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Cortriatriatum in Adults Disease Three New Cases and a Brief Review

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Cortriatriatum in Adults Disease Three New Cases and a Brief Review 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
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