Volume124 Number 6
Brief Communications
1655
Identification of coronary sinus septal defect (unroofed coronary sinus) by color Doppler echocardiography Alvin J. Chin, MD, and John D. Murphy, MD Philadelph,ia, Pa.
Coronary sinus septal defect (CSSD) (unroofed coronary sinus) is a defect in the wall that divides the left atrium from the coronary sinus. It is frequently associatedwith persistent left superior vena cav8.l When it occursin association with intracardiac anomalies,such as tetralogy of Fallot2 or tricuspid atresia,3,4 there can be significant residual right-to-left shunting through the defect in the early postoperative period, if it isnot recognizedat the time of repair. The echocardiographicdetection of this anomaly dependson the identification of the wall between the left atrium and the coronary sinus. Although this may not be difficult in the caseof a neonate who can be scannedfrom multiple windows with 5.0 and 7.5 MHz transducers, resolution of this thin wall in the older infant or child with 2.5 or 3.5 MHz transducerscan be a formidable challenge.Using color Doppler flow echocardiography, we recently prospectively diagnosedcoronary sinusseptal defects in a 20month-old infant (casel), a 32-month-old infant (case2), and a ‘I-year-old child (case3). All three patients were thought to have atrial septal defects according to results of physical examinations. A Hewlett-Packard Sonos500phased-arraycolor flow imager (Hewlett-Packard Co., Imaging Systems Division, Andover, Mass.) with a 5 MHz and a 3.5 MHz medium focus transducer wasused. Subcostal sweeps(frontal, long axial oblique, and sagittal) were employed, along with parasternal short-axis and long-axis sweeps.In an infant with a dilated coronary sinusmouth in whom a coronary sinusroof is present, it can often be seenas a thin line immediately cephaladto the left atrioventricular groove in the subcostal frontal view (Fig. 1). In case1, no roof could be visualized with two-dimensionalimagingfrom the subcostalwindow (Fig. 2). Only the mouth of the coronary sinuswasevident; it wasdelineated inferiorly by the crux and superiorly by the septal attachment of the eustachian valve. The dilated coronary sinus mouth could be distinguished from an ostium primum atria1 septal defect by three criteria: (1) it appeared at a very inferior location in the subcostalfrontal sweep,(2) the dropout disappeared as the subcostalfrontal sweepprogressedmore anteriorly and superiorly, and (3) the mitral valve had a normal structure. Color flow Doppler echocardiography confirmed the presenceof a left-to-right shunt, yhich passedfrom the left atrium through the coronary siFrom the Division of Cardiology, and the Department of Pediatrics, Medicine, Philadelphia. Reprint requests: Alvin Hospital of Philadelphia, PA 19104. 414/41348
The Children’s University
J. Chin, MD, Division 34th Street and Civic
Hospital of Philadelphia, of Pennsylvania School of Cardiology, Center Blvd.,
Children’s Philadelphia,
of
Fig. 1. Subcostalfrontal view of a normal patient with a dilated coronary sinus (and persistent left superior vena cava). Note the presenceof the coronary sinusroof (unlabelled arrows). cs, Coronary sinus; LA, left atrium; RA, right atrium; rpu, right lower pulmonary vein.
Fig. 2. Subcostal frontal view of case1. The mouth of the coronary sinusis enlarged, but no roof is visualized at all. L, left; S, superior; other abbreviations as in Fig. 1.
nus mouth into the right atrium (Fig. 3). Cardiac catheterization was not performed. The diagnosisof CSSD was confirmed during surgery; the coronary sinus ostium was closedwith a patch. In case2, a partial roof could be visualized from the subcostal and parasternal windows. Color flow Doppler echocardiography confirmed the presenceof CSSD. The patient underwent reparative surgery, which confirmed the diagnosis. In case3, a dilated coronary sinusostium wasobserved;
1656
Brief Communications
American
December 1992 Hean Journal
Fig. 3. Subcostalfrontal view: color flow Doppler echocardiographicexamination. Flow is directed toward the transducer (red) and passesfrom the left atrium, through the mouth of the coronary sinus,into the right
atrium. Abbreviations as in Figs. 1 and 2.
however, mostof the roof of the coronary sinuswaspresent. No CSSD could be located with two-dimensional imaging from the subcostalor parasternal windows.Only with color flow Doppler echocardiography wasthe diagnosispossible; a left-to-right shunt, which passedthrough the coronary sinusmouth, wasconspicuous.The diagnosiswassuggested at the time of cardiac catheterization, although the CSSD marginscould not be sharply defined becausethe catheter recoiled during the coronary sinus angiogram; CSSD was subsequently confirmed at operation, and the coronary sinus ostium was closedwith a patch. The possiblecausesof left-to-right shunting at the atria1 level include: (1) sinusvenosusdefect of the superior vena cavatype,5 (2) sinusvenosusdefect of the inferior vena cava type,6 (3) ostium secundum defect (deficiency in septum primum, the flap valve of the foramen ovale), (4) incompetent or fenestrated flap valve of the foramen ovale, (5) ostium primum defect (atrioventricular septal defect), (6) anomalouspulmonary venousconnection, (7) left ventricle to right atrium shunt, (8) coronary artery to right atrium fistulas, and (9) CSSD. In each of our cases,all causesof a right ventricular volume overload other than CSSD were ruled out by two-dimensional imaging; in addition, a left superior vena cava was sought, and none was found. A dropout produced by a sinusvenosusdefect of the inferior uena caua type is adjacent to the right lower pulmonary vein and is thus far to the right of where a CSSD would be (Fig. 4). Ostium primum defects extend more anterosuperiorly, up to the region immediately below the aortic valve; CSSDsare far inferior to the aortic valve. Color flow Doppler findings that are consistentwith left-to-right flow from
the left atrium, passingthrough the mouth of the coronary sinus, were noted from multiple windows in our cases.In our case3, most of the wall betweenthe left atrium and the coronary sinus appeared to be present, and two-dimensionalimaging alone was unsuccessfulin displaying a hole in the wall. This difficulty is not surprising in view of the fact that someCSSDsare not circular but rather markedly elliptical. 4q7 Color flow Doppler echocardiography assists in the detection of CSSD in a way that is similar to its utility in aortopulmonary window,sanother lesionin which the noninvasive diagnosisrests on finding a defect in a relatively thin wall. The occurrenceof CSSD in postmortem specimensfrom subjectswith tricuspid atresia hasbeenreported as2 % by Rumisek et a1.4The difficulty in recognizing them during surgery from a right atria1 view makespreoperative detection an important issue.Severe arterial desaturation after right atria1to right pulmonary artery or right atria1 to right ventricular outlet chamber anastomosis(modified Fontan operation) can result in hemodynamic deterioration. CSSDs are often not suspectedin right heart obstructive diseasebecausethe coronary sinus ostium may not be dilated in thesepatients. In addition to occurring in association with right-sided obstructive lesions,CSSD can also coexist with left atrioventricular valve hypoplasiaor atresia. The modification of Fontan operation that leavesboth the coronary sinusostium and the native left atrium aspart of the pulmonary venous pathway is especially attractive. Even if CSSD goesunrecognizedbefore surgeryin a patient with atrioventricular valve atresia, this type of Fontan operation will still succeed.
Volume Number
124 6
Brief Communications
1657
Fig. 4. Top left, Subcostal frontal view of a patient with sinus venosus defect of the inferior vena cava type (circle). This cut is sufficiently inferior that the ventricles are not displayed. Note that the defect is adjacent to the right lower pulmonary vein entrance site. This is far to the right of where a CSSD would be. L, Left atrium; R, right atrium; rlpu, right lower pulmonary vein; S, superior. TOP right, Subcostal left oblique view of the same case. The arrowheads show the sinus venosus defect of the inferior vena cava type. Bottom left, A subcostal left oblique view slightly further to the left shows the roof of the coronary sinus to be intact. White lines point to the thin roof. A, R, Anterior and rightward; mcs, mouth of coronary sinus. Bottom right, A subcostal left oblique view even further to the left shows the cephalad septum secundum (closed arrowheads) and the septum primum (open arrowheads) to be intact. (The cephalad septum secundum is absent in sinus venosus defect of the superior vena cava type.)
REFERENCES
1. Mantini E, Grondin GM, Lillehei CW, Edwards JE. Congenital anomalies involving the coronary sinus. Circulation 1966; 33:317-27. 2. Yeager SB, Chin AJ, Sanders SP. Subxiphiod two-dimensional echocardiographic diagnosis of coronary sinus septal defects in children. Am J Cardiol 1984;54:686-7. 3. Kurosawa H, Yagi Y, lmanura E, Koyanagi H, Satomi M, Nakazawa M, Takao A. A problem in Fontan’s operation: sinus septal defect complicating tricuspid atresia. Heart Vessels 1985;1:48-50. 4. Rumisek JD, Pigott ,JD, Weinberg PM, Norwood WI. Coronary sinus septal defect associated with tricuspid atresia. J Thorac Cardiovasc Surg 1986;92:142-5. 5. Ross DN. The sinus venosus type of atria1 septal defect. Guys Hosp Rep 1956;105:376-81. 6. McCormack RJM, Pickering D, Smith II. A rare type of atria1 septal defect. Thorax 1968;23:350-2. 7. Freedom RM. Culham JAG. Rowe RD. Left atria1 to coronarv sinus fenes&ion (partially unroofed coronary sinus). Bi Heart J 1981;46:63-8. 8. AIboliras ET, Chin AJ, Barber G, Helton JG, Pigott JD. Detection of aorta-pulmonary window by pulsed and color Doppler echocardiography. AM HEART J 1988;115:900-2.
Transesophageal echocardiographic diagnosis of coronary sinus type atrial septal defect Yasushi Sunaga, MD, Kayo Hayashi, MD, Naohiko Okubo, MD, Yoshio Taniichi, MD, Tetsuro Sugiura, MD, Nobuyuki Tsuda, MD, Toshiji Iwasaka, MD, and Mitsuo Inada, MD Osaka, Japan Coronary sinus type atria1 septal defect is a rare cardiac malformation and is usually diagnosed during a surgical procedure or at autopsy. l, ’ This report describes a case of From the Second Department of Internal Medicine, Kansai Medical University, Osaka, Japan. Reprint requests: Yasushi Sunaga, MD, Second Department of Internal Medicine, Kansai Medical University, 1 Fumizono-cho. Moriguchi City, Osaka 570 Japan. 414141347
Brief Communications
1655
Identification of coronary sinus septal defect (unroofed coronary sinus) by color Doppler echocardiography Alvin J. Chin, MD, and John D. Murphy, MD Philadelph,ia, Pa.
Coronary sinus septal defect (CSSD) (unroofed coronary sinus) is a defect in the wall that divides the left atrium from the coronary sinus. It is frequently associatedwith persistent left superior vena cav8.l When it occursin association with intracardiac anomalies,such as tetralogy of Fallot2 or tricuspid atresia,3,4 there can be significant residual right-to-left shunting through the defect in the early postoperative period, if it isnot recognizedat the time of repair. The echocardiographicdetection of this anomaly dependson the identification of the wall between the left atrium and the coronary sinus. Although this may not be difficult in the caseof a neonate who can be scannedfrom multiple windows with 5.0 and 7.5 MHz transducers, resolution of this thin wall in the older infant or child with 2.5 or 3.5 MHz transducerscan be a formidable challenge.Using color Doppler flow echocardiography, we recently prospectively diagnosedcoronary sinusseptal defects in a 20month-old infant (casel), a 32-month-old infant (case2), and a ‘I-year-old child (case3). All three patients were thought to have atrial septal defects according to results of physical examinations. A Hewlett-Packard Sonos500phased-arraycolor flow imager (Hewlett-Packard Co., Imaging Systems Division, Andover, Mass.) with a 5 MHz and a 3.5 MHz medium focus transducer wasused. Subcostal sweeps(frontal, long axial oblique, and sagittal) were employed, along with parasternal short-axis and long-axis sweeps.In an infant with a dilated coronary sinusmouth in whom a coronary sinusroof is present, it can often be seenas a thin line immediately cephaladto the left atrioventricular groove in the subcostal frontal view (Fig. 1). In case1, no roof could be visualized with two-dimensionalimagingfrom the subcostalwindow (Fig. 2). Only the mouth of the coronary sinuswasevident; it wasdelineated inferiorly by the crux and superiorly by the septal attachment of the eustachian valve. The dilated coronary sinus mouth could be distinguished from an ostium primum atria1 septal defect by three criteria: (1) it appeared at a very inferior location in the subcostalfrontal sweep,(2) the dropout disappeared as the subcostalfrontal sweepprogressedmore anteriorly and superiorly, and (3) the mitral valve had a normal structure. Color flow Doppler echocardiography confirmed the presenceof a left-to-right shunt, yhich passedfrom the left atrium through the coronary siFrom the Division of Cardiology, and the Department of Pediatrics, Medicine, Philadelphia. Reprint requests: Alvin Hospital of Philadelphia, PA 19104. 414/41348
The Children’s University
J. Chin, MD, Division 34th Street and Civic
Hospital of Philadelphia, of Pennsylvania School of Cardiology, Center Blvd.,
Children’s Philadelphia,
of
Fig. 1. Subcostalfrontal view of a normal patient with a dilated coronary sinus (and persistent left superior vena cava). Note the presenceof the coronary sinusroof (unlabelled arrows). cs, Coronary sinus; LA, left atrium; RA, right atrium; rpu, right lower pulmonary vein.
Fig. 2. Subcostal frontal view of case1. The mouth of the coronary sinusis enlarged, but no roof is visualized at all. L, left; S, superior; other abbreviations as in Fig. 1.
nus mouth into the right atrium (Fig. 3). Cardiac catheterization was not performed. The diagnosisof CSSD was confirmed during surgery; the coronary sinus ostium was closedwith a patch. In case2, a partial roof could be visualized from the subcostal and parasternal windows. Color flow Doppler echocardiography confirmed the presenceof CSSD. The patient underwent reparative surgery, which confirmed the diagnosis. In case3, a dilated coronary sinusostium wasobserved;
1656
Brief Communications
American
December 1992 Hean Journal
Fig. 3. Subcostalfrontal view: color flow Doppler echocardiographicexamination. Flow is directed toward the transducer (red) and passesfrom the left atrium, through the mouth of the coronary sinus,into the right
atrium. Abbreviations as in Figs. 1 and 2.
however, mostof the roof of the coronary sinuswaspresent. No CSSD could be located with two-dimensional imaging from the subcostalor parasternal windows.Only with color flow Doppler echocardiography wasthe diagnosispossible; a left-to-right shunt, which passedthrough the coronary sinusmouth, wasconspicuous.The diagnosiswassuggested at the time of cardiac catheterization, although the CSSD marginscould not be sharply defined becausethe catheter recoiled during the coronary sinus angiogram; CSSD was subsequently confirmed at operation, and the coronary sinus ostium was closedwith a patch. The possiblecausesof left-to-right shunting at the atria1 level include: (1) sinusvenosusdefect of the superior vena cavatype,5 (2) sinusvenosusdefect of the inferior vena cava type,6 (3) ostium secundum defect (deficiency in septum primum, the flap valve of the foramen ovale), (4) incompetent or fenestrated flap valve of the foramen ovale, (5) ostium primum defect (atrioventricular septal defect), (6) anomalouspulmonary venousconnection, (7) left ventricle to right atrium shunt, (8) coronary artery to right atrium fistulas, and (9) CSSD. In each of our cases,all causesof a right ventricular volume overload other than CSSD were ruled out by two-dimensional imaging; in addition, a left superior vena cava was sought, and none was found. A dropout produced by a sinusvenosusdefect of the inferior uena caua type is adjacent to the right lower pulmonary vein and is thus far to the right of where a CSSD would be (Fig. 4). Ostium primum defects extend more anterosuperiorly, up to the region immediately below the aortic valve; CSSDsare far inferior to the aortic valve. Color flow Doppler findings that are consistentwith left-to-right flow from
the left atrium, passingthrough the mouth of the coronary sinus, were noted from multiple windows in our cases.In our case3, most of the wall betweenthe left atrium and the coronary sinus appeared to be present, and two-dimensionalimaging alone was unsuccessfulin displaying a hole in the wall. This difficulty is not surprising in view of the fact that someCSSDsare not circular but rather markedly elliptical. 4q7 Color flow Doppler echocardiography assists in the detection of CSSD in a way that is similar to its utility in aortopulmonary window,sanother lesionin which the noninvasive diagnosisrests on finding a defect in a relatively thin wall. The occurrenceof CSSD in postmortem specimensfrom subjectswith tricuspid atresia hasbeenreported as2 % by Rumisek et a1.4The difficulty in recognizing them during surgery from a right atria1 view makespreoperative detection an important issue.Severe arterial desaturation after right atria1to right pulmonary artery or right atria1 to right ventricular outlet chamber anastomosis(modified Fontan operation) can result in hemodynamic deterioration. CSSDs are often not suspectedin right heart obstructive diseasebecausethe coronary sinus ostium may not be dilated in thesepatients. In addition to occurring in association with right-sided obstructive lesions,CSSD can also coexist with left atrioventricular valve hypoplasiaor atresia. The modification of Fontan operation that leavesboth the coronary sinusostium and the native left atrium aspart of the pulmonary venous pathway is especially attractive. Even if CSSD goesunrecognizedbefore surgeryin a patient with atrioventricular valve atresia, this type of Fontan operation will still succeed.
Volume Number
124 6
Brief Communications
1657
Fig. 4. Top left, Subcostal frontal view of a patient with sinus venosus defect of the inferior vena cava type (circle). This cut is sufficiently inferior that the ventricles are not displayed. Note that the defect is adjacent to the right lower pulmonary vein entrance site. This is far to the right of where a CSSD would be. L, Left atrium; R, right atrium; rlpu, right lower pulmonary vein; S, superior. TOP right, Subcostal left oblique view of the same case. The arrowheads show the sinus venosus defect of the inferior vena cava type. Bottom left, A subcostal left oblique view slightly further to the left shows the roof of the coronary sinus to be intact. White lines point to the thin roof. A, R, Anterior and rightward; mcs, mouth of coronary sinus. Bottom right, A subcostal left oblique view even further to the left shows the cephalad septum secundum (closed arrowheads) and the septum primum (open arrowheads) to be intact. (The cephalad septum secundum is absent in sinus venosus defect of the superior vena cava type.)
REFERENCES
1. Mantini E, Grondin GM, Lillehei CW, Edwards JE. Congenital anomalies involving the coronary sinus. Circulation 1966; 33:317-27. 2. Yeager SB, Chin AJ, Sanders SP. Subxiphiod two-dimensional echocardiographic diagnosis of coronary sinus septal defects in children. Am J Cardiol 1984;54:686-7. 3. Kurosawa H, Yagi Y, lmanura E, Koyanagi H, Satomi M, Nakazawa M, Takao A. A problem in Fontan’s operation: sinus septal defect complicating tricuspid atresia. Heart Vessels 1985;1:48-50. 4. Rumisek JD, Pigott ,JD, Weinberg PM, Norwood WI. Coronary sinus septal defect associated with tricuspid atresia. J Thorac Cardiovasc Surg 1986;92:142-5. 5. Ross DN. The sinus venosus type of atria1 septal defect. Guys Hosp Rep 1956;105:376-81. 6. McCormack RJM, Pickering D, Smith II. A rare type of atria1 septal defect. Thorax 1968;23:350-2. 7. Freedom RM. Culham JAG. Rowe RD. Left atria1 to coronarv sinus fenes&ion (partially unroofed coronary sinus). Bi Heart J 1981;46:63-8. 8. AIboliras ET, Chin AJ, Barber G, Helton JG, Pigott JD. Detection of aorta-pulmonary window by pulsed and color Doppler echocardiography. AM HEART J 1988;115:900-2.
Transesophageal echocardiographic diagnosis of coronary sinus type atrial septal defect Yasushi Sunaga, MD, Kayo Hayashi, MD, Naohiko Okubo, MD, Yoshio Taniichi, MD, Tetsuro Sugiura, MD, Nobuyuki Tsuda, MD, Toshiji Iwasaka, MD, and Mitsuo Inada, MD Osaka, Japan Coronary sinus type atria1 septal defect is a rare cardiac malformation and is usually diagnosed during a surgical procedure or at autopsy. l, ’ This report describes a case of From the Second Department of Internal Medicine, Kansai Medical University, Osaka, Japan. Reprint requests: Yasushi Sunaga, MD, Second Department of Internal Medicine, Kansai Medical University, 1 Fumizono-cho. Moriguchi City, Osaka 570 Japan. 414141347
