10. McGuire LB, Nolan TB, Reeve R, Dammann JF Jr. Cor tri- tures on cross sectional echocardiography. Br Heart J 1984; atriatum as a problem of adult heart disease. Circulation 51:211-9. 1965;31 :263-72. 23. Ludomirsky A, Erickson C, Vick GW 3d, Cooley DA. 11. Tanaka F, Itoh M, Esaki H, Isobe J, Inoue R. Asymptomatic Transesophageal color flow Doppler evaluation of cor tria- cor triatriatum incidentally revealed by computed tomogra- triatum in an adult. Am HeartJ 1990;120:451-5. phy. Chest 1991;100:272-4.

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