Intraoperative Echocardiographic Diagnosis of Previously Unrecognized Cor Triatriatum Charles W. Hogue, Jr, MD, Benico Barzilai, MD, Robert Forstot, MD, Todd A. Shuman, MD, and James L. Cox, MD Section of Cardiothoracic Anesthesia, Department of Anesthesiology, Section of Cardiology, Department of Medicine, and Division of Cardiothoracic Surgery, Department of Surgery, Washington University School of Medicine, St. Louis, Missouri
Cor triatriatum is a congenital heart defect resulting in abnormal septation of the left atrium by a fibromuscular membrane. Echocardiography has improved the preoperative diagnosis of this rare congenital heart defect. We report a case where transesophageal echocardiography proved useful in the intraoperative diagnosis of a previously undetected cor triatriatum. (Ann Thorac Surg 1992;54:562-3)
chamber echocardiographic image of the heart failed to reliably identify the presence of the membrane. After the initiation of cardiopulmonary bypass and cardioplegic arrest, left atriotomy was performed confirming the presence of cor triatriatum. The anomalous left atrial membrane was excised and the patient underwent successful coronary revascularization and mitral valve replacement. Reexamination of preoperative transthoracic echocardiograms failed to demonstrate cor triatriatum.
C
Comment
or triatriatum, a rare congenital heart defect resulting from septation of the left atrium by a fibromuscular membrane, can lead to left ventricular inflow obstruction and elevation of pulmonary arterial pressure [l-81. Transthoracic echocardiography has improved the preoperative diagnosis of cor triatriatum [3, 41. We report a case of cor triatriatum, undetected preoperatively despite transthoracic echocardiography, that was diagnosed intraoperatively with transesophageal echocardiography. A 76-year-old man was admitted with congestive heart failure and electrocardiographic myocardial ischemic changes. Diagnostic evaluation included transthoracic echocardiography and cardiac catheterization revealing global left ventricular hypokinesis, severe mitral regurgitation, serious stenosis of two coronary arteries, and a pulmonary artery pressure of 63/25 mm Hg but no other cardiac abnormalities. After a period of stabilization, the patient was scheduled for coronary artery bypass grafting with mitral valve replacement. After the induction of general anesthesia a 5.0-MHz transesophageal echocardiographic probe was inserted and connected to a Hewlett-Packard 77020A imaging system (Hewlett-Packard, Andover, MA). Echocardiographic imaging of the superior left atrium at the level of the left atrial appendage immediately demonstrated an abnormal membrane extending from the atrial septum to the lateral atrial wall (Fig 1) with membrane movement toward the mitral valve during diastole. Color-flow Doppler imaging revealed flow across the abnormal membrane without evidence of flow obstruction. The fourAccepted for publication Dec 23, 1991 Address reprint requests to Dr Hogue, Department of Anesthesiology, Washington University School of Medicine, 660 South Euclid Avenue, Box 8054, St. Louis, MO 63110.
0 1992 by The Society of Thoracic Surgeons
The association of cor triatriatum with other congenital cardiac defects can result in the former condition going undetected until the time of operation. For example, cardiac abnormalities that result in reduced pulmonary blood flow may mask cor triatriatum as can an atrial septa1 defect that decompresses the pulmonary venous chamber of the left atrium. Depending on the degree of left ventricular inflow obstruction, cor triatriatum may go unrecognized until adulthood. Cor triatriatum can be diagnosed during cardiac catheterization if a left atrial pressure to pulmonary artery occlusion pressure gradient is identified; however, these findings are not reliable in the detection or differentiation of cor triatriatum [4]. Furthermore, measurement of left atrial pressure, usually performed by a transseptal technique, is not routinely performed in most adult cardiac catheterization laboratories. Consequently, before the development of echocardiography, cor triatriatum often went undiagnosed until the time of operation [ 3 ] . Although transthoracic echocardiography has improved the preoperative diagnosis of cor iriatriatum, cases of misdiagnosis have been reported due to the presence of other congenital cardiac defects or ultrasound artifact [2, 571. Transesophageal echocardiography may be the preferred method to conclusively diagnose this congenital atrial defect and to assist in excluding other causes of left ventricular inflow obstruction such as mitral stenosis, supravalvular mitral ring, total anomalous pulmonary venous connection to the coronary sinus, and left atrial myxoma [2, 5-81. In prior reports where transesophageal echocardiography was used to image cor triatriatum, the diagnosis was suspected on the basis of other preoperative diagnostic evaluations including transthoracic echocardiography [5, 81. In this report, however, cor triatriatum went undiagnosed until the time of intraoperative echocardiographic examination despite preoperative trans0003-4975/92/$5.00
CASE REPORT HOGUE ET AL INTRAOPERATIVE DETECTlON OF COR TRIATRIATUM
Ann Thorac Surg 1992;54:562-3
563
In conclusion, because of the superior imaging of posterior cardiac structures, intraoperative transesophageal echocardiography may detect previously unrecognized cardiac defects even in a geriatric population.
References 1. Van Praagh R, Corsini I. Cor triatriatum: pathologic anatomy and a consideration of morphogenesis based on 13 postmor-
2. 3.
Fig 1. Transesophageal echocardiogram of left atrium demonstrating cor triatriatum (arrow). ( A 0 = aortic root; LAA = left atrial appendage. )
thoracic echocardiography. Furthermore, retrospective review of the preoperative transthoracic echocardiograms failed to demonstrate cor triatriatum. The superior acoustic window of transesophageal compared with transthoracic echocardiography for posterior cardiac structures may explain the failure of preoperative detection of cor triatriatum in our patient.
4.
5. 6. 7. 8.
tem cases and a study of normal development of the pulmonary vein and atrial septum in 83 human embryos. Am Heart 1 1969;78:379-85. Jacobstein MD, Hirschfeld SS. Concealed left atrial membrane: pitfalls in the diagnosis of cor triatriatum and supravalvular mitral ring. Am J Cardiol 1982;49:780-6. Oglietti J, Cooley DA, Izquierdo JP, et al. Cor triatriatum: operative results in 25 patients. Ann Thorac Surg 1983;35: 415-20. Ostman-Smith I, Silverman NH, Oldershwa P, et al. Cor triatriatum sinistrum: diagnostic features on cross sectional echocardiography. Br Heart J 1984;51:211-9. SchIuter M, Burkhart A, Langenstein BA, et al. Transesophageal two dimensional echocardiography in the diagnosis of cor triatriatum in the adult. J Am Coll Cardiol 1983;S:lOll-5. Lacrte M, Harada K, Williams RG. Echocardiographic features of left ventricular inflow obstruction. Circulation 1976;54: 5624. Snider AR, Roge CL, Schiller NB, et al. Congenital left ventricular inflow obstruction evaluated by two dimensional echocardiography. Circulation 1980;61:848-55. Ludomirsky A, Erickson C, Vick GW, et al. Transesophageal color-flow Doppler evaluation of cor triatriatum in an adult. Am Heart J 1990;120:451-5.
Cor triatriatum is a congenital heart defect resulting in abnormal septation of the left atrium by a fibromuscular membrane. Echocardiography has improved the preoperative diagnosis of this rare congenital heart defect. We report a case where transesophageal echocardiography proved useful in the intraoperative diagnosis of a previously undetected cor triatriatum. (Ann Thorac Surg 1992;54:562-3)
chamber echocardiographic image of the heart failed to reliably identify the presence of the membrane. After the initiation of cardiopulmonary bypass and cardioplegic arrest, left atriotomy was performed confirming the presence of cor triatriatum. The anomalous left atrial membrane was excised and the patient underwent successful coronary revascularization and mitral valve replacement. Reexamination of preoperative transthoracic echocardiograms failed to demonstrate cor triatriatum.
C
Comment
or triatriatum, a rare congenital heart defect resulting from septation of the left atrium by a fibromuscular membrane, can lead to left ventricular inflow obstruction and elevation of pulmonary arterial pressure [l-81. Transthoracic echocardiography has improved the preoperative diagnosis of cor triatriatum [3, 41. We report a case of cor triatriatum, undetected preoperatively despite transthoracic echocardiography, that was diagnosed intraoperatively with transesophageal echocardiography. A 76-year-old man was admitted with congestive heart failure and electrocardiographic myocardial ischemic changes. Diagnostic evaluation included transthoracic echocardiography and cardiac catheterization revealing global left ventricular hypokinesis, severe mitral regurgitation, serious stenosis of two coronary arteries, and a pulmonary artery pressure of 63/25 mm Hg but no other cardiac abnormalities. After a period of stabilization, the patient was scheduled for coronary artery bypass grafting with mitral valve replacement. After the induction of general anesthesia a 5.0-MHz transesophageal echocardiographic probe was inserted and connected to a Hewlett-Packard 77020A imaging system (Hewlett-Packard, Andover, MA). Echocardiographic imaging of the superior left atrium at the level of the left atrial appendage immediately demonstrated an abnormal membrane extending from the atrial septum to the lateral atrial wall (Fig 1) with membrane movement toward the mitral valve during diastole. Color-flow Doppler imaging revealed flow across the abnormal membrane without evidence of flow obstruction. The fourAccepted for publication Dec 23, 1991 Address reprint requests to Dr Hogue, Department of Anesthesiology, Washington University School of Medicine, 660 South Euclid Avenue, Box 8054, St. Louis, MO 63110.
0 1992 by The Society of Thoracic Surgeons
The association of cor triatriatum with other congenital cardiac defects can result in the former condition going undetected until the time of operation. For example, cardiac abnormalities that result in reduced pulmonary blood flow may mask cor triatriatum as can an atrial septa1 defect that decompresses the pulmonary venous chamber of the left atrium. Depending on the degree of left ventricular inflow obstruction, cor triatriatum may go unrecognized until adulthood. Cor triatriatum can be diagnosed during cardiac catheterization if a left atrial pressure to pulmonary artery occlusion pressure gradient is identified; however, these findings are not reliable in the detection or differentiation of cor triatriatum [4]. Furthermore, measurement of left atrial pressure, usually performed by a transseptal technique, is not routinely performed in most adult cardiac catheterization laboratories. Consequently, before the development of echocardiography, cor triatriatum often went undiagnosed until the time of operation [ 3 ] . Although transthoracic echocardiography has improved the preoperative diagnosis of cor iriatriatum, cases of misdiagnosis have been reported due to the presence of other congenital cardiac defects or ultrasound artifact [2, 571. Transesophageal echocardiography may be the preferred method to conclusively diagnose this congenital atrial defect and to assist in excluding other causes of left ventricular inflow obstruction such as mitral stenosis, supravalvular mitral ring, total anomalous pulmonary venous connection to the coronary sinus, and left atrial myxoma [2, 5-81. In prior reports where transesophageal echocardiography was used to image cor triatriatum, the diagnosis was suspected on the basis of other preoperative diagnostic evaluations including transthoracic echocardiography [5, 81. In this report, however, cor triatriatum went undiagnosed until the time of intraoperative echocardiographic examination despite preoperative trans0003-4975/92/$5.00
CASE REPORT HOGUE ET AL INTRAOPERATIVE DETECTlON OF COR TRIATRIATUM
Ann Thorac Surg 1992;54:562-3
563
In conclusion, because of the superior imaging of posterior cardiac structures, intraoperative transesophageal echocardiography may detect previously unrecognized cardiac defects even in a geriatric population.
References 1. Van Praagh R, Corsini I. Cor triatriatum: pathologic anatomy and a consideration of morphogenesis based on 13 postmor-
2. 3.
Fig 1. Transesophageal echocardiogram of left atrium demonstrating cor triatriatum (arrow). ( A 0 = aortic root; LAA = left atrial appendage. )
thoracic echocardiography. Furthermore, retrospective review of the preoperative transthoracic echocardiograms failed to demonstrate cor triatriatum. The superior acoustic window of transesophageal compared with transthoracic echocardiography for posterior cardiac structures may explain the failure of preoperative detection of cor triatriatum in our patient.
4.
5. 6. 7. 8.
tem cases and a study of normal development of the pulmonary vein and atrial septum in 83 human embryos. Am Heart 1 1969;78:379-85. Jacobstein MD, Hirschfeld SS. Concealed left atrial membrane: pitfalls in the diagnosis of cor triatriatum and supravalvular mitral ring. Am J Cardiol 1982;49:780-6. Oglietti J, Cooley DA, Izquierdo JP, et al. Cor triatriatum: operative results in 25 patients. Ann Thorac Surg 1983;35: 415-20. Ostman-Smith I, Silverman NH, Oldershwa P, et al. Cor triatriatum sinistrum: diagnostic features on cross sectional echocardiography. Br Heart J 1984;51:211-9. SchIuter M, Burkhart A, Langenstein BA, et al. Transesophageal two dimensional echocardiography in the diagnosis of cor triatriatum in the adult. J Am Coll Cardiol 1983;S:lOll-5. Lacrte M, Harada K, Williams RG. Echocardiographic features of left ventricular inflow obstruction. Circulation 1976;54: 5624. Snider AR, Roge CL, Schiller NB, et al. Congenital left ventricular inflow obstruction evaluated by two dimensional echocardiography. Circulation 1980;61:848-55. Ludomirsky A, Erickson C, Vick GW, et al. Transesophageal color-flow Doppler evaluation of cor triatriatum in an adult. Am Heart J 1990;120:451-5.
