- Email: [email protected]
Successful surgical correction of symptomatic cor triatriatum dexter
J
THORAC CARDIOVASC SURG
78:573-575, 1979
Successful surgical correction of symptomatic cor triatriatum dexter An unusual case of cor triatriatum dexter is presented. Abnormal embryologic development of the eustachian valve caused nearly complete septation of the right atrium in a 67-year-old woman. Multiple supraventricular arrhythmias prompted clinical evaluation and diagnosis. Surgical excision of the anomalous membrane resulted in complete relieffrom arrhythmias and a clinical cure. Cor triatriatum dexter is rare and is infrequently diagnosed before postmortem study; however, once the diagnosis is established, the condition is amenable to a relatively simple surgical correction. Supraventricular tachycardia has not previously been reported to be associated with cor triatriatum dexter. We believe this is the first case in which such arrhythmias led to early diagnosis and successful surgical correction.
David A. Ott, M.D., Denton A. Cooley, M.D., Paolo Angelini, M.D., and Robert D. Leachman, M.D., Houston, Texas
During embryologic development of the heart, persistence of the right valve of the sinus venosus, in rare instances, may divide the right atrium almost completely into two chambers. Although this lesion was described earlier by Rokitansky, 1 Laurenstein," Chiari," and others, 4-6 a review by Doucette and Knoblich 7 in 1963 states that the term "cor triatriatum dexter" was first used by German pathologists early in this century. A patient with the anomaly is usually asymptomatic unless associated abnormalities of the tricuspid valve exist": 9 or the inferior vena cava is obstructed. 10 Arrhythmias rarely have been reported in association with the defect. 11 We recently treated a patient with cor triatriatum dexter whose supraventricular arrhythmias led to evaluation, early diagnosis, and successful surgical correction.
Case report A 67-year-old woman from Venezuela was referred to the Texas Heart Institute for evaluation of supraventricular tachycardia. She reported a history of paroxysmal atrial tachycardia of 20 years' duration that was refractory to medical management. Current medications included digoxin, 0.25 mg four times a day, and propranolol (Inderal), 40 mg four From the Divisions of Surgery and Cardiology of the Texas Heart Institute, SI. Luke's Episcopal and Texas Children's Hospitals, Houston, Texas. Received for publication Feb. 21. 1979. Accepted for publication April 17, 1979. Address for reprints: David A. Ott, M.D., Texas Heart Institute, P.O. Box 20345, Houston, Texas 77025.
times a day. The patient said that she often could stop the episodes of tachycardia by coughing or pressing on the right hypochondrium. Six years before admission, a chest roentgenogram revealed an abnormality at the right lower border of the heart which her physician thought represented a pericardial cyst (Fig. I). Results of physical examination of the patient on admission were unremarkable. An electrocardiogram demonstrated normal sinus rhythm and left anterior hemiblock. Computerized axial tomography (CAT scan) showed evidence that the suspected pericardial cyst actually was confluent with the right atrium. Cardiac catheterization and angiography demonstrated an abnormal septation of the right atrium (Fig. 2). Operation was performed on Oct. 5, 1978, by means of total cardiopulmonary bypass with aortic cross-clamping and cardioplegic arrest. The ascending aorta was cannulated for return of oxygenated blood. The superior cava was cannulated through the right atrial appendage, and the inferior vena cava was cannulated through the wall of the right atrium. Somewhat surprising was the ease with which the cannula could be passed into the inferior vena cava. With the patient supported by total cardiopulmonary bypass, an incision in the right atrium revealed a large weblike remnant of the right valve of the sinus venosus which nearly completely divided the sinus venarum from the right atrium proper (Fig. 3). This web was excised completely. The postoperative course was complicated by the patient's retention of pulmonary secretions which necessitated brief reintubation on the twelfth postoperative day. She then recovered without further difficulties. Three months after operation, the patient was asymptomatic and had no atrial arrhythmias.
Discussion Variations in the embryologic development of the venous valves of the right atrium are common and have been reviewed extensively by Yater." During the early
0022-5223/79/100573+03$00.30/0 © 1979 The C. V. Mosby Co.
573
5740ttetal.
The Journal of Thoracic and Cardiovascular Surgery
Fig. 2. Angiogram demonstrating septation of the right atrium by an abnormal remnant of the right valve of the sinus venosus (arrows). Catheter is in the sinus venarum posterior to the crista terminalis. Fig. 1. Chest roentgenogram showing mass at right lower border of heart (arrow), which originally was mistaken for a pericardial cyst. stages of cardiac development, the opening of the sinus venosus into the right portion of the dividing common atrium is delineated by two prominent folds: the right and left valves of the sinus venosus which join superiorly to form the septum spurium. The left venous valve eventually fuses with the emerging septum secundum to form the posterior portion of the atrial septum. At 12 weeks' gestation, the right valve has regressed; the crista terminalis is left as a remnant of the superior portion and the eustachian and thebesian valves as remnants of the caudal portion. During the remainder of gestation, the eustachian valve directs blood from the inferior vena cava across the foramen ovale. The normal eustachian valve is a weblike structure just anterior to the orifice of the inferior vena cava and, in the adult, is seldom wider than 1 cm. Chiari's network of bands attached to the crista terminalis is a commonly noted vestige of the regression of the right sinus venosus valve. 13 If the right valve fails to regress, varying degrees of septation of the right atrium result. A patient with such a defect is usually asymptomatic unless there is associated hypoplasia of the tricuspid valve." Rossall and Caldwell-" reported a case in which fatal obstruction of the inferior vena cava resulted; they suggested the possibility of surgical correction. Hansing and associates"
reported an interesting case in which a right-to-Ieft shunt across an atrial septal defect resulted when such a web blocked inflow across the tricuspid valve. Excision of the web resulted in a clinical cure. We believe our patient is the first in whom abnormal embryologic development apparently caused the arrhythmias which were successfully eliminated by excising the anomalous web. In this patient the persistent right valve was associated with a bulging of the sinus venarum posterior to the crista terminalis. This bulge initially was interpreted as a pericardial cyst on chest roentgenography. The exact mechanism whereby these anomalous structures resulted in paroxysmal atrial tachycardia remains nebulous; yet simple excision of the abnormal septum successfully eliminated the arrhythmia. The diagnosis of cor triatriatum dexter should be considered in symptomatic patients with obstruction of the inferior vena cava, abnormalities in the area of the right atrium evident on radiologic study, unexplained right-to-Ieft shunting, or abnormal function of the tricuspid valve. REFERENCES Von Rokitansky C: Die Defecte der Scheidewande des Herzens: Pathologisch-anatomische Abhandlung. Vienna, 1875, W. Braumuller 2 Laurenstein C: Varietatder Kalppen des rechten Atriums. Virchows Arch F Pathol Anat 68:632, 1876 3 Chiari H: Ueber Netzbildungen im rechten Vorhofe des Herzens. Beitr Z Pathol Anat u Z Allg Pathol 22: I, 1897
Volume 78 Number 4 October, 1979
Cor triatriatum dexter
575
Fig. 3. Operative photograph of opened right atrium. The patient's head is to the left, and the superior caval cannula passes behind the atrial retractor at the top of the photograph. The inferior caval cannula passes dorsal to the anomalous septum (arrows) into the inferior vena cava.
4 Sternberg C: Beitrage zur Herzpathologie. Verh Dtsch Ges Pathol Jena, 1913, p 253 5 Ponhold J: Ein Cor Triatriatum Biventriculare. Frankfurt Z Pathol 51:237-241, 1937 6 Gombert H: Beitrage zur Pathologie der Vorhofsscheidewand des Herzens. Beitr Z Pathol Anat u Z AlIg Pathol 91:483-502, 1933 7 Doucette J, Knoblich R: Persistent right valve of the sinus venosus. So-called cor triatriatum dextrum. Review of the literature and report of a case. Arch Pathol 75: 105-112, 1963 8 Gerlis LM, Anderson RH: Cor triatriatum dexter with imperforate Ebstein's anomaly. Br Heart J 38:108-111, 1976 9 Hansing CE, Young WP, Rowe GG: Cor triatriatum dexter. Persistent right sinus venosus valve. Am J Cardiol 30:559-564, 1972
10 Runcie J: A complicated case of cor triatriatum dexter. Br Heart J 30:729-731, 1968 II Jones RN, Niles NR: Spinnaker formation of sinus venosus valve. Case report of a fatal anomaly in a ten-yearold boy. Circulation 38:468-473, 1968 12 Yater WM: Variations and anomalies of the venous valves of the right atrium of the human heart. Arch Pathol 7:418-441, 1929 13 Kjellberg SR, Mannheimer E, Rudhe U, Jonsson B: Diagnosis of Congenital Heart Disease, Odelberg, translator, Chicago, 1955, Year Book Medical Publishers, Inc., p 25 14 Rossall RE, Caldwell RA: Obstruction of inferior vena cava by a persistent eustachian valve in a young adult. J Clin Pathol 10:40-45, 1957
THORAC CARDIOVASC SURG
78:573-575, 1979
Successful surgical correction of symptomatic cor triatriatum dexter An unusual case of cor triatriatum dexter is presented. Abnormal embryologic development of the eustachian valve caused nearly complete septation of the right atrium in a 67-year-old woman. Multiple supraventricular arrhythmias prompted clinical evaluation and diagnosis. Surgical excision of the anomalous membrane resulted in complete relieffrom arrhythmias and a clinical cure. Cor triatriatum dexter is rare and is infrequently diagnosed before postmortem study; however, once the diagnosis is established, the condition is amenable to a relatively simple surgical correction. Supraventricular tachycardia has not previously been reported to be associated with cor triatriatum dexter. We believe this is the first case in which such arrhythmias led to early diagnosis and successful surgical correction.
David A. Ott, M.D., Denton A. Cooley, M.D., Paolo Angelini, M.D., and Robert D. Leachman, M.D., Houston, Texas
During embryologic development of the heart, persistence of the right valve of the sinus venosus, in rare instances, may divide the right atrium almost completely into two chambers. Although this lesion was described earlier by Rokitansky, 1 Laurenstein," Chiari," and others, 4-6 a review by Doucette and Knoblich 7 in 1963 states that the term "cor triatriatum dexter" was first used by German pathologists early in this century. A patient with the anomaly is usually asymptomatic unless associated abnormalities of the tricuspid valve exist": 9 or the inferior vena cava is obstructed. 10 Arrhythmias rarely have been reported in association with the defect. 11 We recently treated a patient with cor triatriatum dexter whose supraventricular arrhythmias led to evaluation, early diagnosis, and successful surgical correction.
Case report A 67-year-old woman from Venezuela was referred to the Texas Heart Institute for evaluation of supraventricular tachycardia. She reported a history of paroxysmal atrial tachycardia of 20 years' duration that was refractory to medical management. Current medications included digoxin, 0.25 mg four times a day, and propranolol (Inderal), 40 mg four From the Divisions of Surgery and Cardiology of the Texas Heart Institute, SI. Luke's Episcopal and Texas Children's Hospitals, Houston, Texas. Received for publication Feb. 21. 1979. Accepted for publication April 17, 1979. Address for reprints: David A. Ott, M.D., Texas Heart Institute, P.O. Box 20345, Houston, Texas 77025.
times a day. The patient said that she often could stop the episodes of tachycardia by coughing or pressing on the right hypochondrium. Six years before admission, a chest roentgenogram revealed an abnormality at the right lower border of the heart which her physician thought represented a pericardial cyst (Fig. I). Results of physical examination of the patient on admission were unremarkable. An electrocardiogram demonstrated normal sinus rhythm and left anterior hemiblock. Computerized axial tomography (CAT scan) showed evidence that the suspected pericardial cyst actually was confluent with the right atrium. Cardiac catheterization and angiography demonstrated an abnormal septation of the right atrium (Fig. 2). Operation was performed on Oct. 5, 1978, by means of total cardiopulmonary bypass with aortic cross-clamping and cardioplegic arrest. The ascending aorta was cannulated for return of oxygenated blood. The superior cava was cannulated through the right atrial appendage, and the inferior vena cava was cannulated through the wall of the right atrium. Somewhat surprising was the ease with which the cannula could be passed into the inferior vena cava. With the patient supported by total cardiopulmonary bypass, an incision in the right atrium revealed a large weblike remnant of the right valve of the sinus venosus which nearly completely divided the sinus venarum from the right atrium proper (Fig. 3). This web was excised completely. The postoperative course was complicated by the patient's retention of pulmonary secretions which necessitated brief reintubation on the twelfth postoperative day. She then recovered without further difficulties. Three months after operation, the patient was asymptomatic and had no atrial arrhythmias.
Discussion Variations in the embryologic development of the venous valves of the right atrium are common and have been reviewed extensively by Yater." During the early
0022-5223/79/100573+03$00.30/0 © 1979 The C. V. Mosby Co.
573
5740ttetal.
The Journal of Thoracic and Cardiovascular Surgery
Fig. 2. Angiogram demonstrating septation of the right atrium by an abnormal remnant of the right valve of the sinus venosus (arrows). Catheter is in the sinus venarum posterior to the crista terminalis. Fig. 1. Chest roentgenogram showing mass at right lower border of heart (arrow), which originally was mistaken for a pericardial cyst. stages of cardiac development, the opening of the sinus venosus into the right portion of the dividing common atrium is delineated by two prominent folds: the right and left valves of the sinus venosus which join superiorly to form the septum spurium. The left venous valve eventually fuses with the emerging septum secundum to form the posterior portion of the atrial septum. At 12 weeks' gestation, the right valve has regressed; the crista terminalis is left as a remnant of the superior portion and the eustachian and thebesian valves as remnants of the caudal portion. During the remainder of gestation, the eustachian valve directs blood from the inferior vena cava across the foramen ovale. The normal eustachian valve is a weblike structure just anterior to the orifice of the inferior vena cava and, in the adult, is seldom wider than 1 cm. Chiari's network of bands attached to the crista terminalis is a commonly noted vestige of the regression of the right sinus venosus valve. 13 If the right valve fails to regress, varying degrees of septation of the right atrium result. A patient with such a defect is usually asymptomatic unless there is associated hypoplasia of the tricuspid valve." Rossall and Caldwell-" reported a case in which fatal obstruction of the inferior vena cava resulted; they suggested the possibility of surgical correction. Hansing and associates"
reported an interesting case in which a right-to-Ieft shunt across an atrial septal defect resulted when such a web blocked inflow across the tricuspid valve. Excision of the web resulted in a clinical cure. We believe our patient is the first in whom abnormal embryologic development apparently caused the arrhythmias which were successfully eliminated by excising the anomalous web. In this patient the persistent right valve was associated with a bulging of the sinus venarum posterior to the crista terminalis. This bulge initially was interpreted as a pericardial cyst on chest roentgenography. The exact mechanism whereby these anomalous structures resulted in paroxysmal atrial tachycardia remains nebulous; yet simple excision of the abnormal septum successfully eliminated the arrhythmia. The diagnosis of cor triatriatum dexter should be considered in symptomatic patients with obstruction of the inferior vena cava, abnormalities in the area of the right atrium evident on radiologic study, unexplained right-to-Ieft shunting, or abnormal function of the tricuspid valve. REFERENCES Von Rokitansky C: Die Defecte der Scheidewande des Herzens: Pathologisch-anatomische Abhandlung. Vienna, 1875, W. Braumuller 2 Laurenstein C: Varietatder Kalppen des rechten Atriums. Virchows Arch F Pathol Anat 68:632, 1876 3 Chiari H: Ueber Netzbildungen im rechten Vorhofe des Herzens. Beitr Z Pathol Anat u Z Allg Pathol 22: I, 1897
Volume 78 Number 4 October, 1979
Cor triatriatum dexter
575
Fig. 3. Operative photograph of opened right atrium. The patient's head is to the left, and the superior caval cannula passes behind the atrial retractor at the top of the photograph. The inferior caval cannula passes dorsal to the anomalous septum (arrows) into the inferior vena cava.
4 Sternberg C: Beitrage zur Herzpathologie. Verh Dtsch Ges Pathol Jena, 1913, p 253 5 Ponhold J: Ein Cor Triatriatum Biventriculare. Frankfurt Z Pathol 51:237-241, 1937 6 Gombert H: Beitrage zur Pathologie der Vorhofsscheidewand des Herzens. Beitr Z Pathol Anat u Z AlIg Pathol 91:483-502, 1933 7 Doucette J, Knoblich R: Persistent right valve of the sinus venosus. So-called cor triatriatum dextrum. Review of the literature and report of a case. Arch Pathol 75: 105-112, 1963 8 Gerlis LM, Anderson RH: Cor triatriatum dexter with imperforate Ebstein's anomaly. Br Heart J 38:108-111, 1976 9 Hansing CE, Young WP, Rowe GG: Cor triatriatum dexter. Persistent right sinus venosus valve. Am J Cardiol 30:559-564, 1972
10 Runcie J: A complicated case of cor triatriatum dexter. Br Heart J 30:729-731, 1968 II Jones RN, Niles NR: Spinnaker formation of sinus venosus valve. Case report of a fatal anomaly in a ten-yearold boy. Circulation 38:468-473, 1968 12 Yater WM: Variations and anomalies of the venous valves of the right atrium of the human heart. Arch Pathol 7:418-441, 1929 13 Kjellberg SR, Mannheimer E, Rudhe U, Jonsson B: Diagnosis of Congenital Heart Disease, Odelberg, translator, Chicago, 1955, Year Book Medical Publishers, Inc., p 25 14 Rossall RE, Caldwell RA: Obstruction of inferior vena cava by a persistent eustachian valve in a young adult. J Clin Pathol 10:40-45, 1957