Cor triatriatum sinister is a rare condition caused by a membrane within left atrium that separates pulmonary veins from mitral valve (10). While the condition is usually diagnosed at childhood, rare presentation during adulthood is observed when the membrane is incomplete. We report two cases of incomplete cor triatriatum sinister diagnosed during adulthood and review literature for this rare anomaly

Balci, B.

Bilgin, S.

Ercakmak, B.

Hasan Kantarci, U.

Ilhan, E.

Karabag, Y.

Ozen, K.

Sevingil, T.

Sinan Guvenc, T.

English

Via Medica Journals

Folia Morphol.Vol. 71, No. 4, pp. 275–279Copyright © 2012 Via MedicaISSN 0015–5659www.fm.viamedica.plC A S E   R E P O R T275Address for correspondence: B. Ercakmak, MD, Department of Anatomy, Faculty of Medicine, Hacettepe University, Ankara 06100, Turkey,e-mail: burcue@hacettepe.edu.trCor triatriatum sinister: two cases diagnosedin adulthood and a review of literatureT. Sinan Guvenc1, S. Bilgin2, U. Hasan Kantarci3, T. Sevingil4, B. Ercakmak5, E. Ilhan6,K. Ozen1, Y. Karabag1, B. Balci11Department of Cardiology, Faculty of Medicine, Kafkas University, Kars, Turkey2Department of Anatomy, Faculty of Medicine, Kafkas University, Kars, Turkey3Department of Radiology, Faculty of Medicine, Kafkas University, Kars, Turkey4Department of Cardiovascular Surgery, Faculty of Medicine, Kafkas University, Kars, Turkey5Department of Anatomy, Faculty of Medicine, Hacettepe University, Ankara, Turkey6Van Ercis State Hospital, Van, Turkey[Received 6 July 2012; Accepted 11 August 2012]Cor triatriatum sinister is a rare condition caused by a membrane within the leftatrium that separates the pulmonary veins from the mitral valve. While thecondition is usually diagnosed in childhood, a rare presentation during adult-hood is observed when the membrane is incomplete. We report two cases ofincomplete cor triatriatum sinister diagnosed during adulthood and review theliterature for this rare anomaly. (Folia Morphol 2012; 71, 4: 275–279)Key words: cor triatriatum sinister, left atrium, three dimensionalechocardiography, transesophageal echocardiography, cardiaccomputerised tomographyINTRODUCTIONCor triatriatum sinister (CTS) is a rare congenitalanomaly that is caused by a fibromuscular mem-brane dividing the left atrium (LA) into two cham-bers. Communication between the pulmonary veinsand anterior chamber is provided by fenestrationson the membrane. As a result of atrial flow obstruc-tion, symptoms of congestive heart failure developduring infancy and childhood. When the membraneis incomplete or has large fenestrations, the condi-tion is diagnosed at adulthood due to lack of symp-toms.In this article, we report two cases of incompleteCTS that are diagnosed in adulthood and review theliterature for this congenital anomaly.CASE 1A 31-year-old female patient with a complaintof shortness of breath during exercise was admit-ted to our institution. She had given birth to onechild, and her previous medical records did not showany chronic medical condition. During her pregnan-cy, she had experienced mild shortness of breaththat began on the 12th gestational week, and thesesymptoms had abated spontaneously after labour.Her family history was unremarkable. On physicalexamination, her blood pressure was 100/60 mm Hgand heart rate was 70 bpm. No abnormal findingswere present during auscultation of cardiac andlung fields. Likewise, there was no jugular disten-tion, hepatomegaly or pretibial oedema. Her elec-trocardiogram showed normal sinus rhythm witha rate of 70 bpm. Amplitude, duration, and axis ofP waves and duration of PR interval were within nor-mal ranges, and there were no signs of right ven-tricular enlargement (Fig. 1A). Her chest X-ray wasalso normal. As she had unexplained dyspnoea, tran-sthoracic echocardiography was performed. Two-276Folia Morphol., 2012, Vol. 71, No. 4-dimensional (2D) echocardiography showed a par-tial membrane in LA, and colour Doppler recordingsshowed that flow was present at the fenestratedpart. Three-dimensional (3D) echocardiographic re-construction of LA revealed a membrane in LA thatseparated the pulmonary veins from the mitral valve.Continuity of the membrane was complete at theposterior aspect, while a large fenestration waspresent on the anterior side, which allowed com-munication between two separate chambers (Fig. 2).Two-dimensional transesophageal echocardiographyallowed measurement of the gradient between thetwo chambers separated by the membrane (Figs. 3, 4).As the pressure gradient was negligible and thepatient had minimal symptoms during treadmillexercise test, surgical intervention was not con-sidered.CASE 2A 27-year-old male patient admitted to our in-stitution with a complaint of chest pain that beganone month previously. He was physically active, hadno chronic disorders, and he had been smoking onepack/day for ten years. His family history was unre-markable. On physical examination, his blood pres-sure was 110/70 mm Hg and heart rate was 65 bpm.Auscultation of cardiac and lung fields showed nor-mal findings, and no signs of right ventricular fail-ure were noted. His electrocardiogram showed si-nus rhythm with normal P waves and PR durationwith a normal QRS axis, and signs of right ventricu-lar enlargement were not present (Fig. 1B). Two-di-mensional echocardiography revealed a membranein the LA, dividing it into two chambers. As his api-cal window was inadequate for a complete 3DFigure 1. Electrocardiograms of Case 1 (A) and Case 2 (B). Both cases had no specific findings for cor triatriatum. An early repolarisationpattern is noted in (A).Figure 2. Three-dimensional transthoracic echocardiographyview of the first patient, showing fibromuscular membrane (aster-isk). Black arrowheads point to the opening (foramen) on themembrane. The membrane is shown as seen from the left ventri-cle. The mitral valve is cropped for a clear view of the membrane.Figure 3. Two-dimensional transesophageal view of the first pa-tient in lower oesophageal four chamber view. The membrane isseen from the long axis of the left ventricle (arrowheads). Posteriorchamber is shown with an asterisk, while the anterior chamber isshown with a dagger sign (†).†**277T. Sinan Guvenc et al., Cor triatriatum review and case reportechocardiographic reconstruction, cardiac comput-ed tomography (CT) was planned. On cardiac CT, anincomplete membrane separating the atrium intoa posterior and an anterior chamber was observed (Fig. 5).All pulmonary veins were in communication with theposterior chamber, while continuation of the mem-brane was interrupted at the anterior aspect of theLA. As he had no symptoms compatible with out-flow obstruction, a treadmill exercise test was per-formed for hidden dyspnoea. However, dyspnoeawas not observed at maximal exercise, and the testwas terminated at the 12th minute due to fatigue.As he remained asymptomatic after the treadmilltest and had an incomplete membrane, surgery wasnot considered and follow-up was planned.DISCUSSIONCor triatriatum sinister is a rare congenital car-diac anomaly [10]. This condition is a result of fail-ure of embryologic common pulmonary vein incor-poration into the back of the LA [5, 17]. As a result,the LA is divided into two chambers by a fibromus-cular membrane [11]. The two cavities, positionedposterior superior and anterior inferior, are anatom-ically and functionally separated [5, 11].The incidence of cor triatriatum is 0.1–0.4% ofpatients with congenital heart disease [11]. This en-tity can present in classical or atypical form [9]. Whilethe classical form is an isolated thin membrane with-in the LA, the atypical form is accompanied by oth-er cardiac anomalies [9].In 1868 Church first described cor triatriatum [1].After the postmortem description of Church, Milleret al. [14] described this entity by angiography atthe Mayo Clinic in 1964. In 1984 Ostman-Smith etal. [15] demonstrated cor triatriatum by echocar-diography for the first time.The classical morphology has been well describedin recent years. The volume of the proximal cham-ber is larger than the distal chamber. Also, the wallof the proximal chamber is thicker, and the pres-sure is higher in the proximal chamber than the dis-tal chamber [10]. The posterosuperior chamber re-ceives the pulmonary veins and the anteroinferiorchamber is continuous with the mitral valve [18].In cor triatriatum the right and left pulmonaryveins join the posterior-superior part of the LA form-ing an accessory chamber [7, 10]. This chamber, whichleads pulmonary venous obstruction, communicateswith the LA through a restricted opening [7, 17]. Cortriatriatum is an anatomical barrier of pulmonaryvenous drainage and a rare, surgically correctablecause of pulmonary arterial hypertension [7].Persistent left superior vena cava (LSVC) is report-ed as the most common coexisting abnormality ob-served with cor triatriatum [9, 10]. Kaneko et al. [9]hypothesised that CTS is caused by a failure of LSVCto disappear during foetal life. As LSVC drains to thecoronary sinus, dilation of the coronary sinus couldlead to a bulging of the posterior atrial wall. Thisbulging of the atrial wall is considered as the causeof obstruction of the LA outflow [9].Figure 4. Two-dimensional transesophageal view of the first pa-tient at mid oesophagus showing the relationship between themembrane (arrowheads) and the orifices of the left superior andinferior pulmonary veins. Doppler flow shows pulmonary flow inleft inferior pulmonary vein (asterisk).Figure 5. Axial maximum intensity projection computed tomogra-phy image demonstrating the membrane (arrowheads) dividingthe left atrium into two distinct chambers and the left upper pul-monary vein (asterisk) draining into the posterior chamber nearthe communication of the membrane to the atrial wall.*278Folia Morphol., 2012, Vol. 71, No. 4One of the variations coexisting with CTS isanomalous pulmonary venous return [10]. Anoma-lous connection of pulmonary veins can be totalor partial. Partial anomalous pulmonary venousreturn can present as right-sided or left-sidedanomalous pulmonary venous return [10, 17]. Inright-sided partial anomalous pulmonary venousreturn, right-sided veins join with each other todrain the anomalous proximal chamber, while left--sided pulmonary veins drain into the LA. In left--sided partial anomalous pulmonary venous re-turn, left-sided pulmonary veins join to form a leftvertical vein. This left vertical vein connects to theleft innominate vein. The remaining pulmonaryveins connect into the anomalous proximal cham-ber as a component of this entity. In some vari-ants, partial anomalous venous return consists ofright pulmonary veins draining into the proximalchamber and left veins connecting to the coro-nary sinus [10].Other cardiac anomalies that accompany CTS areventricular septal defect, coarctation of aorta, te-tralogy of Fallot, atrioventricular septal defect, andmitral stenosis [10, 13, 18]. Park et al. [16] reportedan extremely rare clinical presentation as cerebralinfarct due to mitral stenosis in a patient with CTS.Extracardiac manifestations that may be observedwith CTS include asplenia and polysplenia [13].Depending on the severity of obstruction, CTSmay be symptomatic or asymptomatic. Symptomsare a result of outflow obstruction and include dys-pnoea, orthopnoea, cyanosis, haemoptysis, andchest discomfort. When the obstruction is severe,CTS is usually diagnosed during infancy or adulthood.Some cases may remain asymptomatic or minimallysymptomatic during lifetime [16, 18]. Rarely, ob-structive symptoms may became manifest duringadulthood [8]. In our first case, symptoms attribut-able to CTS developed during pregnancy, while thepatient was minimally symptomatic at diagnosis. Anincrease in blood volume was considered as respon-sible for her symptoms during pregnancy, as pre-viously reported by Davlouros et al. [2]. Our secondcase was asymptomatic.Electrocardiography is often normal in CTS pa-tients, especially when obstruction is not present.When there is significant obstruction, electrocardio-gram may show signs of right ventricular hypertro-phy, such as right axis deviation and prominent R orR’ wave in V1, which may develop due to associat-ed pulmonary hypertension [19]. In adults, CTSmight present with atrial fibrillation (AF), and re-moval of the membrane could abolish the arrhyth-mia [20]. Whether this is a direct consequence ofthe membrane or a result of atrial stretch is un-known. Well-known causes of AF, such as accompa-nying mitral regurgitation, could also play a role inthe development of AF when present [21]. Otherreported arrhythmias include atrial tachycardia andectopic atrial rhythm with abnormal P waves [22,23]. Interestingly, in the latter case, abnormalP waves disappeared after the operative removal ofthe membrane, and the authors demonstratedHCN4 + cells with electrical automaticity in the re-moved material.Cor triatriatum sinister is a complicated cardiacmalformation due to associated anomalies and ob-struction at the level of the LA. Haemodynamic pa-rameters and anatomical structures of the heart canbe well visualised by 2D transthoracic and transe-sophageal echocardiography [3]. Three-dimensionalechocardiography gives more reliable informationabout anatomical features than 2D echocardio-graphy [6]. Combined use of 3D echocardiography,transesophageal echocardiography, and Dopplerassessment of stenosis severity allows a completeevaluation of CTS [6]. Additional modalities thatcould be used for diagnosis of CTS include cardiacCT and cardiac MRI [4, 12]. Both techniques allowa complete anatomic evaluation of the lesion whenechocardiographic assessment is unavailable or in-adequate. In our first case, 3D transthoracic echocar-diography was able to completely visualise the mem-brane, while additional haemodynamic data wasobtained with transesophageal echocardiography.In the second case, cardiac CT was used to evaluateatrial anatomy as transthoracic echocardiographyviews were inadequate.Surgical removal of the membrane abolishesobstructive symptoms in patients. Urgent opera-tion is needed when the membrane is completeand the proximal chamber communicates with theright atrium via an atrial septal defect [10]. Oper-ation is also indicated when the opening is restric-tive and obstruction is manifested, as high deathrates are observed in the infancy period with CTS.In adulthood, surgical removal is generally indi-cated if obstructive symptoms are present. How-ever, additional complications including AF ormitral regurgitation could be observed in adult-hood, and could even present symptom of CTS[18]. Our cases were asymptomatic or minimallysymptomatic at peak exercise, so surgical remo-val was not considered.279T. Sinan Guvenc et al., Cor triatriatum review and case reportCONCLUSIONSCor triatriatum sinister can be observed in adult-hood when the dividing membrane is incomplete.Echocardiography and CT can readily demonstratethe membrane, while 3D reconstruction allows com-plete anatomical evaluation. 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Cor triatriatum sinister: two cases diagnosed in adulthood and a review of literature

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Cor triatriatum sinister: two cases diagnosed in adulthood and a review of literature

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