Transesophageal Echocardiographic Detection of Cardiac Embolic Source in Cor Triatriatum Complicated by Aortic Saddle Emboli
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Brucellosis in patients with heart disease: When should endocardi- Daoud M: Endocardite Brucelliettne sur valve de stam aoitiqiie. tis be diagnosed? Cardiology 1990;77:313-3 I7 ArchMulCoeur kiss 1991:84:269-271(in French) Clin. Cardiol. 20, 294-296 (1997) Transesophageal Echocardiographic Detection of Cardiac Embolic Source in Cor Triatriatum Complicated by Aortic Saddle Emboli TSLJEI-YUEN HUANG,M.D., PING-HSIUNSUNG, M.D. Department of Internal Medicine, Father Fox Memorial Hospital, Tainan, Taiwan, R.O.C. Summary: Transesophageal echocardiographic findings are Key words: cor triatriatum, aortic saddle emboli, spontaneous described in a case of cor triatriatum with atrial fibrillation echo contrast complicated with cerebral and aortic saddle emboli. A sponta- neous echo contrast confined to the dilated proximal chamber of the left atrium was noted and was presumed to be the em- Introduction bolic source in cor triatriatum. Cor triatriatum is an uncommon congenital heart disease in adults. It consists of an abnormal fibromuscular membrane that separates the left atrium into the proximal and distal cham- bers. The clinical presentation usually is pulmonary conges- Address for reprints: tion secondary to the obstruction produced by the intra-atrial Tsuei-Yuen Huang, M.D. membrane. Cerebral emboli have been reported to be one of Father Fox Memorial Hospital the clinical manifestations in cor triatriatum; however, they are Chung Hwa Road encountered rather rarely, and the embolic source in this dis- Yung Kang City order is obscure. This report describes a case of cor triatriatum Tainan, Taiwan, 71010, R.O.C. in an adult, complicated by cerebral and aortic saddle emboli; Received: June 12, 1995 transesophageal echocardiographic findings of the embolic Accxptdbecember 22, 1995 source are also described. T.-Y. Huang and P.-S. Sung: Cardiac embolic source in cor triatriatum detected by TEE 295 Case Report The patient, a 65-year-old woman, had a history of exer- tional dyspnea and high blood pressure. On the day of adrms- sion, she suffered a sudden loss of consciousness after dinner. Upon admission, the patient was unable to be aroused; physi- cal examination revealed blood pressure 136/88mmHg, heart rate 92 beatshin, normal temperature, absence of arterial pulse in both femoral arteries, basal rales over the bilateral pos- terior chest,variablefirst heart sound, a grade WVI pansystolic murmur over the left sternal border and a grade WVI diastolic rumbling murmur over the apex, coldness and cyanosis of both lower extremities, and paralysis of the right amand both legs. Electrocardiography revealed atrial fibrillation, left hy- pertrophy, and ST-T changes. Transthoracic two-dimensional echocardiography and color-coded Doppler study revealed a cor triatriatum with dilated left atrium and mild-to-moderate mitral regurgitation (Fig. 1). The orifice area of the infra-atrial Rc. 1 Transthoracic echocardiography showing an intra-atrial membrane estimated by pressure halftime was 1.2cm2. Trans- membrane the separating the left atrium into proximal and distal chambers in the apical long-axis view. Dist LA = distal chamber of esophageal echocardiography revealed spontaneous echo the left atrium, prox LA =proximal chamber of the left atrium, LV = contrast in the proximal chamber of the left atrium (Fig. 2). left ventricle. Immediate brain computed tomography scan revealed brain swelling only. Emergency aortogram was performed due to suspicion of aortic occlusion. It demonstrated total occlusion of the abdominal aorta at the level of the second lumbar of the ments: the proximal chamber which receives pulmonary spine (Fig. 3). Emergency thrombectomy was performed de- veins and the distal chamber which almost always contains spite severe neurological problems. The postoperative course left atrial appendage and fossa ovales.' Most patients are di- was progressive deterioration to circulatory and, finally, cen- agnosed at pediatric age or in young adulthood with a presen- tral failure. tation of associated congenital heart disease or pulmonary congestion. Few patients remain well until adulthood even Discussion with marked stenosis of the orifice of the intra-atrial mem- brane. Progressive stenosis of the orifice of the membrane, Cor triatriatum is a rare congenital cardiac abnormality development of mitral regurgitation, and atrial fibrillation lat- with a prevalence rate of about 0.1% among all congenital er in life are supposed to precipitate the initial presentation of heart diseases. It usually consists of an abnormal fibromuscu- this disorder in adulthood. The embolic presentation is rare lar membrane that partitions the left atrium into two compart- both in children and adults. In a review of seven cases of cor FIG.2 Transesophageal echocardiography showing swirling spontaneous echo contrast in the proximal chamber (F'rox C) of the left atrium in panels A and B. RA =right atrium, memb = membrane, SEC = spontaneous echo contrast. Other abbreviations as in Figure I. 296 Clin. Cardiol. Vol. 20, March 1997 junct to transthoracic echocardiography in the assessment of cor triatriatum. In a review of 1,575 cases of aortoarterial embolism, the heart was listed as the embolic source in 89% of patients. Heart diseases leading to peripheral arterial emboli include rheumatic heart disease, atrial fibrillation, atherosclerotic heart disease with or without myocardial infarction, infective endocarditis, and prosthetic valves? Other unusual etiologic factors include mural thrombi within aortic aneurysm, athero- matous plaques, paradoxical emboli in the presence of intrac- ardiac shunt, atrial septa1 aneurysm, and pulmonary vein thrombosis. In the era of transesophageal echocardiography, the presence of spontaneous echo contrast in the left atrium has been shown to be associated with increasing risk of sys- temic thromboembolic events. Our case experience docu- mented that the spontaneous echo contrast in the proximal FIG.3 Aortogram showing total occlusion of the abdominal aorta chamber of the left atrium in cor triatriatum is an embolic at the level of the second lumbar of the spine. source and cor triatriatum itself is an etiologic factor of aor- toarterial embolism. triatriatum with initial presentation at middle age, six patients had atrial fibrillation. None of these patients had a history of Conclusion stroke and in none was an intracardiac embolic source de- scribed, although most had an orifice area I 1.0 cm2.2, Our Spontaneousecho contrast may be confined to the proximal patient had atrial fibrillation and systemic embolism. The chamber of the left atrium in cor triatriatum and can be detect- well-confined spontaneous echo contrast in the proximal ed by transesophagealechocardiography alone. It is an embol- chamber of the left atrium was suspected to be the embolic ic source of cerebral and aortoarterialemboli. For complete di- source. It is supposed that atrial fibrillation and stenosis of the agnostic evaluation, the application of transesophageal echo- orifice of the membrane in cor triatriatum predispose to blood cardiography in cor triatriatum is advisable. stasis in the proximal chamber, which manifests as sponta- neous echo contrast by echocardiography,thereby causing in- tracardiac thrombus formation and systemic embolism with a References mechanism similar to that in rheumatic mitral stenosis.